title: Pulmonary hypertension in bronchopulmonary dysplasia: clinical findings, cardiovascular anomalies and outcomes.
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Pediatric Cardiology Department, La Paz Children's Hospital, Madrid, Spain.
authors.forename: Maria Jesús
authors.initials: MJ
authors.lastname: del Cerro
authors.forename: Anna
authors.initials: A
authors.lastname: Sabaté Rotés
authors.forename: Antonio
authors.initials: A
authors.lastname: Cartón
authors.forename: Lucia
authors.initials: L
authors.lastname: Deiros
authors.forename: Montserrat
authors.initials: M
authors.lastname: Bret
authors.forename: Malaika
authors.initials: M
authors.lastname: Cordeiro
authors.forename: Cristina
authors.initials: C
authors.lastname: Verdú
authors.forename: Maria Isabel
authors.initials: MI
authors.lastname: Barrios
authors.forename: Luis
authors.initials: L
authors.lastname: Albajara
authors.forename: Federico
authors.initials: F
authors.lastname: Gutierrez-Larraya
mesh_terms: D001997:Bronchopulmonary Dysplasia / Q000150:complications*; D018376:Cardiovascular Abnormalities / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
keywords: bronchopulmonary dysplasia; cardiovascular anomalies; echocardiography; premature infants; pulmonary hypertension