PMID- 30269081 OWN - NLM STAT- MEDLINE DCOM- 20190502 LR - 20190502 IS - 1468-201X (Electronic) IS - 1355-6037 (Linking) VI - 105 IP - 6 DP - 2019 Mar TI - Hospitalisations for heart failure predict mortality in pulmonary hypertension related to congenital heart disease. PG - 465-469 LID - 10.1136/heartjnl-2018-313613 [doi] 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. CI - (c) Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Ntiloudi, Despoina AU - Ntiloudi D AD - Department of Cardiology, AHEPA University Hospital, Thessaloniki, Greece. FAU - Apostolopoulou, Sotiria AU - Apostolopoulou S AD - Department of Pediatric Cardiology and ACHD, Onassis Cardiac Surgery Center, Athens, Greece. FAU - Vasiliadis, Konstantinos AU - Vasiliadis K AD - Department of Cardiology, General Hospital 'George Papanikolaou', Thessaloniki, Greece. FAU - Frogoudaki, Alexandra AU - Frogoudaki A AD - Second Department of Cardiology, Attikon University Hospital, Athens, Greece. FAU - Tzifa, Aphrodite AU - Tzifa A AD - Department of Congenital Heart Disease, Mitera Children's Hospital, Athens, Greece. FAU - Ntellos, Christos AU - Ntellos C AD - Department of Cardiology, Tzaneio General Hospital of Piraeus, Athens, Greece. FAU - Brili, Styliani AU - Brili S AD - Department of Cardiology, Ippokrateion University Hospital, Athens, Greece. FAU - Manginas, Athanasios AU - Manginas A AD - Department of Cardiology, Mediterraneo Hospital, Athens, Greece. FAU - Pitsis, Antonios AU - Pitsis A AD - Department of Cardiothoracic Surgery, St Luke's Hospital, Thessaloniki, Greece. FAU - Kolios, Marios AU - Kolios M AD - Department of Cardiology, University Hospital of Ioannina, Ioannina, Greece. FAU - Karvounis, Haralambos AU - Karvounis H AD - Department of Cardiology, AHEPA University Hospital, Thessaloniki, Greece. FAU - Tsioufis, Costas AU - Tsioufis C AD - Department of Cardiology, Ippokrateion University Hospital, Athens, Greece. FAU - Goudevenos, John AU - Goudevenos J AD - Department of Cardiology, University Hospital of Ioannina, Ioannina, Greece. FAU - Rammos, Spyridon AU - Rammos S AD - Department of Pediatric Cardiology and ACHD, Onassis Cardiac Surgery Center, Athens, Greece. FAU - Giannakoulas, George AU - Giannakoulas G AD - Department of Cardiology, AHEPA University Hospital, Thessaloniki, Greece. CN - CHALLENGE investigators LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20180929 PL - England TA - Heart JT - Heart (British Cardiac Society) JID - 9602087 RN - 0 (Cardiovascular Agents) SB - IM MH - Adult MH - *Arrhythmias, Cardiac/epidemiology/etiology/therapy MH - Cardiovascular Agents/*therapeutic use MH - Female MH - Follow-Up Studies MH - Greece/epidemiology MH - *Heart Defects, Congenital/complications/mortality MH - *Heart Failure/epidemiology/etiology/therapy MH - Hospitalization/*statistics & numerical data MH - Humans MH - *Hypertension, Pulmonary/diagnosis/etiology/mortality/therapy MH - Male MH - Middle Aged MH - Mortality MH - Prognosis MH - Registries/statistics & numerical data MH - Risk Assessment MH - *Stroke/epidemiology/etiology/therapy OTO - NOTNLM OT - congenital heart disease OT - heart failure OT - secondary pulmonary hypertension COIS- Competing interests: GG has acted as a consultant and/or received unrestricted educational or research grants from Actelion, Bayer, MSD, GlaxoSmithKline, Lilly, Pfizer and United Therapeutics. AF received travel grants from Actelion and GlaxoSmithKline. EDAT- 2018/10/01 06:00 MHDA- 2019/05/03 06:00 CRDT- 2018/10/01 06:00 PHST- 2018/05/18 00:00 [received] PHST- 2018/08/28 00:00 [revised] PHST- 2018/08/30 00:00 [accepted] PHST- 2018/10/01 06:00 [pubmed] PHST- 2019/05/03 06:00 [medline] PHST- 2018/10/01 06:00 [entrez] AID - heartjnl-2018-313613 [pii] AID - 10.1136/heartjnl-2018-313613 [doi] PST - ppublish SO - Heart. 2019 Mar;105(6):465-469. doi: 10.1136/heartjnl-2018-313613. Epub 2018 Sep 29.