In this patient with symptoms that are disproportionate to her resting echocardiographic assessment, a

functional study should be performed (Class 1). At baseline and with stress, transmitral pressure gradient
and TR velocity are obtained and correlate well with invasively derived measurements. In sedentary
patients, exercise-induced dyspnea, along with an increase in mean transmitral pressure gradient to >15
mm Hg and pulmonary artery systolic pressure (PASP) to >60 mm Hg, identifies patients with
hemodynamically significant lesions that may benefit from percutaneous valvotomy if the anatomy is
suitable and MR is mild or less. When exercise results in only minimal changes in transmitral pressure
gradient, but a marked increase in PASP occurs, further evaluation for underlying lung disease is
indicated. In patients who are unable to exercise, dobutamine stress can be used.

This patient does not have severe mitral stenosis at rest and does not have elevated RVSP at rest and so
does not have criteria to be referred to percutaneous balloon mitral commissurotomy or MV surgery.

is diagnosed with AF of uncertain duration and is not on an anticoagulant. He is thus at risk of

development of a LAA thrombus. This occurs along a continuum from spontaneous echo contrast (often
called "smoke"), to sludge, to thrombus. In the clinical scenario presented, LAA thrombus is the most
likely diagnosis based on the clinical data presented and the imaging findings seen in Video 1.

This is not side lobe artifact, which are ambiguous echos generated by off-axis interactions with the
ultrasound beam typically seen next to echo bright structures.

A myxoma is typically a pedunculated mass arising from the interatrial septum.

Prominent pectinate muscles are sometimes mistaken for LAA thrombus, however they are smaller and
communicate with the wall of the appendage.

Pericardial fat is often seen in the transverse sinus adjacent to the LAA, but would not be seen in the
endocardial aspect.

MR is common in patients with hypertrophic cardiomyopathy (HCM) and is related to systolic anterior
motion of the anterior leaflet of the mitral valve (MV) (Video 2). The sequence of systolic events in HCM
leading to MR is eject/obstruct/leak. MR is typically eccentric and posteriorly directed. MR may also result
from mitral annular calcification.

Dilated annulus can cause central or functional MR. Left ventricular size is normal in this image.

Papillary muscle dysfunction and regional wall motion abnormalities can cause tethering of the MV
leaflets, which is not observed in this image.

Cleft MV is a congenital abnormality that causes central regurgitation without increased left ventricular
wall thickness.

This patient is at intermediate risk for CAD and should undergo further non-invasive risk stratification.
An exercise stress test is preferred to gauge functional capacity and has strong prognostic implications.
However, given the presence of LV hypertrophy and repolarization abnormalities on his ECG, an imaging
stress test would be ideal to evaluate for LV morphology and ischemic response. Stress echocardiography
represents a radiation-free method to evaluate both functional capacity and ischemia, and in the
PROMISE trial had similar outcomes to anatomic testing.

A dobutamine stress echocardiogram would be problematic; if palpitations are present an arrhythmia

could be exacerbated by dobutamine. In addition atropine is often used to reach target the heart rate in
dobutamine stress echocardiogram protocols and glaucoma is a contraindication for the use of atropine.
Adenosine or dobutamine cardiac magnetic resonance imaging are reasonable methods for the evaluation
of cardiac ischemia, but would not provide information regarding functional capacity. Exercise stress
would be preferred over regadenoson nuclear imaging

This patient has evidence of tetralogy of Fallot (ToF), including a ventricular septal defect (VSD),
overriding aorta, right ventricular outflow tract (RVOT) obstruction, and RV hypertrophy. The ability of
an infant to survive with TOF depends on the optimal degree of RVOT obstruction. With severe/critical
obstruction, patients have profound hypoxia in infancy and depend on either a Blalock-Tausig shunt or
repair for survival. With minimal obstruction, the hemodynamics are similar to a large VSD with
pulmonary overcirculation and heart failure symptoms. This patient had enough pulmonary blood flow to
survive, but also enough RVOT to protect her pulmonary vasculature. She underwent a complete repair
and had an uncomplicated pregnancy one year later.

It is very unusual to survive to adulthood with transposition of the great arteries. This patient does not
have the characteristic parallel great vessels seen in transposition. Tricuspid atresia is associated with a
thick plate of tissue where the tricuspid valve is usually located. This patient has two atrio-ventricular
valves. Hypoplastic left heart syndrome has stenosis and/or atresia of the mitral and aortic valves, both of
which appear normal in this patient. Pulmonary hypertension from a large VSD can present with clubbing
and cyanosis; however, a loud murmur would be unusual as flow across the VSD is low velocity in the
setting of pulmonary hypertension. The echocardiogram shows evidence of RVOT obstruction, which
implies the RV systolic pressure does not represent the pulmonary pressure and further evaluation is
necessary.
The images demonstrate a papillary fibroelastoma (PFE) on the aortic valve. PFEs are the second most
common primary cardiac tumor after myxoma. They are small round masses with well demarcated
borders. They have a stalk of connective tissue and a characteristic shimmering appearance. More than
80% occur on valvular surfaces, with the aortic valve being the most common, but they can occur on any
endocardial surface including within the left ventricle, left atrium (LA), or left atrial appendage. The most
significant clinical implications are their embolic potential. They rarely cause valve dysfunction. For
patients with a history of TIA or stroke, surgical excision is usually recommended; management of
incidental (asymptomatic) PFE has not been well studied.

Vegetations typically have an irregular border, unlike the smooth, well-demarcated edges of a PFE, and
often cause valve dysfunction. Additionally, a clinical history of fevers, chills, or other symptoms of
infection, as well as elevated inflammatory markers and leukocystosis, would be expected in a patient with
bacterial endocarditis.

This image is not consistent with thrombus. Thrombi usually form on damaged or diseased cardiac
structures, not on structurally normal or normally functioning chambers and valves. Laboratory evidence
of a hypercoagulable state can also be helpful for supporting a diagnosis of valvular or arterial thrombus.

The most common primary tumor of the heart is mxyoma. Most myxomas are pedunculated mobile masses
with a narrow stalk and are often attached to the fossa ovalis (<90%). They have a gelatinous appearance
and can become very large, leading to valvular obstruction. The majority of myxomas are located in the
LA. Ninety percent of myxomas are sporadic, but a high recurrence rate may be found when associated
with the Carney complex. Constitutional symptoms are frequent, which can include fever, dyspnea,
weight loss, and joint pain.

Libman-Sacks or nonbacterial thrombotic endocarditis (NBTE) is usually an asymptomatic condition and

is not commonly associated with valve destruction or embolic phenomenon. It has a nodular appearance
and is always sessile. NBTE typically affects both surfaces of a valve, unlike infective endocarditis, which
is most often seen on the regurgitant surface of a valve. NBTE would be unusual in a previously healthy
individual without any systemic signs of inflammatory illness such as lupus erythematosus.

The image demonstrates a patent ductus arteriosus (PDA), which is a connection between the aorta and
pulmonary artery that results in left to right shunt and increased pulmonary blood flow. Similar to a
ventricular septal defect, the lesion creates volume overload on the left heart with resultant LV and left
atrial (LA) enlargement. However, with a PDA, LVOT VTI is increased, but RVOT VTI is not as the shunt
occurs above the pulmonary valve and outflow tract. Right atrial and RV enlargement develop late only as
a result of pulmonary hypertension. While there is volume overload, there is no pressure overload of the
LV and hence concentric LVH is not a feature of PDA. LV and LA enlargement are Class I indications to
close a PDA.

This patient has an initial calculated AVA of 0.6 cm 2 with low mean gradients (<40 mm Hg) and reduced
LVEF (<50%). A low-dose dobutamine stress echocardiogram can be helpful in differentiating
pseudosevere aortic stenosis (AS) and true AS. A LVOT velocity-time integral (VTI) increase of <20% with
dobutamine indicates absent flow reserve, and the stress test cannot distinguish between true severe AS
and pseudosevere AS. When flow reserve is present, mean and peak AoV gradients will increase with
pseudosevere AS, but the calculated AVA will also increase to >1 cm 2. With true AS, the calculated AVA
with dobutamine would remain <1 cm 2. This patient has a calculated AVA of 0.6 cm 2 at rest and 1.1
cm2 with 20 mcg/kg/min of dobutamine. The LVOT VTI increase also exceeds 20%. Hence, this patient
has pseudosevere AS, and therapy should be directed at improving ventricular function.

Neither surgical AoV replacement nor balloon valvuloplasty are indicated for pseudosevere AS. Ongoing
studies are evaluating transcatheter AoV replacement in patients with pseudosevere AS and low ejection
fraction (EF). Ezetimide was studied for nonsevere AS and did not affect the progression of valve disease.
Guideline-directed medical therapy including angiotensin-converting enzyme inhibitors and beta-
blockers are indicated for patients with low EF. Hydralazine would not be the preferred first-line therapy
for this patient.

When comparing Doppler- and catheter-based measurements, it is important to be aware of the impact of
downstream pressure recovery. This phenomenon refers to the conversion of potential energy to kinetic
energy as flow converges through a stenotic AoV. This results in a reduction of pressure at the vena
contracta (VC). There is then reconversion of kinetic energy to potential energy with recovery of some of
the pressure lost from the LVOT to the VC. The phenomenon is seen more commonly in patients with
small aortas, such as the patient in this question. The implication of pressure recovery is that AVA
calculated by Doppler-based methods can underestimate the valve area (i.e., overestimate severity of aortic
stenosis) when compared with AVA assessment calculated by catheter-based methods, in which aortic
pressure is routinely measured in the aortic root (i.e., distal to the AoV).
The CW Doppler shows a dagger-shaped, late-peaking systolic contour consistent with dynamic
obstruction. M-mode echocardiogram shows mid-systolic closure reflecting diminution of flow during the
peak gradient. Fixed stenosis with degenerative aortic stenosis, bicuspid valve, or supravalvular aortic
stenosis would produce an earlier peaking, rounded contour of CW Doppler. The M-mode tracing of the
subaortic membrane may similarly show mid-systolic partial closure, but can be distinguished by the
rounded, mid-peaking CW Doppler profile

Patients in atrial fibrillation have varying RR intervals resulting in variable peak velocities across a
stenotic AV due to differing stroke volumes. Therefore, it is important to calculate an average velocity of
ten cardiac cycles. A slower sweep speed allows you to see more cardiac cycles per frame allowing for the
calculation of the average velocity.

Widening the sample volume is used when obtaining pulsed wave Doppler and does not pertain to CW
Doppler. The velocities across a stenotic AV are elevated, and therefore the scale needs to be increased to
see the peak velocities.

Shifting the baseline away from the probe is useful for seeing flow that is directed toward the probe. Peak
velocity across the AV is generally obtained in the apical 5-chamber window where the direction of flow
across the AV is away from the probe.

Decreasing the wall filters would display more low frequency signals and is not helpful when assessing
aortic peak velocity.

Using the flow convergence (PISA) method, the EROA is calculated as: EROA = (2πr 2 * Va) / Vp, where r
is the AR PISA radius, Va is the aliasing velocity, and Vp is the peak aortic regurgitation velocity. Using
the values in this case, the EROA is 0.4 cm 2.

Underestimation of the peak AR velocity will result in overestimation of EROA. Undermeasuring the
PISA radius will underestimate the EROA. The Va itself will not affect the calculated EROA, but failing to
decrease the Va will make the PISA radius smaller and more prone to measurement error. The AR
velocity-time integral is multiplied by the EROA to calculate the regurgitant volume but is not used in the
calculation of the AR EROA

For a patient with enteroccus bacteremia, native valves, and an alternative site of infection, TTE is the
first-line test to rule out endocarditis. For all of the other scenarios, TEE is appropirate according to the
American College of Cardiology appropirate use criteria.

Appropriate
Use of TEE when there is a high likelihood of a nondiagnostic TTE due to patient characteristics or
inadequate visualization of relevant structures. Re-evaluation of prior TEE finding for interval change
(e.g., resolution of thrombus after anticoagulation, resolution of vegetation after antibiotic therapy) when
a change in therapy is anticipated. Guidance during percutaneous noncoronary cardiac interventions
including, but not limited to, closure device placement, radiofrequency ablation, and percutaneous valve
procedures. Suspected acute aortic pathology including but not limited to dissection/transection.
Evaluation of valvular structure and function to assess suitability for, and assist in planning of, an
intervention. To diagnose infective endocarditis with a moderate or high pretest probability (e.g., staph
bacteremia, fungemia, prosthetic heart valve, or intracardiac device). Evaluation for cardiovascular source
of embolus with no identified noncardiac source. Atrial fibrillation/flutter: evaluation to facilitate clinical
decision making with regard to anticoagulation, cardioversion, and/or radiofrequency ablation.
Uncertain Evaluation for cardiovascular source of embolus with a previously identified noncardiac source.
Inappropriate Routine use of TEE when a diagnostic TTE is reasonably anticipated to resolve all diagnostic
and management concerns. Surveillance of prior TEE finding for interval change (e.g., resolution of
thrombus after anticoagulation, resolution of vegetation after antibiotic therapy) when no change in
therapy is anticipated. Routine assessment of pulmonary veins in an asymptomatic patient status post
pulmonary vein isolation. To diagnose infective endocarditis with a low pretest probability (e.g., transient
fever, known alternative source of infection, or negative blood cultures/atypical pathogen for
endocarditis). Evaluation for cardiovascular source of embolus with a previously identified noncardiac
source. Atrial fibrillation/flutter: evaluation when a decision has been made to anticoagulate and not to
perform cardioversion.

This patient has classical features of carcinoid syndrome, which is usually caused by serotonin-releasing
tumors of the small bowel. This paraneoplastic syndrome is characterized by symptoms and signs related
to the pharmacologic effects of the secretion of serotonin and kallikrein that causes flushing, diarrhea, and
bronchoconstriction. Serotonin is released into the hepatic portal circulation and therefore interacts with
the right-sided cardiac structures, including the tricuspid and pulmonary valves. The most common valve
lesions associated with this condition include primary tricuspid regurgitation and pulmonary stenosis.
Left-sided cardiac lesions can occur, but are rare and usually secondary to a right-to-left shunt and
increased secretion of serotonin or a bronchial carcinoid.

The echocardiographic images in Video 1 demonstrate a thickened, retracted septal leaflet of the tricuspid
valve with failure of coaptation. The history and echocardiographic images are most consistent with
carcinoid heart syndrome. Other diagnoses to be considered for this echocardiographic image include
iatrogenic changes related to drugs (e.g., phentermine), mediastinal radiation, endomyocardial fibrosis,
and Ebstein anomaly (although this would typically show apical displacement of the tricuspid valve). This
patient would be expected to have severe tricuspid regurgitation. A physical examination would show
prominent jugular V waves. Cannon A waves are seen with atrioventricular dissociation, and there is no
evidence this patient has conduction disease. Based on the information provided, there is no reason to
suspect this patient has significant mitral regurgitation (apical systolic murmur). There are no signs of left
ventricular enlargement or failure.

Color Doppler operation presents anatomic information in the conventional gray-scale form and also
rapidly detects Doppler shift frequencies at several locations along each scan line, presenting them in color
at appropriate locations in the cross-sectional image. The color map encodes the mean velocity of blood
flow at each of the sample locations in the region of interest or "color box." Continuous wave Doppler
measures the peak flow velocity along a single scan line. Pulsed wave (PW) Doppler samples the local
velocity at a specified site. Pulse repetition frequency describes the sampling rate for PW Doppler, which
in turn determines the sampling depth for PW Doppler measurements. Aliasing describes the
phenomenon when Doppler frequency shifts exceed the pulse repetition frequency and may not
accurately reflect the flow velocity being sampled.

