title: A binational registry of adults with pulmonary arterial hypertension complicating congenital heart disease.
abstract: BACKGROUND
  The management of children with congenital heart disease (CHD) has improved over recent decades and several patients surviving with CHD into adulthood are increasing. In developed countries, there are now as many adults as there are children living with CHD. Pulmonary arterial hypertension (PAH) occurs in ∼ 5% of patients with CHD.
  
  
  OBJECTIVE
  We aimed to understand the characteristics and outcomes of this emerging population.
  
  
  METHODS
  We collected data retrospectively and prospectively from 12 contributing centres across Australia and New Zealand (2010-2013). Patients were included if they had been diagnosed with PAH and CHD and had been seen once in an adult centre after 1 January 2000.
  
  
  RESULTS
  Of 360 patients with CHD-PAH, 60% were female and 90% were New York Heart Association functional class II or III at the time of adult diagnosis of PAH. Mean age at diagnosis of PAH in adulthood was 31.2 ± 14 years, and on average, patients were diagnosed with PAH 6 years after symptom onset. All-cause mortality was 12% at 5 years, 21% at 10 years and 31% at 15 years. One hundred and six patients (30%) experienced 247 hospitalisations during 2936 patient years of follow up. Eighty-nine per cent of patients were prescribed PAH specific therapy (mean exposure of 4.0 years).
  
  
  CONCLUSIONS
  Adults with PAH and CHD often have this diagnosis made after significant delay, and have substantial medium-term morbidity and mortality. This suggests a need for children transitioning to adult care with CHD to be closely monitored for this complication.
authors.affiliation: Department of Medicine, University of Notre Dame, Perth, Western Australia, Australia.|Pulmonary Hypertensions Society ANZ Inc., Sydney, New South Wales, Australia.|Murdoch Children's Research Institute, Melbourne, Victoria, Australia.
authors.forename: G
authors.initials: G
authors.lastname: Strange
authors.affiliation: Department of Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia.
authors.forename: M
authors.initials: M
authors.lastname: Rose
authors.affiliation: Department of Respiratory Medicine, The Prince Charles Hospital, Brisbane, Queensland, Australia.
authors.forename: F
authors.initials: F
authors.lastname: Kermeen
authors.affiliation: Department of Cardiology, Auckland City Hospital, Auckland, New Zealand.
authors.forename: C
authors.initials: C
authors.lastname: O'Donnell
authors.affiliation: Department of Cardiology, St Vincent's Hospital, Sydney, New South Wales, Australia.
authors.forename: A
authors.initials: A
authors.lastname: Keogh
authors.affiliation: Department of Cardiology, St Vincent's Hospital, Sydney, New South Wales, Australia.
authors.forename: E
authors.initials: E
authors.lastname: Kotlyar
authors.affiliation: Department of Cardiology, Royal Melbourne Hospital, Melbourne, Victoria, Australia.
authors.forename: L
authors.initials: L
authors.lastname: Grigg
authors.affiliation: Department of Paediatric Cardiology, Royal Perth Hospital, Perth, Western Australia, Australia.
authors.forename: A
authors.initials: A
authors.lastname: Bullock
authors.affiliation: The Royal Adelaide Hospital, Adelaide, South Australia, Australia.
authors.forename: P
authors.initials: P
authors.lastname: Disney
authors.affiliation: Department of Cardiology, Royal Hobart Hospital, Hobart, Tasmania, Australia.
authors.forename: N
authors.initials: N
authors.lastname: Dwyer
authors.affiliation: Department of Respiratory Medicine, The Alfred Hospital, Melbourne, Victoria, Australia.
authors.forename: H
authors.initials: H
authors.lastname: Whitford
authors.affiliation: Department of Cardiology, Westmead Hospital, Sydney, New South Wales, Australia.
authors.forename: D
authors.initials: D
authors.lastname: Tanous
authors.affiliation: Department of Cardiology, University of Otago, Christchurch, New Zealand.
authors.forename: C
authors.initials: C
authors.lastname: Frampton
authors.affiliation: Murdoch Children's Research Institute, Melbourne, Victoria, Australia.|Department of Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia.
authors.forename: R
authors.initials: R
authors.lastname: Weintraub
authors.affiliation: Sydney Medical School, Sydney, New South Wales, Australia.
authors.forename: D S
authors.initials: DS
authors.lastname: Celermajer
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D001315:Australia / Q000453:epidemiology; D003131:Combined Modality Therapy; D004232:Diuretics; D065128:Endothelin Receptor Antagonists / Q000008:administration & dosage*; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000150:complications / Q000453:epidemiology* / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D009520:New Zealand / Q000453:epidemiology; D011788:Quality of Life; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors
chemical_list: D000959:Antihypertensive Agents; D004232:Diuretics; D065128:Endothelin Receptor Antagonists
keywords: PAH; congenital heart disease; pulmonary arterial hypertension