Systolic Dysfunction in Systemic Sclerosis: Prevalence and Prognostic Implications

Tennøe, Anders Heiervang; Murbræch, Klaus; Andreassen, Johanna; Fretheim, H.; Midtvedt, Øyvind; Garen, Torhild; Dalen, Håvard; Gude, Einar; Andreassen, Arne K.; Aakhus, Svend; Molberg, Øyvind; Hoffmann‐Vold, Anna‐Maria

doi:10.1002/acr2.1037

Cited by 14 publications

(19 citation statements)

References 45 publications

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“…Systolic and diastolic cardiac function were evaluated by one of the authors (AHT) on available echocardiographies, applying updated international recommendations as previously shown (9,34). Systolic dysfunction was de ned by a GLS >-17.0 % (10,35), rather than ejection fraction, due to better reproducibility. LV diastolic dysfunction was evaluated according to the 2016 guidelines as described earlier (9,36) and RV systolic dysfunction was de ned by tricuspid annular plane systolic excursion (TAPSE) < 17 mm (10,34).…”

Section: Cardiopulmonary Assessmentmentioning

confidence: 99%

“…Systolic dysfunction was de ned by a GLS >-17.0 % (10,35), rather than ejection fraction, due to better reproducibility. LV diastolic dysfunction was evaluated according to the 2016 guidelines as described earlier (9,36) and RV systolic dysfunction was de ned by tricuspid annular plane systolic excursion (TAPSE) < 17 mm (10,34).…”

Section: Cardiopulmonary Assessmentmentioning

confidence: 99%

“…Cardiac brosis in SSc is thought to be related to repeated focal ischaemia leading to irreversible lesions and/or in ammatory myocarditis, but these issues have not been fully elucidated (7,8). From our recent studies and others, we know that left and right ventricular (LV and RV) systolic dysfunction, as well as LV diastolic dysfunction, are common complications of SSc (9)(10)(11). LV diastolic dysfunction further progresses over time and displays a poor prognosis (9).…”

Section: Introductionmentioning

confidence: 99%

“…LV diastolic dysfunction further progresses over time and displays a poor prognosis (9). We have also presented multivariable models predicting mortality in SSc (9,10). Such models may be improved by additional predictor variables as knowledge of SSc pathology improves.…”

Section: Introductionmentioning

confidence: 99%

“…Such models may be improved by additional predictor variables as knowledge of SSc pathology improves. Early detection of these cardiac complications is believed bene cial to improve outcome (9,10). However, there exist no established guidelines for screening of cardiac involvement as for pulmonary arterial hypertension (PAH) where screening with annual echocardiography is recommended (12,13).…”

Section: Introductionmentioning

confidence: 99%

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Serum Markers of Cardiac Complications In a Systemic Sclerosis Cohort

Tennøe

Murbræch

Didriksen

et al. 2021

Preprint

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Background: Primary cardiac involvement is one of the leading causes of mortality in systemic sclerosis (SSc), but little is known regarding circulating biomarkers for cardiac SSc. Here, we aimed to investigate potential associations between cardiac SSc and candidate serum markers.Methods: Serum samples from patients of the Oslo University SSc cohort and 100 healthy controls were screened against two custom-made candidate marker panels containing molecules deemed relevant for cardiopulmonary and/or fibrotic diseases. Left (LV) and right ventricular (RV) dysfunction was assessed by protocol echocardiography, performed within three years from serum sampling. Patients suspected of pulmonary hypertension underwent right heart catheterization. Vital status at study end was available for all patients. Descriptive analyses, logistic and Cox regressions were conducted to assess associations between cardiac SSc and candidate serum markers. Results: The 371 patients presented an average age of 57.2 (± 13.9) years. Female sex (84%) and limited cutaneous SSc (73%) were predominant. Association between LV diastolic dysfunction and tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) (OR 0.41, 95%CI 0.21-0.78, p=0.007) was identified. LV systolic dysfunction defined by global longitudinal strain was associated with angiopoietin 2 (ANGPT2) (OR 3.42, 95%CI 1.52-7.71, p=0.003) and osteopontin (OPN) (OR 1.95, 95%CI 1.08-3.52, p=0.026). RV systolic dysfunction, measured by tricuspid annular plane systolic excursion, was associated to markers of LV dysfunction (ANGPT2, OPN, and TRAIL) (OR 1.67, 95%CI 1.11-2.50, p=0.014, OR 1.86, 95%CI 1.25-2.77, p=0.002, OR 0.32, 95%CI 0.15-0.66, p=0.002, respectively) and endostatin (OR 1.86, 95%CI 1.22-2.84, p=0.004).Conclusion: ANGPT2, OPN and TRAIL seem to be circulating biomarkers associated with both LV and RV dysfunction in SSc.