The calculation for effective regurgitant orifice area (EROA) in this patient is (2π[0.9] 2 * 40) / 490 = 0.42
cm2.
The equation for regurgitant volume (RVol) = EROA x VTI. The RVol is thus equal to 0.42 x 180 = 75
mL/beat, consistent with severe MR. Systolic pulmonary vein flow reversal is consistent with severe MR
and is the only possible correct answer choice.

Vena contracta of 0.4 cm is consistent with moderate (progressive) MR. Although 65 mL/beat is consistent
with severe MR, it is below the calculated value in this patient. Left atrial volume index >34 mL/m 2 is
considered dilated, which would be expected with chronic severe MR but not necessarily with acute
severe MR. Regurgitant fraction cannot be calculated with the information provided, and a value ≥50%
would be consistent with severe MR.

This patient presented with symptoms suggestive of cardiac sarcoidosis, a granulomatous disease that is
more prevalent among African Americans. Noncaseating granulomas can occur in multiple organs,
including the heart. Twenty-five percent of patients with systemic sarcoidosis have cardiac involvement.
Sarcoid granulomas in the myocardium can cause regional wall motion abnormalities, usually in a
noncoronary distribution. They can also cause conduction abnormalities such as heart block and
predispose a patient to ventricular arrhythmias. Patients with sarcoidosis may have regional wall motion
abnormalities not consistent with a coronary distribution, left ventricular dilation with reduced ejection
fraction, wall thinning, and focal aneurysmal segments. Cardiac magnetic resonance imaging may be
helpful in the further evaluation of these patients.

This patient has classic features of cardiac involvement in sarcoidosis. She had a sudden cardiac arrest with
likely scar-mediated ventricular tachycardia or ventricular fibrillation. Her ECG demonstrated an
intraventricular conduction delay suggestive of disease in her conduction system. Her echocardiogram
demonstrated basal septal thinning as well as a focal inferoseptal aneurysm, both of which are suggestive
of cardiac sarcoidosis.

Cardiac amyloidosis would be expected to show diffuse left ventricular wall thickening, which is not seen
in this case. Hypertrophic cardiomyopathy classically shows asymmetric septal hypertrophy, not septal
thinning. An infarction of the proximal left anterior descending artery would not yield focal thinning
confined to the basal anteroseptum. Eosinophilic cardiomyopathy characteristically shows apical filling
resembling laminar thrombus in the absence of focal wall motion abnormalities.

This patient has severe pulmonary regurgitation (PR) in the setting of repaired ToF. Severe PR is noted by
laminar flow reversals in the main and branch pulmonary arteries and dilated right ventricle (RV) (Videos
1, 2, and 4). The spectral Doppler (Figure 1) demonstrates a dense regurgitant jet and short deceleration
time with early termination of diastolic flow (Table 1). The systolic color Doppler flow profile (Video 3) is
also abnormal likely due to increased flow from pulmonary insufficiency as well as possible residual
pulmonic stenosis seen in patients with ToF.

She should undergo cardiac MRI to document the size and function of her RV. Although
echocardiography is a vital tool in following congenital patients, MRI is the reference standard for RV size
and function; decisions for surgery in asymptomatic patients will be based on quantitation of RV size and
function. Patients with ToF can have anomalous coronary arteries which should be documented prior to
repeat sternotomy, but nuclear stress testing or invasive angiography is not indicated at this time. She
remains at risk for RV dilation and dysfunction, so annual follow-up is recommended and indicated for
patients with severe PR.

AR regurgitant volume can be calculated using either the proximal isovelocity surface area (PISA) method
or the stroke volume (SV) method. Not enough information is provided in this case for the PISA AR
calculations.

The AR volume is the difference between the calculated SV across the LVOT and the volume entering
through the mitral annulus. The SV method calculates regurgitant volume as the difference in SV through
the LVOT compared with SV through either mitral valve (MV) (Figure 1).

SV through the LVOT is calculated using the LVOT VTI and LVOT cross-sectional area: pi r2 x LVOT VTI.

SV through the MV is calculated using the mitral inflow VTI and mitral annular cross-sectional area: pi
r2 x mitral valve area (MVA) VTI.

Use of the MV SV is only valid in the absence of mitral regurgitation (MR), as in this case.

Using the information provided:

• LV SV = pi r2 x LVOT VTI = pi (2.2 / 2)2 * 34 = 129 mL

• MV SV = pi r2 x MVA VTI = pi (2.5 / 2)2 * 20 = 98 mL
• Regurgitant volume = 129 mL – 98 mL = 31 mL
This patient has signs and symptoms of acute pulmonary embolism. An echocardiogram is most likely to
show signs of right ventricular (RV) strain, RV dilation, hypokinesis (with possible McConnell's sign;
hypokinesis of the RV free wall sparing the apex). Pulmonic stenosis with RV hypertrophy is unlikely to
present acutely with hypoxia. Severe biventricular dysfunction can be chronic or acute, but would likely
present with pulmonary congestion and elevation in jugular venous pressure and/or lower extremity
edema. Systolic anterior motion of the mitral valve, a sign of left ventricular outflow tract obstruction, is
seen in hypertrophic cardiomyopathy. Although an interatrial shunt could present with hypoxemia, the
shunt would be predominantly right to left if causing hypoxemia

An athlete's heart would typically fall into the eccentric hypertrophy category

The correct answer is pericardial cyst. Pericardial cysts are benign congenital variants of the pericardium.
They occur at a rate of 1 person per 100,000 and are thought to result from a failure of the fusion of the
mesenchymal lacunae that form the pericardial sac. 75% have no associated symptoms and are usually
found incidentally during chest X-ray or echocardiography, but if the cyst compresses adjacent structures
it may result in chest pain, cough, or dyspnea. Cysts appear as circumscribed echolucent masses with little
attenuation of the ultrasound beam. They commonly occur next to the right atrium and color Doppler can
demonstrate obstruction to flow through the atrium, if present. Microbubble contrast can be used to
confirm that the cyst does not communicate with the vasculature. Computed tomography or magnetic
resonance imaging (Figure 3) can confirm the diagnosis.
Echinococcal cysts would be seen within the myocardium, often in the interventricular septum, typically
would be associated with cysts elsewhere, and usually cause systemic symptoms of infection. A hiatal
hernia would be expected to be posterior to the left atrium and would typically have a mixed echolucent
and echogenic appearance; ingestion of a carbonated beverage can confirm the diagnosis of hiatal hernia
by demonstrating the moving bubbles within the stomach and the hernia. A pericardial fat pad is also a
common finding in the anterior pericardial space, but would be heterogenous and echodense, rather than
echolucent. A persistent left superior vena cava would drain into the coronary sinus, resulting in dilation
of the coronary sinus; a normal coronary sinus is visible in the atrioventricular groove in this patient.

This patient has evidence of mild pulmonary stenosis (PS). Echocardiography is used to assess the degree
of right ventricular outflow tract (RVOT) obstruction, as well as location which can be valvular,
subvalvular, or supravalvular. Peak Doppler velocity or peak gradient is used for quantification (Table 1),
which correlates well with cardiac catheterization. Assessing the estimated RV systolic pressure (RVSP)
can be useful in confirming the gradient. If the estimated RVSP is normal or only mildly elevated, there is
unlikely significant PS. Congenital PS is much more common than acquired. The valve may be trileaflet,
bicuspid, unicuspid, or dysplastic. The most common cause of acquired PS is carcinoid valve disease.

There was not evidence of significant pulmonic regurgitation on color or spectral Doppler, although
significant PR can increase the velocity across the RVOT. Patent ductus arteriosus (PDA) would not be
expected to increase velocity across the RVOT; additionally, the normal left ventricular size is not
consistent with a hemodynamically significant PDA. Pulmonary hypertension is associated with
pulmonary artery dilation and while the elevated tricuspid regurgitation velocity suggests elevated RVSP,
accounting for the increased gradient across the pulmonic valve, the pulmonary arterial pressure is
probably normal. There is no imaging included that suggests tricuspid stenosis.
The correct answer choice is stage A: at risk for mitral stenosis (MS) with mild MV abnormality and
normal hemodynamics, no evidence of abnormalities in the LA or pulmonary vasculature, and no
symptoms (Figure 1). Medical and interventional approaches to the management of patients with valvular
MS depend on the accurate diagnosis of the cause and stage of the disease process.

Figure 1 shows the stages of MVD ranging from patients at risk of MS (stage A) or with progressive
hemodynamic obstruction (stage B) to severe asymptomatic MS (stage C) and symptomatic MS (stage D).
Unlike the staging of aortic stenosis, there are no distinctions or subcategories of stage C. Each of these
stages is defined by valve anatomy, valve hemodynamics, the consequences of valve obstruction on the LA
(i.e., LA volume index >34 mL/m 2), pulmonary circulation, and patient symptoms. The anatomic features
of the stages of MS are based on a rheumatic etiology for the disease because it is the most common cause
of MS.

Hemodynamic severity is best characterized by the planimetered mitral valve area (MVA) and the
calculated MVA from the diastolic PHT. The definition of severe MS is based on the severity at which
symptoms occur as well as the severity at which intervention will improve symptoms: an MVA ≤1.5
cm2 and a gradient ≥5-10 mm Hg at a normal heart rate. However, the mean pressure gradient is highly
dependent on the transvalvular flow and diastolic filling period, and will vary greatly with changes in
heart rate. The diastolic PHT is dependent not only on the degree of mitral obstruction, but also the
compliance of the LV and LA. Other measures of MVA, such as the continuity equation or the proximal
isovelocity surface area, may be used if discrepancies exist

The images shown are consistent with hypereosinophilic syndrome, which is a rare multisystem disease
that can present with cardiac manifestations. Early cardiac involvement includes eosinophilic infiltration,
then a thrombotic stage with thrombus formation, and finally a late fibrotic stage with endomyocardial
fibrosis. Typical echocardiographic findings in hypereosinophilic syndrome include laminar thrombus in
the apex of either or both ventricles or involving the valves (which can lead to valvular regurgitation), as
well as restrictive cardiomyopathy that develops as a result of the endomyocardial fibrosis. In this case, the
abnormalities primarily involve the right side of the heart, with an echogenic mass in the apex of the right
ventricle seen by echocardiography. A complete blood count with differential would most likely reveal
hypereosinophilia.

Cardiac sarcoid presents with wall motion abnormalities that do not follow a typical coronary distribution.
Elevated serum ACE level may be seen in these patients. Cardiac amyloidosis typically results in restrictive
cardiomyopathy with thickened myocardium, valves, biatrial enlargement, and small pericardial effusions.
Patients with amyloid light-chain amyloidosis would be expected to have elevated levels of serum free
light chains and/or an abnormal serum free kappa/lambda ratio. Carcinoid heart disease typically results in
thickening and restriction of the right sided valves with malcoaptation of leaflets. Urinary 5-
hydroxyindoleacetic acid is the end product of serotonin metabolism and is likely to be elevated in these
patients. Fabry disease manifests as a thickened, nondilated left ventricle as well as thickened papillary
muscles and/or systolic anterior motion of the mitral leaflet. Diagnosis is confirmed by reduced levels of
alpha galactosidase A enzyme.

The images show a circular echolucent structure in the atrioventricular (AV) groove. Two structures
reside here, the left circumflex and the coronary sinus. An enlarged coronary sinus due to a persistent left-
sided superior vena cava would perfuse with a left arm injection not noted in this study. The structure
takes up perflutren echocardiographic contrast suggesting an arterial structure. This is likely a coronary
aneurysm. These can be congenital or related to Kawasaki's disease. The catheterization images reveal an
exceptionally large coronary aneurysm of the left circumflex artery as the reason for the echolucent
structure seen in the AV groove on the echocardiogram (Figure 1). The most common cause of coronary
aneurysm is coronary artery disease although it would be unusual for it to lead to a coronary aneurysm of
this size. A coronary cameral fistula is a possibility, but there is no continuous murmur heard on the
physical exam which would be expected with a fistula this large. A hiatal hernia would typically be seen
posterior to the left atrium and would not enhance with echocardiographic contrast.

The ability of a pulsed Doppler system to detect frequency shift (and therefore velocity) is affected by the
pulse repetition frequency (PRF) and the frequency of the transducer. PRF is reduced at high imaging
depths (longer listening time) and increased at shallow depths (less listening time required). The upper
limit of frequency shift that can be detected is PRF/2 (the Nyquist limit). Beyond the Nyquist limit,
aliasing occurs.

The frequency shift is directly related to the transmitted (carrier) frequency. Therefore for the same
detected velocity, the frequency shift will be lower in absolute magnitude for a transducer using a lower
transmitted frequency when compared with a transducer with a higher transmitted frequency. Using a
transducer with lower frequency allows higher velocities to be detected prior to encountering the Nyquist
limit.

This patient has stage D rheumatic mitral stenosis (MS) based on MV area ≤1.5 cm 2 and dyspnea on
exertion (Figure 1). The 2014 American College of Cardiology Foundation/American Heart Association
(ACCF/AHA) guideline de-emphasizes the transmitral gradient, which may vary with heart rate and body
size, in favor of the effective MV area and associated hemodynamic consequences such as left atrial
enlargement and pulmonary pressure.

The Wilkins score (Figure 2) is the most commonly used 2D TTE assessment of the MV for planning
balloon valvuloplasty; it includes severity and extent of leaflet thickening, calcification, and involvement
of the subvalvular apparatus. Each feature is graded on a scale of 1-4, yielding a maximal score of 16. A
score >8 does not preclude percutaneous mitral balloon valvuloplasty (PMBV), but is associated with less
optimal results. In this patient with symptomatic severe MS and a Wilkins score of 8 for normal mobility
at the mid and basal mitral leaflets, thickening of the chords only near the leaflets, thickening (5-8 mm) of
just the leaflet margins, and scattered areas of calcification, PMBV is the treatment of choice.
Contraindications include moderate or greater MR and left atrial thrombus.

PMBV is preferred over MV replacement in patients for whom PMBV is likely to be successful and who
do not have contraindications. In general, the valves of patients with rheumatic MS are not amenable to
repair. Repeat echocardiography and exercise stress testing would not add significant information in this
symptomatic patient.

Guidelines recommend that the sinuses of Valsalva, sinotubular junction, and proximal ascending aorta all
be measured at end-diastole. In contrast, the aortic valve annulus should be measured in mid-systole
(when the valve is open), between the hinge points of the aortic valve leaflets. The left atrial diameter
should be measured at end-systole

Flow propagation velocity (Vp) measures the rate of left ventricular (LV) myocardial relaxation. It is one
method of assessing LV diastolic function. It is acquired in the apical 4-chamber view using color M-mode
echocardiography, with the M-mode cursor parallel to the mitral inflow. It is measured as the slope of
mitral inflow (first aliasing velocity) in early diastole, which represents the Vp of blood flowing toward
the apex. Figure 2 shows examples of Normal, Grade 1, and Grade 2 diastolic filling. Normal Vp is >50
cm/s.

