DISORDERS OF

THE
CARDIOVASCUL
AR SYSTEM

Alma Fe V.G Bascos, FPPS, FPCC, FPSE

September 2019
 FETAL CIRCULATION
• the placenta
provides gas and
metabolite exchange
• lungs ≠ gas exchange
– vessels in the
pulmonary
The RV & LV ≈ parallel circulation
circuit ≠ the series vasoconstricted
circuit of a NB
FETAL CIRCULATION
• 4 shunts:
1. placenta
2. ductus venosus
3. foramen ovale
4. ductus arteriosus
FETAL CIRCULATION
• 4 shunts:
1. Placenta
a. receives the largest
amount of combined
(RV & LV) ventricular
output (CVO=55%)
b. has the lowest
vascular resistance in
the fetus
FETAL CIRCULATION
• 4 shunts:
1. placenta

2. ductus venosus
shunts portion of
the UV blood
directly into the IVC
3. foramen ovale
4. ductus arteriosus
FETAL CIRCULATION
• 4 shunts:
1. placenta
2. ductus venosus

3. foramen ovale
opening in the
interatrial septum that
allows R → L shunting
in the atria
4. ductus arteriosus
FETAL CIRCULATION
• 4 shunts:
1. placenta
2. ductus venosus
3. foramen ovale
4. ductus arteriosus
a. connects the PA to the
proximal desc aorta
b. allows most of the
blood from the RV to
bypass the fetal lungs
 FETAL CIRCULATION
SVC drains upper IVC blood drains
body→RV the lower body
1/3 of the IVC blood with ↑O2
sat directed by the crista
dividens → LA via the foramen
ovale
The remaining 2/3 enters the
RV and PA
 Changes in Circulation after Birth
• shift of blood flow for 1. Removal of placenta :
gas exchange from the a. ↑SVR (because the
placenta →→ lungs placenta had the lowest
• Placental circulation SVR in the fetus)
disappears, and the b. Cessation of blood
pulmonary circulation flow in the umbilical
established vein →→closure of the
ductus venosus

• *SVR-systemic vascular resistance

Changes in Circulation after Birth:
Lung expansion results in the ff:
• a. ↓PVR, ↑PBF, and ↓ PA pressure

b. Functional closure of the foramen ovale due to

↑ LAP > RAP
● ↑ PBF →↑ LAP, ↑PVReturn to the LA
● closure of the DV, ↓ RAP

c. Closure of PDA as a result of increased arterial O2

sat
DISORDERS OF THE
CARDIOVASCULAR SYSTEM
CV diseases significant causes of morbidity
and mortality in all age groups

In 2005, CHD – 6th leading cause of

mortality in the Phils

RHD, obesity and hypertension ↑↑↑

5 Basic Questions in the Evaluation
of Congenital Heart Diseases
1. Is the patient cyanotic or acyanotic?
2. Is the PBF ↑ or ↓
3. Is the malformation originating from the L or
R side?
4. What is the dominant ventricle?
5. Is there pulmonary hypertension? (when
present may alter the clinical manifestation &
PE)
 HISTORY TAKING
•perinatal period: (+) cyanosis, respiratory distress, or
prematurity
Exposure to radiation, infection and
st
drug intake (1 trim)
•maternal cx: gestational DM, meds, SLE, substance
abuse Rubella: PDA and PS
Maternal diabetes ↑ risk of CHD
•frequency and volume per feeding
SLE: congenital heart block
•respiratory distress: rapid breathing, nasal flaring,
cyanosis, chest retractions
•older children, HF: exercise intolerance, difficulty keeping up
with peers
HISTORY AND PHYSICAL
EXAMINATION
When murmur was first noted
Noted at birth: PS, AS
Noted after the NB age (4th to 6th week of life):
(+)shunt lesions when PVResistance lowers

