Anna N. Seale, Hideki Uemura, Steven A. Webber, John Partridge, Michael Roughton, Siew Y. Ho, Karen P. McCarthy, Sheila Jones, Lynda Shaughnessy, Jan Sunnegardh, Katarina Hanseus, Hakan Berggren, Sune Johansson, Michael L. Rigby, Barry R. Keeton,Piers E. F. Daubeney on behalf of the British Congenital Cardiac Association
Royal Brompton Hospital, National Heart and Lung Institute and Imperial College
United Kingdom
Journal of Thoracic and Cardiovascular Surgery
J Thorac Cardiovasc Surg 2013; 145: 1255-1262
DOI: 10.1016/j.jtcvs.2012.06.031
Abstract
Objective: Pulmonary venous obstruction (PVO) is an important cause of late mortality in total anomalous pulmonary venous connection (TAPVC). We aimed to describe current practices for the management of postoperative PVO and the efficacy of the different interventional procedures.
Methods: We conducted a retrospective international collaborative population-based study involving 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. Patients with TAPVC born between January 1, 1998, and December 31, 2004, were identified. Patients with functionally univentricular circulation or atrial isomerism were excluded. All available data and images were reviewed.
Results: Of 406 patients undergoing repair of TAPVC, 71 (17.5%) had postoperative PVO. The diagnosis was made within 6 months of surgery in 59 (83%) of the 71 patients. In 12, serial imaging documented change in appearance of the pulmonary veins. Good-sized pulmonary veins can progress to diffusely small veins and rarely atresia. Patients presenting after 6 months had less severe disease; all are alive at most recent follow-up. Fifty-six (13.8%) of 406 patients underwent intervention for postoperative PVO: 44 had surgical treatment and 12 had an initial catheter intervention. One half underwent 1 or more reinterventions. Three-year survival for patients with postoperative PVO was 58.7% (95% confidence intervals, 46.2%-69.2%) with a trend that those having a surgical strategy did better (P = .083). Risk factors for death included earlier presentation after TAPVC repair, diffusely small pulmonary veins at presentation of postoperative PVO, and an increased number of lung segments affected by obstruction.
Conclusions: Postoperative PVO tends to appear in the first 6 months after TAPVC repair and can be progressive. Early intervention for PVO may be indicated before irreversible secondary changes occur.
Category
Segmental Pulmonary Venous Disease. Without a Focus on Pulmonary Hypertension
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes