Sudden Death as a Presenting Symptom of Hypertrophic Cardiomyopathy: Treatment with an Implantable Cardioverter Defibrillator

Abstract

Aborted sudden death as the presenting manifestation of hypertrophic cardiomyopathy in a 14-year-old child is reported. Documented ventricular fibrillation was the cause of cardiac arrest. No ventricular arrhythmia was induced during programmed electrical stimulation. An implantable cardioverter-defibrillator was indicated. As the patient had a family history of myocardial disease, he had undergone a cardiovascular evaluation 4 years before the major event, and was found normal. It is suggested that normal physical examination, ECG, echocardiogram should not rule out the diagnosis of hypertrophic cardiomyopathy when a family history is present. Left ventricular hypertrophy may develop during childhood in patients with hypertrophic cardiomyopathy.

Documented exercise-induced cardiac arrest in a paediatric patient with hypertrophic cardiomyopathy

“…18,19 Moreover, the information available in the literature on ICD treatment in children with HCM is limited. [2][3][4][5][6] This case offers some confirmation that ICD therapy is effective in young patients with HCM and previous cardiac arrest. The ICD may become established as a therapeutic option in patients with HCM at high risk.…”

“…1 Only a few reports of aborted sudden death in children and adolescent patients, and limited information about the treatment of these patients with automatic implantable cardioverter defibrillators (ICD) is available. [2][3][4][5][6] We report a case of a 12-year-old boy with HCM and aborted sudden death caused by ventricular fibrillation, who underwent ICD implantation. Very shortly thereafter, he experienced an effective defibrillator shock, and the stored electrogram showed ventricular fibrillation following sinus tachycardia during exercise.…”