Cardiac Involvement and Heart Failure Staging in Patients with Systemic Sclerosis Without Pulmonary Arterial Hypertension

<b>Background/Objectives</b>: Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by fibrosis and vascular damage, significantly increasing the risk of heart failure (HF). <b>Methods</b>: This cross-sectional study included 61 SSc patients (92% female, mean age 63 ± 13 years), excluding those with pulmonary arterial hypertension, referred to a tertiary pulmonary hypertension center. HF stages were classified according to updated guidelines. Clinical, echocardiographic, hemodynamic, and functional capacity data were analyzed in relation to HF stages. <b>Results</b>: A total of 48% of patients had pre-symptomatic HF (5% stage A, 43% stage B), while 38% had symptomatic HF (stage C). Advanced HF stages were significantly associated with older age (<i>p</i> = 0.02) and multiorgan involvement (<i>p</i> = 0.045) but not with SSc subtype or autoantibodies. Structural and functional echocardiographic abnormalities were prevalent (77% and 10%, respectively). Markers of elevated ventricular filling pressure such as left atrial volume (<i>p</i> = 0.011) and E/e' ratio (<i>p</i> = 0.03) correlated with HF severity. Functional impairment was observed with lower 6 min walk test (6MWT) distance (<i>p</i> = 0.017), reduced VO<sub>2</sub> peak (<i>p</i> = 0.015), and increased VE/VCO<sub>2</sub> slope (<i>p</i> = 0.002). Resting pulmonary artery wedge pressure did not correlate with HF stage (<i>p</i> = 0.93). VE/VCO<sub>2</sub> slope and 6MWT were independently associated with HF severity. <b>Conclusions</b>: Preclinical and symptomatic HF are highly prevalent in SSc patients. HF staging was linked to disease severity, age, and cardiovascular risk factors. Functional capacity tests (6MWT and CPET) serve as valuable tools for HF risk stratification. These findings highlight the critical need for comprehensive cardiovascular assessment and targeted management strategies to mitigate HF progression in SSc patients.

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Journal
Journal of Clinical Medicine
Published
2025-03-24
DOI
https://doi.org/10.3390/jcm14072211
Citations
4
Primary Topic
Systemic Sclerosis and Related Diseases
Type
article
Field-Weighted Citation Impact
5.89
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article

Cardiac Involvement and Heart Failure Staging in Patients with Systemic Sclerosis Without Pulmonary Arterial Hypertension

Bruno Bragança, Maria Isilda Oliveira, Mário Santos, José Rodrigues Gomes
4 citations
Journal of Clinical Medicine
Systemic Sclerosis and Related Diseases
5.89
article

Cardiac Involvement and Heart Failure Staging in Patients with Systemic Sclerosis Without Pulmonary Arterial Hypertension

Bruno Bragança, Maria Isilda Oliveira, Mário Santos, José Rodrigues Gomes
article en
4 citations

Abstract

<b>Background/Objectives</b>: Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by fibrosis and vascular damage, significantly increasing the risk of heart failure (HF). <b>Methods</b>: This cross-sectional study included 61 SSc patients (92% female, mean age 63 ± 13 years), excluding those with pulmonary arterial hypertension, referred to a tertiary pulmonary hypertension center. HF stages were classified according to updated guidelines. Clinical, echocardiographic, hemodynamic, and functional capacity data were analyzed in relation to HF stages. <b>Results</b>: A total of 48% of patients had pre-symptomatic HF (5% stage A, 43% stage B), while 38% had symptomatic HF (stage C). Advanced HF stages were significantly associated with older age (<i>p</i> = 0.02) and multiorgan involvement (<i>p</i> = 0.045) but not with SSc subtype or autoantibodies. Structural and functional echocardiographic abnormalities were prevalent (77% and 10%, respectively). Markers of elevated ventricular filling pressure such as left atrial volume (<i>p</i> = 0.011) and E/e' ratio (<i>p</i> = 0.03) correlated with HF severity. Functional impairment was observed with lower 6 min walk test (6MWT) distance (<i>p</i> = 0.017), reduced VO<sub>2</sub> peak (<i>p</i> = 0.015), and increased VE/VCO<sub>2</sub> slope (<i>p</i> = 0.002). Resting pulmonary artery wedge pressure did not correlate with HF stage (<i>p</i> = 0.93). VE/VCO<sub>2</sub> slope and 6MWT were independently associated with HF severity. <b>Conclusions</b>: Preclinical and symptomatic HF are highly prevalent in SSc patients. HF staging was linked to disease severity, age, and cardiovascular risk factors. Functional capacity tests (6MWT and CPET) serve as valuable tools for HF risk stratification. These findings highlight the critical need for comprehensive cardiovascular assessment and targeted management strategies to mitigate HF progression in SSc patients.

Journal of Clinical MedicineVol. 14(7)
Universidade do Porto (PT), Administração Regional de Saúde de Lisboa e Vale do Tejo (PT), Centro Hospitalar do Tâmega e Sousa (PT)
Openalex Percentile: Top 4%
Systemic Sclerosis and Related Diseases
5.89
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