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Heart. 2019 Mar;105(6):465-469. doi: 10.1136/heartjnl-2018-313613. Epub 2018 Sep 29.

Hospitalisations for heart failure predict mortality in pulmonary hypertension related to congenital heart disease.

Author information

1
Department of Cardiology, AHEPA University Hospital, Thessaloniki, Greece.
2
Department of Pediatric Cardiology and ACHD, Onassis Cardiac Surgery Center, Athens, Greece.
3
Department of Cardiology, General Hospital 'George Papanikolaou', Thessaloniki, Greece.
4
Second Department of Cardiology, Attikon University Hospital, Athens, Greece.
5
Department of Congenital Heart Disease, Mitera Children's Hospital, Athens, Greece.
6
Department of Cardiology, Tzaneio General Hospital of Piraeus, Athens, Greece.
7
Department of Cardiology, Ippokrateion University Hospital, Athens, Greece.
8
Department of Cardiology, Mediterraneo Hospital, Athens, Greece.
9
Department of Cardiothoracic Surgery, St Luke's Hospital, Thessaloniki, Greece.
10
Department of Cardiology, University Hospital of Ioannina, Ioannina, Greece.

Abstract

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

PMID:
30269081
DOI:
10.1136/heartjnl-2018-313613
[Indexed for MEDLINE]

Conflict of interest statement

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.

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