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.

A 69-year-old man was referred to our hospital because of a right upper mediastinal mass observed on chest roentgenogram. Computed tomography showed a dissecting aneurysm of the right subclavian artery and dissection of the ascending aorta. Furthermore, the ascending aorta was dilated. We subsequently reconstructed the right subclavian artery with a bypass graft and replaced the ascending aorta. Two-thirds of the aortic arch was placed in deep hypothermic circulatory arrest with retrograde cerebral perfusion. His postoperative course was uneventful with no neurological complications. While subclavian artery aneurysms are relatively rare in comparison to other peripheral artery aneurysms, subclavian artery aneurysms with aortic dissections are even rarer. The most important concerns during subclavian artery aneurysm repair are the method of surgical approach and the maintenance of sufficient cerebral flow. We suggested that deep hypothermic circulatory arrest with retrograde cerebral perfusion might prove useful in cases involving an intramural thrombus adherent to cerebral vessels. Therefore, patients with subclavian artery aneurysms must undergo extensive preoperative evaluation.