Background: Behçet's disease (BD) is a multisystemic chronic inflammatory disorder. Cardiac manifestations in BD are extremely rare. There have been no reports of cardiac involvement of BD and especially endomyocardial fibrosis in the left ventricle (LV).
Case summary: A 50-year-old woman presented at the emergency department experiencing palpitations and fatigue, accompanied by elevated levels of B-type natriuretic peptide. Her medical history included mucocutaneous involvement of BD. Vital signs were within normal ranges, and electrocardiography showed a normal sinus rhythm. Physical examination did not reveal any pathological findings. The 24 h ambulatory electrocardiogram monitoring indicated sinus rhythm with premature ventricular contractions. Transthoracic echocardiography demonstrated a reduced LV ejection fraction. Further investigation with cardiac magnetic resonance imaging reported diffused areas of subendocardial enhancement, indicative of fibrosis likely due to vasculitis probably associated with BD. The patient was administered tartrate metoprolol, eplerenone, and dapagliflozin in addition to the ongoing medical treatment for BD, which included methylprednisolone, colchicine, and apremilast. This treatment approach resulted in an improvement in the patient's clinical condition.
Discussion: This case highlights that diffuse subendocardial fibrosis of the LV may be associated with the underlying BD.
Keywords: Behçet’s disease; Cardiac manifestations; Case report; Endomyocardial fibrosis.
© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.