Chest pain common in children and

adolescents (location, character, severity,
triggers)
 HISTORY AND PHYSICAL
EXAMINATION
• Past history: previous hospitalizations and
illnesses
– Recurrent RTI, poor growth, delayed development
• Exercise capacity: school activities/sports
• Family history: CHDs, sudden death,
syndromes
 GENERAL PHYSICAL EXAM
• general survey, ht/wt
• (+)cyanosis, growth abnormalities
• (+) respiratory distress
• (+/-)murmur
• Quality of pulses
• Ventricular heaves
 CYANOSIS
• best observed over the nail beds, lips, tongue,
mucous membranes
• Differential cyanosis: blue LE and pink UE
(usually RArm), (+) R-to-L shunting via a PDA in
with coarc or IAA
• turn "blue around the lips" when crying
vigorously or during breath-holding spells
≠ cyanotic CHDs ≠ acrocyanosis
• HF: hepatomegaly, splenomegaly
• Sites of peripheral edema
• HR of NB: 120 to 140 beats/min and may
↑I70+ beats/min during crying and activity or
↓70-90 beats/min during sleep.
• As the child grows older, the average pulse
rate ↓ and may be as low as 40 beats/min in
athletic adolescents.
 PULSE RATES AT REST
AGE N LOWER LIMITS AVERAGE N UPPER LIMITS
NEWBORN 70 125/min 190/min
1-11
HR mos
of NB: 120 to 140 80
beats/min and may 120 160
↑170+ beats/min during
crying
2 and activity or ↓70-90
80 beats/min during
110 sleep 130
4 80 100 120
6 75 100 115
8 As the child grows
70 older, the average90pulse rate ↓ and may
110
be as low as 40 beats/min in athletic adolescents
10 70 90 110
GIRLS BOYS GIRLS BOYS GIRLS BOYS
12 70 65 90 85 110 105
14 65 60 85 80 105 100
16 60 55 80 75 100 95
18 55 50 75 70 95 90
 PULSE RATES AT REST
AGE N LOWER LIMITS AVERAGE N UPPER LIMITS
NEWBORN 70 125/min 190/min
1-11 mos 80 120 160
2 80 110 130
4 80 100 120
6 75 100 115
8 70 90 110
10 70 90 110
GIRLS BOYS GIRLS BOYS GIRLS BOYS
12 70 65 90 85 110 105
14 65 60 85 80 105 100
16 60 55 80 75 100 95
18 55 50 75 70 95 90
• wide pulse pressure with bounding pulses:
(1) aortic run-off
– PDA, AI, arterial-venous communication
(2)↑CO: anemia, anxiety, or ↑ catecholamine
or TH secretion
• ↓ pulses in all extremities: pericardial
tamponade, LVOTO, or cardiomyopathy
 BLOOD PRESSURE
• cuff that covers ≈ 2/3 of the upper arm or leg
may be used for BP measurement
• too small cuff: falsely ↑ BP
• too large cuff slightly ↓ BP
 BLOOD PRESSURE
• 1st Korotkoff sound = SBP
• DBP = muffled or when they disappear
• For LE BP determination: the steth placed
over the popliteal artery
– 10 mm Hg higher than that in the arms
• In infants, BP determined by auscultation,
palpation, or an oscillometric (Dinamap)
device
 VENOUS PRESSURE
• JVP inspection: info about CVP and RAP
• The neck veins inspected with the patient
sitting at a 900
• The EJV should not be visible above the
clavicles unless CVP ↑
CARDIAC EXAMINATION:
Inspection & Palpation
• precordial bulge with ↑precordial activity →
cardiac enlargement
– Let the child lay supine; with the examiner
looking up from the child's feet.
• Substernal thrust: (+)RVE
• apical heave: (+) LVE
• Hyperdynamic precordium: volume load:
large L-R shunt (may be normal in thin pts)
CARDIAC EXAMINATION:
Inspection & Palpation
• Thrills: palpable murmurs
• Palpate suprasternal notch and neck for aortic
bruits: AS or PS
• RLSB thrills: VSD
• apical systolic thrills: MR
• Diastolic thrills: AV stenosis
• Timing and localization of thrills should be
carefully noted
CARDIAC EXAMINATION:
Auscultation
• The diaphragm of the stethoscope placed
firmly on the chest for high-pitched sounds
• a lightly placed bell is optimal for low-pitched
sounds
• concentrate on the characteristics & their
variation with respirations →→ murmurs
• The patient should be supine, lying quietly and
breathing normally
CARDIAC EXAMINATION:
Auscultation
• 1st heart sound: best heard at the apex
– Closure of MV & TV
• 2nd heart sound: LUSB, RUSB
– closure AV & PV
– During inspiration, ↓intrathoracic pressure, ↑
filling of R side of the heart, → → ↑RV ejection
time → → delayed closure of the PV;
– splitting of the 2nd heart sound ↑ inspiration and
↓ expiration.
CARDIAC EXAMINATION:
Auscultation
• 3rd heart sound: best heard w/ the bell at the
apex in mid-diastole
– may be normal in adolescent with slow HR
– but in a patient with the clinical signs of HF and
tachycardia ≈ gallop rhythm: may merge with a
4th heart sound
• 4th sound: ≈ atrial contraction just before the
1st heart sound in late diastole
LABORATORY EVALUATION:
Radiologic Assessment
CT ratio: estimate
the heart size in
older children

CTR >50%: cardiomegaly

the largest transverse

diameter of the heart to
the widest internal
diameter of the chest
CT ratio = (A + B) ÷ C
LABORATORY EVALUATION:
Radiologic Assessment
• Should be taken in inspiration and in upright
• CTR cannot be used accurately in NB & small
infants
– an estimation of the cardiac volume should
be made by inspecting the PA and lateral
views instead of the CTR
• the thymus may obscure the true cardiac
silhouette
 NORMAL CARDIAC SILHOUETTE
R cardiac silhouette: L cardiac border:
Aortic knob
MPA
SVC
L Left ventricle
V
Right atrium
LAA : between the
IVC MPA and the LV

The RV does
not form the
cardiac border
in the PA view
 NORMAL CARDIAC SILHOUETTE
Anterior: Posterior:

Right ventricle
Left atrium

IVC
 Enlargement of
chambers or major
vessels >>>
(+)prominence of the
areas in which these
are outlined

ECG is a more sensitive

•assess the degree of and accurate index of
pulmonary vascularity ventricular hypertrophy
 ELECTROCARDIOGRAPHY
• 13L ECG in pedia patients, (+)V3R or V4R, to
evaluate RVH

• 1st days of life: RAD, large R waves and

upright T waves in the R precordial leads (PCL)

• 1st 48 HOL, PVR ↓, the R precordial T waves

become (-)
ELECTROCARDIOGRAPHY
• The T wave in V1: should be negative in < 5 y.o
and may remain (-) into adolescence
– represents one of the most important, yet subtle
differences between pediatric and adult ECGs
• Upright T waves that persist in V3R, V4R, or V1
> 1 wk of life: RVH or strain
ELECTROCARDIOGRAPHY:
In the Newborn
• mean QRS axis: +1100 to +1800
• R PCL: R > S wave for months or years: RV
remains relatively thick throughout infancy
• L-sided leads (V5 and V6): R < S (dominant R)
• A dominant R wave: LV forces quickly
becomes evident within the 1st few days of
life
 ELECTROCARDIOGRAPHY
• Ventricular hypertrophy: ↑voltage in R & S
waves in the chest leads
• The height of deflections governed by
proximity of electrode to surface of the heart
• Because the chest wall in infants and children,
and adolescents, relatively thin, the diagnosis
of ventricular hypertrophy should not be
based on voltage changes alone
ELECTROCARDIOGRAPHY
• Approach to evaluating ECGs:
1. assessment of rate and rhythm
2. calculation of the mean frontal-plane QRS axis
3. measurements of segment intervals
4. assessment of voltages
5. assessment of ST and T-wave abnormalities
ELECTROCARDIOGRAPHY:
Sinus rhythm
• P waves precede each QRS complex; with a
normal P axis (0 to +900)
• If the atrium situated normally in the chest,
the P wave should be upright in I and aVF and
inverted in aVR
ELECTROCARDIOGRAPHY:
P Waves
• RAE : Tall (>2.5 mm), narrow, and spiked
– are seen in congenital PS, Ebstein anomaly, TVA,
sometimes cor pulmonale, thyrotoxicosis
• most obvious in leads II, V3R, and V1
• LAE: Broad P waves, bifid, biphasic
– large L-R shunts VSD, PDA, severe MS or MR

