The assessment of left ventricular shape changes during cardiac revolution may be a new step in c... more The assessment of left ventricular shape changes during cardiac revolution may be a new step in clinical cardiology to ease early diagnosis and treatment. To quantify these changes, only point registration was adopted and neither Generalized Procrustes Analysis nor Principal Component Analysis were applied as we did previously to study a group of healthy subjects. Here, we extend to patients affected by hypertrophic cardiomyopathy the original approach and preliminarily include genotype positive/phenotype negative individuals to explore the potential that incumbent pathology might also be detected. Using 3D Speckle Tracking Echocardiography, we recorded left ventricular shape of 48 healthy subjects, 24 patients affected by hypertrophic cardiomyopathy and 3 genotype positive/phenotype negative individuals. We then applied Generalized Procrustes Analysis and Principal Component Analysis and inter-individual differences were cleaned by Parallel Transport performed on the tangent space,...
A positive endomyocardial biopsy (EMB) is a major diagnostic criterion for arrhythmogenic right v... more A positive endomyocardial biopsy (EMB) is a major diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy (ARVC). Nevertheless, its sensitivity is low due to the focal nature of the disease. Moreover, myocardial samples are usually taken from the uncommonly involved interventricular septum to minimize the risk of perforation. In this report, we describe a novel bioptical approach for ARVC diagnosis guided by the identification of right ventricle (RV) affected regions by means of electroanatomical voltage mapping.
Sudden cardiac death (SCD) is one of the most common causes of death in developed countries. In I... more Sudden cardiac death (SCD) is one of the most common causes of death in developed countries. In Italy, an annual incidence of 0.7 per 1000 inhabitants per year can be estimated. SCD represents the main cause of sudden death in children, adolescents and young adults and often occurs in young and previously asymptomatic patients. This issue has acquired even greater relevance since implantable cardioverter-defibrillators have proved to be highly effective in preventing sudden death in high-risk subjects. Autopsy findings of young SCD victims include inherited cardiac disorders with a defined morphologic substrate but also hearts without any identifiable structural abnormalities (sudden unexplained death). The potential heritability of the underlying disorder makes surviving relatives at risk of sudden death. A cardiological workup in these families may allow identification of cardiac disease and may unmask affected surviving relatives in whom the disease had remained unrecognized. Car...
In patients with heart failure (HF), during maximal cardiopulmonary exercise test, anaerobic thre... more In patients with heart failure (HF), during maximal cardiopulmonary exercise test, anaerobic threshold (AT) is not always identified. We evaluated whether this finding has a prognostic meaning. We recruited and prospectively followed up, in 14 dedicated HF units, 3058 patients with systolic (left ventricular ejection fraction <40%) HF in stable clinical conditions, New York Heart Association class I to III, who underwent clinical, laboratory, echocardiographic, and cardiopulmonary exercise test investigations at study enrollment. We excluded 921 patients who did not perform a maximal exercise, based on lack of achievement of anaerobic metabolism (peak respiratory quotient ≤1.05). Primary study end point was a composite of cardiovascular death and urgent cardiac transplant, and secondary end point was all-cause death. Median follow-up was 3.01 (1.39-4.98) years. AT was identified in 1935 out of 2137 patients (90.54%). At multivariable logistic analysis, failure in detecting AT res...
