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.
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Acknowledgments
The authors thank Pr Alain Fraisse (formerly Department of Pediatric Cardiology, Hôpital La Timone- Enfants, AP-HM, Marseille, France, now Brompton Hospital, London, UK), Dr Yves Dulac (Centre hospitalier universitaire de Toulouse, France), Dr Pascal Amedro (Centre regional hospitalier universitaire de Montpellier, France) and Dr Jean-Marc Rosenthal (Centre hospitalier universitaire de Guadeloupe, Antilles, France) for clinical care and/or follow-up data concerning four of the survivors who are followed in the outpatient departments of the respective institutions.
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All procedures performed in this study involving human participants were in accordance with ethical standards of the institutional research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. For this type of study, no formal consent is required. This article does not contain any studies with animals performed by any of the authors.
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Laux, D., Rocchisani, MA., Boudjemline, Y. et al. Pulmonary Hypertension in the Preterm Infant with Chronic Lung Disease can be Caused by Pulmonary Vein Stenosis: A Must-Know Entity. Pediatr Cardiol 37, 313–321 (2016). https://doi.org/10.1007/s00246-015-1279-1
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DOI: https://doi.org/10.1007/s00246-015-1279-1