Article

Echocardiographic alterations in systemic sclerosis: a longitudinal study.

Department of Clinical Medicine, Cardiovascular and Immunological Science, University of Naples Federico II, Italy.
Seminars in Arthritis and Rheumatism (impact factor: 4.97). 04/2005; 34(5):721-7. DOI:10.1016/j.semarthrit.2004.11.001
Source: PubMed

ABSTRACT To investigate the evolution of cardiac alterations in systemic sclerosis (SSc).
Echocardiographic and echo-Doppler findings from 77 unselected SSc patients were analyzed at the first clinical observation and after a follow-up period of 65 +/- 36 months. Data were compared with those obtained from 45 normal subjects matched for age and sex.
Baseline left ventricular (LV) systolic function was normal in all patients and controls while LV diastolic dysfunction (expressed by an inverted E/A ratio which represents early and late filling of the LV during atrial contraction) was present in 23 patients and in 1 control ( P < 0.001). At the end of the follow-up period, while LV systolic function declined in 1 case alone, 6 further patients developed an inverted E/A ratio. Moreover, in the group of SSc patients mean A-wave values, E/A ratio, left atrial dimension, and LV wall thickness significantly changed, all indicating the progression of heart involvement. The alteration of LV diastolic function was independent of other known causes potentially affecting LV relaxation. Moreover, impairment of LV filling parameters was detected in the first phase of follow-up, while the anatomical changes occurred in the last phase.
Our data confirm the significant prevalence of LV diastolic dysfunction in SSc patients and the role of primary myocardial involvement. The long-term follow-up demonstrates that LV filling dysfunction is progressive and precedes the occurrence of LV remodeling.

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    ABSTRACT: To assess the prevalence and patterns of cardiac abnormalities as detected by cardiac magnetic resonance imaging (MRI) in systemic sclerosis (SSc). Fifty-two consecutive patients with SSc underwent cardiac MRI to determine morphological, functional, perfusion at rest and delayed enhancement abnormalities. At least one abnormality on cardiac MRI was observed in 39/52 patients (75%). Increased myocardial signal intensity in T2 was observed in 6 patients (12%), thinning of left ventricle (LV) myocardium in 15 patients (29%) and pericardial effusion in 10 patients (19%). LV and right ventricle (RV) ejection fractions were altered in 12 patients (23%) and 11 patients (21%), respectively. LV diastolic dysfunction was found in 15/43 patients (35%). LV kinetic abnormalities were found in 16/52 patients (31%) and myocardial delayed contrast enhancement was detected in 11/52 patients (21%). No perfusion defects at rest were found. Patients with limited SSc had similar MRI abnormalities to patients with diffuse SSc. Seven of 40 patients (17%) without pulmonary arterial hypertension had RV dilatation. This study shows that MRI is a reliable and sensitive technique for diagnosing heart involvement in SSc and for analysing its mechanisms, including its inflammatory, microvascular and fibrotic components. Compared with echocardiography, MRI appears to provide additional information by visualising myocardial fibrosis and inflammation. RV dilatation appeared to be non-specific for pulmonary arterial hypertension but could also reflect myocardial involvement related to SSc. Further studies are needed to determine whether cardiac MRI abnormalities have an impact on the prognosis and treatment strategy.
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Keywords

1 case
 
45 normal subjects
 
77 unselected SSc patients
 
atrial contraction
 
cardiac alterations
 
dysfunction
 
first clinical observation
 
first phase
 
follow-up period
 
heart involvement
 
inverted E/A ratio
 
last phase
 
long-term follow-up
 
LV diastolic dysfunction
 
LV diastolic function
 
LV relaxation
 
LV systolic function
 
LV wall thickness
 
primary myocardial involvement
 
significant prevalence