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Cardiac involvement in progressive systemic sclerosis
Insights
Progressive systemic sclerosis (PSS) can cause myocardial necrosis without infarction symptoms. Electrocardiograms and hemodynamic studies in PSS patients revealed heart muscle damage, suggesting PSS-related cardiac disease.
Area of Science:
- Cardiology
- Rheumatology
- Pathology
Background:
- Progressive systemic sclerosis (PSS) is a multisystem autoimmune disease.
- Cardiac involvement is a significant cause of morbidity and mortality in PSS.
- Subtle or atypical cardiac manifestations can complicate diagnosis.
Observation:
- A case of PSS presenting with electrocardiographic and vectorcardiographic evidence of myocardial necrosis, but lacking typical myocardial infarction symptoms.
- Coronary angiography revealed no obstructive coronary artery disease.
- Cineventriculography showed a hypokinetic, enlarged left ventricle.
Findings:
- Analysis of 43 PSS patients revealed electrocardiographic signs of myocardial necrosis in 5 individuals, none with clinical myocardial infarction.
- Hemodynamic investigations in 13 PSS patients identified a dip-plateau pattern in the right ventricle pressure curve in 3 cases.
- These findings suggest myocardial disease in PSS, distinct from ischemic heart disease.
Implications:
- Electrocardiographic "necrosis" patterns in PSS may indicate underlying myocardial disease rather than acute infarction.
- Hemodynamic restrictive findings and ventricular enlargement on ventriculography are potential indicators of cardiac involvement in PSS.
- Early recognition of these cardiac manifestations is crucial for managing PSS patients and improving outcomes.
Abstract:
The case of a patient with progressive systemic sclerosis (PSS) who developed electro- and vectorcardiographic patterns of myocardial necrosis without clinical picture of myocardial infarction is reported. The coronarography showed no obstruction of coronary arteries and cineventriculography a hypodynamic enlarged left ventricle. The analysis of electrocardiograms from 43 other patients affected with PSS revealed myocardial necrosis in 5 of them. The clinical syndrome of myocardial infarction was absent in all these cases. Moreover, the hemodynamic investigation in 13 cases allowed to record a dip-plateau figure on the right ventricle pressure curve in 3 of them. In PSS, the electrocardiographic aspects of "necrosis" as well as hemodynamic restrictive findings or ventricular enlargement at ventriculography could indicate myocardial disease.