A 16-year-old boy with a family history of hypertrophic cardiomyopathy died suddenly despite having normal left ventricular mass, showing characteristic histological abnormalities on necropsy.
Case Report (n=1)
Can sudden death occur in patients with hypertrophic cardiomyopathy before the development of macroscopic left ventricular hypertrophy?
Sudden death can occur in patients with hypertrophic cardiomyopathy due to histological abnormalities even before macroscopic left ventricular hypertrophy develops.
An active, healthy, and symptom free 16 year old boy with a family history of hypertrophic cardiomyopathy died suddenly while walking home from school. Necropsy showed absence of left ventricular hypertrophy (that is, normal heart weight), though the characteristic histological abnormalities of hypertrophic cardiomyopathy, such as cardiac muscle cell disorganisation and abnormal intramural coronary arteries, were present. It is likely that this patient had hypertrophic cardiomyopathy and died before left ventricular hypertrophy developed.
Maron et al. (1990) conducted a case report in Hypertrophic cardiomyopathy (n=1). A 16-year-old boy with a family history of hypertrophic cardiomyopathy died suddenly despite having normal left ventricular mass, showing characteristic histological abnormalities on necropsy.