Pulmonary Arterial Hypertension Associated with Congenital Heart Disease in Adults over the Age of 40 Years

Journal of Clinical Medicine

卷:9

SusanneJ. Maurer¹  Claudia Pujol¹  Katharina Stöckemann¹  Oktay Tutarel¹  Peter Ewert¹  Jürgen Hörer² 

[1] Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany;

[2] Department of Congenital and Paediatric Heart Surgery, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany;

关键词: adult congenital heart disease;    pulmonary hypertension;    outcome;   

DOI  :  10.3390/jcm9124071

来源: DOAJ

【 摘 要 】

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 ≥ 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 ± 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 1.2–7.5), 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: 1.2–14.4, p = 0.029) and creatinine (HR: 16.3, 95% CI: 2.2–118.7, 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.

【 授权许可】

Unknown