• Flat P waves: hyperK

ELECTROCARDIOGRAPHY:
QRS Complex
• Criteria for RVH:
– (1) qR pattern in the RV surface leads
– (2) (+) T wave in V3R-V4R and V1-V3 between 5
days and 6yo
– (3) monophasic R wave in V3R, V4R, or V1
– (4) rsR'pattern in RPCL w/ the 2nd R wave taller
than the initial one

*should include the RPCL: V3R &/or V4R

ELECTROCARDIOGRAPHY:
QRS COMPLEX
• Criteria for RVH:
• (5) age-corrected ↑ voltage of the R wave in V3R-V4R or
the S wave in leads V6-V7, or both
• (6) marked RAD >120 0 in patients beyond the NB period
• (7) complete reversal of the normal adult precordial RS
pattern
• (8) right atrial enlargement

– At least 2 of these changes should (+) to support a

diagnosis of RVH
ELECTROCARDIOGRAPHY:
QRS COMPLEX
• systolic overload pattern: tall, pure R in RPCL
• In infants and children <6 yr, the T waves in
V3R-V4R and V1 are abnormally upright
• The diastolic overload pattern: (ASDs): rsR'
pattern and a slightly ↑ QRS duration (minor
RV conduction delay)
 ELECTROCARDIOGRAPHY:
QRS COMPLEX
• Criteria for LVH:
– ( 1 ) depression of ST segments & inversion of the
T waves in LPCL (V5, V6, and V7): LV strain pattern
>>> severe lesion
– (2) deep Q wave in LPCL
– (3) ↑ voltage of the S wave in V3R and V1 or the R
wave in V6-V7, or both
ECHOCARDIOGRAPHY
• to evaluate cardiac structure in CHDs
• estimate intracardiac pressures and gradients across
stenotic valves and vessels
• quantitate cardiac function (both systolic and
diastolic)
• determine the direction of flow across a defect
• examine the integrity of the coronary arteries
• (+) vegetations, cardiac tumors, thrombus
• Exercise testing
• MRI, MRA, CT, Radionuclide studies
• Diagnostic and interventional cardiac
catheterization
5 Basic Questions in the Evaluation
of Congenital Heart Diseases
1. Is the patient cyanotic or acyanotic?
2. Is the PBF ↑ or ↓)
3. Is the malformation originating from the L or
R side?
4. What is the dominant ventricle?
5. Is there pulmonary hypertension? (when
present may alter the clinical manifestation &
PE)
ETI0L0GY
• Cause of most CHDs unknown
– Multifactorial
– combination of genetic predisposition
and environmental stimulus
EVALUATION OF THE INFANT OR
CHILD WITH CHDs
• 3 systematic approaches:
1. Cyanotic or acyanotic – PE & pulse
oximetry
2. Pulmonary Blood Flow (PBF) - CXR
3. (+) biventricular hypertrophy – ECG

• The final diagnosis: echocardiography, CT or

MRI, or cardiac catheterization
ACYANOTIC CHDs
• Classification: according to predominant
physiologic load on the heart
• ↑Volume load:
1. L-R shunts
2. AV regurgitation
3. Cardiomyopathies
• ↑ Pressure load:
1. ventricular outflow obstruction (PS or AS)
2. narrowing of one of the great vessels (coarctation of
the aorta)
ACYANOTIC CHDs:
Increased Volume Load: L-R shunts
• Increased Volume Load: L-R shunts
1. ASD
2. VSD
3. AVSD
4. PDA
(+) communication: systemic and pulmonary
circulation >>> shunting of fully 02 blood
back to lungs
Normal Heart
ACYANOTIC CHDs:
Ventricular septal defect
• most common cardiac malformation
• 25% of CHDs
ACYANOTIC CHDs:
Ventricular septal defect
• may occur in any portion of VS
– membranous type – most common
• posteroinferior position, anterior to septal leaflet of TV
– supracristal VSDs - less common
• superior to the crista supraventricularis
• just beneath PV; may Implnge on aortic sinus → AR
– muscular VSDs - midportion or apex
– Swiss cheese - single or multiple in the septum
Ventricular Septal Defect
direction and
magnitude of
shunt ≈ size of
defect and the
relative PVR &
SVR
QpQs ratio:
2:1 shunt: twice
the normal PBF
Ventricular Septal Defect
Restrictive VSD:
small defect (<0.5
cm2), RV pressure
is normal

