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[Cardiovascular involvement in systemic sclerosis]
Joël Constans1, Sophie Skopinski, Damien Barcat
1Service de Médecine Interne et Pathologie Vasculaire, Hôpital Saint-André, 1, rue Jean-Burguet, 33075 Bordeaux, France. joel.constans@chu-bordeaux.fr
Insights
Systemic sclerosis frequently affects the heart, often leading to cardiomyopathy diagnosed late. Early detection via cardiac imaging reveals myocardial issues and diastolic dysfunction, crucial for managing this condition.
Area of Science:
- Cardiology
- Rheumatology
- Internal Medicine
Background:
- Heart involvement is a common complication of systemic sclerosis.
- Cardiomyopathy is the primary cardiac manifestation, frequently diagnosed late.
- Pulmonary hypertension is another significant cardiac complication, particularly in advanced disease stages.
Purpose of the Study:
- To highlight the early diagnostic methods for cardiac involvement in systemic sclerosis.
- To discuss the pathogenesis and clinical significance of cardiomyopathy and pulmonary hypertension in systemic sclerosis.
- To review current therapeutic options and their limitations for cardiac complications in systemic sclerosis.
Main Methods:
- Cardiac echography and radionuclide imaging for early myocardial involvement detection.
- Doppler echography for diagnosing pulmonary hypertension.
- Review of existing literature on pathogenesis and treatment of cardiac manifestations.
Main Results:
- Early myocardial involvement and left ventricular diastolic dysfunction are detectable via cardiac imaging.
- Pulmonary hypertension, though diagnosable, presents therapeutic challenges with a poor prognosis.
- Systemic sclerosis is associated with an increased frequency of large-artery disease, contrasting with typical small-artery involvement in the hands.
Conclusions:
- Early diagnosis of cardiac involvement in systemic sclerosis is possible with advanced imaging techniques.
- Managing cardiomyopathy and pulmonary hypertension in systemic sclerosis remains challenging.
- Systemic sclerosis impacts both small and large arteries, with implications for cardiovascular health.
Abstract:
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 alterations of diastolic function of the left ventricle or perfusion defects. The pathogenesis of this cardiomyopathy is supposed to be 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.