Abstract

Objective: To evaluate the characteristics of the patients with idiopathic pulmonary arterial hypertension (IPAH) and inoperable chronic thromboembolic pulmonary hypertension (CTEPH) depending on the comorbidity status Design and method: The study included patients with IPAH (n = 88) and inoperable CTEPH (n = 38) with median age 38.5[28.5;51] and 53.5[41;58] years, respectively. At the time of diagnosis verification the assessment of functional status with 6-minute walking test (6MWT) and desaturation index, 2D and 3D Echo, right heart catheterization (RHC) was made. All patients were divided into 5 groups according to the comorbidity status: group 1-pts with IPAH/CTEPH without comorbidity (n = 29); group 2 - with BMI>25 kg/m2 (n = 23); group 3 –with BMI>25 kg/m2 and dyslipidemia (n = 27); group 4 -with essential hypertension, BMI>25 kg/m2 and dyslipidemia (n = 30); group 5 –all of the above in group 4 plus carbohydrate metabolism disorders (n = 17). Results: The pts from group 4 and 5 had significantly more severe functional class (WHO) at baseline (3 [3; 3] and 3 [3; 3,5]) with more reduced distance in 6MWT (314[250;340] m and 280 [205,0; 346,0] m) compared to the pts from group 1 (3[2;3] and 409 [341; 455] m) and group 3 (3[2;3] and 407 [357; 467] m). The pts from group 5 had significantly more reduced SpO2 before the 6MWT (93 [92; 95] %) compared to the pts from group 1 and 3 (97[94; 98]% and 97[94; 98]%). According to 3D Echo the end-diastolic right ventricular volume was significantly larger in pts from group 4 and 5 (135 [92;192]/ 138 [106;170] ml) also as end-systolic right ventricular volume (95 [63; 138]/ 86,8 [68;137] ml, respectively) compared to the pts from group 1 (119 [80;138]/ 77,5 [44;90] ml) and 3 (99 [75;148]/ 59 [41; 84] ml). Belonging to the group 5 associated with an increase of systolic pulmonary arterial pressure (RHC) on B coefficient = 23,62 mmHg (p = 0,01) compared to the group 1.Conclusions: IPAH and inoperable CTEPH pts with BMI > 25 kg/m2, dyslipidemia, essential hypertension, also as with additional carbohydrate metabolism disorders have more severe status, that requires the more aggressive therapy and strict dynamic control.

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