Anudya Kartika Ratri, Ovin Nada Saputri, David Nugraha, Ricardo Adrian Nugraha, Alisia Yuana Putri, Meity Ardiana, Budi Susetyo Pikir, I. Gde Rurus Suryawan
Universitas Airlangga. Dr. Soetomo General Academic Hospital.
Indonesia
Annals of Pediatric Cardiology
Ann Pediatr Cardiol 2025; 18: 26-32
DOI: 10.4103/apc.apc_230_24
Abstract
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.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Heart Dysfunction Associated with Pulmonary Vascular Disease (Right)
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes