Diffuse myocardial fibrosis precedes subclinical functional myocardial impairment and provides prognostic information in systemic sclerosis.

Gotschy, Alexander; Jordan, Suzana; Stoeck, Christian T; von Deuster, Constantin; Peer, Tatiana; Gastl, Mareike; Vishnevskiy, Valery; Wissmann, Lukas; Dobrota, Rucsandra; Mihai, Carina; Becker, Mike O; Maurer, Britta; Kozerke, Sebastian; Ruschitzka, Frank; Distler, Oliver; Manka, Robert (2023). Diffuse myocardial fibrosis precedes subclinical functional myocardial impairment and provides prognostic information in systemic sclerosis. European heart journal - cardiovascular imaging, 24(3), pp. 373-382. Oxford University Press 10.1093/ehjci/jeac094

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AIMS

Myocardial involvement is common in patients with systemic sclerosis (SSc) and causes myocardial fibrosis and subtle ventricular dysfunction. However, the temporal onset of myocardial involvement during the progression of the disease and its prognostic value are yet unknown. We used cardiovascular magnetic resonance (CMR) to investigate subclinical functional impairment and diffuse myocardial fibrosis in patients with very early diagnosis of SSc (VEDOSS) and established SSc and examined whether this was associated with mortality.

METHODS AND RESULTS

One hundred and ten SSc patients (86 established SSc, 24 VEDOSS) and 15 healthy controls were prospectively recruited. The patients were followed-up for a median duration of 7.0 years (interquartile range 6.0-7.3 years). Study subjects underwent CMR including assessment of myocardial fibrosis [native T1 and extracellular volume (ECV)] and measurement of global longitudinal (GLS) and circumferential (GCS) myocardial strain. Native T1 values and ECV were elevated in VEDOSS and SSc patients compared with controls (P < 0.001). GLS was similar in VEDOSS and controls but significantly impaired in patients with established SSc (P < 0.001). GCS was similar over all groups (P = 0.88). There were 12 deaths during follow-up. Elevated native T1 [hazard ratio (HR) 5.8, 95% confidence interval (CI): 1.7-20.4; P = 0.006] and reduced GLS (HR 6.1, 95% CI: 1.3-29.9; P = 0.038) identified subjects with increased risk of death. Only native T1 was predictive for cardiovascular mortality (P < 0.001).

CONCLUSION

Subclinical myocardial involvement first manifests as diffuse myocardial fibrosis identified by the expansion of ECV and increased native T1 in VEDOSS patients while subtle functional impairment only occurs in established SSc. Native T1 and GLS have prognostic value for all-cause mortality in SSc patients.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Rheumatology, Clinical Immunology and Allergology

UniBE Contributor:

Maurer, Britta

Subjects:

600 Technology > 610 Medicine & health

ISSN:

2047-2412

Publisher:

Oxford University Press

Language:

English

Submitter:

Monika van Seeters-Messerli

Date Deposited:

18 Jan 2023 12:14

Last Modified:

18 Feb 2023 00:15

Publisher DOI:

10.1093/ehjci/jeac094

PubMed ID:

35639682

Uncontrolled Keywords:

cardiovascular magnetic resonance imaging feature tracking—strain myocardial tissue characterization prognosis systemic sclerosis

URI:

https://boris.unibe.ch/id/eprint/177634

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