Myocardial hyperintensity on T2-weighted short-tau inversion recovery (STIR) (HyT2) cardiac magne... more Myocardial hyperintensity on T2-weighted short-tau inversion recovery (STIR) (HyT2) cardiac magnetic resonance (CMR) images has been demonstrated in patients with hypertrophic cardiomyopathy (HCM) and is considered a sign of acute damage. The aim of the current study was to evaluate the relationship between HyT2 and both a) markers of ventricular electrical instability and b) clinical and CMR parameters. Sixty-five patients underwent a thorough clinical examination, consisting of 24-h ECG recording and CMR examination including functional evaluation, T2-STIR images and late gadolinium enhancement (LGE). HyT2 was detected in 27 patients (42%), and subjects with HyT2 showed a greater left ventricle (LV) mass index (p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001), lower LV ejection fraction (p = 0.05) and greater extent of LGE (p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001) compared to those without HyT2. Twenty-two subjects (34%) presented non-sustained ventricular tachycardia (NSVT) on the 24-h ECG recording, 21 (95%) of whom exhibited HyT2. Based on the logistic regression analysis, HyT2 (odds ratio [OR]: 165, 95% CI 11-2455, p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001) and LGE extent (1.1, 1.0-1.3, p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001) served as independent predictors of NSVT, while the presence of LGE was not associated with NSVT occurrence (p = 0.49). The presence of HyT2 was associated with lower heart rate variability (p = 0.006) and a higher number of arrhythmic risk factors (p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001). In HCM patients, HyT2 upon CMR examination is associated with more advanced disease and increased arrhythmic burden.
Most patients with hypertrophic cardiomyopathy (HCM) show a limited exercise capacity. A correlat... more Most patients with hypertrophic cardiomyopathy (HCM) show a limited exercise capacity. A correlation between exercise tolerance and diastolic dysfunction has already been demonstrated. On the contrary, the role of rest-induced or exercise-induced obstruction as a determinant of exercise capacity is still open to debate. The aim of the present study was to analyse the exertional behaviour of patients with HCM presenting different left ventricle (LV) obstructive profiles. Thirty-five consecutive patients with HCM (mean age 45 ± 14 years, 23 men) underwent echocardiography during cardiopulmonary exercise (CPX) testing. Non-invasive measurement of cardiac output was obtained with an inert gas rebreathing system at the beginning and at peak of exercise. Fifteen patients (43%) had neither resting nor provocable obstruction (group A: non-obstructive profile), 12 patients (34%) showed provocable obstruction during exercise (group B: latent-obstructive profile) and eight patients (23%) presented obstruction at rest (group C: rest-obstructive profile). Group A and B patients showed higher peak oxygen consumption in comparison with group C patients (24 ± 6 and 23 ± 6 vs. 17 ± 3 ml/kg per min; P = 0.016) and a greater increment of cardiac index during exercise (6.6 ± 1.3 and 6.0 ± 1.4 vs. 4.6 ± 0.8 l/min per m; P = 0.004). In comparison with the rest-obstructive profile, latent and non-obstructive HCM patients seem to share a similar exertional behaviour characterized by a greater increment of cardiac index during exercise and a minor impairment of exercise tolerance. Accordingly, in HCM patients not obstructive at rest, latent obstruction cannot be suspected based on exertional behaviour and functional capacity. Echocardiography performed during CPX test providing an important adjunct, may be valuable in guiding treatment in patients with substantial exercise limitation.
To determine the influence of a positive genetic test for hypertrophic cardiomyopathy (HCM) on cl... more To determine the influence of a positive genetic test for hypertrophic cardiomyopathy (HCM) on clinical outcome. A cohort of 203 unrelated patients with HCM (mean +/- SD age, 50+/-18 years) was enrolled from January 1, 2002, through December 31, 2003. They were followed up for a mean +/- SD time of 4.0+/-1.7 years after genetic testing of the 8 HCM-susceptibility genes that encode key sarcomeric/myofilament proteins. The clinical phenotype of those with a positive genetic test (myofilament-positive HCM) was compared with those with a negative genetic test (myofilament-negative HCM). In this cohort of 203 patients, 87 mutations were identified in 126 patients (myofilament-positive HCM, 62%); the remaining 77 patients (38%) were myofilament-negative. Despite similar baseline features, patients with myofilament-positive HCM showed increased risk of the combined end points of cardiovascular death, nonfatal stroke, or progression to New York Heart Association class III or IV compared with the patients with myofilament-negative HCM (25% vs 7%, respectively; independent hazard ratio, 4.27; P=.008). These end points occurred at any age among patients with myofilament-positive HCM (range, 14-86 years), but only in those aged 65 years and older among patients with myofilament-negative HCM. Moreover, patients with myofilament-positive HCM showed greater probability of severe left ventricular systolic and diastolic dysfunction, defined as an ejection fraction of less than 50% and a restrictive filling pattern (P=.02 and P&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;.02, respectively, vs myofilament-negative HCM). Screening for sarcomere protein gene mutations in HCM identifies a broad subgroup of patients with increased propensity toward long-term impairment of left ventricular function and adverse outcome, irrespective of the myofilament (thick, intermediate, or thin) involved.
