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Ann Rheum Dis. Dec 2006; 65(12): 1669–1670.
PMCID: PMC1798482

Evaluation of cardiac functional abnormalities in systemic sclerosis by dobutamine stress echocardiography: a myocardial echostress scleroderma pattern

In this study, we investigate the possibility of detecting, by dobutamine stress echocardiography (DSE), the presence of early or subclinical myocardial functional changes in patients with systemic sclerosis (SSc) without symptoms of ischaemic cardiac involvement.

Thirty consecutive patients with SSc (26 women and four men; mean age 51 years, range 28–73 years), with a mean disease duration of 8 years (range 1–21 years), were studied. In all, 16 of 30 patients with SSc were affected by diffuse cutaneous (dc)‐SSc, and the remaining 14 patients had limited cutaneous (lc)‐SSc. The study was approved by the local ethics committee, and all patients gave informed consent to participate. All patients fulfilled the American College of Rheumatology preliminary classification criteria for SSc.1 Table 11 lists the demographic and clinical data of patients with SSc. Four patients with dc‐SSc (25%) and two with lc‐SSc (14%) had hypertension, and three patients with dc‐SSc (19%) had diabetes; all were well controlled by appropriate treatment.

Table thumbnail
Table 1 Demographic and clinical features of patients with systemic sclerosis

The DSE protocol was applied as follows: after baseline data were acquired, dobutamine was infused, beginning at a dose of 5 μg/kg/min and increasing every 3 min to a maximum dose of 40 μg/kg/min. Echocardiograms were recorded at baseline, low dose, peak dose and 5 min into recovery. When necessary, atropine (up to 1 mg) was given intravenously at higher dose to augment the heart rate response (fig 11).). The duration of the whole examination was 30 min.

figure ar51110.f1
Figure 1 Histogram illustrating the protocol of dobutamine stress echocardiography.

The electrocardiogram and blood pressure were monitored continuously and recorded at each stage. For segmental analysis of left ventricular function, a 16‐segment model was used, as suggested by the American Society of Echocardiography.2

In 13/30 (43%) patients with systemic sclerosis, a wall motion abnormality, which was absent on baseline rest examination, was observed during dobutamine infusion. These changes were observed in 9/16 (56%) patients with dc‐SSc and in 4/14 (28%) patients with lc‐SSc, showing a higher frequency in the diffuse cutaneous subset. Owing to the small size of the two patient subsets, this difference did not reach significance (p = 0.17).

The wall motion abnormalities usually involved a small myocardial region, often (in eight patients) a single segment, but when present in more than one segment (in five patients: three with dc‐SSc and two with lc‐SSc), it affected scattered segments, with a “patchy” or “spots‐on‐a‐leopard” distribution, falling within different coronary territories. This characteristic picture differed from those usually observed in coronary artery disease involving two or more contiguous segments; it seems to be typical of patients with SSc and might be defined as “myocardial echostress scleroderma pattern” (MESP).

To our knowledge, this is the first report of a DSE picture associated with the myocardial functional change observed in patients with SSc, and consisting of segmental left ventricular wall motion abnormality areas the feature of which seems to be, when multiple segments are involved, the typical of scleroderma heart disease.

Owing to the absence of these left ventricular wall motion abnormalities in the baseline examination, the DSE patchy pattern seems not to be directly referable to the patchy areas of fibrosis reported in the literature,3,4,5 but rather to the expression of a functional defect evidenced by pharmacological stress.

In these patients, evaluation of coronary arteries status is needed; a recent clinical experience in some patients with SSc supports the use of myocardial multidetector computed tomography to avoid coronarography in the absence of a clinical history of coronary artery disease.6

Further studies are required to investigate the potential use of DSE in evaluating the benefits of pharmacological treatment of the functional status of small coronary vessels in SSc.

Footnotes

Competing interests: None declared.

References

1. Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committeee Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980. 23581–590.590 [PubMed]
2. Schiller N B, Shah P M, Crawford M, De Maria A, Devereux R, Feigenbaum H. et al Recommendations for quantitation of the left ventricle by two‐dimensional echocardiography. American Society of Echocardiography Committee on Standards, Subcommittee on Quantitation of Two‐Dimensional Echocardiograms. J Am Soc Echocardiogr 1989. 2358–367.367 [PubMed]
3. Black C, Myers A. Current topics in rheumatology: systemic sclerosis (scleroderma). Proceedings of the International Conference on Progressive Systemic Sclerosis, 20–23 Oct 1981, Austin, TX, USA
4. Roberts N K, Cabeen W R, Moss J, Clements P J, Furst D E. The prevalence of conduction defects and cardiac arrhythmias in progressive systemic sclerosis. Ann Intern Med 1981. 9438–40.40 [PubMed]
5. Ferri C, Emdin M, Nielsen H, Bruhlmann P. Assessment of heart involvement. Clin Exp Rheumatol 2003. 21(Suppl 29)S24–S28.S28 [PubMed]
6. Vacca A, Siotto P, Cauli A, Montisci R, Garau P, Ibba V. et al Absence of epicardial coronary stenosis in systemic sclerosis patients with severe impairment of coronary flow reserve. Ann Rheum Dis 2006. 65274–275.275 [PMC free article] [PubMed]

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