MICA: Pulmonary Hypertension: intensification and personalisation of combination Rx (PHoenix)

Pulmonary arterial hypertension (PAH) is a devastating disease driven by remodelling and constriction of the small blood vessels of the lungs. Approved therapies reduce constriction by targeting three distinct biological pathways. Therapies are changed due to side effects and/or disease worsening. However, due to the invasive, hospital-based investigations required to assess disease severity and demonstrate benefit of treatment, there is no way to match patients to drugs effective for their disease. Through an MRC Confidence-in-Concepts funded feasibility study we have developed the capacity to use regulatory approved, minimally-invasive monitors to make key measurements that allow assessment of disease severity and treatment effect while patients are at home. Data is relayed daily to care teams through regulatory approved, clinically used online portals, making early, remote evaluation of treatment benefit in an individual patient possible. In patients with pulmonary arterial hypertension (PAH) this study will: evaluate the capacity of implantable/remote technology for early evaluation of clinical efficacy and matching patients to drugs that work for them. Forty patients with PAH established on guideline recommended therapy will be implanted with approved medical devices and remote monitoring established. Physiology, activity and quality-of-life will be monitored during a randomised cross-over study of oral prostacyclin IP receptor agonist and soluble guanylate cyclase stimulator. The study will assess the physiology of therapeutic intensification and de-escalation in a manner previously unachievable. If successful, the technological approach will facilitate novel study designs in the area, providing a platform that has the potential to transform experimental medicine, therapeutic development and personalised medicine for patients with pulmonary hypertension.