We experienced a rare case of a patient who had a bicuspid aortic valve associated with acute aortic dissection limited to the sinus of Valsalva involving the left main coronary artery and acute coronary syndrome. The patient was a 36-year-old male who was identified as having a congenital bicuspid aortic valve. He visited our emergency room with a chief complaint of acute chest/back pain. He was diagnosed with acute coronary syndrome based on ECG findings and underwent an emergency coronary angiography. This test revealed filling defects at the entrance of the left main coronary artery, with aortic dissection limited to the sinus of Valsalva suspected. Emergency chest contrast-enhanced CT (ECG gated) led to his being diagnosed as having an acute aortic dissection limited to the sinus of Valsalva. After an intra-aortic balloon pump was inserted in order to maintain the coronary blood flow by surgery, emergency coronary artery bypass surgery and a modified Bentall procedure (the Carrel patch method) were carried out. His postoperative course was good and he was discharged home on the 19th disease day.

Congenital mitral regurgitation (MR) occurs infrequently and the number of reported adult surgical cases is small. A 77-year-old man presented with an exacerbation of congestive heart failure. He had a 19-year history of receiving medical treatment for MR and atrial fibriration. Transthoracic and transesophageal echo cardiograms revealed severe MR due to the restriction of the posterior mitral leaflet with very short chorda tendanea attached beneath the posterior leaflet preoperatively. We diagnosed this case to have congenital MR (Carpentier type III) in an adult based on the specific findings of echocardiography and mitral valve plasty was thus performed. All the dysplastic chordae of the P2 and P3 in the immovable leaflet region were cut and the reconstructed by the fifth artificial chordae. These procedures successfully allowed the posterior mitral leaflet to recover its normal shape and movability. Postoperative echocardiography showed no further mitral regurgitation and normal leaflet motion.