Zhaoyang Zheng, Jinmei Zhang
West China Second University Hospital and Sichuan University.
China
Frontiers in Pediatrics
Front Pediatr 2026; 14:
DOI: 10.3389/fped.2026.1895693
Abstract
Common pulmonary vein atresia (CPVA) is an extremely rare and lethal congenital heart defect characterized by the absence of a functional connection between the common pulmonary vein chamber (CPVC) and both the left atrium and systemic venous system. We report a case of a full-term female infant who successfully underwent primary sutureless repair. The patient developed acute respiratory distress syndrome (ARDS) with severe cyanosis and refractory hypoxemia 12 min after birth and underwent emergent surgery at 15 h of life. Intraoperative findings confirmed CPVA with bilateral superior pulmonary vein stenosis. Sutureless anastomosis was performed, leaving a 2.5 mm interatrial communication. Due to severe pulmonary hypertension and high-dose vasoactive requirements, venoarterial extracorporeal membrane oxygenation (VA-ECMO) support was established immediately. ECMO was weaned on postoperative day 6, the patient was extubated on day 10, and discharged on day 43. Two-month follow-up showed unobstructed pulmonary venous flow without evidence of anastomotic stenosis. To our knowledge, this is the first report of successful primary sutureless repair of this cardiac malformation.
Category
Absence or Atresia of Normal Pulmonary Venous Connections
Diagnostic Testing. Noninvasive
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Year of Publication: 2026
Age Focus: Pediatric
Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes