Riociguat as inpatient rescue therapy for acute decompensation of inoperable chronic thromboembolic pulmonary hypertension.

A man in his 70s, a retired coal miner with prior asbestos exposure and no smoking history, presented with syncope and progressive exertional dyspnoea. Initial CT pulmonary angiogram (CTPA) revealed bilateral lobar pulmonary emboli, and transthoracic echocardiography (TTE) showed evidence of pulmonary hypertension. He was discharged on rivaroxaban and referred to the heart failure and respiratory clinic. At the respiratory clinic follow-up, he reported worsening breathlessness and was initiated on long-term oxygen therapy (LTOT). Outpatient investigations were arranged, including a ventilation/perfusion (V/Q) scan, which demonstrated extensive mismatched perfusion defects consistent with chronic thromboembolic pulmonary hypertension (CTEPH). He was also referred for right heart catheterisation (RHC) and to the Irish National Pulmonary Hypertension Unit.Before he was reviewed in the pulmonary hypertension unit, he re-presented to the emergency department with worsening dyspnoea, haemoptysis and bilateral lower limb oedema, with clinical signs of right heart failure. Repeat imaging with TTE and CTPA confirmed persistent findings of chronic embolic disease. Despite optimal diuresis and anticoagulation, his symptoms remained refractory and required high-flow oxygen without decreasing requirements after 14 days. Riociguat was initiated and titrated inpatient, resulting in rapid clinical improvement and reduced oxygen requirement within 72 hours. Subsequent right heart catheterisation confirmed inoperable CTEPH. He was reviewed at the National Pulmonary Hypertension Clinic, and following multidisciplinary discussion, given insufficient surgically accessible thromboembolic burden and coexisting left ventricular dysfunction, the decision was made to continue riociguat as long-term medical therapy.