Nonrestrictive
VSD: large
(usually >1.0 cm2),
RV pressure = LV
pressure
ACYANOTIC CHDs:
VSD: Pathophysiology
If L-R shunt small
(Qp:Qs <1.75 :1),
cardiac chambers not
enlarged; pulmonary
vascular bed probably
normal
Large shunts: Qp:Qs
>2:1: (+) LA & LV
volume overload ≈
RV & MPA, LA, and
LV enlarged
 ACYANOTIC CHDs:
VSD: Clinical Manifestations
Small VSDs most
common;
asymptomatic

loud, harsh, or
blowing holosystolic
murmur best over
clinical findings ≈ size
the LLSB;
of the defect & PBF
frequently (+) thrill
and pressure
ACYANOTIC CHDs:
Large VSD: Clinical Manifestations
• (+) excessive PBF & PH
1. dyspnea
2. feeding difficulties
3. poor growth
4. profuse perspiration
5. recurrent pulmonary infections
6. cardiac failure
• Such occur in early infancy
ACYANOTIC CHDs:
Large VSD: Clinical Manifestations
1. prominent left precordium
2. parasternal lift
3. laterally displaced apical impulse
4. apical thrust
5. systolic thrill
6. murmur of a large VSD < harsh than of a small
VSD
• P2 of S2 ↑ PH
ACYANOTIC CHDs:
Large VSD: Diagnosis
• CXR:
– (+/-) Cardiomegaly
– ↑ or ↓ PBF
• ECG: LVH, RVH in PS or PAH
• Echo: size and position of the VSD
– (+) ventricular septal aneurysm: partially cover the
defect
– Pulsed Doppler exam shows whether VSD is
pressure restrictive (gradient across the defect)
ACYANOTIC CHDs:
VSD: Treatment
• 30% to 50% close spontaneously < 2 yo
• Small muscular VSDs closure (80%) >
membranous VSDs (35%)
• majority of defects close before 4yo
– although (+) spontaneous closure in adults
– often have ventricular septal aneurysm tissue
limiting the magnitude of the shunt
ACYANOTIC CHDs:
Large VSD: Treatment
• Control HF; maintain normal growth
• Prevent pulmonary vascular disease
• Surgery
ACYANOTIC CHDs:
Large VSD: Treatment
• Indications for surgical closure:
– large defects (+) clinical sxs (failure to thrive)
– infants bet 6 & 12 mo old w/ large defects (+) PAH
– > 24 mo old with Qp:Qs ratio >2 : 1
– (+) supracristal VSD of any size, (+) ↑ risk for AR

• Contraindication to closure of VSD: Severe

pulmonary vascular disease
• PA banding
– With subsequent debanding and repair
• Catheter occlusion devices
ACYANOTIC CHDs:
VSD: Complications
1. Infective endocarditis
2. Arrhythmia
3. Subaortic stenosis
4. Exercise intolerance
5. Aortic Regurgitation – in supracristal VSD
6. Acquired infundibular PS
Atrial Septal Defect
ACYANOTIC CHDs: L-R shunt
lesions Atrial Septal Defect
occur in any portion of
the atrial septum:
1. Secundum
2. Primum
3. Sinus venosus
depends on which
embryonic septal
structure has failed
to develop normally
ACYANOTIC CHDs: L-R shunt
lesions Atrial Septal Defect
ASD secundum: 7% of CHDs

majority of cases: sporadic

AD inheritance: Holt-Oram
syndrome (hypoplastic or
absent radii, 1st-degree
heart block, ASD)

F > M 3:1 in incidence

Patent Foramen Ovale

common echo finding in infancy

usually no hemodynamic significance

may play an important role: PS, TVA

(+) 15-30% of adults

≠ ASD (+) risk for paradoxical (R-L)
systemic embolization
 ACYANOTIC CHDs: Atrial Septal
Defect Ostium secundum
At fossa ovalis: most
common form of ASD

associated with structurally

normal AV valves

may be single or multiple (fenestrated atrial septum)

>2 cm diameter common in symptomatic older children

 ACYANOTIC CHDs: Atrial Septal Defect
Ostium secundum: Pathophysiology
• L-R shunting depends:
1. size of the defect
2. compliance of RV &
LV
3. PVR & SVR

Large ASDs:
L-R shunting + venous return to RA → RV → lungs
Qp:Qs ≥ 2 :1
ACYANOTIC CHDs: Atrial Septal Defect
Ostium secundum: Pathophysiology

• In early life: its muscular

wall thick, ↓compliant
>>> limits L-R shunt

• With age, PVR ↓,

the RV wall
• The large BF via becomes thinner
R side of heart and the L-R shunt
>>> RAE & RVE+ across the ASD ↑
dilated PA
ACYANOTIC CHDs: Atrial Septal Defect
Ostium secundum: Pathophysiology
• Despite large PBF,
PAPressure usually
normal due to (-) ↑
pressure
communication
PVR remains low in between pulmonary
childhood, may begin to ↑ and systemic
in adulthood >>> reversal circulations
of shunt and clinical
cyanosis
ACYANOTIC CHDs: Atrial Septal Defect
Ostium secundum: Clinical Manifestations
In younger children, subtle
failure to (+/-) thrive

• in older children:
varying degrees of
often asymptomatic exercise intolerance
the lesion discovered
inadvertently during PE
ACYANOTIC CHDs: Atrial Septal Defect
Ostium secundum: Physical Examination

a mild L precordial bulge

RV systolic lift (palpable at

the LSB)

A loud S1
S2: wide, fixed
split in all phases • pulmonic ejection click
of respiration
 ACYANOTIC CHDs: Atrial Septal Defect
Ostium secundum: Physical Examination
S2: wide, fixed split in all
phases of respiration

• duration of RV ejection
varies w/ respiration:
In ASD, RV diastolic
– inspiration RV
volume constantly ↑, volume ↑, closure of
ejection time prolonged PV delayed
throughout all phases of
respiration
ACYANOTIC CHDs: Atrial Septal Defect
Ostium secundum: Diagnosis
• CXR: RAE & RVE (depending on shunt size)
– PA large, and pulmonary vascularity ↑

