Mark J Schuuring1, Annelieke C M J van Riel1, Jeroen C Vis2, Marielle G Duffels2, Arie P J van Dijk3, Rianne H A C M de Bruin-Bon2, Aeilko H Zwinderman4, Barbara J M Mulder5, Berto J Bouma2. 1. Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, The Netherlands. 2. Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands. 3. Department of Cardiology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands. 4. Department of Clinical Epidemiology and Biostatistics, Academic Medical Center, Amsterdam, The Netherlands. 5. 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.
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. CONCLUSION: 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.
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. CONCLUSION: 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: Alexander Van De Bruaene; Lukas Meier; Walter Droogne; Pieter De Meester; Els Troost; Marc Gewillig; Werner Budts Journal: Heart Fail Rev Date: 2018-01 Impact factor: 4.214
Authors: Annelieke C M J van Riel; Alexander R Opotowsky; Mário Santos; Jose M Rivero; Andy Dhimitri; Barbara J M Mulder; Berto J Bouma; Michael J Landzberg; Aaron B Waxman; David M Systrom; Amil M Shah Journal: Circ Cardiovasc Imaging Date: 2017-04 Impact factor: 7.792
Authors: M J Schuuring; A P Backx; R Zwart; A H Veelenturf; D Robbers-Visser; M Groenink; A Abu-Hanna; N Bruining; M P Schijven; B J Mulder; B J Bouma Journal: Neth Heart J Date: 2016-11 Impact factor: 2.380
Authors: V J M Baggen; M M P Driessen; M C Post; A P van Dijk; J W Roos-Hesselink; A E van den Bosch; J J M Takkenberg; G T Sieswerda Journal: Neth Heart J Date: 2016-06 Impact factor: 2.380