PRESUMPTIVE Q-FEVER MYOCARDITIS ASSOCIATED WITH COXIELLA-BURNETII INFECTION OF A HOMOGRAFT VALVE IN THE OUTFLOW TRACT OF THE RIGHT VENTRICLE - REVIEW AND CASE-REPORT

A man with pulmonary atresia and a subaortic ventricular septal defect had a radical surgical repair at the age of 16 years with correction of pulmonary atresia by replacement with an aortic valve homograft. Th is later became infected with Coxiella burnetii. He died at the age of 27 years from renal failure caused by glomerulonephritis and was foun d to have a most unusual form of widespread focal myocardial degenerat ion with dystrophic calcification. The diagnosis of Q fever was confir med by positive serology during life and identification of the organis ms in the homograft valve after death, by histology and electron micro scopy. A fatal outcome from involvement of the myocardium in Q fever i s very rare, and histological evidence of such involvement, other than by embolic infarction, is almost unknown. This would also appear to b e the first recorded instance of coxiella colonization of a valve in t he pulmonary outflow tract.