Pulmonary function and lung diffusion capacity in patients with pulmonary hypertension associated with systemic connective tissue diseases compared with other forms of pulmonary hypertension
Progonov S.O., Radchenko G.D. , Bortkevych O.P.
Summary. The aim. To compare the indicators of pulmonary function (PF) and diffusion capacity of the lungs for carbon monoxide (DLCO) in patients with pulmonary arterial hypertension (PAH) associated with systemic connective tissue diseases (SCTD) and other forms of pulmonary hypertension (PH). Materials and methods. 60 patients from 4 statistically comparable groups were included: 15 patients with idiopathic PAH (IPAH), 15 with PAH associated with congenital heart disease (PAH-CHD), 15 with chronic thromboembolic PAH (CTEPH), 15 with PAH-SCTD. There were assessed vital lung capacity (VC), forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), DLCO and total lung capacity (TLC), performed right heart catheterization (RHC). Results. The biggest statistically significant decrease in PF indicators was observed in the group of patients with PAH-CHD, DLCO — in PAH-SCTD. Independent statistical correlations were between VC (p = 0.037) and FEV1 (p = 0.027) and the presence of CHD; between FVC and the volumetric index of right atria (RA) (p = 0.009); between DLCO and the 6-minute walking distance (p=0.009), mean pulmonary artery pressure (p=0.040), mean right ventricular pressure (p=0.002), presence of systemic sclerosis (SS) (p <0.001); between TLC and the volumetric index of RA (p=0.003) and presence of SS (p=0.006). Conclusions. A significant decrease in DLCO and TLC in patients with SCTD against the background of almost normal PF indicators confirmed the need for their determination in all patients with PAH-SCTD, in addition to standard spirometry indicators. DLCO and TLC were significantly associated with the functional state of patients and hemodynamic impairment.
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