Δημοσίευση στις Mar 2019 στο PubMed: https://pubmed.ncbi.nlm.nih.gov/30269081/?from_term=tsioufis&from_sort=date&from_size=200&from_pos=73

 

Despoina Ntiloudi 

, Sotiria Apostolopoulou 

, Konstantinos Vasiliadis 

, Alexandra Frogoudaki 

, Aphrodite Tzifa 

, Christos Ntellos 

, Styliani Brili 

, Athanasios Manginas 

, Antonios Pitsis 

, Marios Kolios 
0
, Haralambos Karvounis 

, Costas Tsioufis 

, John Goudevenos 
0
, Spyridon Rammos 

, George Giannakoulas 

, CHALLENGE investigators


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.


Keywords:

congenital heart disease; heart failure; secondary pulmonary hypertension.

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