Abstract

Right ventricular (RV) function is associated with prognosis in chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed to establish an RV dysfunction score using RV echocardiographic parameters to clarify the clinical characteristics in patients with CTEPH and to compare RV dysfunction score with parameters such as World Health Organization (WHO) functional class, hemodynamics, exercise capacity, and plasma BNP level. We enrolled 35 inpatients with CTEPH (mean age, 62 ± 15 years, 15 males). We constructed ‘an RV dysfunction score’ calculated as the summation of each point awarded for the presence of four parameters: tricuspid annular plane systolic excursion (TAPSE) < 16 mm, 1 point; tissue Doppler-derived tricuspid lateral annular systolic velocity (S′) < 10 cm/s, 1 point; right ventricular fractional area change (RVFAC) < 35%, 1 point; and right ventricular myocardial performance index (RV-MPI) > 0.4, 1 point. TAPSE, S′, RVFAC, and RV-MPI was 18.7 ± 4.8 mm, 11.9 ± 3.1 cm/s, 33.5 ± 13.9%, and 0.39 ± 0.2, respectively. The RV dysfunction score was associated with symptom [WHO functional class (p = 0.026)], hemodynamics [mean PAP (p = 0.01), cardiac index (p = 0.009), pulmonary vascular resistance (p = 0.001), and SvO2 (p = 0.039)], exercise capacity [6-min walk distance (p = 0.046), peakVO2 (p = 0.016), and VE/VCO2 slope (p = 0.031)], and plasma BNP level (p = 0.005). This RV dysfunction score using the four RV echocardiographic parameters could be a simple and useful scoring system to evaluate prognostic factors in patients with CTEPH.

Highlights

  • Chronic thromboembolic pulmonary hypertension (CTEPH) continues to be a disease with poor prognosis, several specific drugs and treatment are available [1, 2]

  • This study aimed to establish an right ventricular (RV) dysfunction score using the four RV echocardiographic parameters (TAPSE, S′, right ventricular fractional area change (RVFAC), and right ventricular myocardial performance index (RV-MPI)) to clarify the clinical characteristics on admission in patients with CTEPH and to compare the RV dysfunction score with parameters such as symptoms of World Health Organization (WHO) functional class, hemodynamics, exercise capacity [6-min walk test (6MWT) and cardiopulmonary exercise test (CPET)], and plasma brain natriuretic peptide (BNP) level used for risk assessment in patents with CTEPH

  • Since there is a possibility that riociguat positively affected RV function and hemodynamics, sub-group analysis was conducted between the patients with riociguat and without riociguat (Supplementary Table 1)

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Summary

Introduction

Chronic thromboembolic pulmonary hypertension (CTEPH) continues to be a disease with poor prognosis, several specific drugs and treatment are available [1, 2]. Echocardiography is widely used for assessing the severity of pulmonary hypertension and right ventricular (RV) function, which is important for prognosis in patients with CTEPH. The American Society of Echocardiography (ASE) recommends four RV echocardiographic parameters for the assessment of RV function: tricuspid annular plane systolic excursion (TAPSE), tissue Doppler-derived tricuspid lateral annular systolic velocity (S′), right ventricular fractional area change (RVFAC), and right ventricular myocardial performance index (RV-MPI) [3,4,5,6,7]. We hypothesized that RV function could be evaluated more precisely if the four RV echocardiographic parameters were combined

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