RESUMO
Arrhythmogenic right ventricular cardiomyopathy (ARVC)/dysplasia is characterized by fibro-fatty replacement of the right ventricular myocardium. A 19-year-old soldier was admitted with sudden cardiac arrest that presented as ventricular fibrillation requiring cardiopulmonary resuscitation. His electrocardiogram (ECG) showed normal sinus rhythm with inverted T waves from leads V1 to V4 and isolated premature ventricular beats with a left bundle branch block (LBBB) pattern. Echocardiography showed that the right ventricle was enlarged with apical trabecular formation. The coronary angiogram was normal. Cardiovascular magnetic resonance imaging (MRI) showed thinning and fatty substitution of both the right and left ventricular free walls. Ventricular tachycardia (LBBB pattern) occurred frequently despite anti-arrhythmic drug treatment. His drug-resistant ventricular tachycardia was treated with an implanted cardiac defibrillator. Left ventricle involvement in ARVC is less common and more severe. Cardiac MRI is considered the best imaging technique for evaluating the right ventricle and diagnosing ARVC.
Assuntos
Humanos , Adulto Jovem , Displasia Arritmogênica Ventricular Direita , Bloqueio de Ramo , Reanimação Cardiopulmonar , Morte Súbita Cardíaca , Desfibriladores , Ecocardiografia , Eletrocardiografia , Ventrículos do Coração , Imageamento por Ressonância Magnética , Militares , Miocárdio , Taquicardia Ventricular , Fibrilação Ventricular , Complexos Ventriculares PrematurosRESUMO
The Truncus arteriosus is a congenital malformation in which only one great artery arises from the base of the heart and gives origin to the systemic, pulmonary and coronary arteries proximal to the aortic arch. Pulmonary blood flow is governed by the size of the pulmonary arteries and the pulmonary vascular resistance. In infancy, pulmonary blood flow is usually excessive because pulmonary vascular resistance is not greatly increased. Thus, despite an obligatory admixture of systemic and pulmonary venous blood in the common trunk, only minimal cyanosis is present. Rarely pulmonary blood blood flow is restricted by hypoplastic or stenotic pulmonary arteries arising from the truncus. The prognosis in persistent truncus arteriosus is very poor. the median age of survival of the 94 patients with the disease reported up to 1962 was only five weeks. The longest survival reported is the case of the man described by Carr et al who lived to the age of 36 years and 2 months. We report the case of a man with persistent truncus arteriosus who lives to the age of 29 years.