- Poster presentation
- Open Access
Prognostic significance of ventricular function and late gadolinium enhancement on CMR in symptomatic patients with scleroderma
© Natarajan et al; licensee BioMed Central Ltd. 2013
- Published: 30 January 2013
- Left Ventricular Ejection Fraction
- Right Ventricular
- Cardiovascular Magnetic Resonance
- Late Gadolinium Enhancement
Cardiac involvement is a leading cause of morbidity and premature mortality in patients with scleroderma. Identification of this offers the opportunity for earlier and more stratified therapeutic intervention. Published data on the prognostic significance of left and right ventricular impairment and myocardial fibrosis in this cohort are limited. The study objective was to determine the prevalence and prognostic significance of abnormalities on cardiovascular magnetic resonance (CMR) in patients with scleroderma who have breathlessness and/or other cardiac symptoms.
This is a retrospective longitudinal study of 126 consecutive patients with confirmed scleroderma and cardiac symptoms, who had undergone CMR. Completed scans were available in 124 of these. All scans were performed at 1.5 Tesla (Siemens Sonata or Avanto). Thinned myocardium was defined as thickness <4mm, right ventricular hypertrophy defined as thickness>5mm. The presence of left ventricular (LV) or right ventricular (RV) dilatation was defined as an increase in indexed LV or RV volumes compared to previously published normal ranges. Late gadolinium enhancement (LGE) was defined as an area of clearly increased signal intensity confirmed on phase swapping. All scans were analysed by two independent operators. All cause mortality was determined from review of hospital records and the national summary care database. A Cox proportional hazards model was used to determine predictors of mortality (IBM SPSS 19, USA).
Demographic data and CMR characteristics of 124 patients with symptomatic scleroderma.
Clinical and CMR characteristics
Number (of n=124)
45% male, 55% female
Patients with at least one cardiac abnormality
Thinned LV myocardium
LV wall motion abnormality
Raised LV mass index
Reduced LV long axis fx
Reduction in RV EF
Reduced RV long axis function
LA enlargement (>moderate)
RA enlargement (>moderate)
Increased signal on T2-STIR
Late gadolinium enhancement
Patients with only 1 cardiovascular abnormality on CMR
Patients with 2 cardiovascular abnormalities on CMR
Patients with 4 or more cardiovascular abnormalities on CMR
This is, to the best of our knowledge, the largest cohort of patients with scleroderma to have undergone CMR. In this selective cohort, cardiovascular abnormalities on CMR are detectable in the majority (81%) of patients with scleroderma and breathlessness and/or other cardiac symptoms. Significant LV dysfunction was the strongest CMR predictor of mortality. Presence of LGE did not predict mortality.
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.