{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T03:30:00Z","timestamp":1760239800037,"version":"build-2065373602"},"reference-count":16,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2020,12,17]],"date-time":"2020-12-17T00:00:00Z","timestamp":1608163200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Pulmonary arterial hypertension associated with adult congenital heart disease (PAH-ACHD) leads to significant mortality at a young age. Risk factors for a negative outcome in older adults are lacking. Methods: PAH-ACHD patients \u2265 40 years of age under active follow-up between January 2005 and December 2018 were included. Demographic data, as well as medical\/surgical history, were retrieved from hospital records. The primary end-point was all-cause mortality. Results: In total, 65 patients (67.7% female, mean age 45.19 \u00b1 6.75 years) were included. Out of these, 46 (70.8%) had a shunt lesion, 12 (18.5%) had PAH associated with complex congenital heart defects, and 7 (10.8%) had segmental pulmonary hypertension due to major aorto-pulmonary collaterals. Down syndrome was present in 13 patients (20.0%). During a median follow-up of 4.2 years (IQR 1.2\u20137.5), 16 patients (24.6%) died. On univariate analysis, NT-proBNP (log), creatinine, and a previous history of ventricular arrhythmias were predictors of all-cause mortality. Upon multivariate analysis, NT-proBNP (log) (HR: 4.1, 95% CI: 1.2\u201314.4, p = 0.029) and creatinine (HR: 16.3, 95% CI: 2.2\u2013118.7, p = 0.006) remained as independent predictors of all-cause mortality. Conclusions: PAH-ACHD patients over the age of 40 years are burdened with significant mortality, of which NT-proBNP and creatinine are independent predictors.<\/jats:p>","DOI":"10.3390\/jcm9124071","type":"journal-article","created":{"date-parts":[[2020,12,17]],"date-time":"2020-12-17T21:21:49Z","timestamp":1608240109000},"page":"4071","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Pulmonary Arterial Hypertension Associated with Congenital Heart Disease in Adults over the Age of 40 Years"],"prefix":"10.3390","volume":"9","author":[{"given":"Susanne J.","family":"Maurer","sequence":"first","affiliation":[{"name":"Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany"},{"name":"Department of Electrophysiology, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katharina","family":"St\u00f6ckemann","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Pujol","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2716-1424","authenticated-orcid":false,"given":"J\u00fcrgen","family":"H\u00f6rer","sequence":"additional","affiliation":[{"name":"Department of Congenital and Paediatric Heart Surgery, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany"},{"name":"Division of Congenital and Paediatric Heart Surgery, University Hospital Munich, Ludwig-Maximilians Universit\u00e4t, 80337 Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Ewert","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany"},{"name":"DZHK (German Centre for Cardiovascular Research), Partner Site Munich Heart Alliance, 80992 Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oktay","family":"Tutarel","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany"},{"name":"DZHK (German Centre for Cardiovascular Research), Partner Site Munich Heart Alliance, 80992 Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,12,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e348","DOI":"10.1161\/CIR.0000000000000535","article-title":"Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement from the American Heart Association","volume":"136","author":"Lui","year":"2017","journal-title":"Circulation"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Hock, J., Schwall, L., Pujol, C., Hager, A., Oberhoffer, R., Ewert, P., and Tutarel, O. 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