Postoperative survival for complete atrioventricular septal defect (cAVSD) is excellent. Common complications of surgery include valvular stenosis/regurgitation, left ventricular outflow tract obstruction, arrythmias, and residual defects. We report a challenging case of a 7-month old girl with Trisomy 21, preoperative obstructive sleep apnea with severe pulmonary hypertension, who underwent AVSD repair and required veno-arterial extracorporeal membrane oxygenation (V-A ECMO) while unable to come off cardiopulmonary bypass and developed left ventricular intramural hematoma during the course. This case highlights the challenges in management of an unusual complication.
Atrioventricular septal defects (AVSD) are characterized by variable deficiency of atrial and ventricular (AV) septum and atrioventricular valves (AVV). The incidence of AVSD has been estimated to be 0.24–0.31 in 1,000 live births and is strongly associated with Trisomy 21.1
