Pulmonary vein stenosis of ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort

Autor: Jeffrey R. Fineman, A. Rebecca Kameny, Ivan M. Rebeyka, Linda Mahgoub, Christopher A. Calderone, Rachel D. Vanderlaan, Tarek Kaddoura, María Jesús del Cerro, Andrew N. Redington, Ashok Kakadekar, Ian Adatia, Frank Dicke, Paloma Lopez Ortego
Rok vydání: 2017
Předmět:
Zdroj: Pediatric Pulmonology. 52:1063-1070
ISSN: 8755-6863
Popis: 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
Databáze: OpenAIRE