title: Pulmonary Hypertension in the Preterm Infant with Chronic Lung Disease can be Caused by Pulmonary Vein Stenosis: A Must-Know Entity.
abstract: Pulmonary hypertension (PHT) in the preterm infant is frequently due to chronic lung disease. Rarely, PHT can be caused by pulmonary vein (PV) stenosis that has been described to be associated with prematurity. This study is a retrospective analysis of all premature infants <37 weeks of gestation with PV stenosis and PHT in two French pediatric congenital cardiac centers from 1998 till 2015. Diagnosis, hemodynamics and outcome are described. Sixteen patients met the inclusion criteria. Median gestational age was 28 weeks (25 + 6-35) with a median birth weight of 842 g (585-1500). The majority of infants (87.5 %) had chronic lung disease and associated cardiac defects. Median age at diagnosis was 6.6 months (1.5-71). Fifty-six percentage (n = 9) had initially unilateral PV stenosis affecting in 89 % the left PV. Median initial invasive mean pulmonary artery pressure was 42 mmHg (25-70). Treatment options included surgical intervention (n = 6), interventional cardiac catheter (n = 3) and/or targeted therapy for pulmonary arterial hypertension (n = 5). In six patients, decision of nonintervention was taken. Global mortality was 44 %. All deaths occurred within 7 months after diagnosis regardless of chosen treatment option. Mean follow-up was 6 years (4.9 months-12 years). At last visit, all eight survivors were in stable clinical condition with five of them receiving targeted therapy for pulmonary arterial hypertension. PV stenosis is an unusual cause of PHT in the premature infant with chronic lung disease. Diagnosis is challenging since initial echocardiography can be normal and the disease is progressive. Treatment options are numerous, but prognosis remains guarded.
authors.affiliation: Department of Congenital Cardiac Surgery, Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-CCML, Centre Chirurgical Marie Lannelongue, 133, Avenue de la résistance, 92350, Le Plessis Robinson, France. d.laux@ccml.fr.
authors.forename: Daniela
authors.initials: D
authors.lastname: Laux
authors.affiliation: Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Pediatric Cardiology, Hôpital Necker Enfants Malades, Paris, France.
authors.forename: Marie-Amélie
authors.initials: MA
authors.lastname: Rocchisani
authors.affiliation: Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Pediatric Cardiology, Hôpital Necker Enfants Malades, Paris, France.
authors.forename: Younes
authors.initials: Y
authors.lastname: Boudjemline
authors.affiliation: Department of Congenital Cardiac Surgery, Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-CCML, Centre Chirurgical Marie Lannelongue, 133, Avenue de la résistance, 92350, Le Plessis Robinson, France.
authors.forename: Marielle
authors.initials: M
authors.lastname: Gouton
authors.affiliation: Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-Necker, Pediatric Cardiology, Hôpital Necker Enfants Malades, Paris, France.
authors.forename: Damien
authors.initials: D
authors.lastname: Bonnet
authors.affiliation: Department of Pediatric Cardiology, Hôpital La Timone- Enfants, AP-HM, Marseille, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Ovaert
mesh_terms: D000800:Angioplasty, Balloon; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D005602:France; D005865:Gestational Age; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D007223:Infant; D062071:Infant, Extremely Premature; D053208:Kaplan-Meier Estimate; D008297:Male; D011667:Pulmonary Veins / Q000002:abnormalities; D012189:Retrospective Studies; D000071078:Stenosis, Pulmonary Vein / Q000000981:diagnostic imaging* / Q000401:mortality* / Q000601:surgery*; D015996:Survival Rate; D016896:Treatment Outcome
keywords: Bronchopulmonary dysplasia; Chronic lung disease; Prematurity; Pulmonary hypertension; Pulmonary vein stenosis