The Vp is normal or increased in constrictive pericarditis and is often >100 cm/s. Vp can be reduced in
restrictive cardiomyopathy and LV systolic dysfunction. The patient is in sinus rhythm based on the end-
diastolic forward flow across the mitral valve. There is age related reduction in myocardial relaxation and
hence an elevated Vp is unlikely to be seen in a 68-year-old patient without cardiac abnormalities
Left to right shunts at the level of the atria or ventricles lead to increased flow across the pulmonary valve.
The degree of shunt can be estimated by calculating and comparing stroke volume (SV) across the RVOT
and LVOT using the following equation: SV=Cross sectional area of outflow tract x velocity time integral
(VTI). Here, Qp/Qs=138.9/61.2=2.3. This suggests significant left to right shunt and so additional imaging
is warranted.

Precise quantification of Qp/Qs by echocardiogram is limited by the dynamic nature of the RVOT
dimensions in systole and ensuring optimal Doppler angle. In the setting of RV enlargement, additional
imaging is indicated even in the setting of a normal calculated Qp/Qs by echocardiogram.

Besides primum and secundum ASDs, other potentially correctable anatomic etiologies of left to right
shunting, particularly anomalous pulmonary venous return and sinus venosus ASD, may not be seen
directly on transthoracic echocardiography. This makes it imperative to further investigate the elevated
Qp/Qs calculated in this patient with additional imaging.

Following up in 3 months alone is not appropriate since a significant shunt by Qp/Qs has been identified.
Initiation of medical therapy is not appropriate since the cause of right heart enlargement has not been
identified. A cardiopulmonary exercise stress will evaluate functional capacity, but will be unable to
identify the cause of the elevated Qp/Qs or RV enlargement.
In the setting of depressed myocardial function, the algorithm for grading diastolic function begins with
assessment of mitral inflow (Figure 4).

If the E/A ratio is ≤0.8 and the E wave velocity is ≤50 cm/s, there is grade I diastolic dysfunction. In this
example, the E/A ratio is >0.8 and the E wave velocity is >50 cm/s. In this setting 3 more criteria must be
assessed for evidence of high filling pressures: E/e' >14, TR velocity >2.8m/s, and LA volume index >34
ml/m2. If one or fewer signs of increased filling pressure is present, then grade I diastolic dysfunction is
present. In this case none of the features of increased filling pressures are present, so there is grade I
diastolic dysfunction.

SAM
Normal
MVP
Severe AI (Austin Flint)
Localization of prolapsing or flailing segments is important prior to MV surgery because of the
implications on the surgical approach. The images demonstrate prolapse and flail of the P2, or middle
scallop, of the posterior MV leaflet (Figure 1). Two-dimensional echocardiography requires combinations
of orthogonal views to localize the involved segment(s) of the MV; posterior leaflet prolapse causes
anteriorly directed MR, as seen in this case. The bicommissural view could then demonstrate which
posterior segment is prolapsed.

Three-dimensional echocardiography allows an en face view of the MV, simplifying the identification of
the MV pathology. The "surgeon's view" of the MV displays the aortic valve at the top of the screen, with
the opening of the left atrial appendage on the left side of the screen and the interatrial septum out of view
along the right side of the screen. The anterior mitral leaflet is at the top and the posterior leaflet at the
bottom of the screen, with an approximately horizontal coaptation line. The scallops of the MV leaflets are
numbered from lateral to medial; most MVs have three well-defined posterior scallops and the
corresponding portion of the anterior leaflet is given the same number, even though scallops are not as
well defined on the anterior leaflet. The three scallops may also be referred to as lateral, middle, and
medial.

This patient has a parachute mitral valve (MV), with a single papillary muscle, narrowed intrachordal
spaces, and abnormal MV leaflet tissue. It can also be a component of Shone complex, the other
components being supravalvular mitral membrane, subaortic membrane, and aortic coarctation.
A supravalvular mitral ring is a rare etiology of MS; rings are usually closer to the MV (and adherent to
the mitral leaflets in some cases) than the fibromuscular membrane of cor triatriatum. The ring often
becomes calcified in tandem with the progression of the valve lesion. Rheumatic valve disease causes a
characteristic "hockey-stick" deformity of the anterior MV leaflet along with commissural fusion of the
valve; this is the most common cause of MS in young and middle-aged patients. A double-orifice MV has
two openings. It appears similar to a MV after percutaneous edge-to-edge repair or Alfieri stitch
procedure. Carcinoid heart disease typically affects the right-sided heart valves, but may affect the left
heart valves if a shunt (such as a patent foramen ovale) or bronchial metastases are present. The serotonin
analogues secreted by the carcinoid tumor cause thickening and retraction of the valve leaflets. Ergot
alkaloids and appetite suppressants can cause similar valvulopathy.

A transthoracic echocardiogram (TTE) to establish new baseline values 6-12 weeks after MV repair is an
appropriate indication for TTE. Once this baseline is established, routine surveillance ≥3 years from the
valve repair/replacement and routine TTE as follow-up are considered appropriate. Figure 1 shows
appropriate indications for postoperative echocardiograms.

Although a transesophageal echocardiogram will provide greater visualization of the MV repair and MR if
present, this would only be the test of choice in a situation in which valve dysfunction is suspected on an
initial TTE. An exercise stress test or stress echocardiogram is not warranted in an asymptomatic patient.

A subaortic membrane leading to subvalvular obstruction should be suspected in young adults with
normal-appearing aortic valves who have elevated transvalvular gradients. The images demonstrate an
aortic valve with normal thickness and mobility with a discrete membrane just below the valve. This
condition is rarely diagnosed before birth or during infancy; rather, it manifests in the first decade of life.
This lesion can occur in isolation or in association with other congenital abnormalities including
ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, bicuspid aortic valve, and
atrioventricular septal defect, among others. As image quality can vary in patients, this lesion should be
suspected in patients who have an increased gradient through the aortic valve that is otherwise
structurally normal. Not infrequently, aortic regurgitation may be seen due to valve damage secondary to
turbulent flow.

Aortic coarctation is identified by increased Doppler gradients in the descending thoracic aorta.
Hypertrophic obstructive cardiomyopathy is characterized by increased subvalvular gradient, often in
association with systolic anterior motion of the mitral leaflets. Supravalvular aortic stenosis is more
commonly seen in association with a calcified ascending aorta, but also in congenital syndromes such as
Williams syndrome.

This patient has echocardiographic findings of BAV and aortic coarctation. Given her physical exam
findings, she most likely has Turner syndrome, a chromosomal abnormality found in girls caused by the
absence of the second X chromosome. Turner syndrome is frequently associated with BAV (16%) and
coarctation of the aorta (11-14%). The prevalence of partial anomalous pulmonary venous return in
patients with Turner syndrome is also high (25%), often co-existing with left sided lesions.
Down syndrome is chromosome 21 trisomy that is manifested by characteristic facial dysmorphism,
development delays, and congenital heart disease (40-50%), most often atrioventricular canal defects.

Williams syndrome is caused by a microdeletion of chromosome 7q11.23. Cardiac malformations are

common (75-80%), mainly consisting of supravalvular aortic stenosis and hypercalcemia.

Noonan syndrome is caused by mutations in genes in the RAS-MAPK pathway. It is characterized by short
stature, hypertelorism, and low-set posteriorly rotated ears. The most frequent cardiac anomalies are
dysplastic pulmonary stenosis, hypertrophic cardiomyopathy, and secundum atrial septal defects.

DiGeorge syndrome is caused by 22q11.2 deletion, which is the most common microdeletion found in
humans. There is wide phenotypic variability. The most frequent cardiac anomalies are conotruncal
defects including tetralogy of Fallot (20%), interrupted aortic arch type B (13%), truncus arteriosus (6%),
aortic arch anomalies, and ventricular septal defects.

Right ventricular index of myocardial performance (RIMP) is an index of global RV function that uses
IVCT, IVRT, and ET from pulsed tissue Doppler of the lateral tricuspid annulus or pulsed wave spectral
Doppler of the RV inflow and outflow. The RIMP is calculated as the (IVCT + IVRT)/ET (Figure 2). RIMP
>0.43 by pulsed Doppler and >0.54 by tissue Doppler indicates RV dysfunction. It can be falsely low in
patients with elevated right atrial pressures.
The effective regurgitant orifice area (EROA) is calculated using the below formula which is based on the
continuity principle using flow rates:

EROA = (2πr2 ×AliasingVelocity(cm/s))/Vmax(cm/s)

The radius (r) of the hemisphere formed by the aliasing of the flow velocity proximal to the restrictive
orifice is measured by moving the color map baseline in the direction of flow (for example moving the
baseline down when measuring the PISA radius of mitral regurgitant [MR] from the apical view) so that
the lower Nyquist limit is in the direction of flow. This aliasing Nyquist limit should be between 35-40
cm/s.

With the Nyquist limit at 40 cm/s and the MR peak velocity assumed to be 500 cm/s, then the formula can
be simplified to EROA is approximately r2/2. This method makes a number of assumptions and should only
be used as a screening tool or when continuous wave Doppler is not obtained or not reliable.

The calculation of the EROA is affected by eccentric jets in multiple ways. First, an eccentric jet makes the
accurate measurement of Vmax difficult. Second, an eccentric jet may cause a large portion of the PISA
shell to be constrained by a valve or chamber wall.

The PISA technique using EROA tends to overestimate MR severity in cases of mitral valve prolapse with
late systolic mitral regurgitation, as the peak flow rate is used to calculate the orifice area, but the actual
regurgitant volume is limited by the short duration of MR. Using the velocity time integral of the
regurgitation envelope to report regurgitation volume using PISA is recommended for these cases.

The PISA technique can be used to calculate the orifice area for both regurgitant and stenotic lesions. In
the setting of valvular regurgitation with multiple, well defined, PISA jets, calculated EROAs can be
summed for total EROA.

Fundamental imaging is based on the reflection of the transmitted frequency as opposed to harmonic
imaging where the harmonic frequency is generated as the ultrasound signal propagates through the tissue
(Figure 1). In fundamental imaging the ultrasound passes through the tissue twice. Single pass harmonic
imaging therefore reduces artifacts and is useful for imaging deeper structures. Doppler echocardiography
is not affected by fundamental or harmonic two-dimensional imaging. The temporal resolution of
harmonic and fundamental imaging modes is the same
Pulmonary vascular resistance (PVR) is determined invasively by dividing the pressure difference across
the pulmonary circuit by the transpulmonary flow. PVR= (Mean PA pressure - Mean left atrial (LA)
pressure)/Qp in L/min. By using the peak tricuspid regurgitation velocity (TRV) as a surrogate for pressure
and the RVOT VTI as a surrogate for flow, PVR can be estimated with the following regression equation:
PVR = TRV/RVOT VTI (cm) x 10 + 0.16.

Using the data from this case,

PVR= 4/14 x 10 + 0.16 = 3.01 Woods units

The LVEF includes the forward stroke volume and the regurgitant volume crossing the mitral valve into
the LA. Therefore, in this patient with severe MR and normal LV systolic function, the LVEF is often
hyperdynamic (>70%). An LVEF <60% in a patient with severe MR is abnormal and consistent with LV
systolic dysfunction. It is a Class I indication for mitral valve surgery in asymptomatic patients with
chronic severe primary MR and LV dysfunction (LVEF 30-60% and/or LV end-systolic diameter ≥40 mm).
This patient's LVEF is 55% (stage C2 primary MR) and therefore should be referred for mitral valve
surgery (Figure 1).
Repeating the echocardiogram is an incorrect answer choice because this patient has already met a Class I
indication for surgery. Lisinopril is not indicated in patients with normotension and severe MR. Cardiac
magnetic resonance imaging is not indicated because there is adequate information from the
echocardiogram. A treadmill stress echocardiogram is not indicated because the patient already meets the
requirements for surgery.

TDI difference vs flow doppler blood: TDI is low frequency (cycles per second)

TDI allows for the measurement of myocardial velocities, and is used to measure the motion of the
myocardium using either pulsed wave Doppler with a sample volume at a specific site in the myocardium
or color Doppler to display myocardial motion in the entire imaging plane. TDI signals are very high
amplitude, so machine power output and gain settings are low. Tissue velocities are low, so the Doppler
velocity range is small. This is in contrast to blood flow Doppler shifts which occur at higher velocities
and therefore are high frequency and low amplitude. In order to distinguish tissue from blood, the
returning low amplitude and high frequency signals are filtered out in tissue Doppler mode.
This patient has paradoxical low-flow, low-gradient AS. The calculated AVA is <1 cm 2, although the mean
gradient is <40 mm Hg and the peak velocity across the aortic valve is <4 m/sec. The stroke volume (SV) is
abnormally low. SV can be calculated by the following formula: SV = LVOT VTI x cross-sectional area
(CSA) LVOT, where CSA LVOT = 3.14 (LVOT diameter / 2)2. Thus, in this case, SV = 18 cm x (3.14 x [1
cm]2) = 56 cm3. The indexed SV = SV / BSA. Thus, in this case, the indexed SV = 56 / 2 = 28 cc/m 2.

An indexed SV <35 cc/m2 is considered low. Although her LVEF appears "normal," she actually had low
flow across her LVOT and aortic valve as a consequence of small chamber size. She is therefore classified
as paradoxical low-flow, low-gradient severe AS (i.e., stage D3) according to the 2014 American Heart
Association/American College of Cardiology (AHA/ACC) Guideline for the Management of Patients With
Valvular Heart Disease.

Bicuspid aortic valve and aortopathy are common in relatives of patients with bicuspid aortic valves.
Bicuspid aortic valve is found in 10% of first degree relatives and nearly a 1/3 of patients with bicuspid
valves have ascending aortic enlargement. Echocardiogram screening is therefore recommended in all first
degree relatives. The incidence in second degree relatives is not felt to be high enough to merit screening.
Genetic testing of unaffected relatives is inappropriate without first testing the affected individual.
Because of the risk of aortic involvement, a physical exam is inadequate for screening.

Bicuspid aortic valves (BAV) are associated with early valve dysfunction. BAV is also associated with
aortopathy and the presence of intracranial aneurysms. While approximately 50% of aortic coarctation
patients have bicuspid valves, only 5-10% of patients with BAVs have coarctations. BAVs are not
associated with a significantly increased risk of atrial septal defects, mitral valve regurgitation, or renal
disease. Partial anomalous pulmonary venous return is not commonly associated with BAVs
TEE is more sensitive for detecting left atrial (LA) or LA appendage thrombus, which are common in
patients with rheumatic MV disease and must be excluded prior to percutaneous interventions for the
valve disease. TEE can also detect spontaneous contrast, which may be a better predictor of thrombosis
risk than LA size.

LA size, most reliably expressed as the LA volume and indexed to body surface area, can be reproducibly
measured from transthoracic windows. The pulmonary vascular resistance can be estimated from Doppler
techniques such as the ratio of peak tricuspid regurgitant velocity to the right ventricular outflow tract
velocity-time integral, but is only measured directly by right heart catheterization. The transmitral
gradient can be readily measured using Doppler echocardiography from the apical (transthoracic) window;
TEE is not required. The transmitral gradient is not the preferred parameter for grading severity of MS
because the gradient is dependent on heart rate and loading conditions (volume status) in addition to the
MVA. Assessment of left ventricular diastolic function is confounded by the presence of hemodynamically
significant MV disease no matter the imaging technique used.

