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

Susanne J Maurer,Oktay Tutarel,Jürgen Hörer,Claudia Pujol,Peter Ewert,Katharina Stöckemann

doi:10.3390/jcm9124071

Abstract

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.

Highlights

The life expectancy for those born with congenital heart disease (CHD) has greatly improved over the last few decades [1]
The requirement for informed consent was waived by the ethics committee due to the retrospective nature of the study. This retrospective, single-center study included all patients with a diagnosis of PAH-adults with congenital heart disease (ACHD) under follow-up at the German Heart Centre Munich who were ≥40 years of age at any point between January 2005 and December 2018
The underlying CHD was classified as follows: (1) Shunt lesions—atrial septal defects (ASD), ventricular septal defects (VSD), atrioventricular septal defects (AVSD), persistent ductus arteriosus, aortopulmonary window; (2) Complex—PAH associated with complex CHD, e.g., transposition of the great arteries after atrial switch surgery with remaining VSD or univentricular hearts; (3) Segmental—PAH due to major aorto-pulmonary collateral arteries

Summary

Introduction

The life expectancy for those born with congenital heart disease (CHD) has greatly improved over the last few decades [1]. The population of adults with congenital heart disease (ACHD) is growing and patients are increasingly reaching older ages [2]. This is true for patients with simple defects, and for those with complex defects [3]. ACHD patients with pulmonary arterial hypertension (PAH-ACHD) are at the extreme end of this spectrum with one of the highest mortality rates and significant morbidity and mortality [4–6] Even in these patients a shift towards mortality at an older age, as well as a median survival of 52 years, has been reported [5]. Pulmonary arterial hypertension associated with adult congenital heart disease (PAH-ACHD) leads to significant mortality at a young age. Conclusions: PAH-ACHD patients over the age of 40 years are burdened with significant mortality, of which NT-proBNP and creatinine are independent predictors

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