Noncoronary sinus of Valsalva rupture into the right atrium with a coexisting perimembranous ventricular septal defect

Ruptured sinus of Valsalva is very uncommon, and is < 1% of all congenital defects. The incidence ranges from 0.1%-3.5%. There is a male to female predominance of 4:1, with the highest incidence in the Asian population. Higher incidence is also seen in patients with Marfan's syndrome and Ehlers Danlos syndrome. There is a higher association of ruptured sinus of Valsalva with ventricular septal defect (VSD), aortic stenosis, and bicuspid valve defect. While most patients with VSD often have rupture of their right coronary sinus of Valsalva into the right ventricle due to poor structural integrity, we present a rare case of a patient with VSD who had rupture of his noncoronary sinus of Valsalva into the right atrium.