This patient has symptomatic low-flow, low-gradient aortic stenosis (AS) with a reduced ejection fraction
(i.e., LVEF <50%) and an AVA <1 cm 2. She is classified as having stage D2 AS according to the 2014
American Heart Association/American College of Cardiology (AHA/ACC) Guideline for the Management
of Patients With Valvular Heart Disease. In stage D2 AS, a low-dose dobutamine stress echocardiogram
(DSE) is reasonable (Class IIa) in patients with all of the following: 1) calcified AoV with reduced systolic
opening; 2) LVEF <50%; 3) calculated AVA ≤1 cm 2; and 4) aortic velocity <4 m/sec or mean pressure
gradient <40 mm Hg.

Severe AS is present in some of these patients. However, others will have only moderate AS with reduced
AoV opening due to myocardial dysfunction resulting in low flow across the AoV. To differentiate
between moderate and severe AS in these patients with low-flow, low-gradient AS and left ventricular
systolic dysfunction (LVEF <50%), a low-dose DSE may be useful. An increase in the mean gradient to >40
mm Hg or peak AoV velocity to ≥4 m/sec would be consistent with severe AS.

This patient does not yet meet the criteria for valve replacement, so referral for any type of valve surgery
would be premature. A transesophageal echocardiogram would not add additional information at this
time. Computed tomography for calcium scoring of the valve may be useful in those with poor flow
reserve on DSE (Figure 1)

The MVA can be calculated using the proximal isovelocity surface area (PISA) technique. At a Va between
18-30 cm/sec, the radius of the proximal isovelocity contour converging on the mitral orifice in the left
atrium can be measured and the peak diastolic mitral velocity (Vp) obtained with continuous-wave
Doppler imaging. When using PISA for mitral stenosis, the PISA shell is often not a hemisphere but is
constrained by the domed mitral leaflets; the surface area of the shell must be corrected using a factor
equal to the angle (a) between the leaflets divided by 180 degrees (Figure 1).
The MVA is then calculated using the following equation: MVA = 2πr 2 x (Va / Vp) x (a / 180), where
radius (r) is in centimeters, Va is in centimeters per second, and Vp is in meters per second. The PISA
equation is an extension of the law of the conservation of mass, based on the principle that all blood
flowing across the PISA shell must subsequently cross the mitral orifice; the product of Va and measured
surface area of the shell must equal the product of peak velocity of the stenotic orifice and the orifice area.
MR, AR, heart rate, and diastolic filling do not affect this calculation.

Endurance athletes frequently have enlarged hearts that can be difficult to differentiate from pathologic
conditions such as hypertrophic cardiomyopathy (Figure 1).

Left atrial and left ventricular chamber enlargement may be benign findings and, in some cases, regress to
normal with detraining. Mild degrees of left ventricular wall thickening are not specific for hypertrophic
cardiomyopathy; most authorities agree that a wall thickness of >13-15 mm may require further
evaluation. Rapid early diastolic filling is also common in young healthy hearts with vigorous passive
relaxation kinetics. Normal mitral annular tissue Doppler velocities would support the diagnosis of an
athlete's heart, whereas any myocardial pathology would generally be associated with low tissue Doppler
velocities

The M-mode image demonstrates premature closure of the mitral valve (MV), with closure of the MV
well before the R wave. This finding is seen with severe aortic regurgitation (AR). Severe AR is associated
with a rapid rise in left ventricular diastolic pressure (LVDP) and rapid diastolic equilibration of pressures
between the aorta and left ventricle. As a result, LVDP may equal or exceed left atrial pressure, resulting
in early closure of the MV. This may also result in late diastolic mitral regurgitation. Other findings in
severe AR relate to the rapid equilibration of aortic diastolic pressure and LVDP, which results in a short
pressure half-time (typically <200 msec).

Figure 1 is an M-mode of the MV, not the aortic valve, so aortic stenosis cannot be evaluated. Systolic
anterior motion of the MV is characterized by movement of the anterior MV leaflet toward the septum in
systole, which is not observed in this case. Similarly, MV prolapse is characterized by posterior bowing of
the mitral leaflets in systole, which is not observed in this case.

Vegetations or masses may appear as independently mobile echoes on M-mode tracings. Only normal
anatomic structures are seen in this tracing

The AR PHT reflects the aortic to left ventricular (LV) diastolic pressure gradient, with shortening of the
PHT reflecting more rapid equilibration of pressures. PHT of >500 msec is suggestive of mild AR, whereas
PHT of <200 msec suggests severe AR. However, factors other than the severity of regurgitation will also
affect the diastolic aortic to LV pressure gradient and PHT. Reduced LV chamber compliance, as seen with
significant LV diastolic dysfunction, will increase LV diastolic pressure, diminish the aortic to LV diastolic
pressure gradient, and shorten the PHT. Underestimation of the peak AR velocity, a common issue
(particularly with eccentric regurgitant jets), will result in an erroneous overestimation of the PHT.
Neither mitral regurgitation nor aortic stenosis should affect the AR PHT. Anemia does not affect the
PHT. MR does not affect the AR PHT. A ventricular septal defect would cause volume loading of the
ventricle and the larger ventricle would lead, if anything, to a longer PHT.
The correct answer choice is undermeasurement of the LVOT diameter, which is a factor in the
numerator of the continuity equation for MVA: MVA = (LVOT CSA x LVOT VTI) / MV VTI, where CSA
is the cross-sectional area and VTI is the velocity-time integral; LVOT CSA is calculated as: π(LVOT
diameter / 2)2. Undermeasurement of the LVOT diameter is a common source of error in the continuity
equation. Use of the continuity equation assumes that all blood volume is accounted for by forward flow
across the MV and the LVOT. This is no longer valid in the case of significant aortic regurgitation (AR) or
mitral regurgitation (MR). AR increases the LVOT VTI (forward SV) and thus overestimates the MVA.
MR increases the MV VTI and thus would underestimate the MVA.

Hyperdynamic circulatory states such as hyperthyroidism, anemia, and pregnancy can increase the
transmitral gradient, but will not affect the continuity equation valve area calculation because SV
measured at both the MV and at the LVOT will increase proportionately. Diastolic dysfunction affects the
deceleration time of diastolic filling and therefore the pressure half-time; diastolic dysfunction leads to
overestimation of the MVA when using the pressure half-time equation or the deceleration time form of
the equation.

This is an asymptomatic patient with severe aortic stenosis (AS). According to the 2014 American Heart
Association/American College of Cardiology (AHA/ACC) Guideline for the Management of Patients With
Valvular Heart Disease, it reasonable to perform an exercise treadmill test to further evaluate exercise
capacity and to confirm the absence of symptoms.

An aortic valve replacement (AVR) would be indicated in severe AS if stress testing confirmed diminished
exercise tolerance (i.e., not truly "asymptomatic"), a decrease in systolic blood pressure below baseline, or
a failure to increase systolic blood pressure by 20 mm Hg with exercise (Figure 1).

AVR in asymptomatic AS is also indicated in the following situations:

1. Asymptomatic severe AS with an LVEF <50%;

2. Asymptomatic severe AS undergoing other cardiac surgery; and
3. Asymptomatic very severe AS (Vmax >5 m/sec) and low operative risk

It is important to note that symptomatic, severe AS has a Class I indication for valve replacement. Exercise
testing should not be performed in symptomatic patients with severe AS (Class III). If this patient had
been symptomatic, he should have been referred for valve replacement and stress testing would have been
contraindicated.
Left heart interventional procedures require precise location of the trans-septal puncture site to allow a
coaxial catheter approach to target structures and provide room within the LA for device manipulation
(working height).

Complementary views of the interatrial septum with transesophageal or intracardiac echocardiography are
important for optimizing trans-septal access. Short-axis (30-75 degrees) views provide anteroposterior
orientation, and bicaval (90-135 degrees) views provide inferosuperior orientation.

As depicted in Figure 1, posterior access to the LA is preferred for MV procedures (red dots).
Anterosuperior access, typically through the patent foramen ovale (PFO) is preferable (yellow dot) when
closing a PFO. Access along the anterior rim of the fossa ovale (orange dot) is beneficial for accessing
posterior structures such as the pulmonary veins. Access at the inferior limbus, ideally somewhat
posteriorly (green dot), allows the catheter to lie coaxially with the LA appendage. When a trans-septal
puncture is performed for hemodynamic studies or for left ventricular assist device implantation, the most
direct and safest approach corresponds to the middle of the oval fossa (blue dot).

The TEE images show the typical findings of a superior sinus venosus defect (Figure 1). These defects are
located superiorly near the superior vena caval entry and often involve partial anomalous pulmonary
venous return of the right upper and middle pulmonary veins. Unrepaired, they are associated with
chronic RV volume overload and pulmonary over-circulation. Symptoms include exercise intolerance,
fatigue, and palpitations. Late complications include atrial arrhythmias, right heart failure, and pulmonary
hypertension. The TEE demonstrates the remaining atrial septum is intact, including the regions involved
in patent foramen ovales, primum ASDs, and unroofed coronary sinuses.

These echocardiographic images are consistent with congenitally corrected transposition of the great
arteries (ccTGA), which is atrioventricular (AV) discordance and ventricular-arterial discordance. The
clinical course is variable, depending on associated lesions, systemic AV valve function, and systemic right
ventricular function. Patients may present for the first time in adulthood when they develop heart failure
or valvular dysfunction. The echocardiographic features of ccTGA include left-sided apically displaced
tricuspid valve with chordal attachments to the inlet septum and the absence of distinct papillary muscles
in the systemic ventricle (Video 1, Video 2). The great arteries have an abnormal relationship with the
aorta anterior and are leftward of the pulmonary artery (Video 5, Video 6). Continuous-wave Doppler
signals can be helpful in identifying great arteries based on the regurgitation velocity which is typically
much higher in aortic regurgitation compared with pulmonary regurgitation (Figure 1). In the parasternal
long axis view, there is a lack of fibrous continuity of the AV valve and the aorta (Video 6). The anterior
position of the aorta is also appreciated.

ccTGA is often confused with left ventricular noncompaction due to the prominent trabeculations of of
the systemic right ventricle. The apical displacement of the AV valve can help distinguish the two
disorders. Dilated cardiomyopathies can be associated with prominent trabeculations as well, but these
patients lack the other features of ccTGA. Ebstein anomaly is an abnormality of the right-sided tricuspid
valve. Although patients with ccTGA can have an Ebsteinoid valve, it is distinct from Ebstein anomaly.
Patients with D-transposition of the great arteries, status post Mustard or Senning atrial switch procedure,
have a systemic tricuspid valve and a systemic right ventricle, but they are located on the right side of the
heart. They are usually diagnosed at birth and surgery is performed during infancy.

The flow convergence method requires adequate visualization of the AR flow convergence on the aortic
aspect of the aortic valve so that the proximal isovelocity surface area (PISA) radius can be accurately
measured and a high-quality, complete continuous-wave (CW) spectral Doppler profile of the AR flow so
that peak AR velocity can be accurately measured. The most common limitations in making these
measurements are significant aortic calcification that results in shadowing of the flow convergence and
the inability to perform an accurate PISA measurement and eccentric AR that results in nonhemispherical
flow convergence and/or incomplete AR CW profile. Although color and CW Doppler assessments are
typically obtained from the apical position, in the case of eccentric jets, they should also be interrogated
from the parasternal position because data quality may be better from this position in such cases. The
presence of aortic stenosis or regurgitant lesions of other valves does not affect the accuracy of the flow
convergence method.

The continuity equation method, also called the quantitative Doppler technique, involves comparing the
calculated stroke volume (SV) based on flow through the left ventricular outflow tract (LVOT) to the
calculated SV based either on flow through the mitral valve or through the pulmonary valve. SV is
calculated using pulsed-wave Doppler through the valve being used for comparison. If significant MR and
PR are both present, the SV method cannot be used.

Theoretically, the LVOT Doppler SV can be compared with the two-dimensional derived SV to quantify
MR volume, but not AR volume.

This patient has peripartum CM, marked by left ventricular dysfunction and heart failure (HF) syndrome.
Most cases present within weeks of delivery, but onset within the second or third trimester of pregnancy
or within 5 months after delivery is possible. Pre-eclampsia, hypertension, multiparity, and increased age
are all risk factors. Intracardiac thrombus may be identified on the first echocardiogram (Video 1), as in
this patient, and in 10-17% of cases; systemic thromboembolism is more common in peripartum CM than
in other CMs. Prolactin has been implicated in peripartum CM on the basis of a mouse model and studies,
suggesting a benefit of prolactin blockade with bromocriptine.

Serelaxin, a vasodilating hormone that is upregulated in pregnancy, was proposed for treatment of
diastolic HF, although clinical studies failed to show benefits in mortality or hospitalization among
patients with acute HF. Pentraxin-3 is an inflammatory biomarker that has been associated with better
outcomes in patients with HF and was able to reduce myocardial damage in a mouse model of ischemic
CM. Aldosterone is a sodium-retaining, vasoconstricting hormone upregulated in HF of any cause.
Endothelin-1 is produced in response to angiotensin II, inflammatory mediators, and vascular shear stress;
it is responsible for vasoconstriction, activation of reactive oxygen species, and ventricular remodeling,
and is upregulated in HF of any cause.

Swirling artifact is typically seen in the near field of the echocardiogram image and is caused by
microbubble destruction from high ultrasound energy. When swirling artifact is seen, several things may
be done to improve the quality of the image; reducing the mechanical index, increasing contrast infusion
rate, reducing frame rate, or moving the focus to the near field. Decreasing contrast infusion rate and
increasing frame rate may worsen swirling artifact (Video 2). Increasing gain or narrowing sector width
would not be expected to reduce swirling

The correct answer is atrial septal defect/patent foramen ovale (ASD/PFO), which is seen in 50% of
patients with Ebstein anomaly. For that reason, it is recommended that patients with Ebstein undergo
exercise testing with oxygen saturations; patients who desaturate with exercise should have their
interatrial defect closed.

This patient has features of Ebstein anomaly based on his echocardiographic findings (apically displaced
septal leaflet of the tricuspid valve, which leads to atrialization of the right ventricle [RV]). This can lead
to conduction abnormalities (e.g., RBBB, or right-sided Wolff-Parkinson-White syndrome) and SVT.

Although ASD/PFO is most associated with Ebstein anomaly, other less-common associations include
ventricular septal defect, patent ductus arteriosus, and left ventricular noncompaction. There is also an
association with pulmonary stenosis or pulmonary atresia. Over-riding aorta is seen in tetralogy of Fallot
or double-outlet RV. In congenitally corrected transposition, Ebstein anomaly may affect the systemic
ventricle (morphologic RV).

Symptoms of chronic primary MR generally occur during exercise. Evaluation during exercise may be
very informative when resting transthoracic echocardiogram and symptomatic status are discordant. In
such cases, the severity of MR and/or pulmonary artery (PA) pressure may increase during exercise, both
helping to explain exercise-induced symptoms and indicating that mitral surgery may be indicated.

Her exercise PA pressure is calculated using the formula for RV systolic pressure: (4 x [TR jet velocity] 2) +
right atrial (RA) pressure. An exercise peak TR jet velocity of 4 m/sec and her PA systolic pressure of 64
mm Hg plus RA pressure are consistent with exercise-induced pulmonary hypertension (PH; PA systolic
pressure >60 mm Hg). Exercise-induced PH is associated with a significantly reduced 2-year symptom-free
survival (Figure 1). Secondary PH is common in patients with left-sided valve disease and carries
important prognostic significance (Figure 2). The peak TR velocity of 3 m/sec may represent a normal
response to exercise.

