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Chronic thromboembolic pulmonary hypertension (CTEPH) is notoriously tricky to diagnose — its symptoms mimic asthma, COPD, and heart failure. Experts at CHEST 2026 emphasized that getting patients to a high-volume CTEPH center is critical, and that most will need more than just medication. Surgery, balloon pulmonary angioplasty, and evolving drug options like sotatercept are reshaping how this rare condition is managed.
CTEPH is a rare but serious condition where lung blood clots fail to dissolve, blocking blood vessels and causing symptoms — fatigue, shortness of breath, chest pain — that are frequently mistaken for asthma, COPD, or heart failure. At the CHEST 2026 Annual Meeting, Dr. Anjali Vaidya stressed that accurate diagnosis is the essential first step, with tools like ventilation-perfusion (V/Q) scans, dual-energy CT, and identification of systemic-to-pulmonary collateral vessels helping to distinguish CTEPH from other forms of pulmonary hypertension.
Once diagnosed, the gold standard for operable patients remains pulmonary thromboendarterectomy (PTE) surgery, ideally evaluated at an expert, high-volume center. For those who aren't surgical candidates, riociguat — the only US-approved drug for CTEPH — combined with balloon pulmonary angioplasty (BPA) offers a viable path. Emerging data on sotatercept (approved for group 1 PAH in 2025) hints at off-label promise for CTEPH, with one case showing a 59-meter improvement on the 6-minute walk test after 6 months, though randomized trial data is still lacking.
Key Takeaways
Why it matters: CTEPH is underdiagnosed and undertreated due to its nonspecific symptoms and lack of a clear prior clot history. A multimodal, expert-led approach — combining surgery, intervention, and evolving medical therapies — is key to improving outcomes in this challenging patient population.
