Strategy of delayed repair of total anomalous pulmonary venous connection in right atrial isomerism and functional single ventricle

Research output: Contribution to journalArticlepeer-review

Abstract

Objective: Repair of total anomalous pulmonary venous connection (TAPVC) in neonates with right atrial isomerism and functional single ventricle is challenging. In our novel strategy, primary draining vein stenting (DVS) was applied to patients with preoperative pulmonary vein obstruction to delay TAPVC repair. This study investigated our initial experience with a strategy of delayed TAPVC repair, incorporating DVS. Methods: Twenty-nine patients with right atrial isomerism and functional single ventricle who had a severe obstruction in the course of draining veins, who required surgical or catheter intervention in their neonatal period were retrospectively reviewed (primary DVS: n = 11; primary TAPVC repair: n = 18). Results: Patients in the primary DVS group had more mixed type TAPVC (primary DVS: n = 5, 45.5%; primary TAPVC repair: n = 2, 11.1%; P = .03) and required more systemic to pulmonary shunt surgeries during their lifetime (primary DVS: n = 9, 81.8%; primary TAPVC repair: n = 6, 33.3%; P = .047). Kaplan–Meier analysis showed that primary DVS repair was associated with improved survival compared with primary TAPVC repair (survival rates at 90 days, 1 year, 3 years and 5 years: primary DVS: 100%, 80%, 68.6%, and 54.9%; primary TAPVC repair: 55.6%, 38.9%, 38.9%, and 38.9%, respectively [P = .04]). Of the 4 patients who underwent stenting of the ductus venosus, 3 had elevated liver enzymes after surgical repair of TAPVC due to ductus venosus steal, which markedly improved after coil embolization of the stent. Conclusions: For neonates with obstructive TAPVC and functional single ventricle, our delayed TAPVC repair using primary DVS appeared to improve survival compared with the conventional strategy.

Original languageEnglish
JournalJTCVS Open
DOIs
Publication statusAccepted/In press - 2022
Externally publishedYes

Keywords

  • congenital heart disease
  • neonate
  • right isomerism
  • stent implantation
  • surgical repair
  • total anomalous pulmonary venous connection

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Pulmonary and Respiratory Medicine

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