Echocardiographic determinants of pulmonary hypertension in patients with idiopathic pulmonary fibrosis: Аssociation with clinical and functional status

Introduction. Pulmonary hypertension (PH) frequently complicates the course of idiopathic pulmonary fibrosis (IPF) and worsens its prognosis; however, echocardiographic signs of right heart chamber remodeling and clinical and functional factors associated with systolic pulmonary artery pressure (PASP) level in IPF remain insufficiently studied.

Aim. To characterize echocardiographic changes in the right heart chambers in patients with idiopathic pulmonary fibrosis depending on the presence of pulmonary hypertension and to identify clinical and functional factors associated with the value of systolic pulmonary artery pressure.

Materials and methods. The study included 121 patients with IPF. Based on echocardiography, patients were divided into groups without PH (PASP < 35 mmHg, n = 35) and with PH (PASP ≥ 35 mmHg, n = 86). Right atrial area (RA area), mean right ventricular diameter (RV diameter), TAPSE, TAPSE/PASP ratio, and left ventricular ejection fraction (LVEF) were assessed. Spearman correlation analysis, univariate and multivariate linear regression analysis with PASP as a continuous variable were performed.

Results. The PH group showed larger RA area (17.0 vs 13.5 cm², p < 0.001), larger RV diameter (4.00 vs 3.75 cm, p < 0.001), and a lower TAPSE/PASP ratio (0.438 vs 0.697, p < 0.001) with no difference in absolute TAPSE (p = 0.941). LVEF was slightly lower in the PH group (61% vs 62%, p = 0.016) but remained within normal limits. PASP correlated positively with RA area (r = 0.491, p = 0.019) and desaturation level during 6MWT (r = 0.355, p < 0.001), and negatively with post-exercise SpO₂ (r = -0.336, p = 0.001). DLCO correlated positively with post-exercise SpO₂ (r = 0.347, p = 0.001) and negatively with mMRC (r = -0.338, p = 0.001) and desaturation (r = -0.270, p = 0.014). Univariate linear regression showed associations of PASP with cardiovascular events (β = 10.8), CHF (β = 10.9), desaturation (β = 1.02 per 1%), post-exercise SpO₂ (β = -0.603), mMRC (β = 4.52), LTOT duration (β = 3.74 per year), and LVEF (β = -0.562). In multivariate linear regression, independent determinants of PASP were the Charlson comorbidity index (β = 2.205 per 1 point; p = 0.006), desaturation level (β = 1.153 per 1%; p < 0.001), and LTOT duration (β = 4.281 per year; p = 0.020).

Conclusion. In patients with IPF, pulmonary hypertension is associated with right heart remodeling (enlarged RA area, RV diameter) and reduced right ventricular-pulmonary arterial coupling (decreased TAPSE/PASP ratio) despite preserved TAPSE. Independent determinants of PASP value are comorbidity burden (Charlson index), desaturation during exercise, and long-term oxygen therapy duration.

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