title: New predictors of mortality in adults with congenital heart disease and pulmonary hypertension: Midterm outcome of a prospective study.
abstract: BACKGROUND
  Patients with CHD-PAH have a limited prognosis. In daily practice, combination therapy is often initiated after a clinical event. Although clinical events have been associated with a poor prognosis in idiopathic PAH, data on this association are limited in CHD-PAH. The aim of this study was to determine whether baseline characteristics and clinical events associate with mortality in patients with pulmonary hypertension (PAH) due to congenital heart disease (CHD).
  
  
  METHODS
  In total 91 consecutive adults (42 ± 14 year) with CHD-PAH were referred for therapy between January 2005 and June 2013. Cox proportional hazard analysis was performed to identify determinants of mortality, including clinical events as time dependent covariates.
  
  
  RESULTS
  Twenty-four patients (nine with Down) died during the median follow-up of 4.7 (range 0.1-7.9) years. The one and eight year mortality rates were 7.3% and 37.3%, respectively. Clinical events included admission for heart failure (n=9), arrhythmias (n=9), haemoptysis (n=5), change to a worse NYHA class (n=16), vascular events (n=1), syncope (n=1) and need for red blood cell depletion (n=4). In univariate analysis, both baseline characteristics and clinical events were associated with mortality. In multivariate analysis, only baseline NT-pro-BNP serum level ≥ 500 ng/L and TAPSE<15mm at echocardiography were significant determinants of mortality. None of the clinical events remained significant. Patients with both a NT-pro-BNP serum level ≥ 500 ng/L and TAPSE<15mm at echocardiography have a nine fold higher mortality rate than patients without both risk factors.
  
  
  CONCLUSIONS
  Prognosis is still poor in contemporary patients with CHD-PAH. Both baseline NT-pro-BNP serum level and right ventricular function are superior to clinical events in prognostication. These two baseline characteristics should have a major impact on therapeutic management in patients with CHD-PAH, such as initiation of combination therapy.
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, The Netherlands.
authors.forename: Mark J
authors.initials: MJ
authors.lastname: Schuuring
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, The Netherlands.
authors.forename: Annelieke C M J
authors.initials: AC
authors.lastname: van Riel
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: Jeroen C
authors.initials: JC
authors.lastname: Vis
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: Marielle G
authors.initials: MG
authors.lastname: Duffels
authors.affiliation: Department of Cardiology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands.
authors.forename: Arie P J
authors.initials: AP
authors.lastname: van Dijk
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: Rianne H A C M
authors.initials: RH
authors.lastname: de Bruin-Bon
authors.affiliation: Department of Clinical Epidemiology and Biostatistics, Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: Aeilko H
authors.initials: AH
authors.lastname: Zwinderman
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, The Netherlands. Electronic address: b.j.mulder@amc.uva.nl.
authors.forename: Barbara J M
authors.initials: BJ
authors.lastname: Mulder
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: Berto J
authors.initials: BJ
authors.lastname: Bouma
mesh_terms: D000328:Adult; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000401:mortality* / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000401:mortality* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009026:Mortality / Q000639:trends; D011237:Predictive Value of Tests; D011446:Prospective Studies; D012307:Risk Factors; D016896:Treatment Outcome
keywords: Congenital heart disease; Mortality; Predictors; Pulmonary hypertension