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Published on: November 18, 2018
[Symptomatic left ventricular dysfunction and Behçet disease. Report of 2 cases]
A Scheuble1, O Belliard, S Robinet
1Hôpital Saint-Antoine, service de cardiologie, 184, rue du Faubourg Saint-Antoine, 75571 Paris.
Insights
Behçet
Area of Science:
- Cardiology
- Rheumatology
- Pathology
Background:
- Behçet's disease, a systemic vasculitis, can affect cardiac structures in 1-6% of cases.
- Cardiac involvement includes pericardial, coronary, and myocardial disease.
- Left ventricular dysfunction, presenting as dilated cardiomyopathy, is a potential complication.
Observation:
- Two cases of Behçet's disease with symptomatic left ventricular dysfunction are presented.
- One case demonstrated dilated cardiomyopathy with normal coronary arteries, suggesting inflammatory myocarditis.
- Echocardiographic studies indicate underdiagnosis of myocardial disease in Behçet's patients.
Findings:
- Left ventricular dysfunction can occur in Behçet's disease, independent of coronary artery disease.
- Myocardial inflammation (myocarditis) is a potential cause of cardiac dysfunction.
- 20-35% of asymptomatic Behçet's patients exhibit left ventricular diastolic dysfunction.
Implications:
- Early detection of myocardial involvement in Behçet's disease is crucial.
- Assessing left ventricular diastolic function aids in timely diagnosis of cardiac complications.
- This highlights the importance of comprehensive cardiac evaluation in Behçet's disease management.
Abstract:
Behçet's disease is associated with cardiac complications which may affect all three cardiac layers in 1 to 6% of cases. Although pericardial and coronary disease are the most common, the myocardium may also be affected. The clinical presentation may be left ventricular dysfunction with signs of dilated cardiomyopathy. The cause of the left ventricular dysfunction is usually coronary artery disease but it can also be inflammatory, resulting in a myocarditis with normal coronary arteries. The authors report two cases of Behçet's disease with symptomatic left ventricular dysfunction presenting as dilated cardiomyopathy with normal coronary arteries in one of the cases. Recent echocardiographic studies suggest that the incidence of myocardial disease is underestimated in this pathology: 20 to 35% of patients with Behçet's disease but no cardiac symptoms had left ventricular diastolic dysfunction. A more attentive investigation of left ventricular diastolic function in these patients should enable earlier diagnosis of this complication.
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