Treatment Experience of the Infants with Functional Univentricular Heart Combined with Decreased Pulmonary Blood Flow and Total Anomalous Pulmonary Venous Connection

Denis Lyazin, L.A. Bockeria, D.O. Berishvili, I.Yu. Baryshnikova , O.A. Stepanicheva, Ye.A. Andreeva

A.N. Bakoulev Scientific Center for Cardiovascular Surgery, Moscow, Russia

Date, time and location: 2018.05.25 13:30, Exhibition area, 1st Floor. Zone – B

Abstract

Background:Congenital heart disease with combination of decreased pulmonary blood flow and total anomalous pulmonary venous connection (TAPVC) is an extremely rare and severe condition. The results of treatment until today remain unsatisfactory.

The aim of the study was the description of our experience in the treatment of infants with functional univentricular heart combined with reduced pulmonary blood flow and TAPVC.

Methods:From 2008 to 2016 60 patients with TAPVC and concomitant complex heart defects with decreased pulmonary blood flow underwent surgical repair at our center. In 8 cases demonstrated in presentation TAPVC was combined with functional univentricular heart and pulmonary atresia (n = 4) or with critical stenosis of the pulmonary artery (n = 4). The average age was 13±8,3 days (range, 3 days – 1 month); the average weight at the time of surgery was 2,6 ± 0,4 kg (…-…kg).

Results:The overall survival rate was 87,7%; a survival rate for 8 newborns with univentricular heart with decreased pulmonary blood flow and TAPVC was 62,5%. In 3 cases SP-shunt as palliative intervention was performed (without TAPVC repair). All these patients died in the early postoperative period. In other 5 cases, patients underwent one-stage repair of TAPVC with SP-shunt. Among this patients there were no fatal outcomes.

Conclusion:TAPVC is characterized by restriction of blood outflow from the lungs even in the absence of stenosis of pulmonary veins collector. A favorable survival prognosis is possible only among those patients who underwent the displacement of anomalous venous connection into the atriums. Depleted pulmonary blood flow may be the cause that limits the detectability of TAPVC using only the ultrasound examination.