Management of failing bidirectional cavopulmonary shunt: Influence of additional systemic-to-pulmonary-artery shunt with classic Glenn physiology.

OBJECTIVES

Severe hypoxemia in the early postoperative period after bidirectional cavopulmonary shunt (BCPS) is a critical complication. We aimed to evaluate patients who underwent additional systemic to pulmonary shunt and septation of central pulmonary artery (partial takedown) after BCPS.

METHODS

The medical records of all patients who underwent BCPS between 2007 and 2020 were reviewed. Patients who underwent partial takedown were extracted and their outcomes were analyzed.

RESULTS

Of 441 BCPS patients, 27 patients (6%) required partial takedown. Most frequent diagnosis was hypoplastic left heart syndrome (n = 14; 52%). Additional complicating factors included pulmonary artery hypoplasia (n = 12) and pulmonary venous obstruction (n = 3). Thirteen patients (48%) underwent partial takedown on the same day of BCPS, and all of them survived the procedure. The remaining 14 patients (52%) underwent partial takedown between postoperative 1 to 64 days. The reasons for partial takedown were: postoperative high pulmonary vascular resistance (n = 4), early BCPS (<90 days) with PA hypoplasia (n = 3), mediastinitis/pneumonia (n = 3), pulmonary venous obstruction (n = 2), ventricular dysfunction (n = 1), and recurrent pneumothorax (n = 1). Four patients experienced hospital deaths. Six patients died after discharge, 10 achieved Fontan completion, and 6 were alive and waiting for Fontan. Overall survival after partial takedown was 54% at 3 years. The pulmonary venous obstruction (P = .041) and genetic/extracardiac anomalies (P = .085) were identified as risks for mortality after partial takedown.

CONCLUSIONS

The partial takedown resulted in a 3-year survival rate of more than 50%. Of these patients, a significant number underwent successful Fontan completion who would exhibit potential early death with conservative treatment.