Familial Hypertrophic Cardiomyopathy: Late Potentials and Other Prognostic Markers

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.

Prognosis of patients with familial hypertrophic cardiomyopathy: A single-center cohort study with ten-year follow-up by propensity score matching analysis

“…After matching, FHCM had higher cardiovascular mortality/cardiac transplant rates, and the SCD rate also tended to be higher, which was largely consistent with the pre-matching results. Meanwhile, the results of our clinical data are mostly consistent with the conclusion that FHCM has a poor prognosis in previous genetic testing studies [ [3] , [4] , [5] , 16 , 17 , [22] , [23] , [24] , [25] ]. Based on our clinical data, we suggest that for patients with FHCM and a positive family history of SCD, clinical cascade screening should be recommended with a focus on management, regardless of whether positive mutations are found.…”

“…Hypertrophic cardiomyopathy (HCM) is the most common heritable cardiomyopathy and manifests as left ventricular hypertrophy (LVH) without a secondary cause and a nondilated left ventricle [ [1] , [2] , [3] ]. Familial HCM (FHCM) is a type of cardiomyopathy most closely associated with sarcomere gene mutations that accounts for 50–70% of all HCM cases [ 2 , [4] , [5] , [6] , [7] ], and patients with sarcomere mutations have been reported to show earlier clinical symptoms and a worse prognosis [ 5 , 8 ].…”

ECG-based risk stratification of sudden cardiac death and life-threatening ventricular arrhythmias

“…Более поздние исследования этого вопроса немногочисленны. Исследование Â.Chaves-Markman et al (2020) не выявило достоверной корреляции наличия ППЖ с синкопальными состояниями, ЖА и семейным анамнезом ВСС у пациентов с семейной ГКМП[47]. У пациентов с ДКМП…”