Rupture of a congenital aneurysm of the sinus of Valsalva

: The ruptured aneurysm of the sinus of Valsalva is a rare congenital anomaly which we encountered in less then 1% of our surgical patients. We recently operated on a young girl suffering from this congenital malformation consisting of a 15 mm long fistula arising from the right coronary sinus and giving rise to a 6 cm aneurysm which dissected the interatrial septum as well as the posterolateral wall of the right atrium before rupturing into this cavity. Because of the unusual presentation of this malformation, which has never been described previously, we though worthwhile reporting this case in details. We also summarized the embryological, anatomopathological, clinical and surgical aspects discussed in the literature.