The Therapeutic Efficacy of Drugs Targeting the NO-sGC-cGMP Pathway in Treatment of Patients With Chronic Thromboembolic Pulmonary Hypertension: A Systematic Review
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct and potentially curable form of pulmonary hypertension; however, a substantial proportion of patients remain inoperable or experience persistent or recurrent disease. Pharmacological therapies targeting the nitric oxide-soluble guanylate cyclase-cyclic guanosine monophosphate (NO-sGC-cGMP) pathway have emerged as promising treatment options. Therefore, this systematic review aimed to comprehensively evaluate the therapeutic efficacy of drugs targeting the NO-sGC-cGMP pathway on clinical outcomes in patients with CTEPH. We conducted a comprehensive search of electronic databases, including Embase, MEDLINE, Cochrane Library, and Scopus databases from inception to December 1, 2025. Studies involving adult patients with confirmed CTEPH receiving NO-sGC-cGMP pathway-targeted therapies were included. Exclusion criteria encompassed case reports, systematic reviews, review articles, conference abstracts with no full text (since they were not peer-reviewed), non-full-text articles, non-English manuscripts, opinion articles, and book chapters. Risk of bias was assessed using the Cochrane RoB 2 tool and the Newcastle-Ottawa Scale. A total of 23 studies involving 2815 patients were included. The most frequently investigated agents were riociguat and sildenafil. Fourteen of 15 studies demonstrated significant improvements in exercise capacity, with consistent findings reported in major trials such as CHEST-1 and its…
How strong is the evidence?
Evidence Hub reads this as a systematic review, done in people. Its evidence level is 1 of 5 (Highest · pooled evidence). Pooled evidence from several studies sits at the top of the evidence pyramid. Reported size: 2,815 participants or records.
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