Pulmonary vein stenosis: Severity and location predict survival after surgical repair.
OBJECTIVE
Pulmonary vein characteristics that influence survival after repair of stenosis have not been defined. We sought to develop a predictive model relating postrepair survival to preoperative pulmonary vein characteristics on computed tomography and magnetic resonance imaging.


METHODS
Patients who underwent pulmonary vein stenosis repair (1990-2012) with preoperative computed tomography and magnetic resonance imaging were reviewed. We measured pulmonary vein short and long cross-sectional diameters at the left atrial junction (downstream), vein bifurcation (upstream), and narrowest point, and calculated the total cross-sectional area indexed for body surface area. The relationship between pulmonary vein dimensions and survival was related via risk-adjusted parametric hazard analyses.


RESULTS
Of 145 patients who underwent surgical repair, 31 had preoperative computed tomography and magnetic resonance imaging and were analyzed. Surgical repairs were sutureless (n = 30) or pericardial patch reconstruction (n = 1). Mean follow-up was 4.28 ± 4.2 years. In-hospital mortality was 9.7%; unadjusted survival was 75% ± 7%, 69% ± 8%, and 64% ± 7% at 1, 3, and 5 years, respectively. Median downstream total cross-sectional area indexed for body surface area was 163 mm(2)/m(2), upstream total cross-sectional area indexed for body surface area was 263 mm(2)/m(2), and total cross-sectional area indexed for body surface area at maximal stenosis, localized at the left atrial junction in approximately two thirds of patients, was 163 mm(2)/m(2). Smaller upstream total cross-sectional area indexed for body surface area (P = .030) and greater number of stenotic pulmonary veins (P = .0069) were associated with increased early (<1 year) risk of death. Smaller downstream total cross-sectional area indexed for body surface area tended to be associated with a late risk of death (P = .059).


CONCLUSIONS
Smaller upstream or downstream total cross-sectional area indexed for body surface area negatively influenced survival. Early survival seemed especially poor for patients with a greater number of stenotic veins and upstream pulmonary vein involvement. The total cross-sectional area indexed for body surface area measurements can help to inform prognosis and stratify patients for enrollment in clinical trials of agents directed at pulmonary vein pathology.
Division of Cardiovascular Surgery, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Mauro
M
Lo Rito
Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, Ontario, Canada.
Tamadhir
T
Gazzaz
Division of Cardiovascular Surgery, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Travis J
TJ
Wilder
Division of Cardiovascular Surgery, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Rachel D
RD
Vanderlaan
Division of Cardiovascular Surgery, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Glen S
GS
Van Arsdell
Division of Cardiovascular Surgery, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Osami
O
Honjo
Division of Cardiovascular Surgery, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario, Canada; Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, Ontario, Canada.
Shi-Joon
SJ
Yoo
Division of Cardiovascular Surgery, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada. Electronic address: chris.caldarone@sickkids.ca.
Christopher A
CA
Caldarone
D001830:Body Surface Area; D002675:Child, Preschool; D003251:Constriction, Pathologic; D005260:Female; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D010690:Phlebography / Q000379:methods; D011237:Predictive Value of Tests; D011652:Pulmonary Circulation; D011667:Pulmonary Veins / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D016019:Survival Analysis; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000009:adverse effects / Q000401:mortality
congenital heart disease, pulmonary vein stenosis, magnetic resonance imaging, computed tomography, predictive model, survival