Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting with ventricular fibrillation in an adult: a case report

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. The usual clinical course is severe left sided heart failure and mitral valve insufficiency presenting during the first months of life. However, in some cases collateral blood supply from the right coronary artery is sufficient and symptoms may be subtle or even absent. Arrhythmias or sudden cardiac death in adult life may be the first clinical presentation in patients with ALCAPA. We report a case, where a 39-year old woman presented with ventricular fibrillation during phycial exertion. Coronary angiography and CT-angiography revealed an anomalous origin of the left coronary artery, and an aortic reimplantation of the left coronary artery was performed followed by ICD implantation. A review of the literature on ALCAPA is presented along with CT images before and after surgery.

Figure 1

Coronary angiography. Coronary angiography of the right coronary artery with collateral filling of the left coronary vascular territory, which connects directly to the pulmonary trunk.

Figure 2

CT-angiography before operation. Coronary CT-angiography showing an anomalous origin of the left main coronary artery (LM) from the pulmonary artery (PA). Normal course of a very large right coronary artery (RCA) is seen. Volume-rendered reformation (A), axial (B) and curved multiplanar reconstruction (C).

Figure 3

CT-angiography after operation. Coronary CT-angiography one week after aortic reimplantation of the left coronary artery confirms patency. Note the myocardial wall thinning in the apical anterior part of the left ventricle demonstrating the old infarction (arrowheads). Volume-rendered reformation (A), axial (B) and curved multiplanar reconstruction (C).