Extract
Pulmonary arterial hypertension (PAH) develops in 5–10% of patients with congenital heart disease (CHD) [1], and PAH associated with congenital heart disease (PAH-CHD) accounts for a significant proportion of PAH cases (34–42%) [2]. Eisenmenger syndrome is at the extreme end of the spectrum of PAH-CHD, with an untreated 10-year mortality rate of 30–40% [3]. Despite decreasing incidence in developed countries [4], Eisenmenger syndrome is likely to remain a common complication of CHD in low- to middle-income countries [5].
Abstract
In this European cohort of Eisenmenger patients, prostanoid therapy, as part of triple combination therapy, was found to be safe and effective in improving exercise capacity, natriuretic peptide levels and haemodynamic parameters over a 2-year period http://bit.ly/2Zl3W1Z
Footnotes
Conflict of interest: M. D'Alto reports grants and personal fees from Actelion, Pfizer, GlaxoSmithKline, Dompè and Bayer/MSD, outside the submitted work.
Conflict of interest: A. Constantine reports grants from Actelion, outside the submitted work.
Conflict of interest: O.H. Balint reports personal fees from Actelion, Lilly, AOP Orphan and Bayer/MSD, outside the submitted work.
Conflict of interest: E. Romeo reports grants and personal fees from GlaxoSmithKline, Dompè and MSD, outside the submitted work.
Conflict of interest: P. Argiento reports grants and personal fees from GlaxoSmithKline, Dompè and MSD, outside the submitted work.
Conflict of interest: L. Ablonczy has nothing to disclose.
Conflict of interest: N. Skoro-Sajer reports grants and personal fees from AOPOrphan Pharmaceuticals, Actelion, Bayer, GlaxoSmithKline, Pfizer and United Therapeutics, outside the submitted work.
Conflict of interest: G. Giannakoulas reports grants and personal fees from Actelion, United Therapeutics, Pfizer, GlaxoSmithKline, Bayer/MSD and Lilly, outside the submitted work.
Conflict of interest: K. Dimopoulos reports grants, personal fees and non-financial support from Actelion, personal fees from Pfizer, GlaxoSmithKline and Bayer/MSD, outside the submitted work.
- Received July 2, 2019.
- Accepted July 26, 2019.
- Copyright ©ERS 2019
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