A positive endomyocardial biopsy (EMB) is a major diagnostic criterion for arrhythmogenic right v... more A positive endomyocardial biopsy (EMB) is a major diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy (ARVC). Nevertheless, its sensitivity is low due to the focal nature of the disease. Moreover, myocardial samples are usually taken from the uncommonly involved interventricular septum to minimize the risk of perforation. In this report, we describe a novel bioptical approach for ARVC diagnosis guided by the identification of right ventricle (RV) affected regions by means of electroanatomical voltage mapping.
ABSTRACT Background Electroanatomic (EA) voltage mapping of the right ventricle (RV) by CARTO Sys... more ABSTRACT Background Electroanatomic (EA) voltage mapping of the right ventricle (RV) by CARTO System has recently been proposed as a new diagnostic tool to identify dysplastic regions in patients affected with arrhythmogenic right ventricular cardiomyopathy (ARVC). Three-dimensional reconstruction of endocavitary electrogram distribution, performed by CARTO System, would allow the identification of RV low voltage areas, reflecting fibrofatty myocardial replacement. The purpose of our study was to compare EA voltage mapping during sinus rhythm with RV non-invasive assessment in ARVC patients. Methods we enrolled ten consecutive patients fulfilling standardized diagnostic criteria for ARVC (age range: 28-46 years, 5 males), with indication to electrophysiological test to evaluate ventricular electrical vulnerability or to validate an empirically selected antiarrhythmic treatment. After an integrated ECHO + MRI evaluation, an invasive EA reconstruction of RV was performed sampling multiple endocardial sites (202 ± 61) during sinus rhythm. Voltage map analysis was performed with a 0.5-1.5 mV colour range setting of voltage display. Results in all patients voltage mapping documented very low voltage areas (&lt;/= 0.5 mV) consisting with transmural fibrofatty replacement. Voltage analysis demonstrated in all patients different degrees of involvement of RV segments and a marked dispersion of amplitude and duration values (unipolar and bipolar). Analysis of EA distribution of low voltage areas documented a greater involvement of outflow and inflow tracts compared with RV apex and evidenced a lesser involvement of septal wall compared with free wall. In all patients concordance was noted between EA mapping results and non-invasive morphological-functional RV evaluation. Conclusions voltage mapping appears to be a promising method to study ARVC.
European journal of preventive cardiology, Jan 26, 2014
Oxygen uptake at the anaerobic threshold (VO2AT), a submaximal exercise-derived variable, indepen... more Oxygen uptake at the anaerobic threshold (VO2AT), a submaximal exercise-derived variable, independent of patients' motivation, is a marker of outcome in heart failure (HF). However, previous evidence of VO2AT values paradoxically higher in HF patients with permanent atrial fibrillation (AF) than in those with sinus rhythm (SR) raised uncertainties. We tested the prognostic role of VO2AT in a large cohort of systolic HF patients, focusing on possible differences between SR and AF. Altogether 2976 HF patients (2578 with SR and 398 with AF) were prospectively followed. Besides a clinical examination, each patient underwent a maximal cardiopulmonary exercise test (CPET). The follow-up was analysed for up to 1500 days. Cardiovascular death or urgent cardiac transplantation occurred in 303 patients (250 (9.6%) patients with SR and 53 (13.3%) patients with AF, p = 0.023). In the entire population, multivariate analysis including peak oxygen uptake (VO2) showed a prognostic capacity (C-...
