Coronary anomaly in Behçet's syndrome

Area of Science:

• Cardiology
• Rheumatology
• Ophthalmology

Background:

• Behçet's syndrome is a multisystemic inflammatory disorder with a predilection for vascular involvement.
• Ocular manifestations, including chorioretinitis and retinal vein thrombosis, are common in Behçet's syndrome.
• Cardiovascular complications, though less frequent, can be severe.

Observation:

• A 39-year-old male with a history of Behçet's syndrome presented with acute posterior-wall myocardial infarction.
• The patient had a 13-year history of Behçet's disease, characterized by recurrent ocular inflammation and retinal vein thrombosis.
• Coronary arteriography revealed circumflex artery occlusion and a left main coronary artery-to-pulmonary artery fistula, with other coronary vessels being normal.

Findings:

• The myocardial infarction occurred 13 years after the initial diagnosis of Behçet's syndrome.
• The only identified vascular risk factor was cigarette smoking.
• The patient was successfully managed non-invasively with no immediate complications post-myocardial infarction.

Implications:

• This case underscores the potential for Behçet's syndrome to manifest with severe and unusual coronary artery pathology, including aneurysms and fistulas.
• Early recognition and management of cardiovascular involvement in Behçet's syndrome are crucial for patient outcomes.
• Further research is warranted to elucidate the mechanisms linking Behçet's syndrome to complex coronary artery abnormalities.