Linda Mahgoub, Tarek Kaddoura, A. Rebecca Kameny, Palmona Lopez Ortego, Rachael D. Vanderlaan, Ashok Kakadekar, Frank Dicke, Ivan Rebeyka, Christopher A. Caldarone, Andrew Redington, Maria Jesus del Cerro, Jeff Fineman, Ian Adatia
Stollery Children’s Hospital and University of Alberta. Benioff Children’s Hospital and University of California San Francisco. Hospital for Sick Children. Royal University Hospital. Alberta Children’s Hospital.
Canada and United States
Pediatric Pulmonology
Pediatr Pulmonol 2017; 52: 1063-1070
DOI: 10.1002/ppul.23679
Abstract
Background: Pulmonary vein stenosis is emerging as an important clinical problem in ex-premature infants.
Methods: 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.
Results: 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).
Conclusion: 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.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Diagnostic Testing. Noninvasive
Diagnostic Testing. Invasive
Year of Publication: 2017
Age Focus: Pediatric
Article Type: Retrospective Observational Cohort Studies (>10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No