Key result
Systemic sclerosis-associated PAH was associated with a significantly reduced TAPSE/PASP ratio compared to idiopathic PAH (0.22 vs 0.27 mm/mmHg, p<0.001), indicating less favorable RV adaptation.
Why the study?
Does right ventricular arterial coupling differ between patients with systemic sclerosis-associated PAH and idiopathic PAH?
Cohort (n=403)
Does right ventricular arterial coupling differ between patients with systemic sclerosis-associated PAH and idiopathic PAH?
Answers are grounded in this paper and cite their sources.
Patients with systemic sclerosis-associated PAH exhibit worse right ventricular-arterial coupling and adaptation to increased afterload compared to matched patients with idiopathic PAH, particularly at higher pulmonary vascular resistance.
Background Pulmonary arterial hypertension (PAH) is a severe complication of Systemic Sclerosis (SSc) leading to high morbidity and mortality. Objective To compare the TAPSE/PASP ratio, as a surrogate of ventricular-arterial coupling, in two matched cohorts of patients with idiopathic and SSc-associated PAH. Methods Two hundred and two consecutive patients with SSc-PAH matched for age, gender, body max index, and pulmonary vascular resistance (PVR), with 201 idiopathic PAH (IPAH) patients, were enrolled. Physical examination, WHO functional class, 6-minute walk distance (6MWD), right heart catheterization, echocardiographic assessment and European guidelines-derived risk score were assessed at baseline. Results the distribution of TAPSE/PASP between IPAH and SSc-PAH patients diverged for PVR≥5 WU (ROC-derived logistic analysis, Sp 99%, Se 51%; AUC=0.89, 95% CI 0.85-0.92, p<0.0001). Considering patients with PVR ≥5, fewer SSc-PAH patients were at low risk compared with IPAH (low risk:11 patients, 7% vs 36 patients, 20%). SSc-PAH had more advanced WHO functional class, more impaired exercise capacity, higher NT-proBNP, higher right atrial pressure, and lower cardiac index (respectively, WHO III/IV:129 patients, 84% vs 126 patients, 72%, p=0.01; 6MWD: 335 ± 106m vs 367 ± 114m, p=0.01; NT-proBNP: 535 ± 696 pg/ml vs 212 ± 407 pg/ml, p=ns, RAP: 9.3 ± 4.6mmHg vs 7.5 ± 3.9mmHg, p<0.001; CI values: 2.2 ± 0.5 l/min/m2 vs 2.4 ± 0.6 l/min/m2,p=0.01). RV systolic function and RV-PA coupling were significantly reduced in the SSc-PAH population compared with the IPAH patients (TAPSE values: 15±4mm vs. 18±4mm, p<0.001;TAPSE/PASP ratio: 0.22±0.09mm/mmHg vs. 0.27±0.08mm/mmHg,p< 0.001). Conclusion SSc-PAH patients with PVR≥5 had less favorable RV adaptation to increased afterload, resulting in increased filling pressures, higher BNP plasma level, more impaired TAPSE/PASP and finally lower cardiac index compared with IPAH.
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Adamo et al. (2025) conducted a cohort in Pulmonary arterial hypertension (n=403). Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) vs. Idiopathic pulmonary arterial hypertension (IPAH) was evaluated on TAPSE/PASP ratio (p=<0.001). Systemic sclerosis-associated PAH was associated with a significantly reduced TAPSE/PASP ratio compared to idiopathic PAH (0.22 vs 0.27 mm/mmHg, p<0.001), indicating less favorable RV adaptation.
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