A 47-year-old female was referred to our department with 6 months intermittent fever and NYHA functional class 2 dyspnea. She was previously evaluated elsewhere for pyrexia of unknown origin, and empirically started on anti-tuberculous therapy. On presentation she was febrile with temperature of 100F. General examination revealed pallor and clubbing. Cardiovascular examination revealed cardiomegaly suggestive of left ventricular enlargement.

The second heart sound was single and loud. A blowing early diastolic murmur decrescendo in quality was present. Laboratory investigation revealed a hemoglobin of 8.5 gm%, elevated ESR of 105 and C –Reactive Protein of 146 mg/L. Peripheral smear revealed microcytic hypochromic anemia and workup for connective tissue disease was negative. Blood culture grew alpha- hemolytic streptococci, sensitive to vancomycin, gentamycin and linezolid. ECG revealed her to be in sinus rhythm, with a rate of 110/ mt. and normal QRS axis. Chest X-ray showed cardiomegaly with prominence of the main pulmonary artery and obliteration of the pulmonary bay. Transthoracic and transesophageal echocardiogram revealed a 10 x 6 mm vegetation on the right coronary cusp of the aortic valve with significant aortic regurgitation (Figure 1(A)), tricuspid valve vegetation of size 11x 14 mm Figure 1(E) and, a coronary fistula from the right coronary artery to the right atrium (Figure 1(B)-1(D)), and a 26x 18 mm vegetation in the fistulous connection from the right coronary artery to the right atrium, Figure 1(E). The right coronary artery was found to be dilated at its origin from the aortic sinus Figure 1(F). In addition, she was found to have severe pulmonary hypertension with right ventricular systolic pressure of 50 mm Hg. In order to study the abnormal cardiac anatomy, the patient was subjected to a cardiac CT, which revealed a fistulous communication between the sinoatrial nodal branch of the right coronary artery and the right atrium Figure 2(A), as well as pulmonary thromboembolism in the left descending pulmonary artery, with pulmonary infarcts. The patient was treated with parenteral vancomycin and gentamycin for a period of 30 days, and was afebrile at the time of discharge, along with normalization of inflammatory markers.

Follow up echocardiogram showed diminution in the size of the vegetations. She underwent an aortogram and coronary angiogram ,which showed the fistulous communication from the right coronary artery to the right atrium Figure 2(B).She successfully underwent correction of the coronary cameral fistula as well as aortic valve replacement.