Early myocardial and skeletal muscle interstitial remodelling in systemic sclerosis: insights from extracellular volume quantification using cardiovascular magnetic resonance.
AIMS
Systemic sclerosis (SSc) may induce cardiac fibrosis and systo-diastolic dysfunction. Cardiovascular magnetic resonance (CMR) can detect replacement myocardial fibrosis with late gadolinium enhancement (LGE) and interstitial myocardial fibrosis with T1 mapping techniques. The aim of the study was to detect subclinical cardiac involvement with CMR in paucisymptomatic SSc patients with no previous history of myocardial disease, comparing it with skeletal muscle remodelling.


METHODS AND RESULTS
Thirty consecutive SSc patients (mean age: 51 ± 12 years, all women) and 10 healthy controls (mean age: 48 ± 15 years, all women) underwent clinical, biohumoral assessment, and CMR. Extracellular volume fraction (ECV) was calculated from pre- and post-contrast T1 values in the myocardium and skeletal muscle. Seventeen patients (57%) were asymptomatic, 13 (43%) paucisymptomatic (effort dyspnoea). All patients had normal biventricular volumes and systolic function, while LGE was present in seven patients (23%). Myocardial ECV was significantly increased in patients with SSc (30 ± 4%) than controls (28 ± 4%, P = 0.03), as was skeletal muscle ECV (23 ± 6% vs. 18 ± 4%, P < 0.01). Myocardial ECV did not differ between patients with and without LGE (P = NS) and showed no significant correlations with clinical data, biventricular volumes, systolic, or diastolic function. Overall, myocardial ECV showed a significant correlation with skeletal muscle ECV (R = 0.58, P < 0.001).


CONCLUSION
SSc is associated not only with myocardial replacement fibrosis, as detected by LGE, but also with interstitial remodelling of the myocardium and skeletal muscles, as detected by an increased ECV also in patients with normal biventricular function, with potential diagnostic, prognostic, and therapeutic clinical implications.
['Barison|Andrea|A|', 'Gargani|Luna|L|', 'De Marchi|Daniele|D|', 'Aquaro|Giovanni Donato|GD|', 'Guiducci|Serena|S|', 'Picano|Eugenio|E|', 'Cerinic|Marco Matucci|MM|', 'Pingitore|Alessandro|A|']
D000328:Adult D016022:Case-Control Studies D015331:Cohort Studies D004719:Endomyocardial Fibrosis D005260:Female D005500:Follow-Up Studies D005682:Gadolinium D006801:Humans D019028:Magnetic Resonance Imaging, Cine D008875:Middle Aged D018482:Muscle, Skeletal D009135:Muscular Diseases D011446:Prospective Studies D011856:Radiographic Image Enhancement D012016:Reference Values D012595:Scleroderma, Systemic D018709:Statistics, Nonparametric D013997:Time Factors D016277:Ventricular Function, Left D016278:Ventricular Function, Right D020257:Ventricular Remodeling
Systemic sclerosis Cardiovascular magnetic resonance Myocardial fibrosis Late gadolinium enhancement Cardiac T1 mapping