Journal of Rare Cardiovascular Diseases

Chronic thromboembolic pulmonary hypertension (CTEPH) is a debilitating disease caused by chronic obstruction of major pulmonary arteries. The median age of patients at diagnosis of CTEPH is 63 years and both genders are equally affected. The symptoms are usually nonspecific, particularly in the early stages of the disease, which significantly delays the diagnosis. All patients with pulmonary hypertension (PH) of the unknown aetiology should be diagnosed for CTEPH, even without the information on pulmonary embolism in the medical history. Ventilation-perfusion scintigraphy of the lungs has the highest sensitivity in the diagnosis of CTEPH. A positive result of scintigraphy suggestive of CTEPH is not enough to diagnose the disease. Right heart catheterization and angiography of the pulmonary arteries definitively confirm the diagnosis. All patients with CTEPH should receive chronic oral anticoagulation using generally vitamin K antagonists (warfarin, acenocumarol). Pulmonary endarterectomy (PEA) is the only effective treatment for eliminating the cause of the disease. However, not all patients can be referred for pulmonary endarterectomy surgery. According to data from the European CTEPH Registry of the years 2007-2009, over 40% of patients is not subjected to the operation mainly due to the distal location of thromboembolic changes. Currently, riociguat is the only approved therapeutic agent for the pharmacological treatment of inoperable/persistent CTEPH. In some cases, Off-lebel use of drugs approved for PAH may be considered in symptomatic patients with inoperable CTEPH/persistent PH after PEA. Recent advances in balloon pulmonary angioplasty make it a promising therapeutic alternative for selected patients with non-operable CTEPH. JRCD 2015; 2 (2): 4–8

riociguat; pulmonary endarterectomy; pulmonary embolism

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