An atrial switch procedure for heart transplantation in an infant with heterotaxy-dextrocardia

Abstract

Video clip is available online.We report an infant with heterotaxy-dextrocardia who underwent heart transplantation using an atrial switch procedure. CLINICAL SUMMARYThe institutional review board of the University of Iowa approved this report with a waiver of consent (IRB 202103110, March 11, 2021). The male patient was prenatally diagnosed with congenital heart disease. He was born at 34 weeks with a birth weight of 2364 g. A postnatal echocardiogram confirmed the diagnosis of left atrial isomerism, dextrocardia, common atrium, hypoplastic left ventricle with mitral and aortic atresia, hypoplastic aortic arch, ipsilateral pulmonary veins (PV), left-sided single superior vena cava (SVC), and interrupted left-sided inferior vena cava (IVC) with hemiazygos connection to the SVC. Both proximal coronary arteries were patent; however, systolic flow reversal was detected with coronary fistulas to the right ventricle. On day 2 of life, he developed persistent lactic acidosis with ST segment changes on electrocardiogram. An urgent bilateral pulmonary artery banding was performed to stabilize the patient on prostaglandin infusion. Cardiac catheterization and ductal stenting were planned on day 24 of life. He developed significant ST segment changes during the procedure and the intervention was aborted. Considering the atypical clinical course and From the Divisions of