Cardiomyopathy, conduction abnormalities, and aortic root dilation in Turner syndrome

We report two patients with Turner syndrome and unusual cardiac anomalies. One is a 15-year-old girl with aortic coarctation, pulmonary valvular stenosis, tricuspid valve dysplasia, systemic hypertension, complete heart block, and cardiomyopathy. The second is a 15-year-old girl with aortic root dilation. We also reviewed the literature on the relationship between karyotype and heart disease in Turner syndrome and the occurrence of aortic root dilation, cardiomyopathy, and conduction abnormalities in this syndrome. The potentially life-threatening nature of aortic root dilation, cardiomyopathy, and severe conduction abnormalities demands special attention from pediatricians and cardiologists caring for girls with Turner syndrome.