Pulmonary flow study predicts survival in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.
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).



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.



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.



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.
Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, and Department of Surgery, University of Toronto, Toronto, Ontario, Canada; Department of Cardiothoracic Surgery, Xinhua Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
Jiaquan
J
Zhu
Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, and Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
James
J
Meza
Cardiology, The Labatt Family Heart Centre, The Hospital for Sick Children, and Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Atsuko
A
Kato
Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, and Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Arezou
A
Saedi
Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, and Department of Surgery, University of Toronto, Toronto, Ontario, Canada; Department of Pediatrics, Faculty of Medicine & Dentistry, University of Alberta, Edmonton, Alberta, Canada.
Devin
D
Chetan
Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, and Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Rachel
R
Parker
Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, and Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Christopher A
CA
Caldarone
Department of Cardiothoracic Surgery, Xinhua Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
Brian W
BW
McCrindle
Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, and Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Glen S
GS
Van Arsdell
Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, and Department of Surgery, University of Toronto, Toronto, Ontario, Canada. Electronic address: osami.honjo@sickkids.ca.
Osami
O
Honjo
D001783:Blood Flow Velocity; D003097:Collateral Circulation; D005260:Female; D006343:Heart Septal Defects / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011237:Predictive Value of Tests; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000601:surgery*; D018633:Pulmonary Atresia / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000601:surgery*; D011652:Pulmonary Circulation / Q000502:physiology; D011669:Pulmonary Wedge Pressure / Q000502:physiology; D012189:Retrospective Studies; D056346:Sternotomy; D015996:Survival Rate; D013908:Thoracotomy; D016896:Treatment Outcome
PA/VSD/MAPCAs; pulmonary flow study; unifocalization