We report a rare case of aortic root replacement after arterial switch operation (ASO). Ten years after undergoing ASO, a 10-year-old boy underwent a Bentall operation because of progressive aortic valve regurgitation and aortic root dilation. The operation was performed under the division of the right pulmonary artery. This view made it easy and safe to dissect the coronary arteries and to perform aortic root surgery.

A sinus of Valsalva aneurysm is a comparatively rare disease, and it has almost no symptoms unless this is rupture, whereas aortic insufficiency, myocardial ischemia and heart failure might be associated with un-ruptured aneurysm of the sinus of Valsalva. We encountered 2 elderly patients (71 years old and 83 years old) with huge un-ruptured aneurysm of the sinus of Valsalva which causes right ventricular outflow tract obstruction. The orifice of the aneurysm of the sinus of Valsalva was closed using ePTFE patches in the both cases. Plication of aneurysm was attempted in both cases, but it failed in case 1 due to undetermined border of the aneurysm on the right side of the heart. Case 2 was required concomitant aortic valve replacement with a bioprosthesis due to associated aortic regurgitation. The repair of un-ruptured aneurysm of the sinus of Valsalva associated with right ventricular outflow tract obstruction can be performed safely and effectively even in elderly patients.

Eight patients with Takayasu's disease underwent cardiac surgery between 1983 and 1998. All were women and the age at the time of operation ranged from 42 to 68 years (mean, 53.8 years). They were divided into two groups according to the coronary artery involvement: group A (n=3) had aortic regurgitation with an intact coronary artery and underwent aortic valve replacement (AVR); group B (n=5) had coronary artery lesion and underwent coronary artery bypass grafting (CABG) concomitant with or without AVR. All AVR procedures were performed using mechanical valves. At the CABG operation, saphenous veins alone were used in three cases and the left internal thoracic artery and saphenous veins in two. The actuarial survival rate was 65.6% at 5 years and 32.8% at 10 years. There were no early or late deaths in group A. On the contrary, there were one hospital death and two late deaths in group B. We discussed the timing of surgical intervention, the kind of prosthetic valve, the material of bypass graft and the procedure of CABG, the postoperative steroid use, and the surgical prognosis. The optimal timing of surgery for cardiac involvement is, needless to say, the inactive phase of inflammation. However, there are some patients who require operations during the active phase because of medically intractable or worsening symptoms. There is a consensus regarding the kind of prosthesis, and the mechanical valve is usually employed. There are still controversies regarding the material of grafts. We do not know the late results of saphenous vein graft in Takayasu's disease although saphenous vein is thought to be the choice of graft and several CABG procedures are advocated. The left internal thoracic artery might be used as a graft if the patient with Takayasu's disease had no subclavian artery lesions and was stable with an antiinflammatory regimen. We recommend the postoperative steroid therapy to control inflammation and also describe the antiinflammatory regimen after cardiac surgery in Takayasu's disease. It is essential that we have to meticulously follow up the patients with Takayasu's disease who underwent cardiac operations, paying especial attention to the side effects of steroid as well as the progression of inflammation.

We performed the Senning operation and pulmonary valvotomy in an 11-month-old baby with transposition of the great arteries (TGA) with an intact ventricular septum (IVS), and bicuspid pulmonary valvular stenosis associated with pulmonary hypertension (PH). Preoperative catheterization showed a pressure gradient (PG) between the left ventricle (LV) and main pulmonary artery (MPA) of 35mmHg, mean pulmonary artery pressure (MPAP) of 56mmHg, and pulmonary vascular resistance (PVR) of 11.2unit·m². The pure oxygen inhalation test showed a decrease in MPAP from 56 to 38mmHg, and a decrease in PVR from 11.2 to 5.5 unit·m². We could not perform lung biopsy to determine the surgical indications in terms of PH due to preoperative progressive congestive heart failure in this patient. Postoperative catheterization (28 days after the Senning operation) showed a decrease in PG between the LV and MPA to 8mmHg, and MPAP also decreased to 17mmHg. Two radical operations were possible in this patient. One was the arterial switch operation (ASO), and the other was the atrial switch operation, i. e. the Senning or the Mustard operation. We selected the Senning operation because there was the possibility that the new aortic valve might develop persistent stenosis and regurgitation after ASO and pulmonary valvotomy. The Senning operation may be an alternative in selected patients with TGA with IVS and pulmonary valvular stenosis.

Coarctation of the aorta (CoA) complicates with right aortic arch (RAA) is very rare, and its surgical treatment in the neonatal period is extremely uncommon. We performed surgical repair for a 27-day-old boy given a diagnosis of double outlet right ventricle (DORV) and CoA with RAA. The procedures consisted of an arterial switch, intra-ventricular re-routing, aortic arch reconstruction using an equine-pericardial roll and right ventricular outflow reconstruction (RVOTR) with autologous pericardium. We performed re-RVOTR 41 days after the operation because the autologous pericardium used for RVOTR showed aneurysmal dilatation. After the second operation, this patient has done well.

