Shock cardiogénico causado por implantación anómala de arteria coronaria

Background: Congenital anomalies of coronary implantation are unfrequent, only 1% of congenital cardiopathies. The anomalous origin of left coronary artery from the pulmonary artery (alcapa) is far more frequent and its manifestations usually arise from myocardial ischemia. Case-report: A male infant 2 month-old that was admitted at our unit with cardiogenic shock and severe metabolic acidosis. The chest X-ray showed cardiomegaly and the echocardiography found diastolic left ventricular dysfunction, with left ventricular shortening fraction of 13%, evidence of dilated cardiomyopathy and anomalous insertion of left coronary artery in the left postero-lateral side of pulmonary artery trunk. Connected to mechanical ventilation, he received crystalloids, hemoderivatives, inotropic hemodynamic support, furosemide and calcium continuous infusion. Moderate hypothermia appeared the second and third day after admission. He developed multiorgan dysfunction with cardiogenic shock and non-oliguric renal failure. At the fifth day, he was stable enough to try surgical repair. Coronary transference from pulmonary artery trunk to aorta was performed without serious adverse events. Finally, a month after his admission, he was discharged home. An ambulatory evaluation 3 months after showed normal cardiac function. Comment: Myocardial ischemia is the main manifestation of alcapa. Its signs and symptoms usually start at the end of the first gestational trimester, according to the lower pulmonary vascular resistance. The clinician must be aware in every patient with cardiogenic shock and dilated cardiomyopathy. Surgical repair must be done, although good outcomes can be achieved only with early and reliable diagnosis.