Portal de Periódicos da CAPES

Ehlers-Danlos Syndrome and Cardiovascular Abnormalities

1973; Elsevier BV; Volume: 63; Issue: 2 Linguagem: Inglês

10.1378/chest.63.2.214

1931-3543

J. A. Antani, H.V. Srinivas,

Protein Tyrosine Phosphatases

During a period of seven years, a diagnosis of Ehlers-Danlos syndrome was made in 44 patients. The cases were studied with particular reference to involvement of the cardiovascular system. Fifteen patients had cardiac abnormalities and one patient had a vascular anomaly. There were two cases of atrial septal defect, one case of aortic incompetence, one case of left ventricular papillary dysfunction and one of dextrocardia. In the remaining cases, the cardiac abnormalities were nonspecific. One patient had a thoracic deformity and an apical systolic murmur. Electrocardiographic abnormalities included sinus bradycardia, grade 1 atrioventricular block, incomplete and complete right bundle branch block and nonspecific ST-T changes. The literature on cardiovascular abnormalities in Ehlers-Danlos syndrome is reviewed. Cardiovascular abnormalities in Ehlers-Danlos syndrome are interesting and not an uncommon feature. During a period of seven years, a diagnosis of Ehlers-Danlos syndrome was made in 44 patients. The cases were studied with particular reference to involvement of the cardiovascular system. Fifteen patients had cardiac abnormalities and one patient had a vascular anomaly. There were two cases of atrial septal defect, one case of aortic incompetence, one case of left ventricular papillary dysfunction and one of dextrocardia. In the remaining cases, the cardiac abnormalities were nonspecific. One patient had a thoracic deformity and an apical systolic murmur. Electrocardiographic abnormalities included sinus bradycardia, grade 1 atrioventricular block, incomplete and complete right bundle branch block and nonspecific ST-T changes. The literature on cardiovascular abnormalities in Ehlers-Danlos syndrome is reviewed. Cardiovascular abnormalities in Ehlers-Danlos syndrome are interesting and not an uncommon feature.