Echocardiographic correlation of right ventricular-Pulmonary artery coupling to exercise tolerance in congenital heart disease-associated pulmonary hypertension.

Background: Pulmonary arterial hypertension (PAH) is a chronic, progressive disease affecting the pulmonary vasculature, with a high mortality rate. Patients with PAH due to congenital heart disease (CHD) (World Health Organization Group 1) exhibit significantly reduced exercise tolerance compared to other PAH groups. Right ventricular (RV) dysfunction is a commonly used independent prognostic parameter in PAH. This study aimed to investigate an echocardiographic-derived parameter of RV-pulmonary artery (PA) coupling to exercise tolerance in PAH-CHD.

Subjects and methods: This cross-sectional study was performed on 51 consecutive patients with a confirmed diagnosis of PAH-CHD between February and March 2024. These patients underwent a 6-minute walk test (6MWT) and echocardiographic evaluation, including the measurement of tricuspid annular plane systolic excursion (TAPSE), pulmonary artery systolic pressure (PASP), TAPSE/PASP, and tricuspid regurgitation velocity (TRV) as parameters of RV function. The data were analyzed using the IBM SPSS Statistics software (version 26.0).

Results: Fourteen men and 37 women aged 36.0 ± 11.2 years participated in this study. The most common CHD was atrial septal defect (88.2%) with left-to-right shunt (51.0%). The mean of 6MWT was 366.8 ± 60.6 m. There was a significant association between 6MWT and TAPSE (r = 0.330, P < 0.05), PASP (r = -0.273, P < 0.05), TAPSE/PASP (r = 0.392, P < 0.05), and TRV (r = -0.354, P < 0.05).

Conclusion: The TAPSE/PASP is a simple echocardiographic parameter that correlates with RV function and exercise tolerance in patients with PAH-CHD.