A 39-year-old woman was found to have a heart murmur by a medical examination at age 37. During a checkup at our hospital, echocardiography revealed mild aortic valve regurgitation (AR) and mild mitral valve regurgitation (MR). When she was 39 years old, echocardiography revealed severe MR and moderate AR. Based on preoperative examinations, antiphospholipid syndrome (APS) was diagnosed. Therefore, she received high-dose prednisolone therapy and underwent plasma exchange before the surgery. We performed double valve replacement using a bioprosthetic valve. On the first postoperative day (POD 1), the number of platelets suddenly decreased. We diagnosed catastrophic APS, and treated her with high-dose prednisolone, high-dose immunoglobulin and plasma exchange. Her blood platelet gradually increased on POD 3. Although she needed time for rehabilitation, she was discharged from our hospital on POD 88. APS can cause a catastrophic event triggered by an operation. Therefore, stringent pre- and postoperative management is necessary in patients with APS.

A 72-year-old man presented with abdominal aortic aneurysm (AAA) and was referred to our hospital by his physician. A computed tomography revealed a 95-mm AAA with three right renal arteries. The main right renal artery branched from the AAA, and two remaining arteries branched from the same level as the left renal artery. The patient underwent AAA repair and main right renal artery reconstruction without any renal protection. After the operation, renal function did not deteriorate. 3D-CT was useful for diagnosing renal artery branching, evaluation of renal blood perfusion, and determining the operation method.

A 69-year-old woman was admitted with severe hypertension and intermittent claudication. The results of further examination, showed that the hypertension and intermittent claudication were due to stenosis of the descending aorta and we diagnosed atypical aortic coarctation. We performed median sternotomy and ventrotomy with side-to-end anastomosis a woven Dacron graft and the ascending aorta. The graft was passed through the lesser omentum, and mesocolon and to abdominal aorta. The postoperative state was stable, and the hypertension and intermittent claudication were remarkably ameliorated. Many cases of extra-anatomical bypass were reported, and the ascending aorta-abdominal aorta bypass was useful method and, very successful with no complications in this case.

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.