Pulmonary vein stenosis of ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort.
Pulmonary vein stenosis is emerging as an important clinical problem in ex-premature infants.



We sought to describe the epidemiology of pulmonary vein stenosis affecting ex-premature infants by a multicenter retrospective cohort study of patients from seven children's hospitals diagnosed between 2000-2014.



We identified 39 ex-premature patients (26 males, median gestational age 28 weeks range 22-36 weeks, birth weight 1.1 kg range 433-2645-g) with pulmonary vein stenosis. Median age at diagnosis was 6.5 months (1 month-6 years). Presentation with pulmonary hypertension occurred in 26/39 (67%) and 29/39 (74%) had bronchopulmonary dysplasia, 15 (39%) were born of twin pregnancies with unaffected twin siblings. A median of 5 (range 1-25) echocardiograms was performed prior to diagnosis. The diagnosis was made using echocardiography in 22/39 (56%), by multi-detector contrast computed tomography scan (CT) in 8/39 (21%), cardiac catheterization in 6/39 (15%) patients, magnetic resonance imaging in 3/39 (8%). Freedom from death or re-stenosis was 73% at 1-year, 55% at 2, 5, and 10 years. Factors associated with shorter survival or re-stenosis were stenosis of  ≥3 pulmonary veins (P < 0.01), bilateral pulmonary vein stenosis (P < 0.01) small for gestational age (P = 0.05), aged <6 months at diagnosis (P < 0.01).



Pulmonary vein stenosis of ex-premature infants is a complex problem with poor survival, delayed diagnosis, and unsatisfactory treatment. The lack of concordance in twins suggests epigenetic or environmental factors may play a role in the development of pulmonary vein stenosis. In ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia a focused echocardiographic assessment of the pulmonary veins is required with further imaging if the echocardiogram is inconclusive.
Stollery Children's Hospital, University of Alberta, Edmonton, Canada.
Linda
L
Mahgoub
Electrical and Computer Engineering, University of Alberta, Edmonton, Canada.
Tarek
T
Kaddoura
Benioff Children's Hospital, University of California San Francisco, San Francisco, California.
A Rebecca
AR
Kameny
La Paz and Ramón y Cajal University Hospitals, Madrid, Spain.
Paloma
P
Lopez Ortego
Hospital for Sick Children, Toronto, Canada.
Rachel D
RD
Vanderlaan
Royal University Hospital, Saskatoon, Canada.
Ashok
A
Kakadekar
Alberta Children's Hospital, Calgary, Canada.
Frank
F
Dicke
Stollery Children's Hospital, University of Alberta, Edmonton, Canada.
Ivan
I
Rebeyka
Hospital for Sick Children, Toronto, Canada.
Christopher A
CA
Calderone
Hospital for Sick Children, Toronto, Canada.
Andrew
A
Redington
Benioff Children's Hospital, University of California San Francisco, San Francisco, California.
Maria Jesus
MJ
Del Cerro
Benioff Children's Hospital, University of California San Francisco, San Francisco, California.
Jeff
J
Fineman
Stollery Children's Hospital, University of Alberta, Edmonton, Canada.
Ian
I
Adatia
D001997:Bronchopulmonary Dysplasia / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000453:epidemiology*; D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000453:epidemiology*; D007223:Infant; D007231:Infant, Newborn; D007234:Infant, Premature; D008279:Magnetic Resonance Imaging; D008297:Male; D011667:Pulmonary Veins / Q000473:pathology; D012189:Retrospective Studies; D000071078:Stenosis, Pulmonary Vein / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000453:epidemiology*; D014057:Tomography, X-Ray Computed
bronchopulmonary dysplasia; epidemiology; premature; pulmonary hypertension; pulmonary vein stenosis; survival