• ECG: volume overload of RV, RAD

– rsR pattern in RPCL: RV conduction delay

• Echocardiogram: location and size of the ASD

appreciated by 2D scanning,
– confirmed by pulsed and color flow Doppler
ACYANOTIC CHDs: Atrial Septal Defect
Ostium secundum: Complications
• Set in usually 3rd decade of life
• PAH
• Atrial dysrhythmias
• Valvular regurg (TR, MR)
• HF
• IE (rare)
– Antibiotic prophylaxis not recommended
ACYANOTIC CHDs: Atrial Septal Defect
Ostium secundum: Treatment
– For all symptomatic pts
– For asymptomatic pts with Qp: Qs ratio of 2: 1
– Timing: elective closure is usually after 1 yo
• Surgical closure: mortality rate of <1%
• Transcatheter device closure:
– occlusion devices implanted in the cardiac cath
lab: device erosion is 0.1 %
• small secundum ASDs & minimal L-R shunts,
consensus: closure not required
ACYANOTIC CHDs: Atrial Septal Defect
Ostium secundum: Prognosis
• After surgical or device closure w/ moderate
to large shunts excellent
• Symptoms disappear rapidly
• Growth is enhanced
• Heart size decreases to normal
• ECG: ↓RV forces
• ↑ complications in those who undergo
correction at >20yo
Atrioventricular Septal Defect
(AVSD)

AV canal defect

Endocardial
Cushion Defect
ACYANOTIC CHDs:
Atrioventricular Septal Defect (AVSD)
• Complete AVSD:
– (+) anterior and
posterior bridging
leaflet related to
VS, with a lateral
leaflet in each
ventricle
ACYANOTIC CHDs:
Atrioventricular Septal Defect (AVSD)
•single AV valve,
common to RV & LV

•common among
children with Down
sx
•occasionally w/ PS
ACYANOTIC CHDs:
Increased Volume Load: Regurgitant Lesions
• Increased Volume Load: Regurgitant lesions
– Complete AVSD : L-R shunt + AV regurg
– Ebsteins Anomaly: isolated TR
 ACYANOTIC CHDs:
AVSD: Clinical Manifestations
Hyperdynamic Auscultation: normal
precordium or accentuated S1;
wide, fixed split S2
Cardiomegaly

pulmonary SEM (+/-) click

low-pitched, mid-diastolic rumbling murmur at the LLSB or apex, or both, due ↑ flow via
AV valves
ACYANOTIC CHDs:
AVSD: Clinical Manifestations
• = ostium secundum ASD

• (+) exercise intolerance

• easy fatigability,
• recurrent pneumonia
– in infants with large L-R shunts and severe MR
ACYANOTIC CHDs:
AVSD: Diagnosis
• ECG:
1. LAD (left axis deviation)
2. BVH
3. isolated RVH
4. RSR’ pattern V3R & V1 (RV conduction delay)
5. normal or tall P waves
6. prolonged PR interval
ACYANOTIC CHDs:
AVSD: Diagnosis
• CXR:
– moderate to severe cardiomegaly
– ↑ PV markings
• Echocardiogram: RVE w/ encroachment of
the MV on the LVOT
– “gooseneck” deformity of the LVOT (also by angio)
• Due to abnormally low position of the LVOT
• Cardaic cath, angiography, oximetry
ACYANOTIC CHDs:
AVSD: Treatment & Prognosis
• Surgical:
– total correction
• 6-12 months due of ↑ risk to develop pulmonary
vascular disease at an early age
• CX: heart block, narrowing of the LVOT, ↑ MR severity
– PA banding
• Prognosis: unrepaired complete AVSD ≈
magnitude of L-R shunt
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 Patent ductus arteriosus

•distal to the origin of

the L subclavian
artery

•the ductus enters

the PA at its
bifurcation
 Patent ductus arteriosus

(+) important
Functional closure
role:
of PDA,tract
outflow soon after
birth
stenosis or atresia
(PS, IAA)
If ductus remains
patent when PVR ↓,
aortic blood shunted
into PA
ACYANOTIC CHDs:
Patent ductus arteriosus
• F>M (2:1)
• associated with maternal rubella infection
during early pregnancy
ACYANOTIC CHDs:
PDA: Pathophysiology
• L-R via the ductus: from aorta to PA
• extent of the shunt ≈ size of the ductus &
Qp:QS
• Patients with a large PDA high risk → Pulmo
vasc disease
• Pulse pressure is wide due to runoff of blood
into PA during diastole
ACYANOTIC CHDs:
Large PDA: Diagnosis
• CXR:
– prominent PA, ↑PV markings
– Aortic knob normal or prominent
– Heart size ≈ size of PDA (degree of L-R shunting)
• ECG: LVH, BVH
• Echocardiogram: LAE, LVE
• cardiac catheterization
ACYANOTIC CHDs:
PDA: Prognosis & Complications
• Spontaneous closure after infancy is
extremely rare
• HF occurs in early infancy w/ large PDA
• Infective Endocarditis
• Pulmonary or systemic emboli
• Pulmonary hypertension (Eisenmenger Sx)
ACYANOTIC CHDs:
PDA: Treatment
• Any age, PDA requires surgical or catheter
closure

• Transcatheter PDA closure: cardiac

catheterization lab
• Surgery: left thoracotomy or using
thoracoscopic techniques
 ACYANOTIC CHDs:
Aortopulmonary Window
defect b/w asc aorta &
MPA ; usually large
HF appear early infancy;
(+/-) mild cyanosis
murmur systolic w/
mid-diastolic
rumble due to ↑ BF
across MV
ACYANOTIC CHDs:
Increased Volume Load: Cardiomyopathies
• Increased Volume Load: Cardiomyopathies
– ↓heart muscle function
– may affect systolic contractility or diastolic
relaxation, or both
– ↓ cardiac function →↑ atrial and ventricular
filling pressure → pulmonary edema (due to
↑capillary pressure)
– Major causes: viral myocarditis, metabolic
disorders, and genetic defects
ACYANOTIC CHDs:
Obstructive Lesions
1. Pulmonary Valve Stenosis with Intact
Ventricular Septum (PS with IVS)
2. Infundibular PS and Double-Chamber RV
3. PS with Intracardiac Shunt
4. Peripheral Pulmonary Stenosis
5. Aortic stenosis
6. Coarctation of the aorta
7. Congenital MS
ACYANOTIC CHDs:
Increased Pressure Load
• Pathophysiology: obstruction to normal BF
a. ventricular outflow: valvar PS, AS, and CoA
b. ventricular inflow: TS, MS, cor triatriatum

