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Aim. To evaluate the effectiveness of balloon pulmonary angioplasty (BPA) in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Materials and methods. Forty patients with inoperable CTEPH were enrolled in this study. The indications were determined by multidisciplinary team. The average age of patients was 53.5 [43; 63] years. In 65% of cases patients had functional class III (according to WHO); the distance in the 6-minute walk test (6MWD) was 327 [280; 400] m; catheterization of the right heart revealed systolic pulmonary artery pressure (SPAP) 82 [64; 100] mm Hg, mean pulmonary artery (mPAP) 48.5 [38; 56] mm Hg, pulmonary vascular resistance (PVR) 784 [525; 1257] dyn·s/cm-5. Each patient underwent 6 BPA. Results and discussion. The effectiveness of BPA was assessed 2 months after the last session. According to the data of right heart catheterization SPAP decreased by 27.3%, mPAP by 26%, PVR by 34.5% from baseline. After all series of BPA echocardiography and magnetic resonance imaging demonstrated reverse remodeling of the right heart. Also significant decrease in the level of BNP by 62%, increasing in 6MWD distance by 39% and improvement of the functional class up to I in 60% cases and up to II in 40% cases were noted. Conclusion. The results of the present study demonstrated a high efficacy of BPA allowing to normalize hemodynamic and clinical parameters, increasing the physical activity. Balloon pulmonary angioplasty is a new highly effective, safe method for treating patients with inoperable CTEPH.

Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the potentially curable forms of pulmonary hypertension, in which pulmonary thromboendarterectomy is the gold standard treatment. However, over the last decade, great attention has been given to a combined therapeutic approach including both drug therapy and surgical treatment and the application of endovascular technologies. This clinical case demonstrates the diagnostic difficulties of CTEPH and the opportunities of a comprehensive approach to therapy for the disease with mandatory assessment of preoperative surgical and medical treatment in order to improve the patient status and to prepare for surgery.

Aim. To evaluate the vascular bed and lung perfusion in patients with chronic thromboembolic pulmonary hypertension (CTEPH) by computed tomography (CT) and to compare the severity of pulmonary arterial (PA) thrombotic lesions concurrent with parenchymal perfusion disorders with angiopulmonographic findings and clinical and hemodynamic characteristics. Subjects and methods. In the period from November 2015 to May 2016, 22 patients (7 men, 15 women) aged 27 to 67 years with a verified diagnosis of CTEPH were examined using an Aquilion ONE VISION Edition 640 (Toshiba Medical Systems, Japan) CT scanner with a 320-row detector. Perfusion defect in this study was evaluated with the new software allowing one to combine contrast and contrast-free images by a subtraction method. CT data analysis included visual assessment of the vascular bed and lung parenchyma and quantitative assessment with perfusion map construction and semi-automatic determination of the obstruction index (OI) and perfusion defect index (PDI). OI was compared with PDI and mean LA pressure according to the data of right heart catheterization and 6-minute walk test. Results. A statistically significant correlation was found between OI and PDI in patients with CTEPH (Pearson r=0.56; p=0.0065). There were no relationships between mean LA pressure and vascular perfusion indices (OI and PDI) or between vascular perfusion parameters and 6-minute walk test results. Conclusion. CT angiopulmonography in conjunction with pulmonary perfusion assessment (within one study) allows evaluation of the severity of vascular lesions and perfusion disorders and determination of the efficiency of treatment in patients with CTEPH.

Thromboendarterectomy is undoubtedly the most widely used method in chronic thromboembolic hypertension treatment. Although surgical treatment is highly effective, it is often associated with high risk of complications. One of the main reasons for impossibility of surgery performance is distal pulmonary artery obstruction. In this case medical therapy or staged balloon angioplasty is considered the treatment of choice. This case report discusses if pulmonary artery angioplasty can be used in patients with proximal pulmonary artery obstruction.

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Aim. To assess the effect of balloon pulmonary angioplasty (BPA) on structural parameters of the pulmonary arteries in inoperable patients with chronic thromboembolic pulmonary hypertension (CTEPH).Materials and methods. The study included 21 patients with an inoperable form of CTEPH. Assessment of clinical and hemodynamic parameters, pulmonary arteries diameters according to angiography, and structural parameters of the pulmonary arteries according to intravascular ultrasound (IVUS) in 11 patients was performed initially, immediately after BPA and after a series of BPA.Results. In total 50 BPA were performed – series of 2.3 ± 1.5 BPA per patient. The observation was carried out for 165 (88; 248) days. After series of BPA there were found out positive changes of clinical and hemodynamic parameters, including functional class, distance in the 6-minute walk test, mean pulmonary artery pressure (mPAP), pulmonary vascular resistance (PVR). According to angiography there was observed a continued dilation of the arteries at the level of segmental arteries and the 1st order branches of subsegmental arteries after the first BPA. According IVUS measurements the greatest changes during the observation period were determined at the 1st and 2d order branches of subsegmental pulmonary arteries – increased outer and inner diameters, vessel lumen area, decreased vascular wall area. A statistical relationship was found out between the diameter of the 1st order branches of subsegmental arteries after BPA and mPAP (r = -0.74, p = 0.006), cardiac output (r = 0.71, p = 0.010), PVR (r = -0, 78, p = 0.003).Conclusion. BPA in inoperable patients with CTEPH leads to a persistent enlargement of the ballooned vessel lumen. In the long term, there is a positive remodeling of the pulmonary arteries with the greatest effect at the level more distal than subsegmental arteries. The diameter of the vessels at this level has a strong correlation with the hemodynamic result of BPA.

The currentguidelines on chronic thromboembolic pulmonary hypertension (CTEPH) treatment consider pulmonary thrombendarterectomy as the first therapy choice. In case of inoperability due to severe patient's condition, distal lesions of pulmonary vessels, extremely high pulmonary vascular resistance, medical treatment for CTEPH includes life-long anticoagulation therapy and reduction of heart failure symptoms. This clinical case of the 59-y-old patient M. with inoperable CTEPH demonstrates the efficacy of phosphodiеsterase-5 inhibitor Sildenafil usedin high doses. The diagnosis was confirmed by the results of the complete diagnostic process including right heart catheterization and pulmonary angiography. The initial functional class was assessed as III in accordance with WHO classification. Sildenafil 60 mg daily was added tothe standard therapy (anticoagulant, calcium channel blocker) for initial four weeks. To the 4wk visit Sildenafil dose was increased to 240 mgdailytaking into account good tolerance of the treatment.The significant improvement of functional class, positive dynamic ofhemodynamic and echo parameters were found to 4 month of follow-up.