Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare congenital cardiac anomaly. ALCAPA syndrome mostly presents in the first few months of life. Left untreated, the mortality rate in the first year of life is 90% secondary to myocardial ischemia or infarction and mitral valve insufficiency leading to congestive heart failure. Sudden death may occur because of inadequate collateral circulation between the left and right coronary artery systems and/or development of arrhythmia. Currently, the prognosis for patients with ALCAPA syndrome is dramatically improved as a result of both early diagnosis and improvements in surgical techniques, including myocardial preservation. This case report presents a four month old infant with ALCAPA syndrome including clinical presentation of severe cardiomyopathy and left-sided heart failure. Child was operated for reimplantation of ALCAPA to aortic root with satisfactory outcome.