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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. 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.

journal_name

Int J Cardiol

journal_title

International journal of cardiology

authors

Schuuring MJ,van Riel AC,Vis JC,Duffels MG,van Dijk AP,de Bruin-Bon RH,Zwinderman AH,Mulder BJ,Bouma BJ

doi

10.1016/j.ijcard.2014.11.222

subject

Has Abstract

pub_date

2015-02-15 00:00:00

pages

270-6

eissn

0167-5273

issn

1874-1754

pii

S0167-5273(14)02447-4

journal_volume

181

pub_type

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New predictors of mortality in adults with congenital heart disease and pulmonary hypertension: Midterm outcome of a prospective study.