Primary prevention of sudden death as a novel treatment strategy in hypertrophic cardiomyopathy.

摘要: Case Report : A 20-year-old asymptomatic man was diagnosed with hypertrophic cardiomyopathy (HCM) after routine physical examination during which a systolic heart murmur detected. Echocardiography showed massive left ventricular (LV) hypertrophy septal thickness of 36 mm extending into the anterolateral wall (30 mm); outflow obstruction absent. Ambulatory (Holter) ECG 3 isolated premature contractions, and blood pressure response to exercise normal. Echocardiographic examinations in parents siblings were negative for HCM. Although 2 centers advised against an implantable cardioverter-defibrillator (ICD) based on presence only 1 risk factor sudden death (ie, extreme hypertrophy), prophylactic device recommended by third cardiac consultant. After uneventful 16-month period ICD neither detected nor treated arrhythmias, unprovoked episode fibrillation triggered defibrillation shock that immediately restored sinus rhythm (Figure 1). Figure 1. Primary prevention Continuous recording stored intracardiac electrogram from young LV (septal mm). The senses automatically delivers (arrow), restores normal rhythm. HCM is most common genetic cardiovascular disease, since its description 45 years ago, has been visible devastating consequence.1–5 Indeed, HCM cause people (including trained athletes).5 Such events usually occur previously healthy individuals without significant symptoms or as initial clinical manifestation thus generating considerable anxiety sense vulnerability among patients families.2–5 Identification high-risk efforts at represent important challenges HCM.6 The introduced 25 ago treatment strategy lethal tachyarrhythmias.7 …