Changes in the peak MR velocity and early mitral inflow (E-wave) velocity during exercise likely reflect a
change in loading conditions and are unlikely to carry significant prognostic value.
Because of equalization of right atrial (RA) and right ventricular (RV) pressures, the RV systolic pressure is
often underestimated by two-dimensional Doppler. This patient has severe tricuspid regurgitation (TR),
which can be associated with RA enlargement, RV dilation, and dilation of the inferior vena cava. These
findings are not always present but are not likely to be underestimated in chronic severe TR. The early
diastolic tricuspid E velocity is usually elevated in severe TR.
The empiric formula for MVA is as follows: MVA = 220 ⁄ PHT.

MVA can also be calculated from the formula: MVA = 759 / deceleration time (DT). In this case MVA =
1.1 cm2.

Deceleration time can also be converted to PHT by the formula: PHT = 0.29 * DT,

The simplified Bernoulli equation (ΔP = 4vmax2) assumes that vmax is significantly greater than the proximal
velocity across a stenosis, and that the proximal velocity is approximately 1m/s (or less). If these
assumptions are not true, the proximal velocity should be included in the calculation: ΔP = 4(vmax2 -
vprox2).

For the restrictive VSD, the pressure gradient can be calculated using the simplified equation:
ΔP = 4(52) = 100 mm Hg.

For the nonstenotic aortic and pulmonic velocities, the proximal velocity (outflow tract velocity) is similar
to the max velocity, and the ΔP across these valves is negligable.

Given a systemic SBP of 140 mm Hg and no aortic stenosis, the left ventricular systolic pressure is
estimated to be 140 mm Hg. The ΔP between the left ventricle and right ventricle is 100 mm
Hg based on the VSD velocity, leaving 40 mm Hg as the RV systolic pressure, which is
also the pulmonary systolic pressure given the nonstenotic pulmonary valve.

Higher frequency (and therefore shorter wavelength) provides increased spatial resolution with
ultrasound imaging. High frequency ultrasound is well suited for near field imaging, but does not
penetrate to visualize deep structures as well as lower frequency ultrasound. Increasing nearfield time-
gain-compensations will make near field targets brighter, but will not change spatial resolution.
Decreasing the depth and narrowing the sector arc will increase temporal resolution, but will not change
the spatial resolution
The most common cause of paradoxical pulse is asthma or chronic obstructive pulmonary disease
exacerbation because there are exaggerated intrathoracic pressure changes with inspiration and expiration
which are transmitted to the circulatory system as the diaphragm moves. Pericardial tamponade is the
second most common cause of paradoxical pulse. Echocardiography is an important diagnostic test because
it can differentiate enhanced ventricular interaction from pericardial causes versus extracardiac causes of
paradoxical pulse. Findings such as pericardial effusion or thickening, plethora of the inferior vena cava,
and diastolic chamber collapse would be seen with pericardial tamponade, but not in respiratory causes of
paradoxical pulse. If diastolic heart failure were the cause of this acute presentation, the inferior vena cava
would be dilated with a lack of respiratory variation. Pneumothorax can be associated with pulsus
paradoxus, however the clinical exam describes equal breath sounds bilaterally. Myocardial ischemia can
cause pulsus alternans due to profound left ventricular dysfunction, but not pulsus paradoxus

Sole reliance on jet area to quantify degree of regurgitation can be misleading. Eccentric wall-impinging
jets appear significantly smaller than centrally directed jets of similar hemodynamic severity. A jet may
appear larger by increasing the driving pressure across the valve (higher momentum); hence the
importance of measuring blood pressure for left heart lesions at the time of the study, particularly in the
intraoperative setting or in a sedated patient. Entrainment will increase jet area as the blood pool in the
receiving chamber is pulled into the regurgitant jet. Increasing the color gain and decreasing the Nyquist
limit will both increase the jet area.
There is a large fluid-filled structure with color Doppler flow predominantly during systole. The
differential includes coronary sinus fistula, pseudoaneurysm, aortic dissection, and coronary artery fistula.
Further investigation with another modality would help clarify this.

A computed tomography angiogram was obtained and showed a large aneurysmal right main coronary
button measuring up to 6 cm across with the neck measuring 17 x 15 mm (Figure 1). Small button
aneurysms can be seen after a Bentall procedure; however, giant button aneurysms are rare.
Pseudoaneurysms are more common, but, as this case illustrates, understanding the anatomy is crucial
prior to consideration for surgery.

There is no clinical evidence of endocarditis and therefore antibiotics or inflammation imaging are not
appropriate. Although blood pressure control is important, there is no evidence of aortic dissection and
therefore parenteral antihypertensive medications are not indicated

Crista terminalis is a well-defined fibromuscular ridge separating a smooth sinus venarum and
trabeculated RA. Externally, it corresponds to the sulcus terminalis, and internally, it extends from the
superior vena cava (SVC) to inferior vena cava (IVC) along the lateral RA wall. Embryologically, crista
terminalis develops from the septum spurium, which corresponds to the fused boundary between the
embryonic sinus venosus and the RA proper. Prominent crista terminalis may be confused for a RA tumor
on transthoracic echocardiography. Echocardiographic findings suggestive of prominent crista terminalis
instead of a tumor include: a nodular mass of similar echogenicity with adjacent myocardium; the location
of the posterolateral wall of the RA near the SVC, which corresponds to the course of crista terminalis
connecting the SVC and IVC; the phasic change in size becoming thicker or larger during atrial systole.

The tip of a catheter is usually bright and independently mobile. The Thebesian valve sits at the ostium of
the coronary sinus and is best noted in the right ventricular inflow view and not the 4-chamber view. The
Eustachian valve would be seen as a linear target traversing the posterior aspect of the RA in the 4-
chamber view. Mass/thrombus is unlikely given the normal structure of the RA

This patient is presenting with left bundle branch pattern VT with superior axis and T wave inversions
throughout the precordium, both of which are major criteria for right ventricular (RV) cardiomyopathy;
the presence of two major criteria confirms the diagnosis. Echocardiographic findings in RV
cardiomyopathy include RV dilation, akinesia or dyskinesia and/or aneurysm formation, and a thin walled
right ventricle due to fibrofatty replacement of the RV wall.

Biventricular dysfunction with a diffusely thickened myocardium would be more consistent with an
infiltrative cardiomyopathy such as amyloidosis. Biventricular dysfunction with apical thrombi describes
eosinophilic myocarditis. RV hypertrophy with strain would more likely be seen in pulmonary
hypertension. Asymmetric septal hypertrophy with systolic anterior motion of the mitral valve would be
expected with hypertrophic obstructive cardiomyopathy
This patient has a left ventricular (LV) pseudoaneurysm characterized by a echolucent space with a
narrow neck and no overlying myocardium. To-and-fro flow is evident during the microbubble contrast
enhanced imaging. These findings distinguish pseudoaneurysms from true aneurysms of the LV, which
have a wide neck and intact layers of (akinetic or dyskinetic) myocardium. In this patient, the
pseudoaneurysm was a result of catheter ablation in the LV apex complicated by "steam pop" due to
excessive power delivery. Incardiac echocardiography during the procedure (Video 3) demonstrates the
small hole left by the catheter that matured into the pseudoaneurysm. This was repaired surgically.

Stress cardiomyopathy would be associated with akinesis or dyskinesis of the apical portions of the LV
with preserved function of the basal segments. Pericardial cyst would not communicate with the LV.
Right ventricular (RV) cardiomyopathy may present with RV apical aneurysms, but these would not
communicate with the LV chamber.

This patient has an elevated gradient across the PV consistent with pulmonic stenosis (PS). In the absence
of PS, the pulmonary artery (PA) systolic pressure is equal to the right ventricular systolic pressure (the
TR gradient plus the RA pressure). In the presence of PS, the PA systolic pressure is equal to the right
ventricular systolic pressure minus the PV gradient (estimated as 4v^2). In this case the PA systolic
pressure would be (50+5)-4(3.0^2)=19 mm Hg.

Image resolution is determined by wavelength, which is the reciprocal of transducer frequency. The depth
of penetration of the ultrasound waves into the body is directly related to wavelength; lower frequency
ultrasounds (longer wavelengths) will penetrate deeper into the body. Thus, there is an obvious tradeoff
between image resolution (shorter wavelength or higher frequency preferable) and depth penetration
(longer wavelength or lower frequency preferable). The lowest frequency transducer (the 3mHz vascular
probe) will therefore have the greatest depth of penetration.
The correct answer choice is a mitral valve area (MVA) of 3 cm 2. Percutaneous edge-to-edge repair of the
MV using the MitraClip system (Abbott Laboratories, Abbot Park, Illinois) is currently the only
commercially available option for percutaneous treatment of MR. In EVEREST I (Endovascular Valve
Edge-to-Edge Repair Study I) and EVEREST II (Endovascular Valve Edge-to-Edge Repair Study II), 107
patients with grade 3 to grade 4+ MR were treated with the device, with a 9% major complication rate and
a 74% procedural success rate. Anatomic eligibility criteria included coaptation ≥2 mm, coaptation depth
<11 mm, flail gap <10 mm, and flail width <15 mm (Figure 1). These characteristics are associated with the
highest success rates; MVs with borderline values for these measurements may still be treated but with
lower anticipated success rates or with multiple devices. Mitral stenosis (MVA ≤2 cm 2) is an absolute
contraindication to edge-to-edge MV repair, and MVA between 2-3.5 cm2 is a relative contraindication
due to the risk of producing significant mitral stenosis.

Fractional shortening gives a rough estimate of LV systolic function using linear dimensions. Linear
internal measurements of the LV at end-diastole and end-systole should be performed in the parasternal
long-axis view, perpendicular to the LV long axis and measured at or below the level of the mitral leaflet
tips using two-dimensional or M-mode echocardiography. Using these measurements to calculate
fractional shortening can be misleading in the setting of regional wall motion abnormalities.

The equation for fractional shortening is: FS % = (LVIDd – LVIDs) / LVIDd x 100. Normal values for
fractional shortening are approximately 25-45%. In this case, where the LVIDd = 6.0 cm and LVIDs = 5.4
cm, the FS = (6.0 – 5.4) / 6.0 x 100 = 10%. This is consistent with severe LV systolic function

Guidelines recommend that measurement of LV wall thickness and chamber diameter be performed in the
parasternal long axis view, perpendicular to the long axis of the LV, at the level of the mitral valve leaflet
tips. To assess the interventricular septum, the measurement is made from the right ventricular (RV)
myocardial blood interface to the LV myocardial-blood interface. Care should be taken to avoid including
RV septum marginal trabeculations (moderator band). LV diameter should be measured from the septal
myocardial-blood interface to posterior wall myocardial-blood interface. Measurement of the posterior
wall should be made from the blood-myocardial interface to the interface between the myocardium and
pericardium.

The correct answer choice is rheumatic mitral stenosis. The equation 220 / PHT = mitral valve area is
widely used for the quantification of mitral stenosis in rheumatic valve disease. This equation assumes
normal left ventricular diastolic function.

Calcific mitral stenosis is usually associated with hypertension, end-stage renal disease, and other disorders
that are likely to cause diastolic dysfunction. Coexisting aortic regurgitation increases the left ventricular
pressure throughout diastole, invalidating the PHT equation for mitral valve area. Mitral regurgitation
markedly increases the early diastolic left atrial pressure, which also invalidates the PHT equation. The
PHT can be used to semiquantitatively evaluate bioprosthetic mitral valves, but the relationship is not
consistent enough to calculate mitral valve area.

The most common cause of PV stenosis is congenital. Normal PVs are trileaflet, but congenitally stenotic
valves may be trileaflet, bicuspid, unicuspid, or dysplastic. Calcification is rare, especially at this patient's
age. Acquired PV stenosis may be from carcinoid disease or, less commonly, from rheumatic disease.
Congenital pulmonary stenosis is almost always associated with dilation of the pulmonary artery,
independent of the degree of the stenosis. Right ventricular (RV) hypertrophy is common with pulmonary
stenosis, particularly with more severe stenosis.

PV stenosis is typically quantified in terms of the peak Doppler velocity and gradient (Figure 2). The mean
Doppler velocity may correlate more closely with catheter peak-to-peak gradients, but is less commonly
used for quantification.

RV enlargement may occur in advanced cases of severe pulmonary stenosis or when associated with
significant pulmonary regurgitation, but would not be expected with moderate stenosis. PV stenosis may
occur in conjunction with ventricular septal defects or as part of complex congenital lesions such as
tetralogy of Fallot or double-outlet RV; it may be seen with Noonan syndrome and Williams syndrome,
but may also occur in isolation. There is no established connection to bicuspid aortic valve, coarctation of
the aorta, or atrial septal defects.
Speckles are random patterns of reflected ultrasound, unique to each area of the myocardium. These
speckles can be tracked, and the change in distance between individual speckles during myocardial
contraction is measured to calculate strain. The three main types of strain used clinically are: 1)
longitudinal strain where the myocardium becomes longer/shorter; 2) radial where the myocardium
becomes thicker, thinner; and 3) circumferential where the circumference of the myocardium gets
bigger/smaller. The benefits of speckle tracking strain include high temporal resolution and angle
independence. Limitations of speckle tracking strain include vendor dependence and the need to measure
in previously acquired images.

Due to shadowing of the mechanical prosthesis in the aortic position, the anterior aortic root is not well
visualized by TEE. A TTE in the parasternal window may have improved visualization of the anterior
aortic root in the setting of acoustic shadowing from the mechanical valve. In the case of TEE, the imaging
probe is posterior to the heart, allowing better visualization of posterior structures including the mitral
valve, left atrium, and posterior aortic root

This patient's findings are most consistent with sarcoidosis, a noncaseating granulomatous disorder that
can affect the heart in isolation or concurrently with other organ involvement. The typical clinical
manifestations of cardiac involvement include conduction abnormalities (high-degree atrioventricular
[AV] block or bundle branch block), atrial or ventricular tachyarrhythmias, cardiomyopathy, and sudden
cardiac death.

The diagnosis should be suspected in any patient (particularly patients <60 years of age) with unexplained
high-degree AV block, ventricular tachycardia, or reduction in LVEF. Echocardiographic findings in
cardiac sarcoidosis are nonspecific, but may include LV cavity dilation, segmental wall thinning or
increased thickness, or regional wall motion abnormalities in a noncoronary distribution. Although data
are limited, treatment strategies are focused on controlling inflammation (when present) with
glucocorticoids or alternative steroid-sparing immunosuppressive regimens. There is no evidence that
beta-blockers, nonsteroidal anti-inflammatory drugs, or statins alter the course of the disease.

Treatment with angiotensin-converting enzyme inhibitors such as lisinopril has been best studied for
patients with LVEF <40%

In a post transplant patient, biopsy related complications must be considered. In this individual with an
elevated JVP (classically CV waves) and lower extremity edema, right heart dysfunction must be
considered. The echocardiography images show no evidence of annulus reversus as would be seen with
constrictive pericarditis. The hepatic vein doppler reveals systolic reversal of flow with every cardiac cycle
consistent with severe tricuspid regurgitation (TR). The echocardiography in Video 1 shows a flail
tricuspid leaflet, likely a result of cardiac biopsy. A large color jet vena contracta coupled with reversal in
the hepatic veins is consistent with severe TR.

Echocardiographic findings consistent with RV dysfunction include a tricuspid annular plane systolic
excursion of <1.7 cm, a fractional area change of <35%, a tissue Doppler tricuspid annular systolic (S')
velocity of <9.5cm/s, and a myocardial performance index of >0.43 by pulsed Doppler and >0.54 by tissue
Doppler. Normal RV ejection fraction derived by three-dimensional imaging is ≥45% (Table 1).

