Dextrocardia and Marfan's syndrome

It is described the occurrence of dextrocardia together with the congenital cyanotic heart disease in 20 year old man included in the fruste forme of the Marfan's syndrome. The diagnosis was made by the physical examination with the evidence of the arachnodactyly by the metacarpal indices and confirmed by autopsy with the following results: dextrocardia, large atrial septal defect, common ventricle, atresia of the pulmonary artery with the collateral lung perfusion from the descending aorta. There were found neither ocular manifestations, nor unambiguous manifestations of the aortic lesions. The ultrastructural examinations showed only greater accumulation of the PAS positive substances. Dextrocardia as the cardiovascular manifestation of the Marfan's syndrome has not yet been reported in the available literature.