Anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA) occur in 1 in 300 000 live births. The most common origin of the abnormal LCA is from the pulmonary truncal sinuses. The rarest form of ALCAPA presents with anomalous left coronary artery arising from the right pulmonary artery. This is a case of 1 month old female presenting with Dyspnea , 2D echocardiography revealed ALCAPA. Intraoperativley, the Left Coronary Artery was found to be originating from the Right Pulmonary Artery. The patient underwent coronary implantation and LeCompte procedure.

In the most common form of ALCAPA, the abnormal coronary artery arises from the adjacent pulmonary valvar sinus, rather than the pulmonary trunk. In this case, the Anomalous Left Coronary Artery originated the Right Pulmonary Artery. Such case has an incident of 1 in 2,000,000 live births. This is the first reported case in a tertiary cardiovascular referral center.  In fetal life, this has no detrimental effect since pressures and saturations are similar in the aorta and pulmonary artery. After birth, however, the pulmonary artery contains desaturated blood at pressures that rapidly fall below systemic pressures. The left ventricle is perfused with desaturated blood at low pressures leading to infarction with ventricular dysfunction. Coronary translocation and Lecompte maneuver was done which provided relief for the patient’s condition.