Video 1 demonstrates a mechanical mitral valve with a fixed leaflet. The most likely etiology in this
patient with a subtherapeutic INR is thrombosis. Other considerations include pannus formation. Given
her acute onset of symptoms and current New York Heart Association (NYHA) class IV status, she should
proceed directly to emergency surgery rather than a trial of fibrinolytic therapy (Figure 1).

As this is a structural valve problem, intra-aortic balloon pumps and inotropes would not be effective.
Although she needs to be on warfarin long term, there is no indication for a higher INR target because she
was subtherapeutic on admission.
Differentiating between pannus and thrombus can be difficult, but transesophageal echocardiography is
often helpful in determining the cause of fixed leaflets in mechanical valves.

Rastelli operation is a 2 ventricle repair of D-TGA, pulmonary stenosis, and VSD in which the VSD is
closed with a patch redirecting the LV to the aorta (Figure 3). A homograft or conduit is used to connect
the RV to the pulmonary artery (PA). This patient's homograft had deteriorated as expected for a
bioprosthetic valve. The homograft lies directly behind the sternum, making it difficult to image on
transthoracic echocardiogram. Because of its location, it is associated with very loud murmurs and
palpable thrills.

Atrial septal defects and Eisenmenger syndrome are not typically associated with murmurs. Although the
tricuspid regurgitation velocity is elevated, consistent with elevated RV systolic pressure, the murmur and
Doppler findings of conduit stenosis suggest RV hypertension without PA hypertension. Eisenmenger
syndrome is associated with hypoxia. There is no evidence of aortic valve stenosis on the echocardiogram
(peak velocity across the aortic valve is 1 m/s). Although the patient has tricuspid regurgitation, it would
not be expected to generate a 4/6 systolic murmur
This patient has cleft mitral valve (MV). This is part of an atrioventricular (AV) septal defect, which can
include a primum atrial septal defect, a ventricular septal defect, both, or neither (Figure 1). The atrial
valve in a patient with an AV septal defect has five leaflets, including a superior bridging leaflet, an
inferior bridging leaflet, a left mural leaflet, a right anterosuperior leaflet, and a right inferior leaflet
(Figure 2). The cleft appearance relates to the commissures of this complex valve. "Dropout" artifacts can
sometimes produce the appearance of a cleft in the MV, but "sweeping" short/long-axis views can confirm
the presence of a true cleft; three-dimensional en face views can also be helpful (Videos 3, 4).

There is no evidence of redundant valve tissue to suggest prolapse, and the leaflet tips are not thickened to
suggest rheumatic disease. The MV is structurally abnormal, so by definition this is not a functional
murmur. A subaortic membrane can also cause a systolic murmur but is not seen here
TEE videos demonstrate a normal appearance of the ridge between the left pulmonary veins and the left
atrial appendage (LAA: so-called Coumadin ridge, warfarin ridge, or 'Q-tip sign'). Based on the shape of
the Coumadin ridge, it projects out into the main beam axis of the ultrasound transducer creating an
artifact that can be confused with thrombus.

In general, altering the angle or position of the transducer will resolve this type of artifact, which is called
a reverberation artifact. Unfortunately, it was not possible to fully exclude the Coumadin ridge and resolve
this imaging artifact. Given that the artifact had an appearance consistent with a reverberation artifact,
pulsed wave Doppler showed normal LAA velocities, and there was no evidence of thrombus on color
Doppler, the patient ended up proceeding to cardioversion.

The administration of echocardiographic contrast can also be used in this situation to exclude the
possibility of a LAA thrombus. Since "proceed to cardioversion" was not an answer option, administration
of echocardiographic contrast is the best answer. In some cases this ridge may be confused with a cardiac
mass particularly when it is thicker. However, this is part of the spectrum of the normal appearance;
therefore there is no need to perform cardiac magnetic resonance imaging or to consult cardiac surgery for
either tumor removal or LAA excision.
Agitated saline contrast injections can be helpful in cases of suspected abnormalities of venous connections
and/or interatrial communication. A dilated coronary sinus can be caused by both a persistent left superior
vena cava (SVC) and partial anomalous pulmonary venous return, the former being more common.

A persistent left SVC affects approximately 0.5% of the general population, and most commonly drains to
the RA via the coronary sinus. In this case, there is no right to left shunt and right heart enlargement is
not expected. Agitated saline contrast into the left arm will result in opacification of the coronary sinus
before the RA and RV, while injection into the right arm will result in normal opacification of the RA and
RV without first filling the coronary sinus.

Partial anomalous pulmonary venous return can result in coronary sinus enlargement if one pulmonary
vein drains into the coronary sinus. Early opacificaiton of the coronary sinus with left arm agitated saline
contrast injection is not expected, nor is the transit of bubbles to the left side. Partial anomalous
pulmonary venous return is present in ~10% of ostium secundum atrial septal defects (ASD), and up to
80% of sinus venosus ASD. While the presence of an ASD alone would not explain the dilated coronary
sinus, it is consistent with the enlarged RV and findings with agitated saline contrast injection.

Therefore, of the options provided, the presence of an ASD with partial anomalous pulmonary venous
return is the most likely diagnosis. While Ebstein anomaly is associated with ASD, apical displacement of
the septal and posterior tricuspid valve leaflets is not noted in this case.

A ventricular septal defect or coronary artery to coronary sinus fistula would not be expected to result in
the RA to left atrial shunting demonstrated here with contrast injection.

Ultrasound duty factor is the percentage of time that the ultrasound system is transmitting sound (pulses).
Deep imaging requires longer "listening" time for the ultrasound pulse to travel through tissue and results
in a lower duty factor. Shallow imaging requires less "listening" time and allows for more frequent pulse
transmission, therefore resulting in a higher duty factor. Decreasing the depth of the image will allow for
higher frequency imaging (shorter wavelength with improved spatial resolution). However, decreasing the
depth of view will not automatically change the frequency; this would need to be changed manually.
Pulse repetition period (the time from the start of one pulse to the start of the next) is inversely related to
the duty factor. Ultrasound propagation speed is determined by the medium through which the
ultrasound waves are traveling and cannot be altered by machine settings.

The patient likely has patient–prosthesis mismatch (PPM). This entity needs to be considered in the
setting of heart failure symptoms in the presence of a normal-appearing valve with elevated transvalvular
gradients and velocities. The indexed estimated orifice area (EOA) is calculated according to the formula:

EOA = (LVOT VTI x cross-sectional area LVOT) / MV prosthesis VTI

EOA is then indexed according to the BSA (i.e., EOA / BSA). The patient's indexed EOA = 0.7 cm 2/m2,
which has similar hemodynamic consequences to true mitral stenosis, including left atrial enlargement
and pulmonary hypertension. Left ventricular (LV) dilation and elevated LV end-diastolic pressure would
not be a consequence of mitral stenosis.

PPM, unlike true valvular obstruction, is generally associated with a normal-appearing valve on two-
dimensional and color Doppler imaging, as shown in Video 1. There is no evidence of valve thrombosis.
PPM does not directly result in valve degeneration.

This patient has pulmonary hypertension in the setting of scleroderma manifested by worsening dyspnea
on exertion, chest pain, and pre-syncope. Her ECG shows RV hypertrophy as manifested by tall R waves
in V1, deep S waves in V6, right axis deviation, and evidence of RV strain (T wave inversions and ST
depressions in V1-V2).

Linear measurement of RV free-wall thickness is performed below the tricuspid annulus in the subcostal
view perpendicular to the long-axis of the RV
This patient has effusive-constrictive pericarditis initially presenting with pericardial effusion and
tamponade, and then findings of pericardial constriction due to the thickened pericardium following
drainage of the effusion. The hemodynamics reflect rapid early ventricular filling due to high atrial
pressure and sudden rise in ventricular diastolic pressure as the left ventricle is constrained by the
abnormal pericardium ("square root sign"). The corresponding echocardiographic Doppler findings are a
high velocity E wave with rapid deceleration and little, if any, late diastolic filling of the ventricle.
Annulus reversus refers to the pattern of decreased lateral annular velocity due to restriction caused by
the abnormal pericardium and exaggerated septal motion due to interventricular interaction. Hepatic vein
flows in constrictive pericarditis will reveal respirophasic diastolic flow reversals, but systolic flow in the
hepatic veins will not be affected; systolic flow reversal in the hepatic veins is specific for severe tricuspid
regurgitation. Other features of effusive-constrictive pericarditis include thickened pericardium,
echogenic material in a pericardial effusion, septal bounce motion, and plethora of the caval veins.
Computed tomography or magnetic resonance imaging can be used to determine pericardial thickness
with greater accuracy than echocardiography
Annulus reversus is most likely echo finding

Whereas brief early diastolic flow reversal in the descending aorta is a normal finding, holodiastolic
reversal is abnormal and generally suggests at least moderate aortic regurgitation. However, holodiastolic
reversal can also be seen in conditions resulting in a decrease in proximal aortic pressure through diastole,
as seen with an upper extremity arteriovenous fistula. Additional causes include reduced aortic
compliance (as often seen in the elderly), left-to-right shunting through a patent ductus arteriosus,
ruptured sinus of Valsalva aneurysm, and an aortic dissection with diastolic flow into the false lumen.

Severe diastolic dysfunction with reduced chamber compliance results in a greater rise in left ventricular
(LV) diastolic pressure for any degree of regurgitant volume, resulting in more rapid equalization of aortic
and LV diastolic pressures and a shorter period of aortic diastolic flow reversal. The presence of LV
dysfunction should not influence, or "pseudonormalize," the vena contracta measurement or proximal
isovelocity surface area–based quantitative measures.

M-mode echocardiography is the ideal modality for this type of measurement. It is able to accurately
measure longitudinal motion along the plane of interrogation (for example it is used to measure tricuspid
annular plane systolic excursion), and its high temporal resolution allows for easy discrimination of early
versus late systolic annular plane longitudinal displacement. The temporal resolution of M-mode on
modern machines is approximately 1000 Hz, compared with less than 100 Hz for two-dimensional
echocardiography. The temporal resolution for three-dimensional echocardiography is even lower. Tissue
doppler velocity does not measure displacement, but rather the rate of displacement of a structure.

Patients with bicuspid valve and aortic root enlargement are at increased risk of aortic dissection.
Guidelines do not recommend aortic root replacement for dimensions <5 cm unless there are increased
risk factors such as family history of dissection or a rapid rate of increase. Replacement of the ascending
aorta is reasonable if dimensions are >4.5 cm and the patient is undergoing aortic valve surgery.

When following patients with a dilated aorta in the setting of a bicuspid valve who have dimensions >4.5
cm, it is recommended that imaging be repeated at at least one year intervals. When the ascending aortic
diameter is ≥5.5 cm in the setting of a bicuspid valve, surgical intervention is recommended.

The correct answer is improved visualization of atrial septal anatomy with ICE as opposed to TEE. This is
in part because the ICE catheter, placed in the inferior vena cava and advanced to the right heart, is quite
close to the septum and in particular the inferior rim of most secondum ASDs, an area not well seen with
TEE. ICE has very high spatial and temporal resolution, as the close proximity to the structures being
imaged allows use of higher frequency transducers than are typical for TEE. ICE can be performed with
conscious sedation and local anesthesia (at the femoral vein puncture site) without the need for general
anesthesia. Three-dimensional (3D) imaging is available with both TEE and ICE systems, although with
the small nature and short distance to target of ICE, the advantages of 3D over two-dimensional imaging
are modest. ICE carries risks of intravascular access and potential, although rare, damage to intracardiac
structures, which TEE does not have, although TEE does carry risks of oropharyngeal trauma that ICE
does not. In some instances, ICE may also offer benefits in terms of less fluoroscopic time and therefore
less radiation use, because the smaller probe does not interfere with fluoroscopic visualization and is
manipulated by a single operator

Paravalvular leak (PVL) jets are often multiple, eccentric, and irregular in shape, as this case illustrates.
These features make PVL difficult to quantify. However, moderate and greater PVL have been associated
with worse outcome following TAVR, making accurate identification of moderate or greater aortic
regurgitation (AR) important so that it can be appropriately treated (Figure 1).

This patient has mild-to-moderate PVL, based on the width of the anterior jet at its origin, two jets, and
circumferential extent (<20%). There are two anterior jets (at 2 and 11 o’clock in the parasternal short-axis
view; Video 1). One of the anterior jets is very eccentric and has a larger origin width. There is a small
posterior jet visible in the apical three-chamber (AP3) view (Video 4). There is also trace central AR
visible in the apical five-chamber view (Video 3) and AP3 views (Video 4). Associated quantitative and
semiquantitative findings in such a case of mild-moderate PVL would be an AR index ≥25% (Figure 2),
which is the correct answer choice. The AR index is calculated as: (diastolic blood pressure – left
ventricular end-diastolic pressure) / systolic blood pressure. It is expressed as a percentage; the index tends
toward zero as AR becomes more severe. Partition values for other semiquantitative and quantitative
measures of PVL are presented in Figures 1 and 2.

All the other answer choices would be consistent with severe PVL.
The distribution of hypertrophy in hypertrophic cardiomyopathy (HCM) is variable in location and
pattern. The most common form of HCM is asymmetric left ventricular (LV) hypertrophy involving the
septum with normal systolic and abnormal diastolic function, which may have dynamic outflow
obstruction in association with systolic anterior motion of the mitral valve and resulting mitral
regurgitation. However, hypertrophy can also be concentric or localized to the LV free wall or apex.
Apical hypertrophy, as depicted here, can be difficult to fully characterize with standard
echocardiographic views, and it is important to use an echocardiographic contrast agent to define the
extent of hypertrophy and assess for apical aneurysm and/or apical thrombus (Video 2).

The use of agitated saline contrast is not specifically helpful in the evaluation of hypertrophic
cardiomyopathy, unless there is suspicion for an intracardiac shunt.

Speckle-tracking echocardiography can demonstrate reduction in strain and abnormal myocardial motion
despite normal LV ejection fraction in patients with HCM, however would not change the management of
this case.

Transesophageal echocardiography has poor visualization of the LV apex and would not provide additional
information here.
Administration of intravenous dobutamine for stress echocardiography does not play a specific role in the
diagnosis of apical HCM

This image demonstrates a right atrial mass. The differential diagnosis of an RA mass is myxoma,
vegetation, thrombus, and malignancies. It is important to image the IVC in this case since malignancies
may extend into the venous structures connected to the RA. This is especially true in the case of renal cell
CA or Wilms tumor. Metastatic tumors can also come from the lungs with infiltration into the pulmonary
veins, from the thyroid with infiltration into the SVC, and from the liver or adrenals with infiltration into
the IVC. Venous thrombi may also be imaged either extending from the IVC into the right heart or "in
transit" from the IVC to the pulmonary circuit

In acute severe MR, there is no time for atrial remodeling; therefore, the left atrial volume will be normal.
In acute MR, the pulmonary artery pressure will always be high; however, chronic MR can also lead to
pulmonary hypertension. In both acute MR and compensated chronic severe MR, the left ventricular
ejection fraction will be hyperdynamic to provide a larger total stroke volume to maintain forward stroke
volume. Whereas most patients with flail leaflet will present in the acute setting, some patients with
myxomatous mitral valve disease may have partial chord rupture over the years, resulting in progressive
development of severe regurgitation. An inferolateral wall motion abnormality can cause restricted
motion of the posterior leaflet resulting in severe MR; however, this alone does not tell the clinician about
the timing of the development of MR
Normal left atrial systolic index is normal in severe acute MR

In certain settings, echocardiography Doppler assessment may overestimate transaortic valve gradients
relative to direct assessment by cardiac catheterization (Figure 1). Just beyond the stenotic aortic valve,
blood velocity is highest and pressure lowest. Distally, the blood slows, leading to an increase in blood
pressure and hence a decrease in pressure gradient compared with LV pressure. Cardiac catheterization
measures pressure more distally than the peak pressure assessed by echocardiography, and thus will tend
to yield lower gradients. This pressure-recovery phenomenon is most pronounced in patients with
ascending aortic diameters of <3 cm, aortic coarctation, or fixed obstruction in the proximal ascending
aorta. In cases in which a significant discrepancy exists between mean gradients measured by cardiac
catheterization and echocardiography, the former is more clinically relevant.

