PO:31:171 | Uncovering subclinical cardiac involvement in VEDOSS: An echocardiographic driven study

Eugenio Capparelli¹, Eleonora Zaccara¹, Irene Rota², Sara Facetti², Daniela Bompane¹, Laura Castelnovo¹, Antonino Mazzone³, Paola Faggioli¹ | ¹UOC Reumatologia, ASST Ovest-Milanese, Legnano Milano Italy; ²UOC Cardiologia, ASST Ovest-Milanese, Legnano Milano, Italy; ³UOC Medicina Interna, ASST Ovest-Milanese, Legnano, Milano, Italy

Background. The 2011 Very Early Diagnosis of Systemic Sclerosis (VEDOSS) criteria include patients at risk of progression and those with mild, non-progressive forms of Systemic Sclerosis (SSc). Based on MRI proved case series, cardiac involvement has been already detected in VEDOSS. However, data suggests that early diastolic and systolic dysfunction, detected through echocardiography, can be indicative of myocardial fibrosis even in asymptomatic state in SSc patients. This study aimed to identify subclinical echocardiographic changes and predictive markers of cardiac dysfunction in both very early and mild-longstanding forms of VEDOSS.

Methods. We conducted a cross-sectional observational study involving 61 patients meeting VEDOSS criteria followed up regularly within our Scleroderma Unit. Patients were categorized as early VEDOSS (eVEDOSS, n=37) or mild-longstanding VEDOSS (mlVEDOSS, n=24) based on disease duration (more than 10 years). We analyzed clinical and demographic data, focusing on echocardiographic parameters such as the E/A ratio, as indicative of left ventricular diastolic dysfunction (LVDD) and interventricular septum (IVS) and posterior wall at end diastole (PWED) thicknesses for LV systolic dysfunction (LVSD). Areas and dimensions of both atriums were also assessed. Statistical analyses included Chi-square and Student's T tests, with a significance threshold of p<.05.

Results. Higher comorbidities burden was found in patient with longer disease duration (ml-VEDOSS), specifically cardiovascular, hematological and gastrointestinal disorders (Table 1). Ml-VEDOSS patients presented also with a greater prevalence of anti-centromere antibodies positivity compared to e-VEDOSS. However, no differences emerged for LVDD and LVSD comparing ml-VEDOSS and e-VEDOSS patients, while patients exhibiting SSc specific autoantibodies positivity exhibited lower E/A ratios and increased left atrial (LA) size (Table 2). Late nailfold videocapillaroscopic pattern patients exhibited increased PWED thickening and aortic valve insufficiency. Notably, patients undergoing Iloprost iv administration only experienced larger right atrial (RA) volume measured in the superior-inferior direction at end-systole (18.1±5.1 mm/m2), while further increasing in patients receiving both Iloprost and CCBs (21.7±4.5 mm/m2), compared to patients receiving no vasodilators (16.6±2.1 mm/m2). Renin-Angiotensin-Aldosterone System (RAAS) inhibitors patients reported reduced E/A ratio (p=0.01) and larger LA diameter measured in the superior-inferior direction at end-systole (49.5±5.8 vs 45.3±8.4 mm/m2, p=0.04) and a greater prevalence of aortic valve leaflets sclerosis (35.3% vs 11.6%, p=0.03) (Fig 1). E/A ratios, PWED and IVS thickening were included in multiple general regression model as independent variables. Multivariable regression analysis confirmed DLCO% as the sole predictor of both LVDD and LVSD in VEDOSS population.

Conclusions. Careful monitoring of cardiac function in VEDOSS patients is crucial as alterations may occur even in the absence of symptoms. DLCO% emerged as an important predictor of both LVDD and LVSD.