Cardiac involvement of Whipple disease is a common finding in autopsies [1], but it still attracts little clinical attention [2]. We describe a patient who had severe aortic regurgitation when Whipple disease was diagnosed.

A 55-year-old man became ill for the first time in his life 4 years before his admission with intermittent arthralgias; migratory, often symmetrical, joint swellings; muscle weakness; and attacks of fever. One year later, he had abdominal pain, diarrhea and weight loss. During the 2 months before admission, the symptoms were aggravated. Examination showed a cachetic, weakened man. His blood pressure was 110/45 mm Hg, his pulse was 100 beats per minute, and his body temperature was normal. Palpation and auscultation revealed the signs of severe aortic regurgitation. Transthoracic and transesophageal echocardiography showed a dilated, hyperdynamic left ventricle and severe aortic regurgitation with several small echodense vegetations (2 to 3 mm in diameter) at the aortic cusps. The other values showed no abnormalities. Duodenal biopsy revealed accumulation of distended macrophages positive on periodic acid-Schiff staining (PAS) in the mucosa. After treatment with trimethoprim-sulfamethoxazole, the gastrointestinal and rheumatic symptoms disappeared within a few weeks. The aortic valve was replaced. Macroscopically, we found some brownish spots and focal friable vegetations. Microscopic examination of the aortic valve tissue showed superficial ulceration. The granulation tissue beneath included a chronic inflammatory infiltrate with numerous macrophages containing typical PAS-positive granules, the hallmark of Whipple disease [1, 2] (Figure 1).

View larger version (93K): [in this window] [in a new window]   Figure 1. Changes characteristic of Tropheryma whippelii-induced endocarditis. A. Section of the aortic valve with a superfgicial vegetation. Inflammatory infiltrate is made up of lymphocytes, few granulocytes, and (most characteristically) numerous macrophages with a distended cytoplasm positive on periodic acid-Schiff (PAS) staining (original magnification, x350). B. High-power magnification of macrophages containing the characteristic rod-shaped inclusion bodies (original magnification, x731).

Cardiac symptoms in Whipple disease are rare [3-5], but cardiac involvement of Whipple disease is commonly seen on autopsy [1, 2]. After a history of arthralgias and gastrointestinal symptoms, the patient in this case developed severe aortic regurgitation due to cardiac involvement of Whipple disease. Infective endocarditis should be considered a possible complication of Whipple disease.