In this patient, who is of smaller stature, the pressure-recovery phenomenon is most likely to explain the
findings. Although anemia and the presence of a hemodialysis fistula may increase transaortic valve
gradients, this could occur with both echocardiography and cardiac catheterization. Oversedation during
cardiac catheterization would be expected to decrease the mean gradient, but not to this degree. Proper
measurement of LV outflow tract size is critical to accurately gauge aortic stenosis (AS) severity, but
would not affect assessment of mean gradient.

Figure 2 shows the correlation between cardiac catheterization and echocardiography in the assessment of
AS. The peak instantaneous/maximum gradient measured on catheterization correlates with peak gradient
measured by continuous-wave (CW) Doppler on echocardiography except in cases in which there is
significant pressure recovery. The shaded area depicts mean gradient on cardiac catheterization, which is
also calculated by CW on echocardiography.

The differential diagnosis for cardiac masses includes thrombus, tumor, and vegetation. These
echocardiographic images show a large left ventricular (LV) apical mass with contrast uptake, which is
most suggestive of a vascular intracardiac tumor. Neither a thrombus nor a vegetation would be expected
to demonstrate contrast uptake. Contrast enhancement may help rule out artifacts, but in this case, close
inspection of the contrast image shows a persistent LV apical mass. LV noncompaction is characterized by
a deeply trabeculated LV apex.

This M-mode image of an aortic valve demonstrates an eccentric closure line during diastole that is seen
with bicuspid aortic valves.
The AR vena contracta is the narrowest portion of the AR color Doppler signal as it passes through the
aortic valve (AoV). It is therefore smaller than the jet width in the left ventricular outflow tract (LVOT).
It is best assessed in the parasternal long-axis view, zoomed on the LVOT, AoV, and aortic root, with the
insonifying ultrasound beam perpendicular to the flow of the regurgitant jet (Figure 1). Values of <0.3 cm
suggest mild AR whereas values of >0.6 suggest severe regurgitation. One strength of the vena contracta is
that it is valid with eccentric AR jets. An important limitation is that it may be inaccurate when multiple
regurgitant jets are present, as the vena contracta of only one jet is measured.

Apical views orient the insonifying ultrasound beam parallel to the AR jet; the lateral resolution of the
ultrasound beam does not permit reproducible measurements. AR at the level of the AoV is not likely to
be visualized in either the right parasternal or suprasternal notch views.

TEE reveals severe valvular aortic regurgitation with continuous color flow throughout the cardiac cycle
at the level of the aortic valve leaflets consistent with a fistula between the aortic root and the right
atrium. Visualization of the ostium of the left main (2 o'clock position) and all three aortic valve leaflets
confirms that the imaging is above the aortic annulus. In the context of the clinical presentation, this is
consistent with aortic valve endocarditis.

A typical ventricular septal defect is systolic flow between the left and right ventricle. A Gerbode defect is
an unusual subset of ventricular septal defects that is between the left ventricle and right atrium. In a
Gerbode defect, high-velocity flow is seen only during systole. Although the tricuspid valve is seen at 9
o'clock, there is no evidence of vegetation. Although the ostium of the left main coronary artery is seen,
the dimension is normal and therefore is not a coronary aneurysm. There is an echolucency in the 11
o'clock position adjacent to the noncoronary cusp that is suspicious for a root abscess or pseudoaneurysm.

Chronic, severe AR exposes the left ventricle (LV) to chronically increased LV preload and afterload,
resulting in progressive LV dilatation, hypertrophy, and ultimately systolic dysfunction. In asymptomatic
patients, the presence of LV systolic dysfunction and systolic chamber enlargement predict the onset of
symptomatic heart failure (HF); among symptomatic patients, they are associated with worse outcomes
following valve replacement. In asymptomatic patients with severe AR, the current guideline recommends
aortic valve replacement (AVR) if LVEF is <50% (Class I recommendation), LVESD is >5 cm with LVEF
>50% (Class IIa recommendation), or LVEDD is >6.5 cm with LVEF >50% if surgical risk is low (Class IIb
recommendation).

In a patient with normal systolic function, the 2016 guidelines for the evaluation of diastolic function
recommend the assessment of 4 features to determine if diastolic dysfunction is present:

1. LA size >34 ml/m2

2. TR velocity of >2.8 m/s
3. E/e' ratio of >14
4. Medial e' 50% of these criteria are met, then diastolic dysfunction is present.

Healthy women frequently have cardiovascular symptoms during pregnancy, including edema (59%),
palpitations (42%), fainting (31%), and shortness of breath on exertion (73%). This
patient's echocardiogram is normal. Situs solitus of the atria is normal. Situs inversus indicates right/left
reversal. D-looped ventricles indicate that the inflow portion of the morphologic RV lies to the right of
the morphologic LV, and is also normal. Ventriculoarterial concordance indicates the morphologic RV
gives rise to the pulmonary trunk and the morphologic LV gives rise to the aorta (normal). Levocardia is
when the cardiac apex is to the left, which is normal. Fractional shortening is an assessment of LV
function which is more commonly used in children, particularly in the absence of regional wall motion
abnormality. Normal fractional shortening for women is 27-45%. The systolic RV to right atrial gradient is
the peak tricuspid regurgitant gradient. Even if the estimated right atrial pressure is 15 mm Hg, the
estimated RV systolic pressure is normal.

Because the symptoms are consistent with normal pregnancy and the previous echocardiogram was
normal, reassurance is appropriate at this time. There are no indications to terminate the pregnancy,
obtain cardiac catheterization, or refer to genetics. Repeating the echocardiogram is not necessary.
This patient has pulmonary hypertension with associated RV dilation. Normal RV dimensions are <41 mm
at the base, <35 mm at the midline, RV outflow tract (RVOT) in the parasternal long axis view of <30 mm,
proximal RVOT of <35 mm, and distal RVOT of <27 mm (Table 1).
This patient has a history of valve replacement and a systolic murmur. The spectral Doppler profile
displayed (Figure 1) is of an aortic valve (AoV), not a mitral valve (MV) based on the contour, maximal
velocity (Vmax), and duration. Specifically, it demonstrates a normally functioning AoV prosthesis with
triangular contour, early peaking Vmax, and short acceleration time. Because the valve is functioning
normally, there is no indication for surgical or transcatheter AoV replacement.

An example of AoV prosthesis obstruction/stenosis is shown in Figure 2. Progressive obstruction often

results in a rounded contour, delayed Vmax, and prolonged acceleration time. There are several caveats to
this assessment and, therefore, one must combine multiple two-dimensional Doppler and clinical
parameters when evaluating prosthetic valve function. Evidence of MV dysfunction was not provided, so
MV intervention would not be not indicated.

Figure 3 demonstrates a normally functioning MV prosthesis based on the peak E velocity, mean gradient,
pressure half-time (PHT), and calculated effective orifice area (EOA) using the continuity equation: EOA =
(CSA LVOT x LVOT VTI) / MV prosthesis VTI, where CSA is cross-sectional area, LVOT is left
ventricular outflow tract, and VTI is velocity-time integral.

An example of a Doppler profile of significant MV bioprosthetic stenosis is shown in Figure 4.

There are several caveats and pitfalls with the calculation of EOA based on the continuity equation,
particularly with prosthetic heart valves, including measurement of LVOT diameter and positioning of the
pulsed-wave Doppler sample volume. The continuity equation cannot be relied on in the setting of more
than mild mitral or aortic regurgitation. PHT cannot be used to estimate EOA of MV prosthesis. However,
absolute changes in PHT including significant prolongation (>200 msec) or increases in PHT can be useful
to detect progressive obstruction/stenosis physiology.
TIA, echo shows positive bubble study after 2 cycles

This patient most likely has a patent foramen ovale (PFO) given the normal right ventricular size.
Compared with an atrial septal defect (ASD), PFO is more commonly associated with an atrial septal
aneurysm, prominent Eustachian valves, and Chiari network. In contrast, an ASD is more likely to be
associated with right-sided enlargement due to left-to-right shunting than a PFO
The speed of ultrasound in soft tissue is 1,540 m/s. The posterior annulus in this question is 5 cm deep, so
10 cm of travel time is required (time out + time to return). 10 cm = 0.1 m, and 0.1m ÷ 1540 m/s =
0.000065s = 65µs. A shortcut to remember this is that for every 2 cm in soft tissue (i.e., 1 cm of imaging
depth) it takes ultrasound 13 µs to travel.

The echocardiography images demonstrate severe right ventricular (RV) enlargement with RV
hypertrophy and septal flattening (Videos 1 and 2) and increased RV systolic pressure (Figure 2) all
consistent with severe pulmonary hypertension. There is a large secundum ASD with low volume,
bidirectional shunting consistent with Eisenmenger syndrome (Videos 3 and Figure 1). Surgical closure of
the ASD in the absence of significant left to right shunting is contraindicated. This patient is likely to be
cyanotic with right to left shunt and functional status may improve with treatment with pulmonary
vasodilators and oxygen therapy. Iron therapy maybe beneficial in the setting of iron deficiency with
secondary erythrocytosis in these patients. Diuretic therapy maybe useful in the presence of significant
right heart failure. Survival with pulmonary hypertension with Eisenmenger's syndrome is better than
with other causes of pulmonary hypertension
This sharp rise represents subaortic membrane which requires surgery
Resection of the membrane is indicated with symptoms, peak gradient above 50 mm Hg, or with
significant or progressive aortic regurgitation. In this case, with a the peak velocity of 4.2 m/s, the peak
gradient exceeds 50 mm Hg (4.2 squared x 4 = 70 mm Hg). Alcohol septal ablation or beta-blocker will not
help in the setting of subaortic membrane
After repair, lifelong follow-up is recommended because of the possibility of recurrence

This patient has chronic severe MR. Typical echocardiographic findings associated with chronic severe
MR include left atrial and left ventricular enlargement, pulmonary vein systolic flow reversal, and an E
wave–dominant mitral inflow pattern. The E wave–dominant mitral inflow pattern is due to increased
flow across the mitral valve (MV) during diastole caused by the additional volume from the MR (i.e.,
regurgitant volume plus stroke volume). The increased diastolic volume is associated with an increased
MV velocity-time integral (VTI); the left ventricular outflow tract (LVOT) VTI is not affected. Thus, the
MV VTI/LVOT VTI ratio should be >1. An elevated septal e' is not a finding generally associated with
severe, chronic MR
A 68-year-old man with history of Hodgkin lymphoma with history of mantle radiation is seen for
evaluation of dyspnea, abdominal bloating, and peripheral edema. Physical examination reveals elevated
jugular vein pressure, +S3, and 1+ pitting edema bilaterally. A transthoracic echocardiogram is obtained.
Which of the following echocardiographic findings would be most helpful establishing this patient's
diagnosis?
A: >25% increase in expiratory mitral inflow velocity

Echocardiography can be helpful in differentiating between constriction and restriction. Respirophasic

flow variation across both the tricuspid and mitral valves is present in constriction, but not in restriction.
During inspiration, right ventricular (RV) filling is increased at the expense of left ventricular (LV) filling
and stroke volume (commonly referred to as ventricular interdependence). Conversely, mitral flow is
increased during expiration as RV filling decreases. A specific finding for constriction is end expiratory
diastolic flow reversal in the hepatic veins.

Other Doppler echocardiography findings that can help identify constriction are annulus reversus (medial
e' is greater than lateral e') or annulus paradoxus (E/e' is inversely proportional to filling pressures). While
normal medial tissue Doppler is characteristic of constriction, it is not sufficient to establish the diagnosis.
Short mitral deceleration time does not discriminate well between constriction and restriction.

The continuous wave Doppler velocity curve of mitral regurgitation (MR) reflects the instantaneous
pressure difference between the left ventricle (LV) and the left atrium (LA) in systole. In the setting of
normal LV systolic function and low LA pressure, the rapid increase in LV pressure translates to a rapid
increase in MR jet velocity. The slope of the MR jet velocity is the change in pressure over time, or dP/dt.
The dP/dt is calculated by measuring the time it takes for the MR jet velocity to increase from 1 m/s to 3
m/s and then applying the Bernoulli equation as follows (Figure 1):
dP/dt = [4(v1)^2 – 4(v2)^2] / time interval
= [4(3)^2 – 4(1)^2] / time interval
= 32 mm Hg / time interval (in sec)

In the setting of abnormal LV systolic function, the time interval over which the regurgitant jet velocity
increase is prolonged yielding a lower dP/dt. Accepted reference values:

<1000 mm Hg/sec (abnormal)

>1000 mm Hg/sec (normal)

Of note, the assessment of dP/dt requires the presence of a recordable MR jet, and it assumes a constant
and parallel intercept angle between the MR jet and the ultrasound beam. dP/dt does not provide an
assessment of LV diastolic function or aortic valve disease.
Aortic intramural hematoma (AIH) has the appearance of focal thickening of the aortic wall without a
demonstrable intimal flap. Unlike an aortic dissection with a true lumen and a false lumen, AIH has flow
only in the true lumen. Most patients (80%) with AIH present with chest and/or back pain. The sensitivity
and specificity of TEE in the diagnosis of AIH is >90%. Computed tomography is a confirmatory test and
can demonstrate the extent of the hematoma.

Coarctation is not considered an acute aortic syndrome and does not usually present with chest pain.
Aortic aneurysms that are dilated can cause pain, but would not necessarily be associated with wall
thickening. The patient's presentation does not clinically suggest pericarditis

What would necessitate continued anticoagulation post Watchman implantation:

The correct answer choice is an 8 mm peridevice leak. Based on the PROTECT-AF (Percutaneous Closure
of the Left Atrial Appendage Versus Warfarin Therapy for Prevention of Stroke in Patients With Atrial
Fibrillation) study protocol, anticoagulation should be continued in patients with peridevice leaks >5 mm
measured at a color Doppler aliasing velocity of -50 cm/sec; in that study, the LAA closure device was
noninferior to warfarin even if a leak of up to 5 mm in maximal diameter was present.

