Bianca Lo Verde1, Sunjay Kaushal2, Michael C Slack3 and Sripriya Sundararajan1*
1Department of Pediatrics, University of Maryland Medical Center, USA
2Department of Cardio-Thoracic Surgery, University of Maryland, USA
3Department of Pediatric Cardiology, University of Maryland, USA
Cor-triatriatum dexter is an extremely rare congenital heart disease which can present with varied clinical manifestations. These range from being asymptomatic, incidentally diagnosed during cardiac surgery, right-sided heart failure, cyanosis and death in the neonatal period. This heart defect results from remnants of the right valve of the sinus venosus causing varying degrees of obstruction to the tricuspid valve in flow. We present a neonate with cor-triatriatum dexter who initially failed a car seat tolerance screening test and rapidly deteriorated into cardiogenic shock with tricuspid valve inflow occlusion after closure of Patent Ductus Arteriosus (PDA). The PDA was completely unresponsive to prostaglandins, necessitating emergent institution of venous-arterial extra corporeal membrane oxygenation (VA-ECMO), which provided a vital bridge for survival followed by early primary surgical resection of the cor-triatriatum dexter membrane. Without VAECMO, an adverse outcome may have ensued from a surgically correctable congenital heart defect. In this report, we stress both the need for a high index of suspicion of this rare anomaly and the value of VA-ECMO as a bridge to successful surgical correction in a symptomatic neonate with a cardiac defect that offers excellent prognosis post-surgical correction.
Cor-triatriatum dexter; VA-ECMO; Neonate
Verde BL, Kaushal S, Slack MC, Sundararajan S. Venous-Arterial ECMO as a Vital Bridge for Survival in a Neonate with Cor-triatriatum Dexter. Ann Clin Case Rep. 2017; 2: 1409.