When more is more: the role of additional upfront therapy in pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare disease characterised by pulmonary vascular remodelling and related increased pulmonary vascular resistance. This disease, if left untreated, will ultimately lead to death from right ventricular failure [1]. Currently available therapies target one of three known pathogenic pathways: the endothelin, prostacyclin or nitric oxide pathway [2, 3]. The current treatment paradigm emphasises the role of upfront combination therapy, although the permutation and timing of which remain unclear [4]. Despite advances in the understanding and treatment of this disease, mortality from PAH remains unacceptably high [5]. Ongoing investigation into drug combination strategies and timing of therapy initiation provides clinicians and patients with the opportunity to maximise benefit from currently available therapies, as novel drug discovery can take decades. Early combination therapy targeting several relevant pathogenic pathways, including the prostacyclin pathway, is likely to be of benefit to most patients with PAH <https://bit.ly/3GisHnA>