The manufacturer recommends 8-20% compression of the device (compared with the manufactured/ ex
vivo diameter of the device) to avoid peridevice leak. Iatrogenic septal defects are common after trans-
septal puncture for left-sided procedures and have a high spontaneous closure rate (decreasing from 11%
of patients at 6 months to 7% at 12 months) but have not been associated with stroke or systemic
embolization to date. Thrombus distal to the occlusion device (that is, present in the LAA) is a common
finding after endovascular LAA occlusion procedures and is not necessarily an indication for systemic
anticoagulation. In contrast, thrombus attached to the device or within the left atrium would warrant
anticoagulation. Any mitral A wave implies sinus rhythm, but anticoagulation decisions in AF should
reflect stroke risk (as estimated by the CHA2DS2-VASc score) and not whether the AF is paroxysmal versus
continuous; therefore, mitral A wave velocities are not useful for decision making regarding
anticoagulation in this population

Mitral valve area (MVA) is measured at peak mitral valve (MV) opening during diastole. Of the answer
choices, P wave is the best answer choice because this shows the MV prior to the A wave. The other
answer choices are systolic events.

Measurement of the MVA by planimetry is relatively independent of hemodynamics and minimally

affected by the presence of other valvular lesions. 3D planimetry, using any multiplanar reformatting, on-
image planimetry, or reference grid visualization techniques, has emerged as the "echocardiographic gold
standard" for MVA determination. Unlike two-dimensional planimetry, 3D images allow precise
identification of the edge of the mitral leaflets and do not require any assumptions of geometric alignment
along the tips of the leaflets.

Trastuzumab is a monoclonal antibody that targets human epidermal growth receptor 2 that is used in
patients with breast cancer that overexpress that receptor. This therapy has been associated with cancer
therapeutics–related cardiac dysfunction (CTRCD). Echocardiography is the modality of choice for
monitoring for CTRCD and is performed prior to starting therapy, and every 3 months during treatment.
This patient did receive anthracyclines and trastuzumab which carries an increased risk of CTRCD
compared to either therapy alone.

The patient is experiencing symptoms of dyspnea and may be at risk for heart failure preserve ejection
fraction (HFpEF) given known hypertension, age, obesity, and female sex. Recent data suggest that there is
worsening in diastolic function with doxorubicin with or without trastuzumab, and abnormal or
worsening diastolic function was associated with subsequent LVEF declines. In this patient, it is important
to assess the diastolic function and determine her estimated filling pressures through using E/e'. Elevated
filling pressure could explain the patient's dyspnea on exertion.

Moreover, accurate calculation of LVEF should be performed with each study using the best method
available. Three-dimensional echocardiography is ideal but may not be possible if two-dimensional
echocardiography (2DE) image quality is poor, as in this case. When 2DE is used, the modified biplane
Simpson technique is considered the method of choice. Moreover, the use of newer technologies such as
GLS by speckle-tracking echocardiography (STE) is also recommended for the early detection of
subclinical LV dysfunction

This apical four-chamber view shows dyssynchronous septal contraction and "swinging" motion of the left
ventricular apex. These findings are common in patients with left bundle branch block.
Echocardiography in dextrocardia would be expected to demonstrate a normal heart in mirror position
within the chest.

Echocardiography in left ventricular hypertrophy would be expected to demonstrate a hypertrophied but

normally contracting left ventricle.

Echocardiography in pericarditis may demonstrate pericardial effusions or enhanced brightness or

thickening of the pericardium. Abnormalities of septal wall motion may also be present in some patients
with pericarditis but, as the other findings of pericarditis are not present in the videos shown, this is not
the preferred answer choice. Additionally, the respirophasic anterior septal shift associated with
constrictive pericarditis would not explain the side-to-side motion of the apex seen with every heartbeat.

Takotsubo cardiomyopathy may produce both electrocardiographic findings of long QT and diffuse T-
wave inversions, as well as echocardiographic findings of extensive anterior wall motion abnormalities.
However, it would not explain the dyssynchronous septal motion seen in the video

Question asked: RA and RV enlargement, what’s the area most amenable to transcatheter
treatment
A: superior rim of fossa ovalis

TEE, with three-dimensional visualization and analysis, is a critical imaging tool for assessing the
interatrial septum and planning percutaneous closure of ASDs. Key determinants of successful
percutaneous closure are shown in Figure 1. Patients with ostium secundum ASDs, the most common
septal defect, can often be treated successfully with percutaneous techniques. Secundum-type defects are
commonly septated or multiple, which can affect the selection of device used for percutaneous closure;
however, as long as adequate rims are present on all sides of the defect, percutaneous closure is usually safe
and effective.

A defect adjacent to the atrioventricular (AV) valve annulus is a septum primum–type defect; this term is
interchangeable with the terms partial or incomplete AV canal defect. Because it borders the AV valve
leaflets, surgical treatment is preferred. Sinus venosus defects are caused by a deficiency or absence of the
sinus venosus, which separates the superior vena cava from the right upper pulmonary vein and the
inferior vena cava from the right lower and (when present) middle pulmonary vein(s). Superior and
inferior sinus venosus defects are often associated with partial anomalous pulmonary venous return.
Neither form is typically treated with percutaneous device closure because the pulmonary venous flow
must be redirected to the left atrium once the defect is closed. A defect in the wall of the left atrial aspect
of the coronary sinus, sometimes called an "unroofed" coronary sinus, allows left atrial blood to enter the
right atrium through the coronary sinus ostium, which is usually dilated. There have been case reports of
successful percutaneous closure of partially unroofed coronary sinus defects, but this is not usually
amenable to percutaneous closure with commercial devices.
MVA can be assessed by planimetry using either two-dimensional (2D) or three-dimensional (3D)
imaging, pressure half-time (PHT), the continuity equation, and the proximal isovelocity surface area
(PISA) method. 2D planimetry of the MVA is performed in a parasternal short-axis view at the tip of the
leaflets when maximal excursion of the leaflets is seen. The inner edge of the mitral valve (MV) orifice is
traced in mid-diastole. The entire MV orifice should be seen. High-gain settings should be avoided
because they may lead to underestimation of the MVA. Planimetry has been shown to have the best
correlation with anatomic MVA as assessed by explanted valves. 2D planimetry tends to overestimate
MVA compared with 3D transesophageal echocardiographic measurements, especially in patients with a
large left atrium (LA). 3D echocardiography provides better alignment of the image plane at the mitral
tips, rendering a more accurate and reproducible planimetric measurement with excellent interobserver
and intraobserver agreement. Therefore, 3D is preferred over 2D when available

Percutaneous PVs are associated with an increased incidence of infective endocarditis (IE), and a high
index of suspicion is required for making a correct diagnosis. Due to acoustic shadowing from the stent
structure, it is often difficult to visualize the valve leaflets on TTE or TEE. Therefore a negative TTE or
TEE does not rule out endocarditis in this setting. Because this patient has a prosthetic valve in place and
positive cultures for Staphylococcus aureus, early surgical intervention is appropriate. This patient
underwent intracardiac echocardiography (Video 6) which demonstrated a vegetation on the prosthetic
PV. The increase in transvalvular gradient suggests valve dysfunction and supports the decision to proceed
with surgery.

This patient should be treated with antibiotics, but four weeks of antibiotics would not be considered an
adequate treatment for IE. Performing repeat percutaneous PV placement alone would not address the
infection and the new valve would likely become infected as well. Repeating the TTE would not be
appropriate. Right heart catheterization would not change the patient's management and could potentialy
be contraindicated in the setting of vegetation in the rigth ventricular outflow tract.

The correct answer choice is 1 cm 2. In Figure 1, the correct line to use is the red line, as this represents the
predominating slope. The DT (or the time it takes the early diastolic filling velocity [E wave] to decay to
baseline, or 0 m/sec) and the pressure half-time (PHT; or the time it takes the peak early diastolic filling
gradient to decay by 50%) are related through the equation: PHT = 0.29 x DT, as visualized in Figure 2.
Substituting DT for PHT in the empiric MVA equation (MVA = 220 / PHT) gives MVA = 759 / DT. In this
case, the PHT would be equal to 0.29 x 760 = 220 msec, and the MVA can be calculated as 759 / 760 = 220
/ 220 = 1 cm2

Doppler tissue imaging is useful in identifying constriction. “Annulus reversus” is shown here, and refers
to the finding that the lateral mitral annular e' is usually lower than the septal mitral annular e' in
constrictive pericarditis, most likely due to tethering of the lateral mitral annulus to the thickened
pericardium. This is in contrast to restrictive cardiomyopathy (i.e., cardiac amyloidosis) in which the
septal and lateral e' are both typically reduced.

Another typical finding in constriction is "annulus paradoxus" which refers to the increase in septal e' as
the severity of constriction worsens; as a result, the E/e' ratio is inversely proportional to the left atrial
pressure. This is in contrast to restrictive cardiomyopathy where there is a linear relationship between E/e'
and left atrial pressure due to the typical tall and narrow transmitral E and reduced tissue e'.

None of the other options of pulmonary sarcoidosis, systemic amyloidosis, prior mitral valve repair, or
paroxysmal atrial fibrillation would be expected to show annulus reversus.
Indications for fetal Echocardiography: maternal pre-gestational diabetes

With continuous wave (CW) Doppler, signals are received along the entire length of the beam. Therefore,
the site of flow acceleration cannot be localized. CW is used to measure high velocities in both valve
stenosis and regurgitation. Pulsed wave (PW) Doppler samples velocities from a specific site and is used to
record low velocity signals at a specific sampling volume. PW Doppler could therefore localize the
abnormal flow acceleration in this case. Three-dimensional imaging of the aortic valve would not be
expected to add further information if the valve is normal. CW Doppler of the tricuspid regurgitation,
color flow Doppler of the septum, and M-mode recordings of the mid-left ventricular cavity would not
help explain the cause of the high velocity seen in the region of the aortic valve and the left ventricular
outflow tract

Pregnancy is associated with progressive increases in circulating blood volume as well as modest increases
in all cardiac chamber sizes. Increases of 5-10% from baseline in left ventricular (LV) diastolic diameter,
LV wall thickness and mass, left atrial size, and right ventricular size are all therefore expected during the
course of pregnancy. The aortic annulus and aortic root diameters also increase by 2-3 mm during
pregnancy. Stroke volume of both ventricles and the flow velocities in both outflow tracts rise as well,
commonly reaching -2 m/sec and contributing to flow murmurs heard during pregnancy.

Despite the easily measured changes in cardiac size, there are conflicting data on changes in cardiac
function during pregnancy, suggesting that any changes in LV ejection fraction or myocardial strain are
small and may be explained by changes in loading conditions rather than changes in intrinsic myocardial
contractility. The increases in blood volume seen in normal pregnancy are matched by decreases in
vascular resistance, so that pulmonary artery pressure does not change during pregnancy and systemic
arterial pressure typically falls during midpregnancy. Elevated peak velocity of tricuspid regurgitation
would therefore be unexplained by pregnancy.

Partial anomalous pulmonary venous connection is frequently with associated with sinus venosus ASDs
(85% of cases), but also occasionally with secundum (10-15%) and coronary sinus ASDs (Table 1). If
pulmonary veins were not assessed prior to percutaneous closure, they should be assessed now. Cardiac CT
is the correct answer.

Providing reassurance in this case prior to evaluating veins is not appropriate. There is no evidence of ASD
occluder device dislodgement or pulmonary hypertension on the echocardiogram (Figure 1; Videos 1-4).
She does not have risk factors for coronary artery disease (CAD). None of the other choices would be the
most likely cause of dyspnea in this patient nor would CAD explain the persistently dilated RV.
Pulmonary function testing would not be indicated at this point.
Routine use of echocardiography in place of an endomyocardial biopsy is not recommended to diagnose
rejection (Class III), although echocardiography may be important for patient management in addition to
biopsy.

Increased left ventricular (LV) wall thickness related to increased edema may be a marker of rejection.

A filling pattern that appears restrictive in patients with preserved ejection fraction is a common finding
after heart transplantation and is observed in patients with normal LV diastolic function, as donor hearts
are usually obtained from healthy young individuals. Myocardial tissue velocities are lowest early after
surgery and tend to increase during the weeks and months after surgery. Nonetheless, LV diastolic
dysfunction has often been described as a sensitive sign of early graft rejection, but no single diastolic
parameter appears reliable enough to predict graft rejection.

Pericardial effusion, inferior vena cava dilation, and LV enlargement are not specific signs of rejection. LV
dysfunction is more likely a late manifestation of rejection
The TEE images provided show thickened prosthetic AoV leaflets with mobile elements seen in the long-
axis view. There is periaortic thickening most prominently seen in the region between the aorta and the
left atrium consistent with aortic root abscess.

A postoperative periaortic hematoma would have been resorbed by now. Thrombosed aortic aneurysm
would be unusual in the absence of aortic root dilation, and no dissection is seen. There is dropout seen in
the proximal interventricular septum. This is not a ventricular septal defect, but is an artifact that
represents the shadows of the bioprosthetic AVR strut. The AoV leaflets show restricted opening
consistent with some degree of aortic stenosis. The electrocardiogram tracing also shows first-degree heart
block. Aortic root rupture would be expected to be associated with hemopericardium.

Patients receiving treatment for cancer with bevacizumab, a monoclonal antibody targeting the vascular
endothelial growth factor pathway, which is associated with an increased risk of ischemia. New symptoms
should be evaluated appropriately. In this patient who can exercise but has left ventricular hypertrophy on
her electrocardiogram, an exercise stress echocardiogram is the most appropriate test.

Depending upon the pre-test probability, coronary angiography would typically not be considered prior to
functional evaluation. Pharmacologic testing in a patient who can exercise is not the preferred choice of
stress

Eccentric MR jets frequently do not have a planar surface. When this is the case, a correction factor needs
to be applied to the EROA calculation, such that: EROA = (2[π]r 2 x aliasing velocity x [∝ / 180]) / MR peak
velocity, where ∝ is the angle subtended by the mitral leaflets (Figure 1). In this example without angle
correction, the calculated EROA is 0.5 cm 2. However, when the angle correction of 120/180 is applied
(EROA x 0.67), the corrected EROA is 0.33 cm 2
Evaluation of valve prosthesis regurgitation requires a detailed two-dimensional, Doppler, and color
Doppler assessment. When evaluating mechanical valve function, it is critical to determine the origin of
the regurgitation: physiologic washing jets, paravalvular, or transvalvular. In contrast with pathologic
regurgitant jets, physiologic washing jets are narrow, symmetric, and of short duration. These physiologic
jets are normal, expected findings associated with mechanical valves, helping to prevent thrombus
formation on the prosthesis.

Severe regurgitation is not present. The regurgitation seen in Figure 1 is not consistent with an unstable
valve ring. Patient–prosthetic mismatch would cause elevated anterograde gradients, not regurgitation.
The most likely cause of this patient's symptoms is exercise-induced dynamic LVOT obstruction.
According to the American College of Cardiology/American Heart Association guidelines, patients with
HCM who do not have a resting peak instantaneous gradient of ≥50 mm Hg, exercise echocardiography is
reasonable for the detection and quantification of exercise-induced dynamic LVOT obstruction. (Class IIa,
level of Evidence B).

In this patient with new exertional symptoms and no significant gradient at rest or with Valsalva, stress
echocardiography should be performed to evaluate for dynamic LVOT obstruction mitral regurgitation.
Cardiac magnetic resonance imaging (MRI) is indicated in patients with known HCM when additional
information that may have an impact on management regarding intervention (such as evaluating
morphology and scar burden) is not adequately defined with echocardiography. In this patient, cardiac
MRI would be unlikely to demonstrate the cause of his symptoms.

Although a Holter monitor is indicated for sudden death risk stratification in HCM, it would not be useful
for discerning the etiology of this patient's symptoms. His history suggests a low probability of CAD and
hence perfusion stress testing or coronary computed tomography angiography would be less useful here
than stress echocardiography.