CARDIOVASCULAR SIGNS OF HEREDITARY DYSPLASIA OF THE CONNECTIVE-TISSUE

The purpose of this study was to demonstrate the special features of c ardiovascular effects in connective tissue disorders through a group o f fifteen cases observed over a period of ten years. The group consist ed of eleven cases of Marfan syndrome (or Marfan-like syndrome), two c ases of pseudoxanthoma elasticum and two cases of Ehlers-Danlos diseas e. The cardiovascular lesions were as follows: 1) dissection of the as cending aorta which was confirmed and had been treated surgically in 2 cases 2) aneurysmal dilatation of the ascending aorta in 5 cases 3) m oderate mitral valve prolapse, which was isolated in 2 cases and combi ned with dilatation of the initial aorta in 2 other cases ; 4) distent ion of the mitral valve in 2 cases ; 5) mitral valve prolapse combined with tricuspid valve prolapse in 1 case ; the mitral incompetence was severe and made it necessary to carry out mitral valve replacement ; 6) moderate aortic valve prolapse combined with tricuspid prolapse in a case of type-I Ehlers-Danlos disease ; 7) Fallot's tetralogy combine d with Marfan's syndrome and treated surgically in one case; 8) severe hypertension with abnormalities of the iliac and renal arteries in on e case of elastic pseudoxanthoma elasticum. In three cases complicatio ns occurred leading to death, extension of the dissection of the abdom inal aorta and global recalcitrant heart failure respectively. The out come in the other 12 cases, with a mean follow-up-time of 3 and one ha lf years (range : 3 years to 7 years) was not marked by complications.