Arrhythmogenic left ventricular cardiomyopathy managed with CRT-D: A case report

Abstract

Rationale: Arrhythmogenic left ventricular cardiomyopathy (ALVC) is an arrhythmogenic cardiomyopathy characterized by nonischemic left-ventricular scar, ventricular arrhythmias, and risk of sudden cardiac death. Diagnosis is often guided by cardiac magnetic resonance (CMR) and clinical criteria.

Patient concerns: A 51-year-old man presented with 10 months of intermittent chest tightness and dyspnea, which worsened over 1 month, and 1 episode of syncope.

Diagnoses: Transthoracic echocardiography showed severe left ventricular systolic dysfunction (left ventricular ejection fraction 19%). CMR demonstrated nonischemic subepicardial fibrosis and diffuse left ventricular free-wall thinning consistent with ALVC. Coronary angiography revealed no significant stenosis. A clinical cardiomyopathy panel test reported findings supportive of ALVC.

Interventions: Guideline-directed medical therapy was initiated, and a cardiac resynchronization therapy defibrillator was implanted for left ventricular dysfunction with dyssynchrony and arrhythmic risk.

Outcomes: Ten days after discharge, a localized device-pocket infection occurred and was managed with debridement, negative-pressure therapy, and intravenous vancomycin, with full recovery. At 3 and 6 months, left ventricular ejection fraction improved to 27% and 36%, respectively, and device interrogation documented no malignant ventricular arrhythmias.

Lessons: This case underscores the diagnostic value of CMR and supports the combined strategy of cardiac resynchronization therapy defibrillator in left ventricular-dominant disease with dyssynchrony, aligning with contemporary guidelines.

Keywords: arrhythmogenic left ventricular cardiomyopathy; implantable cardioverter-defibrillator; magnetic resonance imaging; myocardial fibrosis; ventricular tachycardia.