THE IMPACT OF COMORBIDITY STATUS ON PROGNOSIS IN PATIENTS WITH IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION AND INOPERABLE CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION

Abstract

Objective: To evaluate the influence of comorbidity status on the features of the disease and prognosis in patients with idiopathic pulmonary arterial hypertension (IPAH) and inoperable chronic thromboembolic pulmonary hypertension (CTEPH) 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 baseline and after 13[12;20] months the 6-minute walking test, cardiopulmonary exercise testing, transthoracic Echo, right heart catheterization with acute vasoreactive test were made. The risk of the death was evaluated using risk stratification scale according to the ESC/ERS 2015 guidelines on the diagnosis and treatment of PH. 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 IPAH patients from group 1 and 3 had a positive acute vasoreactive test significantly more often (34,5% and 48,2%) as compared to the group 2 (17,4%), group 4 (6,7%) and group (5,9%). The period from the diagnosis verification to the addition of the second specific drug was significantly less in IPAH/CTEPH pts from the group 5, also as period till aneurysmal dilation of main pulmonary artery. Where as in pts from the group 3 these periods were significantly longer. At baseline and after 13[12;20] months of treatment the majority of IPAH pts had high risk of the death. In IPAH patients from group 1 and 3 the high risk of fatal events to 13 [12; 20] months was observed significantly more rarely as compared to the patients from group 5. Conclusions: The presence of BMI > 25 kg/m2, dyslipidemia, essential hypertension, also as additional carbohydrate metabolism disorders is associated with a less favourable course of the disease and indicated the importance of initial combination therapy in this cohort of IPAH and inoperable CTEPH pts.