A case of complete atrioventricular septal defect in which extracorporeal membrane oxygenation could be removed after performing the bilateral Glenn procedure for severe cardiac dysfunction after tricuspid valve replacement: a case report.

Background: Complete atrioventricular septal defect (AVSD) is a congenital heart disease (CHD) in which patients require surgery in early infancy. Tricuspid valve regurgitation (TR) is a complication that can occur a long time after intracardial repair for AVSD, and surgical intervention is occasionally necessary. However, to date, there have been no reports of TR occurring in the acute phase after surgery for AVSD in infancy. In addition, the mortality rate is high for patients with severe symptomatic TR who undergo surgical intervention to correct the tricuspid valve position.

Case presentation: The patient was a 17-year-old adolescent girl with severe scoliosis, who was diagnosed as having complete AVSD in the neonatal period owing to a heart murmur detected after birth. The 2-patch repair method was performed at 2-months old, but severe TR was presented from an early phase after the operation. In addition, myxomatous degeneration of the atrioventricular valve was observed as an intraoperative finding. Subsequently, the patient was admitted to our hospital owing to chest discomfort at 17-years old, and tricuspid valve replacement (TVR) was performed. As substantial deterioration of cardiac contraction was observed after the TVR, the patient was placed on extracorporeal membrane oxygenation (ECMO). However, because it was subsequently difficult to remove the patient from ECMO, the bilateral Glenn procedure was performed to increase right ventricular (RV) protection. After the bilateral Glenn procedure, the patient's cardiac contractile function improved, and she could be taken off ECMO. The patient began treatment with 2new types of therapeutic agents for heart failure, and was discharged from our hospital on the 305th hospital day.

Conclusions: If severe TR appears in a patient, it is important to intervene surgically at the asymptomatic phase with no dilatation of the RA. However, in cases of severe symptomatic TR in patients with CHD, the Glenn procedure might be a useful treatment strategy to increase RV protection.

Clinical trial number: Not applicable.