“Portrait” of patients with idiopathic pulmonary hypertension and chronic thromboembolic pulmonary hypertension depending on comorbid status: current and prognosis features

Changing the "portrait" of patients with idiopathic pulmonary hypertension (IPH) and chronic thromboembolic pulmonary hypertension (CTPH) over the past decade suggests the need to develop models of phenotypes in patients with this cohort.

The purpose of the study was to estimate the nature of the disease course and prognosis of patients with IPH and inoperable CTPH based on the formation of phenotypic groups depending on the comorbid status.

Materials and Methods. Patients with IPH (n = 88) and inoperable CTPH (n = 38) aged 38.5 [28.5; 51] and 53.5 [41; 58] years respectively were enrolled in the study. 6-minute step test (6-MST) and spiroveloergometry, transthoracic echocardiography (EchoCG), catheterization of the right side of the heart was performed at the moment of diagnosis verification and in 13 [12; 20] months. All patients were divided into 5 groups according to comorbid status: Group 1 (n = 29) – patients with IPH/CTPH without comorbid pathology; Group 2 (n = 23) with body mass index (BMI) ≥ 25 kg/m² ; Group 3 (n = 27) – with BMI ≥ 25 kg/m² and dyslipidemia; Group 4 (n = 30) with hypertension, BMI ≥ 25 kg/m² and dyslipidemia; Group 5 (n = 17) with the listed comorbid pathologies and carbohydrate metabolic disorders.

Results. Initially more severe functional status was observed in patients of the 4th and 5^th groups as compared to those in the 1^st and 3^rd groups. According to the data of the 3-dimensional EchoCG, in patients of the 4^th and 5^th groups a reliably more expressed increase in the volume of the right ventricle was revealed in comparison with such values in the 1^st and 3^rd groups. In IPH groups 1 and 3, the positive results of the vasoreactivity test (34.5% and 48.2%) were significantly more frequent in comparison with groups 4 (6.7%) and 5 (5.9%). The period from the diagnosis verification to adding the 2^nd specific drug was significantly shorter in patients with IPH/CTPH of the 5th group compared to patients of the 3^rd group. a high risk of lethal outcome after 13 [12; 30] months of treatment was significantly less frequently observed with IPH in Groups 1 and 3 as compared to Group 5.

Conclusion. The combination of BMI ≥ 25 kg/m², dyslipidemia, hypertension, as well as the additional presence of carbohydrate metabolism disorders is associated with a less favorable course of the disease in patients with IPH and inoperable CTPH.

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