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Pulmonary hypertension in bronchopulmonary dysplasia: Clinical findings, cardiovascular anomalies and outcomes
Author(s) -
del Cerro Maria Jesús,
Sabaté Rotés Anna,
Cartón Antonio,
Deiros Lucia,
Bret Montserrat,
Cordeiro Malaika,
Verdú Cristina,
Barrios Maria Isabel,
Albajara Luis,
GutierrezLarraya Federico
Publication year - 2014
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.22797
Subject(s) - medicine , pulmonary hypertension , bronchopulmonary dysplasia , cardiology , cardiac catheterization , incidence (geometry) , pulmonary artery , stenosis , radiology , pregnancy , physics , biology , optics , genetics , gestational age
Objective Pulmonary hypertension (PH) worsens the prognosis of bronchopulmonary dysplasia (BPD). The following items have not been fully established for PH in BPD: clinical characterization, incidence of cardiovascular anomalies (CVAs), response to PH treatment, and outcome. Study Design A review of clinical records, computed tomography (CT) images and catheterization data of 36 patients with PH‐BPD referred to our PH Unit (March 2006 to December 2011) was performed. Twenty‐nine patients without major congenital heart defects and with complete follow‐up data were included. Results The diagnosis of PH was made at a median age of 4.5 months (IQR 2.4–7.8), with an echocardiography estimated median right ventricular pressure/systemic pressure ratio of 70% (IQR 60–80%). CT scanning was performed in 21 patients and catheterization in 14 patients. CVAs were found in 19 patients (65.5%): aortopulmonary collaterals (n = 9), pulmonary vein stenosis (n = 7), ASD (n = 4), and PDA (n = 9). Hemodynamic data: PVRI 4.3 UW m 2 (2.7–7); PVRI/SVRI 0.44 (0.32–0.8); and transpulmonary gradient 28 mmHg (19–40). At a median follow‐up of 35 months (IQR 21–91), 6 patients had undergone shunts closure, 22 received specific PH drugs, 3 spontaneously improved of their PH, and 8 (26%) had died. Conclusion PH in BPD is not always a transient condition; it can be diagnosed at later stages and can have a protracted course. The incidence of associated CVAs is high. Prompt diagnosis, detection, and treatment of CVAs, and specific drug therapy can improve the outcome in these patients, although the mortality rate remains high. Pediatr Pulmonol. 2014; 49:49–59. © 2013 Wiley Periodicals, Inc.