Congenital quadricuspid aortic valve is a very rare malformation. Most cases have been discovered as an incidental finding at aortic valve surgery or at autopsy. It frequently evolves to aortic regurgitation, which can manifest in adulthood and may require surgical treatment. A 66-year-old man was admitted because of dysprea. Echocardiogram revealed aortic regurgitation, mitral regurgitation, and tricuspid regurgitation. We performed aortic valve replacement, mitral annuloplasty, and tricuspid annuloplasty successfully. The aortic valve showed one large, two intermediate and one smaller cusp, which were classified as typed by Hurwitz's classification. An accessory cusp was situated between the right and left coronary cusps. No coronary abnormality was involved. The postoperative course was uneventful and he is doing well 6 months after operation.

A 78-year-old woman with abnormal shadows on computed tomography (CT) was given a diagnosis of right-sided aortic arch and Kommerell diverticulum (KD), accompanied by aberrant left subclavian artery. Although no symptoms were observed, the maximum diameter of the aneurysm was 63 mm, and surgical intervention was chosen because of the possibility of rupture. At first, a 4-branched blood vessel prosthesis with a side branch was anastomosed to the ascending aorta. Next, after reconstructing the cervical branches, a Conformable GORE® TAG® (W.L. Gore and Associates, 34 mm×200 mm) was inserted from the side branch and expanded in the range of Zones 0 to Th 7. Finally, ALSA coil embolization was performed. She was discharged on postoperative day 36, and at her 2-year follow-up, she was doing well, with shrinkage of Kommerell diverticulum.