Chronic thromboembolic pulmonary hypertension in a patient with myxoma of the heart and thrombophilia of mixed genesis

Objective. To describe the clinical case of a young patient with chronic thromboembolic pulmonary hypertension (CTEPH) with heart myxoma and thrombophilia of mixed origin, who underwent surgical treatment.

Materials and methods. Patient Z., 24 years old, was admitted to the cardiology department with cough with sputum production that occurs during physical activity, including streaks of blood, shortness of breath with slight physical exertion, legs and feets edema, weakness and dizziness. The examination was carried out: electrocardiography; multislice computed tomography pulmonary angiography; echocardiography (EchoCG); transesophageal EchoCG; magnetic resonance imaging of the heart with contrast; screening for coagulopathy.

Results. Based on clinical data, medical history, laboratory, instrumental diagnostic, we have put the diagnosis: Myxoma of the right atrium. Patent foramen ovale. Thrombophilia of mixed origin: hereditary – heterozygous mutation of the prothrombin gene (factor 2), acquired – hyperhomocysteinemia. Postthrombotic deep vein disease of the left lower limb. Recurrent pulmonary embolism (last relapse dated October 19, 2022). CTEPH functional class III (World Health Organization), moderate risk. Congestive heart failure with reduced right ventricular ejection fraction, functional class III (by the New York Heart Association). Relative tricuspid valve regurgitation grade 3. The patient underwent surgery: thromboendarterectomy from the right pulmonary artery under conditions of circulatory arrest and deep hypothermia, removal of a right atrium myxoma under conditions of artificial circulation and blood cold cardioplegia: tricuspid valve plasty according to De Vega. After surgery patient’s condition was significantly improved.

Conclusion. The features of diagnosis and treatment of a patient suffering from CTEPH and cardiac myxoma are outlined. Surgery: Pulmonary thrombectomy and cardiac myxoma removal may improve the patient’s condition with CTEPH and left atrial myxoma.

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