title: Prognosis in children with pulmonary arterial  hypertension: 10-year single-centre experience.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) is a rare progressive disease of the pulmonary arterioles with an unfavourable prognosis.
  
  
  OBJECTIVE
  To evaluate survival and prognostic factors in patients with PAH diagnosed and treated at a single centre in the years 2004–2013.
  
  
  METHODS
  The study included 55 children (33 girls; 66%, 22 boys; 33%), with an average age 6.2 ± 6.0 years, with idiopathic PAH — n = 23 (42%), PAH associated with systemic-to-pulmonary shunts — n = 17 (31%), and PAH after corrective cardiac surgery — n = 15 (27%). Forty-seven of them (87%) were treated with advanced therapy.
  
  
  RESULTS
  During the follow-up with an average time of 5.6 ± 4.7 years 15 (27.3%) children died. The one-, three-, five-, and ten-year survival was, respectively, 83.1%, 77.1%, 70.7%, and 65.2%. The analysis of the survival curves revealed a better prognosis in patients with baseline N-terminal pro-B-type natriuretic peptide (NT-proBNP) level < 605 pg/mL (p = 0.024) and a higher probability of survival of three and five years in children at baseline I/II World Health Organisation functional class (WHO-FC). The higher risk of death was associated with a higher pressure in the right atrium (HR 1.23, p < 0.01) and higher pulmonary resistance (HR 1.1, p < 0.01), whereas no history of syncope had a better prognosis (HR 0.31, p = 0.03).
  
  
  CONCLUSIONS
  Survival in the study group was comparable to the currently published register data. Mortality risk factors were connected with the severity of the disease at diagnosis.
authors.affiliation: Department of Cardiology, The Children's Memorial Health Institute, Warsaw, Poland. m.zuk@czd.pl.
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Żuk
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Mazurkiewicz-Antoń
authors.forename: Anna
authors.initials: A
authors.lastname: Migdał
authors.forename: Dorota
authors.initials: D
authors.lastname: Jagiełłowicz-Kowalska
authors.forename: Anna
authors.initials: A
authors.lastname: Turska-Kmieć
authors.forename: Lidia
authors.initials: L
authors.lastname: Ziółkowska
authors.forename: Grażyna
authors.initials: G
authors.lastname: Brzezińska-Rajszys
authors.forename: Maria
authors.initials: M
authors.lastname: Zubrzycka
authors.forename: Wanda
authors.initials: W
authors.lastname: Kawalec
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D007223:Infant; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D011379:Prognosis; D012307:Risk Factors
chemical_list: D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain