PMID: 9417175Jan 7, 1998Paper

Cardiovascular malformations and complications in Turner syndrome

Pediatrics
V P Sybert

Abstract

Turner syndrome (gonadal dysgenesis with sex chromosome abnormalities) is recognized to be a disorder in which cardiovascular malformations are common. The prevalence and natural history of these findings, the risk for aortic dissection, and the occurrence of cardiovascular disease have all been the subject of debate, as have been the American Academy of Pediatrics recommendations for cardiac screening of patients with Turner syndrome. To evaluate a large population of patients both cross-sectionally and longitudinally to determine the prevalence of cardiovascular malformations, the risk for dissection of the aorta, to determine whether there are phenotype:karyotype correlations that can allow for specific recommendations, and to devise an appropriate screening protocol. Data have been collected for patients with Turner syndrome. These individuals have been seen in an ongoing clinic established for the study of the natural history of Turner syndrome. Data from physical examinations, evaluations by cardiologists, echocardiography results, medical and surgical complications, medical records, and causes of death were analyzed. A total of 244 of 462 individuals in this population with karyotype-proven Turner syndrome could be evalu...Continue Reading

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