CHRONIC POST-THROMBOEMBOLIC PULMONARY HYPERTENSION DEVELOPMENT IN A PATIENT WITH HEREDITARY THROMBOPHILIA: A CASE REPORT

Objective: to describe a clinical case of chronic post-thromboembolic pulmonary hypertension (CTEPH) development and progression in a patient with recurrent pulmonary thromboembolism (PTE) and hereditary thrombophilia.

Materials and methods. Patient K., female, 50 years old, was hospitalized in the 1st therapeutic department of N.I. Pirogov First City Clinical Hospital with complaints of shortness of breath, occurring at rest and exacerbating after minimal physical activity, dry cough, chest heaviness, swelling of the lower extremities (mainly right one). The patient had a history of deep venous thrombosis (DVT) of the lower extremities, PTE, splenectomy, and long glucocorticosteroid drugs intake for thrombocytopenic purpura. The patient underwent different examinations in the hospital, including evaluation of laboratory tests in dynamics, echocardiography, contrast-enhanced multislice computed tomography of the pulmonary artery and its branches, perfusion lung scintigraphy, right heart catheterization.

Results. After examination the patient was diagnosed with multiple segmental and subsegmental perfusion defects of both lungs; we also observed signs of pulmonary hypertension (PH) at echocardiography, proved by right heart catheterization. Also the patient was diagnosed with inherited thrombophilia. The patient was included in the register of PH-patients, thromboendarterectomy together with administration of special medications, approved for use in patients with pulmonary arterial hypertension (PAH-specific therapy) were recommended.

Conclusion. This article describes the main features of CTEPH, its diagnostics and treatment in patients with CTEPH after PTE.