Sandra de Barros Cobra
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Marcelo Palmeira Rodrigues
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Felipe Xavier de Melo
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Nathali Mireise Costa Ferreira
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Viviane Vieira Passini Soares
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...
Abstract
Background: Pulmonary hypertension worsens the prognosis of idiopathic pulmonary fibrosis (IPF) and is associated with chronic respiratory failure in advanced disease. However, little is known about the occurrence of pulmonary hypertension and subsequent right ventricular dysfunction in non-advanced IPF patients without hypoxemia at rest. We evaluated right ventricular dysfunction in non-advanced IPF patients and examined the association of right ventricular dysfunction with mean pulmonary artery pressure (mPAP) at rest and during exercise. Methods: This cross-sectional study included patients with blood oxygen saturation levels ≥92% at rest, Gender-Age-Physiology Index ≤5, modified Medical Research Council scale score ≤3, and no history of oxygen therapy. Patients underwent transthoracic echocardiography at rest and during cardiopulmonary exercise. Ventricular function was analyzed by measuring conventional parameters and right ventricular global longitudinal strain using two-dimensional speckle-tracking echocardiography. The t-test was used to compare means of independent samples. Results: Abnormal speckle-tracking echocardiography findings were identified in 10/27 patients (37%), indicating right ventricular dysfunction (mean mPAP: 21.6 ± 5.5 mmHg at rest, 42.2 ± 14.1 mmHg during maximal exercise). Significant differences in mPAP were observed between patients with right ventricular dysfunction and those without dysfunction (at rest: 26.0 ± 4.8 vs. 19.1 ± 4.2 mmHg, p = 0.001; during exercise: 51.3 ± 6.4 vs. 36.9 ± 14.7 mmHg, p = 0.002). Right ventricular dysfunction was detected in 37% of non-advanced IPF patients and was associated with increased mPAP at rest and during exercise. Early recognition was only possible using speckle-tracking echocardiography. Conclusions: Right ventricular dysfunction was detected in 37% of non-advanced IPF patients and was associated with increased mPAP at rest and during exercise. Special attention should be given to these patients, as right ventricular dysfunction is suggestive of worse prognosis. These patients could benefit from new specific drugs or even oxygen therapy for transitory hypoxia.