SUMMARY The association between aortic dissection and Turner syndrome has been described in the literature as rare. We report a case of a Stanford type-A chronic aortic dissection in a woman suspected of Turner syndrom. Our goal is to recall the association between these two diseases for early detection and follow-upIntroduction Turner syndrome was first described by Henry Turner in 1938. It is one of the most common chromosomal abnormalities. More than 95% of adult women with Turner syndrome have short stature and infertility [1]. Turner syndrome is associated with an increased risk of aneurysms and aortic dissection, which increases the morbidity and mortality of the disease [2].

Left atrioventricular valve aneurysm is rare. We present a rare case of partial atrioventricular septal defect with an extremely thin left atrioventricular valve aneurysm mimicking valve perforation. The preoperative echocardiography demonstrated severe left sided atrioventricular valve regurgitation on the “cleft” and leaflet perforation. But in per-operative, we discovered a left sided atrioventricular valve aneurysm instead of valve perforation. The “cleft” edge and a aneurysm were closed.