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Background/Introduction: Despite the progress in the management of congenital heart disease (CHD), pulmonary arterial hypertension (PAH) still affects a significant proportion of adult patients with CHD (ACHD).

Purpose: We aimed to highlight the rate of the long-term complications in ACHD and the predicting factors of cumulative mortality risk in this population.

Methods: Data from February 2012 until November 2016 were obtained from the national registry of ACHD in Greece (CHALLENGE).

Results: A total of 2086 patients were included in CHALLENGE registry of which 52 (2.5%) suffered from PAH-CHD (mean age 41.8±14.2 years, 61.5% females). Eisenmenger syndrome was diagnosed in 78.8%, left to right shunt in 9.6%, small defect in 1.9% and PAH after correction in 9.6%. Of patients, 55.8% were heavily symptomatic (NYHA III/IV). Prior to administration of advanced therapy for PAH, right heart catheterization was performed in 69.2% of patients. The majority received monotherapy, mainly endothelin receptor antagonists (75.8%), while sequential combination therapy was administered in 36.5% of patients. Prostanoids were prescribed in 5.8%. In terms of history of complications at baseline, arrhythmia was reported in 40.4%, endocarditis in 1.9%, stroke in 5.8%, pulmonary thrombosis in 7.7% and hospitalization due to heart failure (HF) in 21.2%. Over a median follow-up of 3.5 years (IQR 2.0–5.0), 11 (21.2%) patients died. Univariable predictors of cumulative mortality risk were history of hospitalization for HF (HR 5.04, p=0.008) and NYHA class III/IV (HR 8.57, p=0.04).

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Abstracts > Saturday 26 August 2017
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