A 44-year-old man had been pointed out to have a heart murmur during his elementary school days. He came to us complaining of chest pain and palpitation. He was diagnosed to have severe aortic regurgitation caused by prolapse of the right coronary cusp, and the left ventricular function was depressed. At surgery, the aortic valve was repaired with the RCC resuspension technique, but regurgitation was not controlled because the RCC annulus was retracted by natural supracristal closure of the ventricular septal defect. We proceeded to the Ross procedure. A pulmonary autograft was harvested and sewed in the aortic annulus. The right ventricular outflow tract was reconstructed using a stentless bioprosthetic valve with glutaraldehyde-treated bovine pericardium. He was discharged after a straightforward postoperative course.

A 47-year-old woman was incidentally found to have a thoracic aortic aneurysm by CT scan. There was an aneurysm in the ascending aorta and aortic arch, and the Valsalva sinus was enlarged. Echocardiography showed a severe aortic regurgitation. Valve-sparing aortic root replacement and total arch replacement was performed. The pathology of the ascending aorta was consistent with giant cell arteritis. Her postoperative course was straightforward, and she was discharged 23 days after surgery. In Japan, there are few reports of valve-sparing surgery for patients with giant cell aortitis, and we report our experience of a rare case.

A 35-year-old man was referred to our hospital for surgical repair of grade IV/IV aortic regurgitation secondary to a congenital unicuspid aortic valve accompanied by aneurysm of the ascending aorta. The aortic valve was the unicuspid unicommissural type and a fully developed commissure was located in the left lateral position (left coronary/right coronary). The anterior (non-coronary/right coronary) and posterior (non-coronary/left coronary) borders were rudimentary with calcified raphe. We performed aortic valve repair in combination with valve sparing root replacement (reimplantation) and partial arch replacement. We converted the unicuspid into a bicuspid aortic valve by preserving his own free margin tissue and creating a neocommissure to the 180 degrees opposite side of the left lateral commissure at the same height by enlarging the cusp with a glutaraldehyde-treated autologous pericardium patch to the cusp belly. The patient was discharged on the 17th postoperative day with trace aortic regurgitation. We successfully repaired the unicuspid aortic valve by augmenting the cusp size using a pericardium patch in order to preserve the free margin of the cusp.

A 67-year-old man with ascending aortic aneurysm was referred to our hospital. Transthoracic echocardiography showed severe aortic regurgitation with annuloaortic ectasia and transesophageal echocardiography revealed a quadricuspid aortic valve. This patient underwent aortic root replacement with a valve sparing technique. Under deep hypothermic circulatory arrest with retrograde cerebral perfusion, replacement of the ascending aorta was successfully performed. The postoperative course was uneventful. This patient is doing well 6 months after surgery without recurrence of aortic regurgitation.

Idiopathic hypereosinophilic syndrome (IHES) is a rare systemic disease, but frequently associated with the eosinophil-infiltrated end organ (cardiac, hematologic, cutaneous, neurologic, pulmonary, splenic and thromboembolic) failure. Mechanical valve replacement for valvular heart disease in cases of IHES has the potential of thromboembolic accidents in the early post-operative period, even though the strict anti-coagulant therapy was performed. We reported a younger IHES case with congestive heart failure due to severe mitral regurgitation who underwent mitral valve repair instead of replacement. In this case, only the lateral scallop of the posterior mitral leaflet was involved with endocarditis due to the eosinophyl infiltration, with no other symptoms, i.e., initial hyperplasia and thrombus in the left ventricle that were detected in the most cases with IHES endocarditis. Mitral annuloplasty with the complete ring was simply performed because of the presence of sufficient intact anterior leaflet. The eosinophil count had been strictly controlled with imatinib mesilate in addition to the anti-coagulant therapy, and any thromboembolic event had been detected post operatively. In case of valvular heart disease due to IHES endocarditis, valve repair should be firstly attempt and imatinib mesilate is very useful and effective for preventing from post-operative thromboembolic accidents in FIP1L1-PDGFRα gene positive cases.

