Susanne J. Maurer, Veronika Habdank, Jürgen Hörer, Peter Ewert, Oktay Tutarel
German Heart Centre Munich and Technical University of Munich. Ludwig-Maximilians Universität.
Germany
Journal of Clinical Medicine
J Clin Med 2023; 12
DOI: 10.3390/jcm12093101
Abstract
Background: About 5-10% of adults with congenital heart disease (ACHD) will develop pulmonary arterial hypertension (PAH), which is associated with significant mortality. Studies on risk factors for poor outcome in a contemporary cohort of these patients with PAH associated with CHD (PAH-CHD) are rare.
Methods: In this retrospective, single-center study, adult patients with the diagnosis PAH-CHD who had at least one contact as an outpatient or inpatient at the German Heart Centre Munich during the period January 2010-September 2019 were included. Patients with PAH without a CHD were excluded. The primary endpoint was all-cause mortality.
Results: Altogether, 158 patients (mean age 39.9 ± 15.4 years, female 64.6%) were included in the study. A pre-tricuspid shunt was present in 17.7%, other shunts in 51.3%, PAH associated with complex CHD in 22.8%, and segmental PAH in 8.2%. An NT-proBNP measurement at baseline was available in 95 patients (60.1%). During a median follow-up of 5.37 years [IQR 1.76-8.63], the primary endpoint occurred in 10 patients (6.7%). On univariate analysis, CRP (log) (HR 3.35, 95% CI (1.07-10.48), p = 0.037), NT-proBNP (log) (HR: 7.10, 95% CI: 1.57-32.23, p = 0.011), and uric acid (HR: 1.37, 95% CI: 1.05-1.79, p = 0.020) were predictors of the primary endpoint. On multivariate analysis, only NT-proBNP (log) (HR: 6.91, 95% CI: 1.36-35.02, p = 0.0196) remained as an independent predictor.
Conclusion: NT-proBNP is an independent predictor of all-cause mortality in a contemporary cohort of PAH-CHD patients. The role of CRP and uric acid should be further assessed in future studies.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Diagnostic Testing for Pulmonary Vascular Disease. Risk Stratification
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
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