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Acute type A aortic dissection is the most serious cardiovascular complication of Turner's syndrome, and is often fatal. We report a case involving a 38 year old woman with karyotype proven Turner's syndrome (46 X iso X), who presented with sudden chest pain. An angiographic computed tomographic scan (right) revealed the presence of a type A aortic dissection. Upon hypothermic (28°C) femoro-femoral cardiopulmonary bypass, valvar commissural repair and prosthetic vascular replacement of the ascending aorta were performed with the use of GRF glue. One month later, the patient was asymptomatic.
Cardiovascular malformations are often associated with Turner's syndrome, but aortic dilation and its complications are not so far recognised as being associated with this gonadal dysgenesis. Although the rate of aortic dissection associated with this syndrome is higher than that in the population at large (0.8 %v 0.01 %, respectively), reports in the literature are uncommon. A number of chronic aortic dissections, some acute type B aortic dissections, and several deaths by intrapericardial aortic rupture have been reported, but only two cases of acute type A aortic dissection with surgical success have been described. Dissections in Turner syndrome are often associated with coarctation, bicuspid aortic valve, and hypertension, but the enlargement of the aorta (6%) is not recognised as a risk factor. Furthermore, several publications have demonstrated the correlation between aortic dilation and aortic dissection. These results indicate that echocardiographic follow up should be undertaken for all patients with Turner's syndrome in order to avoid this complication by operating on them at the aortic dilation stage.