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.
Highlights
In approximately one percent of the general population a coronar artery anomaly (CAA) is found [1,2]
Most CAAs are discovered as incidental findings during coronary angiography and are clinically insignificant
Some CAAs are associated with clincal manifestations such as sudden cardiac death, arrhythmias, myocardial ischemia or congestive heart failure [3]
Summary
In approximately one percent of the general population a coronar artery anomaly (CAA) is found [1,2]. In patients with ALCAPA the pulmonary vascular resistance and pulmonary arterial pressure decrease shortly after birth, along with oxygen content of the pulmonary artery This causes a drop in antegrade flow and oxygen content of the anomalous left coronary artery, leading to myocardial ischemia. This may progress to myocardial infarction during periods of increased myocardial oxygen (page number not for citation purposes). In adult life symptoms may range from dyspnoea, chest pain and exercise intolerance to sudden cardiac death due to acute ischemia during exercise or malignant ventricular arrhythmias generated from myocardial scar tissue [6,7]. Based on the clinical history, with a documented episode of ventricular fibrillation (VF) without evidence of acute ischemia, a cardioverter defibrillator (ICD) was implanted
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