Recurrent sudden cardiac death secondary to anomalous right coronary artery: Insights into prevalence and management

Abstract

A 32-year-old woman presented after ventricular fibrillation arrest requiring three defibrillations. The episode coincided with an upper respiratory infection and physical exertion. Eight years prior, she survived another cardiac arrest of unknown cause during childbirth. This time, imaging revealed an anomalous right coronary artery connecting to the left coronary cusp, with a small, slit-like osteal orifice coursing between the aorta and pulmonary artery. Surgical exploration revealed an intramural segment of the right coronary artery, which was surgically unroofed with improvement in cardiac function. An implantable cardioverter-defibrillator was implanted for secondary prevention of sudden cardiac death. Surgery is recommended for malignant anomalous coronary arteries, with a very low risk of recurrence of arrhythmia and sudden cardiac death after surgery. However, with growing evidence for persistent risk of arrhythmia and sudden cardiac death even after surgical correction of the anomalous coronary arteries, more experts choose to take secondary prevention measures as a component of initial management.