A 50-year-old man with Marfan syndrome, was given a diagnosis of pseudoaneurysm in an anastomotic site of the left coronary artery after Bentall operation, with severe calcification. He was successfully treated with reanastomosis of a new graft to the left main trunk by the removal of a calcified intima. Coronary artery bypass grafting was not possible because his coronary arteries were covered with thickened fatty tissue due to a previous omental flap procedure for mediastinitis, and therefore we chose left main trunk coronary angioplasty. The whole calcified intima was excluded with a dissector and resected at both ostias of the left descending artery and left circumflex artery. An 8-mm woven Dacron graft was anastomosed at the left main trunk by large stitches of adhesive tissue around the adventitia, to the inside of the lumen of the left main trunk. The patency of the left main trunk was confirmed by CT and he was discharged in a good condition. Close observation is needed for long-term morbidity.

A 39-year-old woman, who had undergone aortic valve replacement with a Carpentier-Edwards pericardial bioprosthesis 16 years previously, was admitted to our hospital with a diagnosis of acute heart failure due to acute aortic regurgitation. An emergency operation was undertaken with the patient in a state of shock due to sudden cardiac arrest. The ascending aorta was cross clamped, and cardiac arrest was induced, and aortotomy was done. One of the leaflets of the CEP was entirely collapsed and dislocated to the LV side, which caused acute aortic regurgitation. Although there was no evidence of endocarditis, slight calcification and small perforation of the leaflet of the valve was observed. Aortic valve replacement was performed with a mechanical heart valve but it was impossible to wean from ECC, and therefore we additionally performed mitral valve annuloplasty with a prosthetic ring for moderate mitral regurgitation. After 4 h cardiopulmonary assistance, ECC was successfully withdrawn. She was discharged in a good condition an post operative day 29th.

Background : The improvement in surgical results for congenital heart disease has resulted in an increase in the number of adult congenital heart disease (ACHD) cases. Some ACHD patients are known to develop thoracic aortic aneurysm (TAA) at a young age, so we examined TAA in ACHD patients presenting at our institute over a 10-year interval. Methods : From 2002 to 2011, we performed 32 cases of surgery for TAA in ACHD patients. We excluded 5 cases of adult bicuspid aortic valve, 2 of TAA with untreated congenital heart disease (CHD), 1 of Marfan syndrome with CHD, and 9 of coarctation of the aorta (CoA) repair for the same site ; 15 patients were included. Results : The male/female ratio was 13/2, and the age of reoperation was 33.3±10.8 years. The 15 ACHD patients included 5 cases of the tetralogy of Fallot (TOF), 4 of congenital aortic stenosis (AS), 3 of ventricular septal defect (VSD), and 1 of each CoA complex, polysplenia/double outlet right ventricle (DORV), and polysplenia/corrected transposition of the great arteries (cTGA). Twelve cases of root dilatation and 2 of ascending aortic aneurysm were observed and 10 cases were concomitant with moderate to severe aortic regurgitation. Thirteen cases underwent elective surgery and the other two cases were emergency surgeries : a Bentall procedure for type 2 acute aortic dissection of polysplenia/DORV, and a Bentall and right ventricular outflow reconstruction (RVOTR) for ascending aorta/right ventricle rupture due to Konno patch detachment in congenital AS. The 13 elective cases included 11 cases of Bentall procedure, 1 of ascending aorta/hemi arch replacement, and 1 of ascending aorta replacement. Concomitant procedures were 1 case of aortic valve replacement, 1 of mitral valve replacement, 1 of subaortic stenosis release, and 2 of RVOTR. Operation time was 572.8+/-101.4 min, cardiopulmonary bypass time was 295.8+/-100.2 min, and aorta clamp time was 188.1+/-58.8 min. One hospital death was observed in 1 emergency case due to methicillin-resistant Staphylococcus aureus (MRSA) sepsis, but no 30-day mortality was observed. Intensive care unit (ICU) stay was 9.4+/-10.1 days and hospital stay was 34.4+/-18.2 days. Conclusion : The most common ACHD found during TAA surgery in our institute was Tetralogy of Fallot. ACHD had various complications and restrictions for surgery but TAA surgery in ACHD patients was safe and feasible.