[Stanford Type A Acute Aortic Dissection with Leg Ischemia and Visceral Ischemia].

A 52-year-old man developed Stanford type A acute aortic dissection and suffered severe pain in the back and the chest, and numbness of the left lower limb. Contrast-enhanced computed tomography (CT) revealed occlusion of the left iliac artery due to dissection. The celiac artery was also obstructed and the superior mesenteric artery was severely narrowed, but there was no clear sign of organ ischemia. Endovascular treatment in the iliac artery was performed, but the patient complained of right upper abdominal pain 6 hours after the procedure. Serum liver enzyme levels were significantly elevated. Repeat contrast-enhanced CT revealed liver infarction, ischemic cholecystitis, and duodenal ischemia due to decreased collateral flow. We immediately carried out cholecystectomy and revasculariza-tion of the celiac and superior mesenteric arteries using a saphenous vein. The patient underwent central repair for aortic dissection several days after recovery of liver function, and was discharged uneventfully 24 days later.

[Complete Anterior Papillary Muscle Rupture with Angiographically Normal Coronary Arteries;Report of a Case].

We present a case of complete anterior papillary muscle rupture (PMR) due to localized papillary muscle infarction, without significant coronary artery disease. A 69-year-old woman was transferred to our hospital because of acute orthopnea and cardiogenic shock. Echocardiography showed severe mitral regurgitation due to anterior PMR, and emergency coronary angiography revealed no obstructive disease in the coronary arteries. Emergency mitral valve replacement was performed with intra-aortic balloon pumping support. Intraoperatively, the anterolateral papillary muscle was found to be completely ruptured. With maximal posterior leaflet preservation, mitral valve replacement was successfully performed. Her postoperative course was uneventful. Pathologic examination showed both old fibrosis and new ischemic lesion in the same resected papillary muscle. It could be speculated that repeated localized subendocardial infarction caused so-called spontaneous PMR.

[Extended Sandwich Patch Repair with the Right Ventricular Approach for Posterior Ventricular Septal Perforation].

Ventricular septal perforation(VSP) is a catastrophic complication of acute myocardial infarction, and posterior VSP reportedly has worse outcomes. We present a case of a 79-year-old man with posterior VSP following acute myocardial infarction. He underwent percutaneous coronary intervention for acute postero-inferior myocardial infarction due to occlusion of the mid-portion of the right coronary artery. Ultrasound Cardiography (UCG) revealed the VSP on the posterior ventricular septum with right ventricle(RV) inferior wall akinesis. Surgical repair was planned 4 weeks later because his hemodynamic state remained stable without intraaortic balloon pumping (IABP) support. Post-infarction posterior VSP was successfully repaired through inferior right ventricular incision. Two patches were fixed to the ventricular septum using the sandwich patch technique with BioGlue surgical adhesive reinforcement. The postoperative course was uneventful, and there was no residual shunt after the operation. This method of approaching via right ventricular incision was considered to be useful in patients who have severe necrosis near the free-wall side of the posterior VSP.

IgG4-related Periarteritis Successfully Diagnosed by an Alternative Prostate Biopsy.

A 56-year-old man was referred to our facility after developing edema in his right leg. Enhanced computed tomography (CT) revealed a periarterial soft tissue mass surrounding the right iliac artery compressing the iliac vein. His elevated serum IgG4 level indicated IgG4-related disease (IgG4-RD). Both a percutaneous and surgical biopsy of this periarterial lesion proved challenging and were not pursued. A prostate biopsy, however, finally provided a histological diagnosis of IgG4-RD. Oral steroid therapy successfully resolved his leg edema and periarterial lesion. This case illustrates the usefulness of an alternative prostate biopsy for the histological diagnosis of IgG4-RD when approaching the primary affected lesion is difficult.

[Two Surgical Cases of Loeys-Dietz Syndrome in Childhood].

Loeys-Dietz syndrome( LDS) is a recently recognized autosomal dominant connective tissue disorder. Mutations in the genes encoding transforming growth factor-beta( TGF-ß) receptor 1 and (2 TGFBR1, TGFBR2)have been associated with LDS. We report here 2 cases of LDS in childhood. Case 1 was a 10-year-old man, who had aneurysm of both the pulmonary trunk and the ascending aorta, associated with pulmonary and aortic valve insufficiency. Surgical repair was performed successfully at the age of 17. The aortic valve was replaced with a mechanical valve. The aneurysmal ascending aorta was replaced with a Dacron graft. Pulmonary valvuloplasty and pulmonary arterioplasty was performed. Case 2 was a 3-month-old female infant, who had a patent ductus arteriosus( PDA) and aortic root dilation. A detailed physical examination revealed hypertelorism, bifid uvula, retrognathia, talipes equinovarus, and camptodactyly. Computed tomography and echocardiography demonstrated PDA, Valsalva sinus dilation, and arterial tortuosity. These findings were consistent with the clinical manifestations of LDS. Surgical ligation and clipping of the PDA was performed with good results. A molecular genetic analysis subsequently demonstrated a heterozygous missense mutation of the TGFBR2. Since aortic dissection occurs at smaller aortic diameters, early diagnosis and close monitoring are important for patients with LDS.