Destructive aortic valve endocarditis or poor controlled aortitis cause the development of left ventricular-aortic discontinuity. We reported our experience with aortic root replacement for cases of severe aortic annular destruction. Between 1999 and 2006, 9 patients with severe aortic annular destruction underwent aortic root replacement at our institute. There were 8 men and one women with a mean age of 55 years. Seven patients were in New York Heart Association functional class III. Four of 9 patients had native valve endocarditis, 4 had prosthetic valve endocarditis (previous aortic valve replacements in 2, aortic root replacements in 2) and one had active aortitis with a detached mechanical valve. Radical debridement of the infected cavity and necrotic tissue was performed in all cases, followed by reconstruction of the aortic annulus using autologous or xenogenic pericardium in 3 cases. Antibiotic-saturated fibrin glue was applied to the cavity. Aortic root replacement was achieved with a pulmonary autograft (Ross procedure) in 4 and stentless aortic root xenograft in 4. One patient who had advanced liver cirrhosis underwent aortic valve replacement with a stentless xenograft by subcoronary implantation method. No mortality was observed during hospitalization and follow-up. Reoperation within 5 years was not necessary in 66.7% of the patients. Excellent outcome can be achieved by radical exclusion of the abscess cavity and viable pulmonary autograft or stentless aortic root xenograft in patients with severe aortic annular destruction.

A 71-year-old woman who had Takayasu's disease underwent Y-grafting, bypass grafting between the abdominal aorta and left renal artery with 8mm ePTFE graft and right nephrectomy for infrarenal abdominal aortic aneurysm and renovascular hypertension (RVH). Four years after the first operation, the bypass graft became occluded and hypertension was exacerbated. Magnetic resonance angiography revealed that the left renal artery was supplied by the collateral arteries. We performed replacement of the thoracoabdominal aorta and reconstruction of the left renal artery using the saphenous vein. Postoperatively serum creatinine level decreased and hypertension was controllable. She was discharged from the hospital and has been well for three years.

A 52-year-old man who had liver cirrhosis sufferred ruptured thoraco-abdominal aortic aneurysm. This patient was classified as having Child's class B liver cirrhosis preoperatively. The thoracoabdominal aorta was successfully replaced with reconstruction of the renal arteries, superior mesenteric artery, celiac artery, and 10th intercostal artery. Omentopexy was added. As persistent ascites continued postoperatively, peritoneovenous shunting was performed on the 29th postoperative day. Ascites disappeared and 20 days later the patient was discharged from hospital and has been well for two years.

Infectious arterial aneurysm in a rare condition with a high mortality because of rapid aneurysmal growth and subsequent rupture. We encountered 3 cases of arterial aneurysm due to Salmonella infection. We evaluated there 3 cases and an additional 14 cases reported in the Japanese literature. Eleven patients with Salmonella infection had bacteremia. The incidence of bacteremia in patients with Salmonella infection was more frequent than that in patients with other bacillary infections. The location of the aneurysm was the abdominal aorta in 14. Rupture or impending rupture of the aneurysm was identified in 12. Fifteen patients underwent operation including in situ reconstruction in 9 and extra-anatomic bypass in 6. Among 15 patients who underwent an operation, 14 survived. These data suggest that accurate preoperative diagnosis, long-term antibiotic therapy, and immediate surgical intervention are essential for effective treatment of arterial aneurysm due to Salmonella infection.

A 73-year-old man who had undergone Y-grafting suffered from septicemia. A culture of arterial blood yielded Bacteroides fragilis. Computed tomography showed abscess and gas around the prosthetic graft. Under a diagnosis of a prosthetic graft infection caused by Bacteroides fragilis, removal of the infected graft, extra-anatomic bypass and omental grafting were performed 13 days after the first operation. After intensive treatment, he recovered and was discharged on the 45th day postoperatively.