Bicuspid Aortic Valve Endocarditis and Q Fever

Patients with bicuspid aortic valves (BAVs) are at increased risk of infective endocarditis. Coxiella burnetii is a rare cause of culture-negative infective endocarditis.

A young male with BAV presented with 3 months of fevers and abdominal distension. Initial workup showed pancytopenia and massive splenomegaly. Blood cultures were negative. Echocardiography revealed endocarditis of the bicuspid valve, aortic regurgitation, and aortic pseudoaneurysm. He underwent annulus debridement, valve replacement, and concurrent splenectomy. Serologic and tissue studies revealed C burnetii and Q fever.

Chronic Q fever is a clinical syndrome that presents months to years after C burnetii infection with nonspecific symptoms. Due to its intracellular and fastidious nature, treatment requires a prolonged antibiotic course and standard surgical indications for infective endocarditis.

Patients with BAVs are at increased risk of endocarditis, and C burnetii should be considered in all cases of culture-negative endocarditis.