[Pediatric case of congenital coronary artery fistula; surgical result and late changes in coronary artery aneurysm].

Congenital coronary artery fistula is an uncommon heart anomaly involving the coronary arteries. We report here a case of a 4-year-old boy who had a coronary fistula from the right coronary artery to the right ventricle, with a coronary aneurysm. He was asymptomatic, but the calculated ratio of pulmonary blood flow to systemic blood flow was shown to be high [pulmonary flow (Qp)/systemic flow(Qs)=1.78]. The coronary angiography showed that the right coronary artery was dilated beginning at the ostium and had an aneurysm at the acute marginal portion. A large spherical aneurysm approximately 20 mm in diameter was found to have been connected with coronary fistula opening into the right ventricle. Surgical repair by closure of the fistula under direct vision, partial resection and suture closure of the aneurysm was performed. Plication of the proximal portion of the right coronary artery was not performed, and the diffusely dilated artery was left untouched. After this operation, he recovered well under anticoagulant treatment with warfarin and aspirin. Postoperative angiography was performed 17 months after the surgery to evaluate morphological changes in the coronary artery. The angiography confirmed the closure of the fistula and the regression of coronary artery dilatation.

[Redo off-pump coronary artery grafting through left thoracotomy].

An 80-year-old man successfully underwent reoperative coronary artery bypass grafting( CABG) via left thoracotomy without cardiopulmonary bypass. The proximal end of the saphenous vein graft( SVG)was connected to the thoracic aorta using an automated proximal anastomosis system. Then the SVG routed beneath the pulmonary hilum was anastomosed to the high lateral branch and the anterior descending artery in a sequential mode. He recovered uneventfully and is free of chest pain after redo CABG. In selected patients, redo CABG for the left coronary artery can be safely performed through left thoracotomy.

Limited resection for noninvasive bronchioloalveolar carcinoma diagnosed by intraoperative pathologic examination.

BACKGROUND: The establishment of limited resection procedures for non-small cell lung cancer is expected. Many groups have suggested noninvasive bronchioloalveolar carcinoma (BAC) to be a potential indication for limited resection. METHODS: We designed a prospective phase II study evaluating limited resection for noninvasive BAC diagnosed by intraoperative pathologic examination. From 1999 to 2007, limited resection was the procedure in 46 patients (16 men and 30 women; median age, 69 years; range, 49 to 83) who were diagnosed intraoperatively as having noninvasive BAC. The first end point was the predictive value of the intraoperative pathologic examination for noninvasive BAC diagnosis. The second end point was overall survival, disease-free survival, and cancer-specific survival, calculated using the Kaplan-Meier method. RESULTS: We performed wedge resections for 44 patients and segmentectomy for 2 patients. Permanent pathologic examination revealed 3 patients had primary lung adenocarcinomas other than noninvasive BAC. The predictive value of intraoperative pathologic examination for noninvasive BAC diagnosis was 94%. During a median 51-month follow-up, there were only 2 cancer unrelated deaths. The 5-year overall survival rate and the disease-free survival rate were 93%, and the 5-year cancer-specific survival rate was 100%. CONCLUSIONS: The results of our prospective phase II study indicate that limited resection, mainly by wedge resection, is a potentially curative surgical procedure and may be an acceptable alternative to lobectomy for patients with noninvasive BAC. Furthermore, an intraoperative pathologic diagnosis of noninvasive BAC is strongly predictive and allows for an intraoperative decision to perform a limited resection in these patients.

Primary malignant schwannoma of the lung.

A 72-year-old male referred for surgical treatment underwent chest computed tomography to further investigate a well defined mass with a maximal diameter of 3.2 cm at the left S10. There was no pathological diagnosis established by either bronchoscopic biopsy specimens or computed tomography-guided percutaneous needle biopsy. The lower lobe and mediastinal lymph nodes were excised (complete resection). The 6.5-cm encapsulated grayish mass showed extensive areas of hemorrhage and necrosis on cut surface. Results from histological and immunohistochemical study yielded a definitive diagnosis of malignant schwannoma. Primary malignant schwannomas of the lung are extremely rare and the prognosis is poor in most cases. Our patient is currently well without recurrence more than 2 years after complete resection. Complete surgical resection is the best therapeutic option for primary malignant schwannoma of the lung.