• If mild, CO maintained , clinical sx of HF

subtle or absent

• ↑cardiac wall thickness (hypertrophy), but

later → dilatation
 ACYANOTIC CHDs: Obstructive Lesions
PS with Intact Ventricular Septum
isolated valvular PS: most common
Valve cusps deformed,
opens incompletely during
systole
Bicuspal or tricuspal
If severe: pinhole opening
If not severely thickened:
dome-like obstruction
(systole)
Pulmonic valve dysplasia:
most common in Noonan Sx
ACYANOTIC CHDs:
Increased Pressure Load: severe obstruction
• critical PS : Severe PS in the NB
– R-sided HF (hepatomegaly, peripheral edema)
– cyanosis R-L shunting across the PFO

• critical AS:
• L-sided HF (pulmonary edema, poor perfusion)
• R-sided failure →→ total circulatory collapse
ACYANOTIC CHDs:
Increased Pressure Load: severe obstruction
• CoA in older children : upper body HPN &
↓pulses in LE
– In the NB: occurrence delayed due to (+)PDA
• PDA as conduit for blood flow to partially bypass the
obstruction
• (+) symptomatic, when the PDA closes
CYANOTIC CHDs

• divided according to pathophysiology based

on CXR:

– ↓PBF: TOF, PVA with IVS, TAPVR with

obstruction

– ↑ PBF: TGA, SV, TA, TAPVR w/o obstruction

 TGA: increased
PVM; narrow
TOF: decreased PVM; mediastinal shadow
RVH with upturn apex; secondary to a
absent MPA; "boot TAPVR: increased PVM small thymus; "egg
shaped heart." or Pulm. edema, figure on side" or "apple
of 8 on a string
 CYANOTIC CHDs

PBF PBF

LVH or CVH RVH CVH LVH RVH

•Truncus •TGA •Truncus •Tricuspid •TOF

arteriosus atresia
arteriosus •PVOD
•TAPVC w/hypoplastic •PA with
•Single PA hypoplastic
•Ebstein
•HLHS RV
Ventricle •Single ventricle
with PS
•TGA + VSD
 CYANOTIC DEFECTS

PBF

CVH LVH RVH

•Truncus •PVA w •TOF

arteriosus intact IVS •DORV
w/hypoplastic
•Tricuspid w PS
PA atresia •PVOD
•Single ventricle •PA with
with PS hypoplastic
•Ebstein
RV
CYANOTIC CHDs:
Decreased Pulmonary Blood Flow
• Obstruction to PBF: at the TV, RV, PV level

• a pathway by which systemic venous blood

can shunt from R-L and enter the systemic
circulation (via PFO, ASD, or VSD)

• TVA, TOF, SV with PS

CYANOTIC CHDs:
Decreased Pulmonary Blood Flow
• degree of cyanosis≈ obstruction to PBF

• mild cyanosis vs tet spells

• if obstruction is severe, PBF dependent on

PDA
 CYANOTIC DEFECTS

PBF

LVH or CVH RVH

•Truncus •TGA
arteriosus
•TAPVC
•Single
•DORV
Ventricle
•HLHS
•TGA + VSD
CYANOTIC CHDs:
Increased Pulmonary Blood Flow
• Cyanosis:
– due to abnormal ventriculo-arterial
connections
– total mixing of systemic venous and
pulmonary venous blood within the heart
CYANOTIC CHDs:
Increased Pulmonary Blood Flow
TGA: most common

PFO & PDA: when PDA begins

allows for to close>>>
mixing NB extremely cyanotic
period
PA from LV

aorta the RV
O2 blood from
the lungs to the
Systemic venous LA is pumped
blood to the RA back into the
pumped back to lungs
the body
CYANOTIC CHD
• Cyanosis:
1. RVOTO (obstruction to RV outflow) →→ R-L
shunting
• (+) R-L shunting across PFO & PDA (RVOTO or PPHPN
of the NB)

2. when, w/o PS (admixture of PVReturn and

complex anatomic defect sSVReturn )

3. >>> pulmonary edema may also develop in HF

 CYANOTIC CHILD

Difficult to detect
CYANOTIC
unless
NEONATE
O2 sat ≤ 85%
WHAT SHOULD BE DONE?
DIAGNOSTICS:
❑ Confirm presence of cyanosis
Pulse oximetry
ABG

❑ If hypoxemic, do 100% O2 challenge

with caution
WHAT SHOULD BE DONE?

HYPEROXITEST:
• response of arterial PO2 to 100% O2
●cardiac vs pulmonary
ABG: PaO2 ↑ to >150 mm Hg
PULMONARY
PaO2 does not ↑ to this value
(remains < 100 mmHg):
CARDIAC
• “If echocardiography not immediately
available, the clinician caring for a NB with
possible cyanotic CHD should not hesitate to
start a prostaglandin infusion (for a possible
ductal-dependent lesion).”
Cyanotic CHD: Lesions with ↓PBF
Tetralogy of Fallot

Conotruncal lesion
1. Obstruction to RV
outflow (PS)
2 . Ventricular Septal Defect
3 . Overriding of the aorta
4. Right ventricular hypertrophy
Cyanotic CHD: Lesions with ↓PBF
PS in Tetralogy of Fallot
• 0 cyanosis ≈ severity of the obstruction
• PV annulus nearly normal size or quite small
– often bicuspid but rarely the only site of stenosis
– More common: (+) hypertrophy of the
subpulmonic muscle, the crista supraventricularis,
which contributes to the infundibular/ subvalvar
stenosis
•INFUDIBULOVALVAR
Cyanotic CHD: Lesions with ↓PBF
PS in Tetralogy of Fallot
1. PVA
a) PDA
b) MAPCAs (major
aortopulmonary
collateral A)
2. Imperforate PV
3. Absent MPA
4. Discontinuous RPA &
LPA
Cyanotic CHD: Lesions with ↓PBF
VSD in Tetralogy of Fallot

