Pulmonary flow study predicts survival in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.
Journal Article (Journal Article)
Background
We hypothesized that mean pulmonary artery pressure (PAP) detected on a pulmonary flow study may predict medium-term survival and right ventricular systolic pressure (RVSP) in patients with pulmonary atresia (PA), ventricular septal defect (VSD), and major aortopulmonary collateral arteries (MAPCAs).Methods
Fifty patients with PA/VSD/MAPCAs underwent unifocalization between 2000 and 2013, and 40 of these patients had a pulmonary flow study since 2003. Predictability of the mean PAP on VSD status, medium-term survival, reintervention, and RVSP were analyzed.Results
Forty-seven of the 50 patients (94%) had complete unifocalization at a median age of 11 months (range, 1-194 months), and 37 patients (74%) achieved VSD closure. Among the 40 patients who underwent a pulmonary flow study, the VSD was closed in 34 (85%), with salvage VSD fenestration in 4 (10%), and was intentionally left open in 6 (15%). Survival was 85.5% at 1 year and 78.5% at 5 years. A mean PAP ≥25 mm Hg was associated with worse survival (P = .011). Cox regression analysis identified a mean PAP ≥25 mm Hg as the sole predictor for death (P = .037). Patients with an open VSD had an increased risk of reoperation (P = .001) and pulmonary artery reintervention (P = .010), and had a trend toward increased risk of death (P = .059), compared with those with a closed VSD.Conclusions
PAP obtained from the intraoperative pulmonary flow study is associated with medium-term survival and late RVSP in patients with PA/VSD/MAPCAs. VSD closure for patients with a mean PAP ≥25 mm Hg on a flow study is considered high risk, and sensible judgment and a low threshold for VSD fenestration are required.Full Text
Duke Authors
Cited Authors
- Zhu, J; Meza, J; Kato, A; Saedi, A; Chetan, D; Parker, R; Caldarone, CA; McCrindle, BW; Van Arsdell, GS; Honjo, O
Published Date
- December 2016
Published In
Volume / Issue
- 152 / 6
Start / End Page
- 1494 - 1503.e1
PubMed ID
- 27692766
Electronic International Standard Serial Number (EISSN)
- 1097-685X
International Standard Serial Number (ISSN)
- 0022-5223
Digital Object Identifier (DOI)
- 10.1016/j.jtcvs.2016.07.082
Language
- eng