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[Cardiac involvement in Behçet's disease. 12 cases]
O Bletry1, A Mohattane, B Wechsler
1Service de Médecine interne, Groupe hospitalier Pitié-Salpêtrière, Paris.
Insights
Behçet
Area of Science:
- Cardiology
- Rheumatology
- Internal Medicine
Background:
- Behçet's disease is a multisystem inflammatory disorder.
- Cardiac involvement is a serious complication of Behçet's disease.
- Understanding cardiac manifestations is crucial for patient management.
Observation:
- A retrospective study identified 15 cardiac manifestations in 12 out of 196 Behçet's disease patients.
- Pericarditis (5 cases) and myocardial infarction (4 cases) were the most frequent.
- Other rare manifestations included ventricular aneurysm, endomyocardial fibrosis, and valvular insufficiencies.
Findings:
- Pericarditis, though common, typically resolves quickly.
- Coronary artery lesions, including stenosis and pseudoaneurysm, may necessitate surgical intervention.
- Myocardial and right heart endomyocardial fibrosis are rare but characteristic, suggesting Behçet's disease.
Implications:
- Early recognition of cardiac involvement in Behçet's disease is vital.
- Specific cardiac lesions require tailored management strategies.
- Further research into the pathogenesis and treatment of cardiac Behçet's disease is warranted.
Abstract:
Fifteen cardiac manifestations observed in 12 of a series of 196 patients with Behçet's disease are reported. In this retrospective study conducted in an internal medicine department, 5 cases of pericarditis and 4 cases of myocardial infarction were encountered. The other cardiac manifestations were observed only once. They included ventricular aneurysm, endomyocardial fibrosis of the right heart, aortic insufficiency, mitral valve insufficiency, mitral valve prolapse, and right heart failure consecutive to pulmonary arterial hypertension. There was no relationship between the severity of cardiac lesions and that of the extracardiac manifestations of the disease. Comparison of these data with those found in the literature showed that pericarditis is the most frequent pathology, but it usually regresses rapidly. Lesions of the coronary arteries, with or without myocardial infarction, consist of stenosis, occlusion or pseudoaneurysm requiring surgical treatment. Myocardial lesions (with the possibility of pseudoaneurysm) and endomyocardial right heart fibrosis are exceptional but fairly characteristic of Behçet's disease which they should suggest. Hughes-Stovin's syndrome may be complicated by pulmonary arterial hypertension, although death is generally caused by massive haemoptysis.