284: CONGESTIVE HEART FAILURE AND CARDIOGENIC SHOCK SECONDARY TO LEFT MAIN CORONARY ARTERY ATRESIA

Abstract

Learning Objectives: Diaphragmatic hernias without previous trauma are a rare occurrence. The most common type being a Bochdalek hernia occurring in the posterolateral portion of the diaphragm, with an estimated frequency of 1/2,500 live births. The subject of this case suffered from an atrial septal defect (ASD) and an uncommon anterior right sided diaphragmatic hernia. Methods: We present the case of a 20 days old cyanotic neonate. The patient presented with respiratory distress, decreased peripheral circulation, suspected pneumonia, had polydactyly and was saturating 80 to low 90%. Prior perinatal findings suggested a ventricular septal defect and a foramen ovale, which prompted to consult a pediatric cardiologist. Multiple echocardiograms suggested only mild tricuspid and aortic regurgitations, moderate enlargement of the left ventricle, and a secundum ASD. Chest X ray revealed an elevation of the right hemidiaphragm. To rule out a tracheoesophageal fistula, due to cyanotic episodes during feeding, a barium swallow was performed revealing gastroesophageal reflux but no hiatal hernia. The patient was transferred to our institution with a high flow nasal cannula for an interventional cardiology evaluation. Initial echocardiogram showed a diaphragmatic eventration, but was later confirmed to be a right diaphragmatic hernia. The subject was found to have an ASD and an anterior right diaphragmatic hernia, which is considered even more uncommon due to the extra support provided by the liver. After stabilization, patient was scheduled for laparoscopic surgical repair of the hernia. Results: ASDs are usually left to right shunts, presenting as a noncyanotic lesion. However, this case highlights the possibility of an ASD behaving as a cyanotic lesion, due to external constriction of the enlarged liver over the right ventricle, rendering it unable to handle right atrial flow. This results in the uncommon occurrence of a bilateral atrial shunt, but increased right to left, resulting in cyanosis.