Yuki Nakayama, Takeshi Hiramatsu, Yusuke Iwata, Toru Okamura, Takeshi Konuma, Goki Matsumura, Kenji Suzuki, Kyoko Hobo, Toshio Nakanishi, Hiromi Kurosawa, Kenji Yamazaki
Tokyo Women’s Medical University.
Japan
Annals of Thoracic Surgery
Ann Thorac Surg 2012; 93: 606-613
DOI: 10.1016/j.athoracsur.2011.09.038
Abstract
Background: Surgical results for functional univentricular heart with total anomalous pulmonary venous connection (TAPVC) have been unsatisfactory to date.
Methods: During a 25-year period until December 2009, 207 TAPVC patients underwent surgical repair at our institute, including 56 with a univentricular heart. The 10-year survival rate was 51.1% with univentricular heart and 84.7% with biventricular heart (p<0.0001; log-rank, 27.6). Surgical outcomes and risk factors for early and late death after TAPVC repair in univentricular hearts were retrospectively analyzed.
Results: Patients were aged 3.8±4.3 years and weighed 12.3±10.7 kg at operation. Preoperative diagnoses included heterotaxy syndrome in 55, asplenia in 48, preoperative pulmonary venous obstruction in 35, and pulmonary atresia in 20. TAPVC was classified as I in 22, II in 26, III in 5, and IV in 3. Concomitant procedures included Fontan procedure in 29, bidirectional Glenn procedure in 5, systemic-pulmonary shunt in 11, and pulmonary artery banding in 5. There were 17 hospital deaths and 11 late deaths. Fontan completion was undertaken in 31 (55.3%). Postoperative pulmonary venous obstruction was found in 15. Multivariate analysis identified TAPVC III and IV and pulmonary atresia as risk factors for hospital death. Univariate analysis identified postoperative pulmonary venous obstruction and concomitant systemic-pulmonary shunt as risk factors for hospital and late death.
Conclusions: TAPVC III, IV, and pulmonary atresia are risk factors for early postoperative death. Intensive intervention, including perioperative management and operation, is required in these complex patients.
Category
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Incidence or Prevalence of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2012
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: Yes