Neonatal Presentation Of Alcapa Syndrome. A Case Report

The case is presented of neonatal ALCAPA with severe left ventricular dysfunction and moderate mitral regurgitation. The patient had good postoperative course. Despite needing extracorporeal membrane oxigenation support in the first postoperative days, the patient recovered ventricular function with persistence of mild mitral regurgitation 10 months after the surgery. Along with this case, a review is carried out on the syndrome, mainly focusing on the surgical treatment. The syndrome consists of the origin of the left coronary artery from the pulmonary trunk. Once the physiological pulmonary pressure of the newborn falls, ischemia phenomena occur due to steal from the pulmonary trunk. Depending on the level of collateral circulation developed from the right corollary artery, the patient will present with ischaemia and heart failure in childhood, or can go unnoticed until adulthood. The treatment of choice, especially in infantile form, is currently aortic reimplantation. In the vast majority of patients, recovery of left ventricular function and improvement or resolution of preoperative mitral regurgitation occur in the first year after surgery. (C) 2020 Published by Elsevier Espana, S.L.U. on behalf of Sociedad Espanola de Cirugia Cardiovascular y Endovascular. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).