Quadricuspid Aortic Valve with Sinus of Valsalva Rupture

A 22-year-old female with no medical history presented to the emergency room with 2 weeks of rapidly worsening dyspnea on exertion, orthopnea, and cough. On cardiac auscultation, she was noted to have to-and-fro murmurs and a continuous murmur with signs of right heart failure. Echocardiographic images obtained showed moderate to severe aortic regurgitation, severe tricuspid regurgitation, and a "windsock" originating in the right coronary sinus of Valsalva and terminating in the right atrium. The aortic valve had four leaflets, with the right leaflet function compromised by the ruptured sinus, causing aortic regurgitation. The patient underwent resection of the sinus aneurysm and aortic valve replacement with a bioprosthetic valve. Quadricuspid aortic valves are uncommon and are rarely associated with sinus of Valsalva aneurysm. The prevalence in the general population, clinical progression, and prognosis of this unique congenital abnormality are reviewed.