The role of the heart team in the diagnosis and treatment of chronic thromboembolic pulmonary hypertension

The aim – to present to the medical community the functioning algorithm of the heart team concept in the diagnosis and surgical treatment of chronic thromboembolic pulmonary hypertension (CTEPH) in Ukraine. The concept of the CTEPH team implies a multi-disciplinary approach to the diagnosis and, consequently, surgical treatment of CTEPH with the participation of a radiologist, a pulmonologist, a cardiologist, a hematologist, a neurologist, and a cardiac surgeon. Considering the low incidence of the disease, the need for high-tech diagnostic tools, including CT angiography and angiopulmonography, as well as challenges of evaluating the operability of patients and the technical complexity of operations, patients with CTEPH are get together in the so-called reference centers. Within the framework of the CTEPH team concept, specific protocols and «road maps» have been developed for both diagnosis and treatment of the disease. In order to eliminate subjectivity in determining the operability of a patient with CTEPH, a special algorithm has been developed, which is applied to a clinical case for illustration. Monitoring patients with pulmonary embolism who are qualifed for a newly coined definition – postembolic pulmonary syndrome, has become a new task of the reference centers. The new syndrome is suggested to include CTEPH, as well as a similar, but not identical pathological condition, named chronic thromboembolic lung disease. The treatment of choice for CTEPH is pulmonary thrombendarterectomy. Mandatory elements of the surgical protocol include the creation of a «dry» operating field by means of a temporary circulatory arrest under deep hypothermia of 18 °C. Operations for distal lesions of the pulmonary arteries have become a recent achievement. The success of the operation depends on the anatomical type of the lesions, the degree of distal arteriopathy, the extent of the intervention, and the comorbid factors. Immediate and long-term results of the operation are evaluated as good and very good. The long-term survival of patients after surgery is significantly higher than of those who were administered medical treatment.Conclusions. Patients with suspected CTEPH should be referred to a reference expert center for diagnosis verification and operability determining. The standard diagnosis of CTEPH is angiopulmonography with simultaneous measurement of pressure in the right heart; the standard treatment is pulmonary thrombendarterectomy.