Objectives: The goal of this study was to assemble a profile and assess the significance of arrhythmias in a nontertiary- based hypertrophic cardiomyopathy(HCM) cohort. Background: Hypertrophic cardiomyopathy is assoc...Objectives: The goal of this study was to assemble a profile and assess the significance of arrhythmias in a nontertiary- based hypertrophic cardiomyopathy(HCM) cohort. Background: Hypertrophic cardiomyopathy is associated with arrhythmia- related consequences, particularly sudden death. Ventricular tachyarrhythmias on Holter electrocardiograms(ECG) have been reported as markers for sudden death in highly selected HCM populations. Methods: We assessed the profile of ventricular and supraventricular ectopy and bradyarrhythmia on ambulatory 24- h Holter ECG and also related these findings to clinical outcome in 178 HCM patients. Results: Of the 178 study patients, 157(88% ) had premature ventricular complexes(PVCs), including 21(12% ) with< 500 PVCs, 74(42% ) had couplets, 67(37% ) had supraventricular tachycardia(SVT), and 56(31% ) had nonsustained ventricular tachycardia(NSVT). Mean number of PVCs was 330± 763(range 1 to 5,435) and increased with age(p< 0.01); NSVT was associated with greater left ventricular hypertrophy(p=0.01) and severe symptoms(New York Heart Association functional classes III and IV)(p=0.04); SVT occurred more commonly in patients with outflow obstruction(p=0.02). Over a follow- up of 5.5± 3.4 years, 11(6% ) patients died suddenly(annual mortality rate, 1.1% ) including 5 patients with NSVT. For sudden death,NSVT on Holter ECG had negative and positive predictive values of 95% and 9% , and sensitivity and specificity of 45% and 69% , respectively. Conclusions: In this nontertiary- based HCM cohort, ventricular and supraventricular tachyarrhythmias were particularly frequent and demonstrated a broad spectrum on ambulatory(Holter) ECG. Paradoxically, despite such a highly arrhythmogenic substrate, sudden death events proved to be relatively uncommon. Ventricular tachyarrhythmias had a low positive and relatively high negative predictive value for sudden death in this HCM population.展开更多
OBJECTIVES: The goal of this study was to assess gender-related differences in a multicenter population with hypertrophic cardiomyopathy(HCM). BACKGROUND: Little is known regarding the impact of gender on the heteroge...OBJECTIVES: The goal of this study was to assess gender-related differences in a multicenter population with hypertrophic cardiomyopathy(HCM). BACKGROUND: Little is known regarding the impact of gender on the heterogeneous clinical profile and clinical course of HCM. METHODS: We studied 969 consecutive HCM patients from Italy and the U.S. followed over 6.2±6.1 years. RESULTS: Male patients had a 3:2 predominance(59%), similar in Italy and the U.S.(p=0.24). At initial evaluation, female patients were older and more symptomatic than male patients(47±23 years vs. 38±18 years; p< 0.001; mean New York Heart Association[NYHA] functional class 1.8±0.8 vs. 1.4±0.6; p< 0.001), and more frequently showed left ventricular outflow obstruction(37%vs. 23%; p< 0.001). Moreover, female patients were less often diagnosed fortuitously by routine medical examination(23%vs. 41%in male patients, p< 0.001). Female gender was independently associated with the risk of symptom progression to NYHA functional classes III/IV or death from heart failure or stroke compared with male gender(independent relative hazard 1.5; p< 0.001), particularly patients≥50 years of age and with resting outflow obstruction(p< 0.005). Hypertrophic cardiomyopathy-related mortality and risk of sudden death were similar in men and women. CONCLUSIONS: Women with HCM were under-represented, older, and more symptomatic than men, and showed higher risk of progression to advanced heart failure or death, often associated with outflow obstruction. These gender-specific differences suggest that social, endocrine, or genetic factors may affect the diagnosis and clinical course of HCM. A heightened suspicion for HCM in women may allow for timely implementation of treatment strategies, including relief of obstruction and prevention of sudden death or stroke.展开更多
文摘Objectives: The goal of this study was to assemble a profile and assess the significance of arrhythmias in a nontertiary- based hypertrophic cardiomyopathy(HCM) cohort. Background: Hypertrophic cardiomyopathy is associated with arrhythmia- related consequences, particularly sudden death. Ventricular tachyarrhythmias on Holter electrocardiograms(ECG) have been reported as markers for sudden death in highly selected HCM populations. Methods: We assessed the profile of ventricular and supraventricular ectopy and bradyarrhythmia on ambulatory 24- h Holter ECG and also related these findings to clinical outcome in 178 HCM patients. Results: Of the 178 study patients, 157(88% ) had premature ventricular complexes(PVCs), including 21(12% ) with< 500 PVCs, 74(42% ) had couplets, 67(37% ) had supraventricular tachycardia(SVT), and 56(31% ) had nonsustained ventricular tachycardia(NSVT). Mean number of PVCs was 330± 763(range 1 to 5,435) and increased with age(p< 0.01); NSVT was associated with greater left ventricular hypertrophy(p=0.01) and severe symptoms(New York Heart Association functional classes III and IV)(p=0.04); SVT occurred more commonly in patients with outflow obstruction(p=0.02). Over a follow- up of 5.5± 3.4 years, 11(6% ) patients died suddenly(annual mortality rate, 1.1% ) including 5 patients with NSVT. For sudden death,NSVT on Holter ECG had negative and positive predictive values of 95% and 9% , and sensitivity and specificity of 45% and 69% , respectively. Conclusions: In this nontertiary- based HCM cohort, ventricular and supraventricular tachyarrhythmias were particularly frequent and demonstrated a broad spectrum on ambulatory(Holter) ECG. Paradoxically, despite such a highly arrhythmogenic substrate, sudden death events proved to be relatively uncommon. Ventricular tachyarrhythmias had a low positive and relatively high negative predictive value for sudden death in this HCM population.
文摘OBJECTIVES: The goal of this study was to assess gender-related differences in a multicenter population with hypertrophic cardiomyopathy(HCM). BACKGROUND: Little is known regarding the impact of gender on the heterogeneous clinical profile and clinical course of HCM. METHODS: We studied 969 consecutive HCM patients from Italy and the U.S. followed over 6.2±6.1 years. RESULTS: Male patients had a 3:2 predominance(59%), similar in Italy and the U.S.(p=0.24). At initial evaluation, female patients were older and more symptomatic than male patients(47±23 years vs. 38±18 years; p< 0.001; mean New York Heart Association[NYHA] functional class 1.8±0.8 vs. 1.4±0.6; p< 0.001), and more frequently showed left ventricular outflow obstruction(37%vs. 23%; p< 0.001). Moreover, female patients were less often diagnosed fortuitously by routine medical examination(23%vs. 41%in male patients, p< 0.001). Female gender was independently associated with the risk of symptom progression to NYHA functional classes III/IV or death from heart failure or stroke compared with male gender(independent relative hazard 1.5; p< 0.001), particularly patients≥50 years of age and with resting outflow obstruction(p< 0.005). Hypertrophic cardiomyopathy-related mortality and risk of sudden death were similar in men and women. CONCLUSIONS: Women with HCM were under-represented, older, and more symptomatic than men, and showed higher risk of progression to advanced heart failure or death, often associated with outflow obstruction. These gender-specific differences suggest that social, endocrine, or genetic factors may affect the diagnosis and clinical course of HCM. A heightened suspicion for HCM in women may allow for timely implementation of treatment strategies, including relief of obstruction and prevention of sudden death or stroke.
