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

Our plain-English summary of Riociguat as inpatient rescue therapy for acute decompensation of inoperable chronic thromboembolic pulmonary hypertension, by R.H. Alsufyani, S. Imran, B. Mccullagh, et al (BMJ Case Reports, 2026).

The study

• Authors: R.H. Alsufyani, S. Imran, B. Mccullagh, T. Hassan
• Journal: BMJ Case Reports
• Published: 2026;19(9)
• Type of article: Case report
• Read it: PubMed · Publisher · Free full text

Abstract

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.

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In plain English

This case report from Ireland described one man in his 70s, a retired coal miner with a history of asbestos exposure and no history of smoking. The article did not describe that exposure further or discuss any link to his illness. He had chronic thromboembolic pulmonary hypertension (CTEPH), raised pressure in the lung arteries caused by long-standing blood clots. After he came back to hospital, he needed high-flow oxygen for 14 days despite diuretic treatment. Riociguat was then started in hospital at 0.5 mg three times daily and raised every two weeks to 1.5 mg three times a day. He improved markedly within 72 hours and went back to his usual home oxygen. A blood marker, NT-proBNP, fell from 7020 to 3720 pg/mL, and no adverse events were observed. Specialists later judged him unsuitable for surgery or balloon angioplasty, so riociguat was continued. The authors concluded that the case supports the view that riociguat may be used effectively and safely in such inpatients. They noted that there are no clinical trial data on this use.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case report (single patient)
• Who was studied: One man in his 70s, a retired coal miner with a history of asbestos exposure and no smoking history, treated at hospitals in Ireland (Our Lady of Lourdes Hospital, Drogheda, and the National Pulmonary Hypertension Unit, Mater Misericordiae University Hospital, Dublin)
• Exposure: The article mentions a history of asbestos exposure and coal mining only as background, with no fiber type, duration or dose. The treatment studied was riociguat, started in hospital at 0.5 mg three times daily and increased every two weeks to a maximum of 1.5 mg three times a day.
• Main results:
  • Before riociguat, and despite diuretic treatment, the patient needed high-flow oxygen (FiO2 83%, flow rate 50 L/min) for 14 days.
  • The patient showed marked clinical improvement within 72 hours of starting riociguat.
  • The patient was weaned off high-flow oxygen and returned to his baseline long-term oxygen therapy.
  • The patient's NT-proBNP decreased from 7020 pg/mL to 3720 pg/mL.
  • No adverse events were observed during dose increases.
  • Right heart catheterisation after discharge was consistent with CTEPH: mean pulmonary artery pressure 40 mm Hg, pulmonary capillary wedge pressure 8 mm Hg, pulmonary vascular resistance 10 Wood units.
  • Lung function tests in this patient showed FVC 119%, FEV1 114%, FEV1/FVC 75.71% and DLCO 93%.
  • The patient was judged unsuitable for pulmonary endarterectomy or balloon pulmonary angioplasty, and riociguat was continued long term.
• The authors' conclusion: The authors concluded that the case supports the emerging view that riociguat, usually started in stable outpatients, may be effectively and safely used in acutely decompensated inpatients with CTEPH.
• Limitations the authors state:
  • The authors state that there are no clinical trial data on the safety, efficacy or routine use of riociguat as inpatient rescue therapy in inoperable CTEPH.

Topics

Medical terms in this study, explained in our Lexicon: Pulmonary Hypertension, Asbestos Exposure, Bilateral, Supplemental Oxygen.

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