The assessment of left ventricular shape changes during cardiac revolution may be a new step in c... more The assessment of left ventricular shape changes during cardiac revolution may be a new step in clinical cardiology to ease early diagnosis and treatment. To quantify these changes, only point registration was adopted and neither Generalized Procrustes Analysis nor Principal Component Analysis were applied as we did previously to study a group of healthy subjects. Here, we extend to patients affected by hypertrophic cardiomyopathy the original approach and preliminarily include genotype positive/phenotype negative individuals to explore the potential that incumbent pathology might also be detected. Using 3D Speckle Tracking Echocardiography, we recorded left ventricular shape of 48 healthy subjects, 24 patients affected by hypertrophic cardiomyopathy and 3 genotype positive/phenotype negative individuals. We then applied Generalized Procrustes Analysis and Principal Component Analysis and inter-individual differences were cleaned by Parallel Transport performed on the tangent space,...
A positive endomyocardial biopsy (EMB) is a major diagnostic criterion for arrhythmogenic right v... more A positive endomyocardial biopsy (EMB) is a major diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy (ARVC). Nevertheless, its sensitivity is low due to the focal nature of the disease. Moreover, myocardial samples are usually taken from the uncommonly involved interventricular septum to minimize the risk of perforation. In this report, we describe a novel bioptical approach for ARVC diagnosis guided by the identification of right ventricle (RV) affected regions by means of electroanatomical voltage mapping.
Sudden cardiac death (SCD) is one of the most common causes of death in developed countries. In I... more Sudden cardiac death (SCD) is one of the most common causes of death in developed countries. In Italy, an annual incidence of 0.7 per 1000 inhabitants per year can be estimated. SCD represents the main cause of sudden death in children, adolescents and young adults and often occurs in young and previously asymptomatic patients. This issue has acquired even greater relevance since implantable cardioverter-defibrillators have proved to be highly effective in preventing sudden death in high-risk subjects. Autopsy findings of young SCD victims include inherited cardiac disorders with a defined morphologic substrate but also hearts without any identifiable structural abnormalities (sudden unexplained death). The potential heritability of the underlying disorder makes surviving relatives at risk of sudden death. A cardiological workup in these families may allow identification of cardiac disease and may unmask affected surviving relatives in whom the disease had remained unrecognized. Car...
In patients with heart failure (HF), during maximal cardiopulmonary exercise test, anaerobic thre... more In patients with heart failure (HF), during maximal cardiopulmonary exercise test, anaerobic threshold (AT) is not always identified. We evaluated whether this finding has a prognostic meaning. We recruited and prospectively followed up, in 14 dedicated HF units, 3058 patients with systolic (left ventricular ejection fraction <40%) HF in stable clinical conditions, New York Heart Association class I to III, who underwent clinical, laboratory, echocardiographic, and cardiopulmonary exercise test investigations at study enrollment. We excluded 921 patients who did not perform a maximal exercise, based on lack of achievement of anaerobic metabolism (peak respiratory quotient ≤1.05). Primary study end point was a composite of cardiovascular death and urgent cardiac transplant, and secondary end point was all-cause death. Median follow-up was 3.01 (1.39-4.98) years. AT was identified in 1935 out of 2137 patients (90.54%). At multivariable logistic analysis, failure in detecting AT res...
