Abstract
Familial hypertrophic cardiomyopathy is an autosomal dominant genetic disease considered the most common cause of sudden cardiac death in individuals under 35 years old, especially the athletes. This study aimed to investigate the association between the presence of late potentials and a family history of sudden death, syncope, and complex ventricular arrhythmias on patients with hypertrophic cardiomyopathy. A case series study was carried out from March 2001 to December 2002, including 22 patients with hypertrophic cardiomyopathy according to transthoracic echocardiogram criteria. Patients on a cardiac pacemaker, right bundle branch block, cardiac transplant, and under no possibilities to realize the exams were excluded. The results showed that asymmetric septal hypertrophy was the most common type (73%), 63% had a positive familial history of hypertrophic cardiomyopathy, 55% sudden cardiac death, and 23% syncope. Also, complex ventricular arrhythmias were detected in 14% and late potentials in 23% of patients. According to this study, the presence of late potentials was not associated with familial sudden death, syncope, and complex ventricular arrhythmias.
Highlights
Hypertrophic cardiomyopathy (HCM) is an uncommon cardiac condition that has a 1:500 population prevalence and is the most common cause of sudden death in the young, especially in the athletes [1,2]
This study aimed to investigate the association between the presence of late potentials and a family history of sudden death, syncope, and complex ventricular arrhythmias on patients with hypertrophic cardiomyopathy
The results showed that asymmetric septal hypertrophy was the most common type (73%), 63% had a positive familial history of hypertrophic cardiomyopathy, 55% sudden cardiac death, and 23% syncope
Summary
Hypertrophic cardiomyopathy (HCM) is an uncommon cardiac condition that has a 1:500 population prevalence and is the most common cause of sudden death in the young, especially in the athletes [1,2]. The clinical presentation is usually asymptomatic, but symptoms such as dyspnea, syncope, arrhythmias, and sudden cardiac death can be found [1]. Even considering that ethnic and racial differences can affect the familial history and genetic profile of the affected subjects, around 1,500 mutations in at least 20 genes encoding the myofilaments of the sarcomere or Z-disc have been studied and associated to this condition [3,4,5,6]. HCM is related to other medical conditions such as Friedreich’s ataxia, Noonan’s syndrome, and some metabolic disorders, for example, amyloidosis, Pompe, and Fabry’s disease [7]
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