Ehlers-Danlos syndrome with cardiac involvement

Congenital Polyvalvular Disease

“…In Ehler-Danlos disease, there is a difference of opinion as to elastification of the valves in this disease. According to Green et al54 there is an increase in elastic tissue, but according to Madison et al 55 there is no undue elastification. In either case no mention is made of loss of elastic tissue and disruption of architecture.…”

Cardiac abnormalities in the Ehlers-Danlos syndrome.

“…It is likely that the man's lesion was due to calcification of the valve, while the woman's abnormality was probably the result of her childhood rheumatic fever. There have been previous reports of mitral valvular disease in patients with Ehlers-Danlos syndrome (Mc-Kusick, 1966;Madison, Bradley, and Castillo, 1963), and it is possible that this patient had a lesion ofthis nature. Frieden, Hurwitt, and Leader (1962) discussed the Ehlers-Danlos syndrome when they reported their patient who died after an operation for aortic incompetence.…”

Repair of Atrial Septal Defect in a Patient with the Ehlers-Danlos Syndrome

“…A number of cardiovascular abnormalities accompanying the Ehlers-Danlos syndrome have Open-heart surgery in Ehlers-Danlos syndrome 117 been described in a small number of patients: a partial endocardia1 cushion defect (Fantl et al), aortic insufficiency and aneurysm of the sinus of Valsalva (Tucker, Miller & Jacoby, 1963), fatal dissecting aortic aneurysms (McKusick), tetralogy of Fallot (Wallack & Burkhart, 1950), insufficiency of mitral and tricuspid valves (Madison, Bradley & Castillo, 1963), and a probable atrial septa1 defect (Freeman, 1950). T o our knowledge, only one of these patients has been subjected to operation: in a boy, aged 11, open-heart surgery was performed with repair of a partial endocardial cushion defect (Fantl et al).…”