Myocardial hyperintensity on T2-weighted short-tau inversion recovery (STIR) (HyT2) cardiac magne... more Myocardial hyperintensity on T2-weighted short-tau inversion recovery (STIR) (HyT2) cardiac magnetic resonance (CMR) images has been demonstrated in patients with hypertrophic cardiomyopathy (HCM) and is considered a sign of acute damage. The aim of the current study was to evaluate the relationship between HyT2 and both a) markers of ventricular electrical instability and b) clinical and CMR parameters. Sixty-five patients underwent a thorough clinical examination, consisting of 24-h ECG recording and CMR examination including functional evaluation, T2-STIR images and late gadolinium enhancement (LGE). HyT2 was detected in 27 patients (42%), and subjects with HyT2 showed a greater left ventricle (LV) mass index (p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001), lower LV ejection fraction (p = 0.05) and greater extent of LGE (p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001) compared to those without HyT2. Twenty-two subjects (34%) presented non-sustained ventricular tachycardia (NSVT) on the 24-h ECG recording, 21 (95%) of whom exhibited HyT2. Based on the logistic regression analysis, HyT2 (odds ratio [OR]: 165, 95% CI 11-2455, p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001) and LGE extent (1.1, 1.0-1.3, p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001) served as independent predictors of NSVT, while the presence of LGE was not associated with NSVT occurrence (p = 0.49). The presence of HyT2 was associated with lower heart rate variability (p = 0.006) and a higher number of arrhythmic risk factors (p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001). In HCM patients, HyT2 upon CMR examination is associated with more advanced disease and increased arrhythmic burden.
Most patients with hypertrophic cardiomyopathy (HCM) show a limited exercise capacity. A correlat... more Most patients with hypertrophic cardiomyopathy (HCM) show a limited exercise capacity. A correlation between exercise tolerance and diastolic dysfunction has already been demonstrated. On the contrary, the role of rest-induced or exercise-induced obstruction as a determinant of exercise capacity is still open to debate. The aim of the present study was to analyse the exertional behaviour of patients with HCM presenting different left ventricle (LV) obstructive profiles. Thirty-five consecutive patients with HCM (mean age 45 ± 14 years, 23 men) underwent echocardiography during cardiopulmonary exercise (CPX) testing. Non-invasive measurement of cardiac output was obtained with an inert gas rebreathing system at the beginning and at peak of exercise. Fifteen patients (43%) had neither resting nor provocable obstruction (group A: non-obstructive profile), 12 patients (34%) showed provocable obstruction during exercise (group B: latent-obstructive profile) and eight patients (23%) presented obstruction at rest (group C: rest-obstructive profile). Group A and B patients showed higher peak oxygen consumption in comparison with group C patients (24 ± 6 and 23 ± 6 vs. 17 ± 3 ml/kg per min; P = 0.016) and a greater increment of cardiac index during exercise (6.6 ± 1.3 and 6.0 ± 1.4 vs. 4.6 ± 0.8 l/min per m; P = 0.004). In comparison with the rest-obstructive profile, latent and non-obstructive HCM patients seem to share a similar exertional behaviour characterized by a greater increment of cardiac index during exercise and a minor impairment of exercise tolerance. Accordingly, in HCM patients not obstructive at rest, latent obstruction cannot be suspected based on exertional behaviour and functional capacity. Echocardiography performed during CPX test providing an important adjunct, may be valuable in guiding treatment in patients with substantial exercise limitation.
To determine the influence of a positive genetic test for hypertrophic cardiomyopathy (HCM) on cl... more To determine the influence of a positive genetic test for hypertrophic cardiomyopathy (HCM) on clinical outcome. A cohort of 203 unrelated patients with HCM (mean +/- SD age, 50+/-18 years) was enrolled from January 1, 2002, through December 31, 2003. They were followed up for a mean +/- SD time of 4.0+/-1.7 years after genetic testing of the 8 HCM-susceptibility genes that encode key sarcomeric/myofilament proteins. The clinical phenotype of those with a positive genetic test (myofilament-positive HCM) was compared with those with a negative genetic test (myofilament-negative HCM). In this cohort of 203 patients, 87 mutations were identified in 126 patients (myofilament-positive HCM, 62%); the remaining 77 patients (38%) were myofilament-negative. Despite similar baseline features, patients with myofilament-positive HCM showed increased risk of the combined end points of cardiovascular death, nonfatal stroke, or progression to New York Heart Association class III or IV compared with the patients with myofilament-negative HCM (25% vs 7%, respectively; independent hazard ratio, 4.27; P=.008). These end points occurred at any age among patients with myofilament-positive HCM (range, 14-86 years), but only in those aged 65 years and older among patients with myofilament-negative HCM. Moreover, patients with myofilament-positive HCM showed greater probability of severe left ventricular systolic and diastolic dysfunction, defined as an ejection fraction of less than 50% and a restrictive filling pattern (P=.02 and P&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;.02, respectively, vs myofilament-negative HCM). Screening for sarcomere protein gene mutations in HCM identifies a broad subgroup of patients with increased propensity toward long-term impairment of left ventricular function and adverse outcome, irrespective of the myofilament (thick, intermediate, or thin) involved.
