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Over the past two decades, we have witnessed significant improvements in the therapy of pulmonary arterial hypertension (PAH), in general, but also specifically in PAH associated with congenital heart disease (CHD-PAH). This progress has been largely driven by the availability of new therapeutic agents that have been gradually introduced into clinical practice and have been demonstrated to improve exercise capacity, quality of life and possibly survival in selected patients subgroups.1 ,2 Unlike in patients with idiopathic PAH, recognised to have a median life expectancy <3 years if left untreated,3 many physicians have not felt the urgency of treating patients with CHD-PAH equally aggressively. This is related to the notion that survival prospects may be superior in this patient population. Unfortunately, this belief is not well supported by scientific data.4 Especially patients with CHD-PAH with small haemodynamically irrelevant defects and those presenting with severe PAH after shunt closure have a very limited prognosis.5 However, even the dogma of the relative longevity of patients with Eisenmenger syndrome has been recently challenged.4 As a consequence, there is a trend towards earlier and a more proactive treatment approach in this population.
The current study by Rosenkranz et al6 explores the efficacy and safety of riociguat in patients with CHD-PAH. To this end, the authors performed a subanalysis of patients with persistent or recurrent PAH after defect closure in patients with CHD included in the Pulmonary Arterial Hypertension sGC-stimulator Trial-1 (PATENT-1) and its open-label …
Contributors Both authors contributed to the writing and critical review of the manuscript.
Competing interests AEL and G-PD have received grants from Actelion and Pfizer, UK and Global.
Provenance and peer review Commissioned; internally peer reviewed.