증례보고 : 심정지로 응급실에 내원한 부정맥야기성 우심실이형성/심근병증 1례

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disorder characterized by patch replacement of right ventricular myocardium by adipose and fibrous tissue and by electrical instability of the right ventricular myocardium. ARVD/C is rare in South Korea but is an important cause of cardiac arrest in young patients. The clinical manifestations of ARVD/C vary from sudden cardiac death of previously asymptomatic patients to chronic symptoms in patients with recurrent palpitations and/or right or biventricular failure. Diagnosis is based on several major and minor criteria involving structural, histologic, eletrocardiographic, arrhythmic, and genetic factors. Therapy is directed to prevent and/or treat malignant vetricular tachyarrhythmias with medications, implantable cadioverter defibrillator, radiofrequency ablation, and surgery in selected cases. We experienced a case of ARVD/C with ventricular fibrillation in the emergency department. The electrocardiography of postresuscitation showed QRS prolongation and inversion of T waves in the right precordial leads. Echocardiography revealed a dilated right atrium and right ventricle, and right ventricular dysfunction. Multislice computed tomography and cardiac magnetic resonance imaging showed multifocal fat infiltration involving right ventricular myocardium, right ventricular trabeulae, and the right ventricular side of the ventricular septum with dilatation of the right ventricle and right ventricular outflow tract and abundant epicardial fat. An implantable cardioverter defibrillator was implanted, and the patient was transferred to another hospital.