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.

An asymptomatic 16-year-old girl with anomalous left coronary artery from pulmonary artery (Bland-Withe-Garland syndrome)

“…In 85% of cases the symptoms of Bland-White-Gar land syndrome occur in the first two months of life [3,5]. Similar pressure in the aorta and pulmonary trunk existing during fetal life enables a similar blood flow in the RCA arising from the aorta and the LCA originating from the PA. After birth there is a decrease in blood pressure and blood oxygenation in the PA and the LCA, which causes myocardial ischemia of the segments supplied by the LCA.…”

“…Survival is possible only after development of potent collateral circulation between the RCA and the LCA with reversal of blood flow in the LCA (in the direction of the PA) [3][4][5]. Such a situation is present in adult patients with Bland-White-Garland syndrome described in the literature: a large, widened and tortuous RCA providing many collaterals to the LCA with reverse flow of blood in the LCA into the PA.…”

“…Prognosis in adult patients with ALCAPA is uncertain or even unfavourable [3][4][5]. The asymptomatic period is often followed by the onset of heart failure or sudden cardiac death [5,6].…”

Anomalous Origin of Left Coronary Artery from the Pulmonary Artery (ALCAPA) in an Infant - A Case Report

“…10 ALCAPA usually manifests as an isolated defect, but in 5% of cases it may be associated with other cardiac anomalies such as Atrial septal defect, Ventricular septal defect, and Coarctation, 11 it also may be associated with Tertralogy of Fallot (TOF), Transposition of great arteries (TGA) and Patent ductus arteriosus (PDA). 5 In the neonatal period, the baby is asymptomatic as there is anterograde flow of desaturated blood from the pulmonary artery to the left coronary artery. As pulmonary arterial pressure drops, the combination of low flow and desaturated blood causes myocardial ischaemia, especially during exertion.…”

“…3,4 A coronary artery anomaly may involve an abnormal number, origin and/ or course, termination or structure of the coronary arteries. 5 The left main coronary artery normally arises from the left coronary sinus of valsalva. Instead of arising from the left aortic sinus, it may arises from the right sinus of valsalva or the proximal right coronary artery, from the non-coronary (posterior) aortic sinus, from the proximal part of the ascending aorta as well as from the pulmonary trunk or artery.…”