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Section: Cardiopulmonary Assessmentmentioning

confidence: 99%

Section: Cardiopulmonary Assessmentmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Serum Markers of Cardiac Complications In a Systemic Sclerosis Cohort

Tennøe

Murbræch

Didriksen

et al. 2021

Preprint

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Systemic sclerosis is associated with increased in‐patient mortality in patients hospitalized for heart failure

Sherif,

Gilvaz,

Abraham

et al. 2024

ESC Heart Failure

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AimsWe aimed to analyse the characteristics and in‐hospital outcomes of patients hospitalized for heart failure (HF) with co‐morbid systemic sclerosis (SSc) and compare them to those without SSc, using data from the National Inpatient Sample from years 2016 to 2019.Methods and resultsInternational Classification of Diseases, Tenth Revision diagnosis codes were used to identify hospitalized patients with a primary diagnosis of HF and secondary diagnoses of SSc from the National Inpatient Sample database from 2016 to 2019. Patients were divided into two groups: those with and without a secondary diagnosis of SSc. Baseline characteristics including demographics and co‐morbidities, outcomes of mortality, length of stay (LOS), and costs were compared between the two groups. Multivariable logistic regression analysis was performed to adjust for confounders and assess the impact of SSc on in‐hospital mortality, cost, and LOS. A total of 4 709 724 hospitalizations for HF were identified, with 8150 (0.17%) having a secondary diagnosis of SSc. These patients were predominantly female (82.3% vs. 47.8%; P = 0.01), younger (mean age of 67.4 vs. 71.4; P < 0.01), and had significantly lower rates of traditional cardiovascular risk factors such as coronary artery disease (35.8% vs. 50.6%; P < 0.01), hyperlipidaemia (39.1% vs. 52.9%; P < 0.01), diabetes (22.5% vs. 49.1%; P < 0.01), obesity (13.2% vs. 25.0%; P < 0.01), and hypertension (20.2% vs. 23.8%; P < 0.01). Higher rates of co‐morbid pulmonary disease in the form of interstitial lung disease (23.1% vs. 2.0%; P < 0.01) and pulmonary hypertension (36.6% vs. 12.7%; P < 0.01) were noted in the SSc cohort. Unadjusted in‐hospital mortality was significantly higher in the HF with SSc group [5.1% vs. 2.6%; odds ratio: 1.99; 95% confidence interval (CI): 1.60–2.48; P < 0.001]. Unadjusted mortality was also higher among female (86.7% vs. 47.0%; P < 0.01), Black (15.7% vs. 13.0%; P < 0.01), and Hispanic (13.3% vs. 6.9%; P < 0.01) patients in the SSc cohort. After adjusting for potential confounders, SSc remained independently associated with higher in‐hospital mortality (adjusted odds ratio: 1.81; 95% CI: 1.44–2.28; P < 0.001). Patients with HF and SSc also had longer LOS (6.4 vs. 5.4; adjusted mean difference [AMD]: 0.37, 95% CI: 0.05–0.68; P = 0.02) and higher hospitalization costs ($67 363 vs. $57 128; AMD: 198.9; 95% CI: −4780 to 5178; P = 0.93).ConclusionsIn patients hospitalized for HF, those with SSc were noted to have higher odds of in‐hospital mortality than those without SSc. Patients with HF and SSc were more likely to be younger, female, and have higher rates of co‐morbid interstitial lung disease and pulmonary hypertension at baseline with fewer traditional cardiovascular risk factors.

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Heart involvement in patients with systemic sclerosis—what have we learned about it in the last 5 years

Nadel,

Taborska

et al. 2024

Rheumatol Int

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This review provides a detailed examination of original research and previously published reviews regarding cardiovascular involvement in systemic sclerosis (SSc). Our study aims to evaluate the current understanding of SSc-associated heart involvement (SHI), focusing on its most prevalent forms, diagnostic methods and treatment options. A comprehensive search of PUBMED, Medline, Web of science, Scopus and DOAJ databases was conducted, involving articles published between January 2019 and August 2024, available in English, both original research and reviews. Additionally, the authors examined the references cited in the selected articles, reviewed relevant literature, and included key publications dating back to 2010. Systemic Sclerosis (SSc) is an autoimmune connective tissue disease characterized by skin and internal organs fibrosis with accompanying vasculopathy. SHI encompasses both primary and secondary cardiac disease with a prevalence rate of up to 39%. It constitutes one of the leading causes of death among affected individuals. Systemic sclerosis- primary heart involvement comprises a wide range of conditions including arrhythmias, heart failure, pericardial disease, valvular abnormalities, and myocardial inflammation. However, its subclinical course, often misinterpreted as other forms of cardiomyopathy, poses true diagnostic challenges, requiring diagnostic tools like transthoracic echocardiography with tissue Doppler echocardiography and cardiac magnetic resonance imaging. The review underscores the importance of SHI and a holistic approach to managing patients with systemic sclerosis. Furthermore, it emphasizes the need for further investigation into potential pathogenetic mechanisms and biomarkers crucial for targeted treatment to fully optimize recommendations for this patient subgroup.

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Systolic Dysfunction in Systemic Sclerosis: Prevalence and Prognostic Implications

Cited by 14 publications

References 45 publications

Serum Markers of Cardiac Complications In a Systemic Sclerosis Cohort

Serum Markers of Cardiac Complications In a Systemic Sclerosis Cohort

Systemic sclerosis is associated with increased in‐patient mortality in patients hospitalized for heart failure

Heart involvement in patients with systemic sclerosis—what have we learned about it in the last 5 years

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