A positive endomyocardial biopsy (EMB) is a major diagnostic criterion for arrhythmogenic right v... more A positive endomyocardial biopsy (EMB) is a major diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy (ARVC). Nevertheless, its sensitivity is low due to the focal nature of the disease. Moreover, myocardial samples are usually taken from the uncommonly involved interventricular septum to minimize the risk of perforation. In this report, we describe a novel bioptical approach for ARVC diagnosis guided by the identification of right ventricle (RV) affected regions by means of electroanatomical voltage mapping.
ABSTRACT Background Electroanatomic (EA) voltage mapping of the right ventricle (RV) by CARTO Sys... more ABSTRACT Background Electroanatomic (EA) voltage mapping of the right ventricle (RV) by CARTO System has recently been proposed as a new diagnostic tool to identify dysplastic regions in patients affected with arrhythmogenic right ventricular cardiomyopathy (ARVC). Three-dimensional reconstruction of endocavitary electrogram distribution, performed by CARTO System, would allow the identification of RV low voltage areas, reflecting fibrofatty myocardial replacement. The purpose of our study was to compare EA voltage mapping during sinus rhythm with RV non-invasive assessment in ARVC patients. Methods we enrolled ten consecutive patients fulfilling standardized diagnostic criteria for ARVC (age range: 28-46 years, 5 males), with indication to electrophysiological test to evaluate ventricular electrical vulnerability or to validate an empirically selected antiarrhythmic treatment. After an integrated ECHO + MRI evaluation, an invasive EA reconstruction of RV was performed sampling multiple endocardial sites (202 ± 61) during sinus rhythm. Voltage map analysis was performed with a 0.5-1.5 mV colour range setting of voltage display. Results in all patients voltage mapping documented very low voltage areas (&lt;/= 0.5 mV) consisting with transmural fibrofatty replacement. Voltage analysis demonstrated in all patients different degrees of involvement of RV segments and a marked dispersion of amplitude and duration values (unipolar and bipolar). Analysis of EA distribution of low voltage areas documented a greater involvement of outflow and inflow tracts compared with RV apex and evidenced a lesser involvement of septal wall compared with free wall. In all patients concordance was noted between EA mapping results and non-invasive morphological-functional RV evaluation. Conclusions voltage mapping appears to be a promising method to study ARVC.
European journal of preventive cardiology, Jan 26, 2014
Oxygen uptake at the anaerobic threshold (VO2AT), a submaximal exercise-derived variable, indepen... more Oxygen uptake at the anaerobic threshold (VO2AT), a submaximal exercise-derived variable, independent of patients' motivation, is a marker of outcome in heart failure (HF). However, previous evidence of VO2AT values paradoxically higher in HF patients with permanent atrial fibrillation (AF) than in those with sinus rhythm (SR) raised uncertainties. We tested the prognostic role of VO2AT in a large cohort of systolic HF patients, focusing on possible differences between SR and AF. Altogether 2976 HF patients (2578 with SR and 398 with AF) were prospectively followed. Besides a clinical examination, each patient underwent a maximal cardiopulmonary exercise test (CPET). The follow-up was analysed for up to 1500 days. Cardiovascular death or urgent cardiac transplantation occurred in 303 patients (250 (9.6%) patients with SR and 53 (13.3%) patients with AF, p = 0.023). In the entire population, multivariate analysis including peak oxygen uptake (VO2) showed a prognostic capacity (C-...
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