Abstract

Background: An anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is an uncommon congenital anomaly associated with a high mortality rate within the first year of life. The rare adult patient who survives this anomaly usually presents with a moderate to severe ischaemia-induced systolic function impairment that raises the question of the risk-benefit balance of surgical correction. To assess this balance, advanced imaging modality is helpful to assess the potential postoperative recovery of the left ventricular myocardium by distinguishing between necrotic and viable myocardium. Case presentation: We report a case of late diagnosis of an abnormal connection of ALCAPA in a 67-yearold man. Preoperative scintigraphy assessed the myocardium status and found moderate to severe hypoperfusion of the anterior and inferior walls as well as the apex that was not reversible at rest. These sequences suggesting an apical, inferior and anterior necrosis (8 myocardial segments). Despite early postoperative assessment performed by transthoracic echocardiogram showed mild initial LV function improvement, scintigraphy performed at 3 years showed similar myocardial function to preoperative and a mild improvement on myocardial perfusion with 5 myocardial segments affected. Conclusion: Late diagnosis of ALCAPA in adults is rare and often associated with severely impaired LVEF due to chronic ischaemia. The risk-benefit balance of surgical decision-making in such complicated patients could be supported by a multiparametric approach including age, preoperative LVEF and advanced imaging modalities providing accurate data on myocardial perfusion to evaluate potential postoperative LV function recovery

Keywords:Bland-white-garland syndrome; Imaging; Congenital

Abbreviations: ALCAPA: Abnormal Left Coronary Artery From The Pulmonary Artery; LVEF: Left Ventricular Ejection Fraction; LGE: Late Gadolinium Enhancement; LV: Left Ventricular; MRI: Magnetic Resonance Imaging