Objective : To investigate the efficacy of aortic valve replacement with annular enlargement for congenital aortic valve stenosis. Methods : Eleven patients underwent aortic valve replacement with annular enlargement for congenital aortic valve stenosis in our institute between January 2002 and July 2012. The clinical status of these patients, including preoperative and postoperative echocardiography, was evaluated in this study. Results : The median age of the patients was 15.5 years (range : 9-38 years). The patients had a mean body surface area of 1.48±0.3 m² (range : 1.00-1.92 m²). Mechanical prostheses were used in all patients and the techniques of aortic annular enlargement were the Nick procedure in 4 patients, Manouguian procedure in 3 (modified Manouguian in 2), Yamaguchi procedure in 2, and Konno procedure in 2. The average follow-up period was 32.1 months (range : 1-117 months). There was neither operative death nor late death. The peak/mean pressure gradient of aortic valve improved from 77.9±31.7/46.6±18.0 mmHg preoperatively to 27.9±7.7/14.8±4.7 mmHg postoperatively and to 28.3±11.1/14.1±7.0 mmHg at intermediate-term follow-up. The estimated left ventricular mass also improved from 206.8±93.4 g preoperatively to 179.7±61.1 g postoperatively and to 100.4±76.3 g at intermediate-term follow-up, respectively. Conclusions : Our series shows the efficacy and safety of aortic valve replacement with annular enlargement for congenital aortic valve stenosis.

We describe our surgical treatment in a patient with subvalvular aortic stenosis due to pannus formation beneath a monocusp mechanical valve. In this case, transvalvular removal of subvalvular pannus using a CUSA (Cavitron ultrasonic surgical aspirator) was performed successfully. A 77-year-old woman underwent aortic valve replacement with a monocusp tilting-disk mechanical valve (Björk-Shiley, 23 mm) 30 years previously. Reoperation for severe aortic stenosis due to calcified subvalvular pannus formation was required. Intraoperative findings revealed no limitation of leaflet motion of the valve but presence of left ventricular outflow tract obstruction caused by subvalvular pannus formation under the major orifice of the prosthesis. Because of difficulty of exposure of the prosthetic valve due to severely calcified valsalva sinus wall, simple re-do aortic valve replacement seemed to be almost impossible. Therefore, we tried transvalvular removal of the pannus. A scalpel could not be applied due to severe calcification of the pannus. Then we used CUSA and removed the pannus successfully. Finally, subvalvular stenosis (LVOTO) was ameliorated and a decrease of trans-aortic valve velocity was recognized. She is doing well without recurrence 1.5 years after the surgery.

A 56-year-old man, who underwent aortic valve replacement with a stentless artificial valve for aortic valve endocarditis at age 52, found to have left ventricular outflow pseudoaneurysm by transthorasic echocardiography, transesophageal echocardiography and enhanced computed tomography. We repaired the pseudoaneurysm, combined with valve re-replacement. Left ventricular outflow pseudoaneurysm is a rare disease, and is often associated with active endocarditis. Transesophageal echocardiography and CT scan are useful to diagnose this disease, especially to rule out annular abscess. Operative indication is recommended soon after the diagnosis was made to prevent rupture of pseudoaneurysm, or development of either mitral regurgitation or coronary ischemia due to compression from the pseudoaneurysm. Combined aortic valve replacement, with or without mitral valve replacement is necessary to repair the pseudoaneurysm.

Intracardiac repair for cardiac anomalies associated with a transposed aorta from the right ventricle is a technically demanding operation. We present two cases of left ventricular outflow tract (LVOT) obstruction after the use of an ePTFE flat patch to reconstruct the LVOT. Case 1 : A 10-year-old boy had undergone the Rastelli operation, VSD enlargement, and intraventricular re-routing using an ePTFE flat patch for repair of the DORV with noncommitted VSD and pulmonary stenosis at the age of 5. Five years later, catheter examination revealed severe LVOT obstruction. Intraventricular re-routing using a part of the ePTFE graft concomitant with re-replacement of an extracardiac conduit was successfully performed. Case 2 : A 13-year-old girl had undergone a double-switch operation (Senning operation, the Rastelli operation, and intraventricular re-routing by the use of an ePTFE flat patch) for the repair of corrected TGA, PA and VSD at the age of 7. Six years later, catheter examination revealed severe LVOT obstruction. Intraventricular re-routing using part of the ePTFE graft concomitant with re-replacement of an extracardiac conduit was successfully performed. We consider that the use of a flat patch for reconstruction of a left ventricular out flow tract in cases with transposition of the aorta from the right ventricle involves a risk of future development of LVOT obstruction.

We have used absorbable suture material for the repair of pediatric cardiovascular anomalies where subsequent growth of the suture line was requied since 1986. We reviewed long-term results of three groups of patients using absorbable suture (1. cardiovascular surgery in the high-pressure areas, 2. semicircular annuloplasty for mitral regurgitation, 3. cardiovascular surgery in the low pressure areas). In group 1, six patients after Jatene procedure using PDS^® were examined. Subsequent growth of the neo-aortic anastomotis were well preserved without formation of pseudoaneurysm 6-56 (average 18.6) months after the operation. In group 2, seven patients with mitral regurgitation (mitral valve regurgitation 4, endocardial cushion defect 3) were examined. The semicircular annuloplasty was applied using Vicryl^® and Dexon^® and mitral regurgitation was improved and in two patients normal growth of the mitral annulus was recognized 4 and half years after the operation. In group 3, sixteen patients in the repair of total anomalous pulmonary venous drainage (Darling type I, III) using PDS^® were examined. Pulmonary venous obstruction were encountered 1 and 5 months after the anastomotis between common pulmonary vein and left atrium in two patients and anastomotic stenosis was thought to be induced by tissue overgrowth. On the basis of our clinical experiences, we believe that application of absorbable suture in pediatric cardiovascular surgery in the high-pressure areas could be recommended as an alternative treatment, but that should be avoided in the neonatal small low-pressure areas on the assumption that anastomotic stenosis might be induced by tissue overgrowth.