Takaya Hoashi, Koji Kagisaki, Kenichi Kurosaki, Masataka Kitano, Isao Shiraishi, Hajime Ichikawa
National Cerebral and Cardiovascular Center.
Japan
Pediatric Cardiology
Pediatr Cardiol 2015; 36: 432-437
DOI: 10.1007/s00246-014-1031-2
Abstract
The aim of this study was to review the surgical outcomes of conventional repair in patients with total anomalous pulmonary venous connection (TAPVC). Between 1999 and 2012, 46 patients underwent conventional TAPVC repair; of those, 14 required emergent surgery within the first 24 h after their birth for coexisting intrinsic obstruction in pulmonary venous drainage pathway (Group 1). The remaining 32 were operated on after the first day of life and were divided into two groups: with (Group 2, n = 10) and without (Group 3, n = 22) subsequently progressed pulmonary venous obstruction (PVO) after birth. A follow-up was completed on all patients, and the mean follow-up period was 7 ± 4 years (range 0.9-14). Group 1 required a significantly prolonged nitric oxide inhalation (12 ± 11 days) and mechanical ventilation support (29 ± 36 days) compared to Group 2 and Group 3. The actuarial survival rate at 10 years was 69% in Group 1, 88% in Group 2, and 96% in Group 3 (Group 1 vs. Group 3: p = 0.05). Freedom from postoperative pulmonary vein stenosis at 10 years was 39% in Group 1, 70% in Group 2, and 86% in Group 3 (Group 1 vs. Group 3: p = 0.002). However, all the ten survivors in Group 1 showed an NYHA functional status of I or II, and 60% of survivors were free from medication. Outcomes of patients with TAPVC requiring surgical repair at the day of birth for coexisting intrinsic PVO were still dismal; however, the acceptable status of current survivors has encouraged us to treat this challenging group surgically.
Category
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Incidence or Prevalence of Disease
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2015
Age Focus: Pediatric
Article Type: Retrospective Observational Cohort Studies (>10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No