TY - JOUR T1 - Hospitalisations for heart failure predict mortality in pulmonary hypertension related to congenital heart disease JF - Heart JO - Heart SP - 465 LP - 469 DO - 10.1136/heartjnl-2018-313613 VL - 105 IS - 6 AU - Despoina Ntiloudi AU - Sotiria Apostolopoulou AU - Konstantinos Vasiliadis AU - Alexandra Frogoudaki AU - Aphrodite Tzifa AU - Christos Ntellos AU - Styliani Brili AU - Athanasios Manginas AU - Antonios Pitsis AU - Marios Kolios AU - Haralambos Karvounis AU - Costas Tsioufis AU - John Goudevenos AU - Spyridon Rammos AU - George Giannakoulas A2 - , Y1 - 2019/03/01 UR - http://heart.bmj.com/content/105/6/465.abstract N2 - Objective Despite the progress in the management of patients with adult congenital heart disease (ACHD), a significant proportion of patients still develop pulmonary hypertension (PH). We aimed to highlight the rate of the complications in PH-ACHD and the predicting factors of cumulative mortality risk in this population.Methods Data were obtained from the cohort of the national registry of ACHD in Greece from February 2012 until January 2018.Results Overall, 65 patients receiving PH-specific therapy were included (mean age 46.1±14.4 years, 64.6% females). Heavily symptomatic (New York Heart Association (NYHA) class III/IV) were 53.8% of patients. The majority received monotherapy, while combination therapy was administered in 41.5% of patients. Cardiac arrhythmia was reported in 30.8%, endocarditis in 1.5%, stroke in 4.6%, pulmonary arterial thrombosis in 6.2%, haemoptysis in 3.1% and hospitalisation due to heart failure (HF) in 23.1%. Over a median follow-up of 3 years (range 1–6), 12 (18.5%) patients died. On univariate Cox regression analysis history of HF hospitalisation emerged as a strong predictor of mortality (HR 8.91, 95% CI 2.64 to 30.02, p<0.001), which remained significant after adjustment for age and for NYHA functional class.Conclusions Long-term complications are common among patients with PH-ACHD. Hospitalisations for HF predict mortality and should be considered in the risk stratification of this population. ER -