致心律失静I生右室心肌病（arrhythmogenic right ventficular dys-plasia／cardiomyopathy-ARVD／C）是一种主要以右心室心肌组织不同程度地被纤维脂肪组织所取代的心肌疾病，其临床特征主要表现为室性早搏、室性心动过速、猝死。其是...致心律失静I生右室心肌病（arrhythmogenic right ventficular dys-plasia／cardiomyopathy-ARVD／C）是一种主要以右心室心肌组织不同程度地被纤维脂肪组织所取代的心肌疾病，其临床特征主要表现为室性早搏、室性心动过速、猝死。其是在1977年由Fontaine G首次报道的典型的右室明显增大合并严重室性心律失常的病例。展开更多
We sought to determine the impact of implantable cardioverter-defibrillator(ICD) therapy in patients with familial arrhythmogenic right ventricular cardiomyopathy(ARVC). Arrhythmogenic right ventricular cardiomyopathy...We sought to determine the impact of implantable cardioverter-defibrillator(ICD) therapy in patients with familial arrhythmogenic right ventricular cardiomyopathy(ARVC). Arrhythmogenic right ventricular cardiomyopathy is a cause of sudden cardiac death, which may be prevented by ICD. We studied 11 families in which a 3p25 deoxyribonucleic acid(DNA) haplotype at locus ARVD5 segregated with disease and compared mortality in subjects who received an ICD with that in control subjects who were matched for age, gender, ARVC status, and family. Subjects(n=367) at 50%a priori risk of inheriting ARVC were classified as high risk(HR)(n=197), low risk(n=92), or unknown(n=78) on the basis of clinical events,DNA haplotyping, and/or pedigree position. Forty-eight HR subjects(30 males, [median age 32 years] and 18 females [median age 41 years]) were followed after ICD(secondary to ventricular tachycardia [VT] in 27%). Survival was compared with 58 HR control subjects who were alive at the same age to-the-day at which the ICD subject received the device. In the HR group, 50%of males were dead by 39 years and females by 71 years: relative risk of death was 5.1(95%confidence interval 3 to 8.5) for males. The five-year mortality rate after ICD in males was zero compared with 28%in control subjects(p=0.009). Within five years, the ICD fired for VT in 70%and for VT >240 beats/min in 30%, with no difference in discharge rate when analyzed by ICD indication. The unknown mutation at the ARVD5 locus causing ARVC results in high mortality. Risk stratification using genetic haplotyping and ICD therapy produced improved survival for males.展开更多
文摘致心律失静I生右室心肌病（arrhythmogenic right ventficular dys-plasia／cardiomyopathy-ARVD／C）是一种主要以右心室心肌组织不同程度地被纤维脂肪组织所取代的心肌疾病，其临床特征主要表现为室性早搏、室性心动过速、猝死。其是在1977年由Fontaine G首次报道的典型的右室明显增大合并严重室性心律失常的病例。
文摘We sought to determine the impact of implantable cardioverter-defibrillator(ICD) therapy in patients with familial arrhythmogenic right ventricular cardiomyopathy(ARVC). Arrhythmogenic right ventricular cardiomyopathy is a cause of sudden cardiac death, which may be prevented by ICD. We studied 11 families in which a 3p25 deoxyribonucleic acid(DNA) haplotype at locus ARVD5 segregated with disease and compared mortality in subjects who received an ICD with that in control subjects who were matched for age, gender, ARVC status, and family. Subjects(n=367) at 50%a priori risk of inheriting ARVC were classified as high risk(HR)(n=197), low risk(n=92), or unknown(n=78) on the basis of clinical events,DNA haplotyping, and/or pedigree position. Forty-eight HR subjects(30 males, [median age 32 years] and 18 females [median age 41 years]) were followed after ICD(secondary to ventricular tachycardia [VT] in 27%). Survival was compared with 58 HR control subjects who were alive at the same age to-the-day at which the ICD subject received the device. In the HR group, 50%of males were dead by 39 years and females by 71 years: relative risk of death was 5.1(95%confidence interval 3 to 8.5) for males. The five-year mortality rate after ICD in males was zero compared with 28%in control subjects(p=0.009). Within five years, the ICD fired for VT in 70%and for VT >240 beats/min in 30%, with no difference in discharge rate when analyzed by ICD indication. The unknown mutation at the ARVD5 locus causing ARVC results in high mortality. Risk stratification using genetic haplotyping and ICD therapy produced improved survival for males.
