Journal Article

PO:32:180 | Clinical and hemodynamic profile of patients with SSC-PAH with and without pulmonary veno-occlusive disease: a single-center study

Di Reumatologia, Società Italiana

2025

Overview

Introduction. Signs of pulmonary veno-occlusive disease (PVOD) may be found in patients with pulmonary arterial hypertension (PAH), particularly in systemic sclerosis (SSc)¹. This association (PVOD/PAH) remains poorly characterized in SSc. We aimed to compare the clinical, echocardiographic and hemodynamic profile of patients with SSc-PAH with and without a concomitant diagnosis of PVOD, and their potential response to vasodilators. Materials and Methods. We retrospectively analyzed 23 patients with SSc-PAH diagnosed by right heart catheterization, followed at our center between 2017 and 2023. Data collected at diagnosis and after 12 months included clinical, laboratory, functional and imaging parameters. Multidisciplinary assessment allowed the identification of cases with clinical and radiological features suggestive of PVOD/PAH¹. Patients were divided into two groups (PVOD/PAH and non-PVOD/PAH) and data were compared at baseline and 12-month follow-up. Results. Twenty-three patients with SSc-PAH were enrolled in the study, of whom 6 (26%) had signs of PVOD/PAH. At PAH diagnosis, PVOD/PAH patients had higher values of mean pulmonary arterial pressure compared to non-PVOD/PAH (47.5±5.3 vs. 36.7±8.8 mmHg, p=0.011), but similar pulmonary vascular resistance. Age, sex, cardiovascular risk factors, SSc features, echo findings as well as cardiac biomarker values were similar between the two groups (Table 1). At follow-up, most PVOD/PAH patients (67%) were on monotherapy with endothelin receptor antagonists (ERA), and 33% on dual therapy (ERA + phosphodiesterase inhibitors). Only one case of pulmonary edema was recorded. In the non-PVOD/PAH group, most patients (53%) were on dual vasodilator therapy. On echocardiography, PVOD/PAH patients showed higher tricuspid regurgitation velocity (4.10±0.68 vs. 3.07±0.91 m/s; p=0.035) and worse right ventricular function (FAC 25.6±8.4% vs. 33.9±6.8%; p=0.046). Changes in NT-proBNP levels from baseline differed between groups (p=0.012), showing a trend toward increase in PVOD/PAH (p=0.076) and reduction in non-PVOD/PAH (p=0.069). At 12 months, 5 hospitalizations for heart failure in each group, and a total of 5 deaths (2 PVOD/PAH and 3 non-PVOD/PAH) were recorded, with similar event-free survival (p=0.101). Conclusions. PVOD is frequently associated with PAH in SSc and does not seem to be associated with specific SSc features. Vasodilators, at least on monotherapy, should be considered in PVOD/PAH, as they seem to be well tolerated. Nevertheless, at follow-up these patients exhibit unfavorable laboratory and echo profiles compared to those with SSc-PAH alone, highlighting the importance of early referral for lung transplantation.

• Publisher: PAGEPress Publications
• Subject: echocardiography / PAH / PVOD