Abstract
Background. Pulmonary hypertension is an abnormal hemodynamic condition classified into 5 major groups. Chronic thromboembolic pulmonary hypertension (CTEPH) is the main representative of group 4 and the only potentially curable form. This review aimed to summarize current evidence on the epidemiology, pathophysiology, diagnostic approaches, and treatment options for CTEPH.
Methods. In this narrative review, we searched PubMed, Scopus, and Google Scholar databases up to June 2025 using the keywords “Chronic Thromboembolic Pulmonary Hypertension”, “CTEPH”, “diagnosis”, and “treatment”. Relevant review articles, original studies, clinical guidelines, and authoritative Persian-language sources published in English or Persian were screened, and pertinent data were extracted.
Results. CTEPH can occur at any age and in both genders, and early diagnosis is challenging because of its non-specific symptoms. Organized residual thrombi and small-vessel involvement constitute the core pathophysiology. Risk factors include coagulation disorders, prior splenectomy, and the presence of lupus anticoagulant. Diagnosis begins with echocardiography and ventilation–perfusion scanning and is confirmed by right-heart catheterization and detailed imaging. Treatment consists of pulmonary endarterectomy as the first-line option, balloon pulmonary angioplasty for inoperable patients, and medical/supportive therapy such as lifelong anticoagulation.
Conclusion. CTEPH is a treatable cause of pulmonary hypertension, and early detection combined with appropriate treatment strategies can significantly improve disease course and patient survival. Multidisciplinary management in specialized centers, advanced imaging, and regular follow-up are essential to optimize outcomes and minimize complications.
Practical Implications. Timely recognition in patients with a history of pulmonary embolism or persistent dyspnea is critical. Prompt referral to experienced centers for surgery or endovascular intervention can significantly enhance survival and quality of life.