Cardiovascular involvement in systemic sclerosis

Heart involvement is frequent in systemic sclerosis. Cardiomyopathy is the main localization but its diagnosis is often late. Cardiac echography or radionuclide imaging show early involvement of the myocardium while showing altertaions of diastolic function of the left ventricle or perfusion defects. The pathogenesis of this cardiomyopathy is supposed to he related to myocardial ischemia in relation with vasospasm, or with organic lesions of small arteries or coronary microcirculation. Pericarditis rarely is of clinical significance. Pulmonary hypertension concerns patients with proximal and advanced systemic sclerosis or limited forms such as CREST. It can be efficiently diagnosed by doppler echography but its therapy is difficult and its prognosis is poor. Epoprostenol in continuous venous infusion seems to be efficient but the accessibility to this therapy is difficult. While the involvement of middle-sized arteries of the hands is common, systemic sclerosis seems to be associated to an increased frequency of large-sized arteries disease.