NT-proBNP Is a Predictor of Mortality in Adults with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease
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 ra...
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MDPI AG
2023-04-01
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Online Access: | https://www.mdpi.com/2077-0383/12/9/3101 |
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author | Susanne J. Maurer Veronika Habdank Jürgen Hörer Peter Ewert Oktay Tutarel |
author_facet | Susanne J. Maurer Veronika Habdank Jürgen Hörer Peter Ewert Oktay Tutarel |
author_sort | Susanne J. Maurer |
collection | DOAJ |
description | 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), <i>p</i> = 0.037), NT-proBNP (log) (HR: 7.10, 95% CI: 1.57–32.23, <i>p</i> = 0.011), and uric acid (HR: 1.37, 95% CI: 1.05–1.79, <i>p</i> = 0.020) were predictors of the primary endpoint. On multivariate analysis, only NT-proBNP (log) (HR: 6.91, 95% CI: 1.36–35.02, <i>p</i> = 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. |
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language | English |
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spelling | doaj.art-7356e80237c44927a1e9ad18901f72052023-11-17T23:10:44ZengMDPI AGJournal of Clinical Medicine2077-03832023-04-01129310110.3390/jcm12093101NT-proBNP Is a Predictor of Mortality in Adults with Pulmonary Arterial Hypertension Associated with Congenital Heart DiseaseSusanne J. Maurer0Veronika Habdank1Jürgen Hörer2Peter Ewert3Oktay Tutarel4Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, TUM School of Medicine, Technical University of Munich, 80636 Munich, GermanyDepartment of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, TUM School of Medicine, Technical University of Munich, 80636 Munich, GermanyDepartment of Congenital and Paediatric Heart Surgery, German Heart Centre Munich, Technical University of Munich, 80636 Munich, GermanyDepartment of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, TUM School of Medicine, Technical University of Munich, 80636 Munich, GermanyDepartment of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, TUM School of Medicine, Technical University of Munich, 80636 Munich, GermanyBackground: 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), <i>p</i> = 0.037), NT-proBNP (log) (HR: 7.10, 95% CI: 1.57–32.23, <i>p</i> = 0.011), and uric acid (HR: 1.37, 95% CI: 1.05–1.79, <i>p</i> = 0.020) were predictors of the primary endpoint. On multivariate analysis, only NT-proBNP (log) (HR: 6.91, 95% CI: 1.36–35.02, <i>p</i> = 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.https://www.mdpi.com/2077-0383/12/9/3101adult congenital heart diseasepulmonary hypertensionoutcome |
spellingShingle | Susanne J. Maurer Veronika Habdank Jürgen Hörer Peter Ewert Oktay Tutarel NT-proBNP Is a Predictor of Mortality in Adults with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease Journal of Clinical Medicine adult congenital heart disease pulmonary hypertension outcome |
title | NT-proBNP Is a Predictor of Mortality in Adults with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease |
title_full | NT-proBNP Is a Predictor of Mortality in Adults with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease |
title_fullStr | NT-proBNP Is a Predictor of Mortality in Adults with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease |
title_full_unstemmed | NT-proBNP Is a Predictor of Mortality in Adults with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease |
title_short | NT-proBNP Is a Predictor of Mortality in Adults with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease |
title_sort | nt probnp is a predictor of mortality in adults with pulmonary arterial hypertension associated with congenital heart disease |
topic | adult congenital heart disease pulmonary hypertension outcome |
url | https://www.mdpi.com/2077-0383/12/9/3101 |
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