RT Journal Article
SR Electronic
T1 Hospitalisations for heart failure predict mortality in pulmonary hypertension related to congenital heart disease
JF Heart
JO Heart
FD BMJ Publishing Group Ltd and British Cardiovascular Society
SP 465
OP 469
DO 10.1136/heartjnl-2018-313613
VO 105
IS 6
A1 Despoina Ntiloudi
A1 Sotiria Apostolopoulou
A1 Konstantinos Vasiliadis
A1 Alexandra Frogoudaki
A1 Aphrodite Tzifa
A1 Christos Ntellos
A1 Styliani Brili
A1 Athanasios Manginas
A1 Antonios Pitsis
A1 Marios Kolios
A1 Haralambos Karvounis
A1 Costas Tsioufis
A1 John Goudevenos
A1 Spyridon Rammos
A1 George Giannakoulas
A1 ,
YR 2019
UL http://heart.bmj.com/content/105/6/465.abstract
AB 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.