• Usually nonrestrictive
& large

• located just below

the aortic valve;
related to the
posterior and right
aortic cusps
Cyanotic CHD: Lesions with ↓PBF
Aorta in Tetralogy of Fallot
• aortic root is large
• overrides the VSD
<50%
• aortic arch R-sided in
20% of cases

• ≠ DORV
– Overriding of the
aorta >50%
Cyanotic CHD: Lesions with ↓PBF
TOF: Pathophysiology
degree of RVOTO:
≈timing of onset of sx
≈ 0 cyanosis
≈ 0 RVH

Pink TOF: RVOTO

mild to moderate + a
balanced shunt in
VSD
 CYANOSIS
(-)cyanosis at birth RV infundibular hypertrophy

(+) Cyanosis
 Cyanotic CHD: TOF
Clinical Manifestations & PE
Systolic ejection murmur
LUSB
•caused by turbulence
through the RVOT

Murmur ↓ prominent
with severe
obstruction,
especially during a
tet spell
Cyanotic CHD: TOF
Clinical Manifestations & PE

Dyspnea on exertion

children assume
squatting position to
relieve dyspnea
caused by physical
effort
Cyanotic CHD: TOF
Clinical Manifestations & PE
Cerebral thromboses:
common w/
polycythemia &
dehydration

Brain abscess: less

common
Cyanotic CHD: TOF
Clinical Manifestations & PE
Cerebral thromboses:
polycythemia &
dehydration
1. Adequate
hydration
2. Phlebotomy
3. Volume
replacement with
albumin or saline
Cyanotic CHD: TOF
Clinical Manifestations & PE
Brain abscess: less
common than cerebral
events

Antibiotic therapy

Surgical drainage
Cyanotic CHD: TOF
Diagnosis: Chest Xray
"boot-shaped heart"

•↓PVM
•RVH w/ upturn apex
•absent MPA
•Aorta large
20% R-sided

"coeur en sabot"
AP view
Cyanotic CHD: TOF
Diagnosis: ECG
• RAD
• RVH (dominant R in R precordial leads)
• RAE (p waves: tall, peaked, bifid)
Cyanotic CHD: TOF
Diagnosis: 2D-Echo, Cardiac Cath

2D-Echocardiogram
Cardiac Carth: R ventriculogram
 Cyanotic CHD: TOF
• CATCH 22: cardiac defects, abnormal facies,
thymic hypoplasia, cleft palate, hypocalcemia
• deletions of large segment of chromosome
22q11known
Cyanotic CHD: TOF
Treatment
• Depends on the severity of RVOTO
– Medical treatment
– Surgical intervention

– Increase PBF to prevent sequelae of severe

hypoxia
TOF: Palliative Surgery
Modified BTS

Very small PAs:

1. Severely
cyanotic
2. severely
hypoxemic
(+) recurrent
tet spells
 TETRALOGY OF FALLOT:
TOTAL CORRECTION
1. VSD closure
2. RVOTO repair

• under CPBypass
•adequate size pulmonary
arteries
 HYPERCYANOTIC SPELLS
≈ hypoxic ≈ tet spells
– major complication of cyanotic CHD
– 6 mos-3 yrs of age
– Precipitated by:
•exertion, feeding,
•pulmonary disease, anemia
• May be followed by features of an
acute CVA
 HYPERCYANOTIC SPELL
(+) hyperpnea , restlessness, worsening of
cyanosis, & disappearance of murmur

occur more frequently in the morning or after

episodes of vigorous crying
MECHANISM OF HYPOXIC SPELL
SVR RVOT obst
Crying Spasm
or defecation Tachycardia

R to L shunting
of blood
PBF
pO2
Systemic venous return
ph
Systemic Vascular resistance
pCO2

Hyperpnea or
Hyperventilation
 TREATMENT OF HYPOXIC SPELL

1. Knee-chest position
– ↓ SVReturn and ↑ SVResistance
2. Humidified O2 by mask
3. Sedation: Morphine 0.01-0.1 mg/kg
SQ or IV
4. IV fluids - correct dehydration
5. NaHCO3: correct acidosis
 TREATMENT OF HYPOXIC SPELL

6. Propanolol 0.1 mg/kg IV –

– ↓ RVOT spasm
– prevent sudden ↓ in SVR

7. General anesthesia (Ketamine): if

spell does not subside after 30 mins.

6. If still no response: BTS or surgery

 TREATMENT OF HYPOXIC SPELL
SVR RVOT obst
Crying Spasm Propranolol
or Tachycardia
defecatio
n Knee
AIM: BREAK THE
R to L shunting
chest
of blood
VICIOUS CYCLE position
PBF
O2 pO2
Systemic venous return
NaHCO3 pH
pCO2 Systemic Vascular
resistance

MSO4 Hyperpnea or Vasoconstrictors

Hyperventilation
MANAGEMENT OF A CYANOTIC
NEWBORN
A. Stabilize before transport
– echo &/or cardiac catheterizaton
•Thermoregulation
•Reduce metabolic requirements to
a minimum
•Prostaglandin E1 infusion
MANAGEMENT OF A CYANOTIC
NEWBORN: MEDICAL
– Prostaglandin E1 infusion
0.01 mcg/k/min to 0.20 mcg/k/min

Indications:
O2 saturation <80%
pO2 <30-35 mmHg
Presence of Metabolic Acidosis
MANAGEMENT OF A CYANOTIC
NEWBORN: MEDICAL
Side effects of PGE1
Apnea
Hypotension with peripheral
vasodilation
Jitteriness
Increased risk of infection
Frank seizure
– Fluid administration to maintain
arterial pressure
 MANAGEMENT OF A CYANOTIC
NEWBORN: MEDICAL

