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.
- congenital heart disease
- secondary pulmonary hypertension
- heart failure
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Contributors All authors have participated in the work. All enrolled the patients from the different ACHD centres and collected the required data. DN reviewed the literature, organised and wrote the various sections of the paper along with the author GG. All authors have reviewed critically and edited the final version.
Funding This work was supported by Hellenic Cardiological Society.
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.
Patient consent Not required.
Ethics approval Scientific committees and administrative councils of the participating hospitals (protocol number 2464/19.1.2012).
Provenance and peer review Not commissioned; externally peer reviewed.
Collaborators Maria Papaphylactou, Tereza Mousiama, Christos Tourmousoglou, Despoina Parcharidou, Nikolaos Kampouridis, Aikaterini Chamaidi, Georgios Papadopoulos, Alexandros Douras, Periklis Davlouros, Sophia Anastasia Mouratoglou, George Krasopoulos, John Kanakakis.
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