title: Pulmonary hypertension specific treatment in infants with bronchopulmonary dysplasia.
abstract: When bronchopulmonary dysplasia (BPD) is complicated by pulmonary hypertension (PH), morbidity and mortality are significantly increased. BPD-associated PH is not included in the current indications for PH medications. However, limited data demonstrate hemodynamic improvement and decreased mortality with PH-specific treatment. This report describes our 6-year experience treating BPD-associated PH with PH medications, mainly sildenafil.
  
  
  
  The medical records of 20 infants diagnosed with BPD-associated PH at a tertiary pediatric pulmonary hypertension clinic in 2008-2014 were reviewed. Clinical improvement was defined as a decrease in Ross functional class by at least one degree. PH severity was classified by echocardiography as mild, moderate, or severe. Hemodynamic improvement was defined as a decrease in PH severity by at least one level.
  
  
  
  Eighteen out of 20 patients were treated with PH medications: 12 sildenafil, 5 sildenafil and bosentan, and 1 bosentan. Median follow-up time was 2 years. Mean functional class significantly decreased from 3.2 ± 0.9 at diagnosis to 1.7 ± 0.9 at the last follow-up. Improvement in functional class was observed in 15/16 children (94%). Moderate or severe PH was found in 13/18 children (72%) at diagnosis, and in three (17%, all moderate PH) at the last follow-up. Improvement in PH class by echocardiography was demonstrated in 14/18 children (78%). The survival rate was 95%.
  
  
  
  Treatment of BPD complicated by PH with PH-specific medications, mainly sildenafil, is associated with improvement in both clinical and hemodynamic parameters and a low mortality rate. Pediatr Pulmonol. 2017;52:77-83. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Pediatric Intensive Care Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Gili
authors.initials: G
authors.lastname: Kadmon
authors.affiliation: Cardiac Intensive Care Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Ofer
authors.initials: O
authors.lastname: Schiller
authors.affiliation: Heart Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Tamir
authors.initials: T
authors.lastname: Dagan
authors.affiliation: Heart Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Elchanan
authors.initials: E
authors.lastname: Bruckheimer
authors.affiliation: Heart Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Einat
authors.initials: E
authors.lastname: Birk
authors.affiliation: Pulmonary Outpatient Clinic, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Tommy
authors.initials: T
authors.lastname: Schonfeld
mesh_terms: D000959:Antihypertensive Agents / Q000494:pharmacology / Q000627:therapeutic use*; D000077300:Bosentan; D001997:Bronchopulmonary Dysplasia / Q000150:complications* / Q000000981:diagnostic imaging; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D004452:Echocardiography; D005260:Female; D006439:Hemodynamics / Q000187:drug effects; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D000068677:Sildenafil Citrate / Q000494:pharmacology / Q000627:therapeutic use*; D013449:Sulfonamides / Q000494:pharmacology / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000068677:Sildenafil Citrate; D000077300:Bosentan
keywords: bosentan; mortality; sildenafil; treatment