1. Avoid dehydration
Assess fluid status of the child
Hydrate

2. Hematocrit level to ≈ 55 vol%

Hydrate - NSS
Phlebotomy
 MANAGEMENT OF A CYANOTIC
INFANT OR OLDER CHILD

1. Correct coagulopathies
2. Dental prophylaxis
3. Adequate and balanced nutrition
4. Manage hypoxic spells
 Cyanotic CHD: Lesions with ↓PBF
PVA with IVS
Pulmonic atresia with intact IVS

no VSD, no egress of
blood from the RV

PV leaflets completely
fused → membrane →
atretic RVOT
Cyanotic CHD: PVA with IVS
Pathophysiology
RA pressure ↑: BF →PFO
→ LA

no VSD, no egress
of blood from the RV

In a NB w/ PVA, the only source of PBF: PDA

 Pulmonic atresia with intact IVS

1. Prostaglandin

2. BAS

3. PPBV (Percutaneous
Pulmonic Balloon
Valvotomy)

4. Surgery
 Pulmonic atresia with intact IVS:
Surgical options
• closed pulmonic valvotomy

• open heart surgery to enlarge the RVOT

– (+) BTS or (-) BTS

• For small cavity or hypoplastic RV, with atretic

outflow tract, Fontan procedure is
recommended
Cyanotic CHD: Tricuspid Atresia
Pathophysiology
Cyanotic CHD: Tricuspid Atresia
Pathophysiology
Tricuspid atresia

no outlet from RA to RV

R-L via PFO or ASD

LV blood usually flows

into RV via a VSD
 Tricuspid atresia

• interatrial
communication
• PDA

• prostaglandin infusion

• BAS
 Tricuspid atresia

• BTS done when the PBF is

• Glenn shunt deficient

• PA banding - pulmonary overcirculation

• Fontan procedure - definitive surgery
• DORV with PS
• TGA, IVS with PS
• Ebstein Anomaly of the TV
Cyanotic CHD: Lesions with ↑PBF
d-Transposition of Great Arteries
• accounts for approximately 5% of all CHDs
• aorta arises from the RV and the PA from the
LV
• The systemic and pulmonary circulations
consist of two parallel circuits
 Cyanotic CHD: Lesions with ↑PBF
d-Transposition of Great Arteries
systemic &
pulmonary
circulations: two
parallel circuits
5% of all CHDs
aorta arises from
the RV & the PA
from the LV
 TGA
Prostaglandin infusion
PDA stenting
Balloon Atrial
Septostomy (BAS)
- promote mixing in at
the atrial level
•best done in neonates < 3 weeks old
•when the LV walls not thinned out
•can handle eventual systemic pressure
 Cyanotic CHD: Lesions with ↑PBF
Total Anomalous Pulmonary Venous
Return
complete anomalous
drainage into the
systemic venous
circulation
the heart has no direct
pulmonary venous
connection into LA
May drain into the RA, coronary sinus,
SVC via vertical veins or below the
diaphragm
 TAPVR

• BAS

• Surgery
•anastomosis of the pulmonary
venous confluence with the LA with
closure of the atrial defect to create
a larger LA
 Truncus arteriosus

•large arterial trunk,

large VSD
•no main PA
•truncus overrides VSD,
receiving blood form RV
& LV
• entire cardiac output
pumped into a main
trunk
↑↑↑ PBF flow due to
↓ PVR

→ mild or absent
cyanosis

•(+) CHF as soon

as the PVR falls
 Truncus arteriosus

• SURGERY
• PA banding
• Rastelli operation – definitive repair
•VSD is closed
•PAs connected to the RV with a
homograft
 DORV
•both PA & aorta
arise from the RV
•only outlet of the
LV is the VSD
•(+) PS ≈ TOF
•(-) PS ≈ large VSD:
mild cyanosis
•PVOD
 Hypoplastic left heart syndrome

• Interatrial communication necessary

• Mixing of blood in RA
 Hypoplastic left heart syndrome
1. hypoplastic LV
2. atresia or critical
stenosis of aortic &/
or mitral valves
3. hypoplasia of the
asc aorta & aortic
arch
4. Small LV
(non-functional or
totally atretic)
Hypoplastic left heart syndrome
hypoplastic LV
Aortic or Mitral valve
atresia &/or critical
stenosis
hypoplasia of the asc
aorta & aortic arch
Small LV
(non-functional or
totally atretic)
 Hypoplastic left heart syndrome

• Become severely ill soon after birth

• (+) CHF
• Low cardiac output
•(+) cyanosis, may be mild
•(+) pallor
•(+) poor pulses
 Hypoplastic left heart syndrome

• PDA closure → Prostaglandin infusion

• Restrictive PFO → BAS
• Heart transplant
• 3-stage operation (Norwood, Sano, or hybrid
procedure, cavopulmonary connection and
completion of Fontan)
• 1ST stage operation and hybrid procedures
done but serious problems besets survivors
5 T’s in Cyanotic Heart Disease
 APPROACH TO
NEONATES WITH CCHD R -> L SHUNTING

CYANOTIC
IMPEDED FLOW TO
NEONATE
THE LUNGS

PARALLEL SERIES
OF CIRCULATION

PDA CLOSURE

RESTRICTIVE PFO

PROSTAGLANDIN
INFUSION

BALOON ATRIAL
SEPTOSTOMY
APPROACH TO A CHILD
WITH CCHD R -> L SHUNTING

CYANOTIC CHILD
IMPEDED FLOW TO
THE LUNGS

PARALLEL SERIES
OF CIRCULATION

UNDERSTAND PATHOPHYSIOLGY

INSTITUTE MANAGEMENT

REFER TO PEDIATRIC CARDIOLOGIST

INTERVENTION/SURGERY