NT-proBNP Is a Predictor of Mortality in Adults with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

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.

Association of preoperative NT-proBNP with postoperative inflammatory factors levels among children of unrestricted left-to-right shunts congenital heart disease

“…PAH is an essential factor for morbidity and mortality during the perioperative period of cardiac surgery, especially in pediatric CHD for the closure of a large left-to-right shunt and use of cardiopulmonary bypass (CPB) that causes an acute increase in pulmonary artery pressure leading to ventricular dysfunction [4][5][6]. Susanne et al detected that the N-terminal pro-B-type natriuretic peptide (NT-proBNP) independently predicts all-cause mortality in a contemporary cohort of PAH-CHD patients [7].…”

Diagnosis of Pulmonary Arterial Hypertension in Adult Congenital Heart Disease

“… 1 The main reason for this finding is that a cure of the CHD is seldom achieved, while in the longer term, complications and sequela like heart failure or arrhythmias that require therapeutical interventions and are major causes of morbidity and mortality are common. 2 , 3 Pulmonary arterial hypertension (PAH) is one of the most impactful complications regarding morbidity and mortality in this patient group. While its association with uncorrected shunt defects like large ventricular septal defects has been well described and known for decades, its occurrence late in adulthood even after the successful closure of so-called simple shunt defects in childhood has recently become a focus of attention.…”