[Chronic thromboembolic pulmonary hypertension - the hidden disease]

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but potentially curable form of pulmonary hypertension. Its pathophysiological background involves incomplete thrombus resolution following acute pulmonary embolism, in situ thrombosis, and vascular remodeling. An increase in pulmonary vascular resistance leads to persistent pulmonary hypertension, which, if left untreated, may progress to fatal right heart failure. CTEPH is often considered a "hidden" disease, as its nonspecific symptoms are frequently misattributed to other conditions.

To provide an update overview of the epidemiology, pathophysiology, diagnostic challenges, and therapeutic options of CTEPH, with particular emphasis on the role of the general practitioners and the internists in early recognition.

A narrative literature review based on the 2022 ESC/ERS guidelines, the most recent European and international registries, and contemporary review.

The incidence of CTEPH following pulmonary embolism is estimated at 2-4%, although its true prevalence is likely higher. Diagnostic delays are common, with patients typically presenting to pulmonary hypertension centers 18-24 months after symptom onset. Pulmonary endarterectomy offers a curative treatment option for patients with surgically accessible central lesions. For inoperable or residual CTEPH, balloon pulmonary angioplasty and targeted pharmacological therapy, such as riociguat, have opened new therapeutic perspectives.

Diagnosis of CTEPH remains a complex challenge. General practitioners have a pivotal role by considering CTEPH in patients with recurrent dyspnea, exercise intolerance, signs of right heart failure, or persistent symptoms following pulmonary embolism, and by ensuring timely referral to specialized pulmonary hypertension centers.

Early diagnosis and evidence-based management of CTEPH can significantly improve both quality of life and survival. As general practitioners and internists are on the frontline, their awareness and education are crucial for early disease recognition. Orv Hetil. 2025; 166(48): 1908-1915.