A Rare Association of Dextrocardia and Transitional AV Canal Defect in a Child with Conjugated Hyperbilirubinemia and Subclinical Congenital Hypothyroidism

Abstract

Dextrocardia is an abnormal situs which occurs when the apex of the heart points to the right and inferiorly. Dextrocardia could occur in isolation or may occur concomitantly with other cardiac lesions. It could also occur with abnormal rotation of the atrium and visceral organs (situs inversus totalis). Complex cardiac anomalies coexisting with dextrocardia with certain endocrinepathies are rare occurrence. We report a 2-month-old female who presented with breathlessness, cyanosis and subclinical features of congenital hypothyroidism from birth. The child was previously managed as neonatal jaundice, but after phototherapy, clinical signs of jaundice persisted. Further examination revealed splitting of the second heart sound and a very faint grade 2 ejection systolic murmur located at the second left intercostal space. Chest radiograph and echocardiography revealed dextrocardia and transitional Atrio-Ventricular (AV) canal defect respectively. He is presently been worked up for AV canal repair and thyroid assay result is been waited. Dextrocardia coexisting with AV canal defect and congenital hypothyroidism are rare combination of complex cardiac defect. High index of suspicion, timely diagnosis and appropriate referral will help avert morbidities and mortality associated with this lesion