[Concomitant Operations for Thoracic Aortic Aneurysm and Myasthenia Gravis;Report of a Case].

A 77-year-old man, who had been under medical treatment for myasthenia gravis without thymoma, was diagnosed with aortic arch aneurysm. He underwent total aortic arch replacement and total resection of the thymus through median sternotomy. His symptoms relating to myasthenia gravis dramatically disappeared after the surgery. The serum anti-acetyl chorine receptor antibody decreased from 2.7 to 0.7 nmol/l (N<0.2) with the reduction of oral predonisolone from 12.5 to 5 mg/day at 4 years after the surgery. The concomitant operations significantly improved his quality of life.

Capillary Degeneration and Right Ventricular Remodeling Due to Hypoxic Stress with Sugen5416.

BACKGROUND: Sugen5416 (semaxinib) is an inhibitor of the vascular endothelial growth factor (VEGF) receptor. A rat model of Pulmonary Arterial Hypertension (PAH), created with Sugen5416 and chronic hypoxia, is known to have similar histological findings to those of PAH patients. OBJECTIVE: To evaluate the pathophysiological mechanisms of cardiac remodeling due to hypoxic stress with Sugen5416 in vivo. METHODS: Male Sprague-Dawley rats were exposed to hypoxia (10 ± 1% O2) for 2 weeks after a single injection of Sugen5416 (SU-hypoxia group) or the vehicle (V-hypoxia group). RESULTS: Hypoxia elevated right ventricular (RV) systolic pressure and caused RV remodeling on Day 14. By electron microscopy, metamorphosis of capillaries with endothelial cell occlusive degeneration was observed in the RV myocardium of the SU-hypoxia group from Day 3. After reoxygenation, progressive RV remodeling with extensive degeneration of cardiomyocytes was observed in the SUhypoxia group, associated with a significant increase of oxidative stress and TUNEL-positive cells in both RV and left ventricular myocardium on Day 84. The expression of VEGF mRNA in the RV myocardium was significantly suppressed in the SU-hypoxia group on Day 3, whereas delayed activation of VEGF/extracellular signal-regulated kinase (ERK) signaling pathway on Day 14 were observed. CONCLUSION: Capillary degeneration and activation of VEGF/ERK signaling pathway might be crucial to accelerate the cardiac remodeling due to hypoxic stress with Sugen5416.

[Left ventriculoplasty in a patient with suspected takotsubo cardiomyopathy followed by a left ventricular aneurysm].

A 42-year-old woman was admitted with chest pain. Coronary angiography did not reveal any significant stenosis, but left ventriculography showed akinesis and ballooning of the apex with a hyperkinetic basal segment indicating Takotsubo cardiomyopathy. Cerebral embolism occurred after one and a half years because of a left ventricular thrombus. The apical akinesis had worsened to a left ventricular aneurysm (maximum diameter 43 mm). The left ventricle was reconstructed to avoid repeated thrombus formation and cerebral infarction despite anticoagulant therapy. A pathological assessment revealed a fibrotic myocardium, but the cause of the cardiac aneurysm remained unknown. Although the outcome of Takotsubo cardiomyopathy is relatively good, careful observation and appropriate treatment are needed considering the possibility of aggravation.

[Aortic regurgitation in a patient with polyarteritis nodosa].

Polyarteritis nodosa (PN) is vasculitis of small- to medium-sized arteries. A 57-year-old woman with PN developed aortic valve regurgitation. Aortic valve replacement (AVR) was performed. We found inflammatory change of the ascending aorta which was rarely involved in PN. Abnormal thickness of the aortic wall was recognized during operation, which had not been detected by preoperative computed tomography. Perioperative course was uneventful, and the patient underwent anti-inflammatory therapy after the operation. No perivalvular leakage has been detected for 3 years since AVR. We should consider the possibility of aortitis, when cardiovascular operations are performed in patients with vasculitis for small- to medium-sized arteries.

[Malignant disease identified after the emergency cardiac operation].

We experienced 2 patients who died early after emergency cardiac operation because of malignant diseases. In one case, we operated on a 67-year-old woman for infective endocarditis and performed aortic valve replacement, but she died of terminal pancreatic cancer on the 26 postoperative day. In the other case, a 53-year-old woman underwent emergency operation for cardiac tumor of the left atrium. However, the tumor was diagnosed as cardiac sarcoma during the operation, and it could not be completely resected. The sarcoma recurred after 2 months and she passed away. An emergency cardiac operation without enough preoperative examinations sometimes causes a poor prognosis.

Minimizing cerebral embolism in resection of distal aortic arch aneurysm through a left thoracotomy.

BACKGROUND: In order to reduce the risk of cerebral embolism during aortic replacement through a left thoracotomy, we performed ascending or arch aortic cannulation (AAC) as well as early extracorporeal perfusion (EEP) under deep hypothermic circulatory arrest (DHCA). In this study we examined the effectiveness of these modifications in preventing cerebral embolism after distal arch replacement. METHODS: Between January 2006 and March 2010, 40 patients underwent distal arch replacement through a left thoracotomy, using 2 pieces of an artificial graft. In all patients, AAC, EEP, and the open technique for aortic anastomosis were performed under DHCA. The AAC resulted in the proximal aortic perfusion from the proximal site of the diseased aorta. The EEP was induced by aortic distal perfusion from the side branch of a distal graft. After completion of the proximal anastomosis under EEP and DHCA, anastomosis between the proximal and distal grafts was made during rewarming. Neurologic deficit in the brain and spinal cord, as well as early surgical results, were clinically evaluated. RESULTS: There was no permanent neurologic deficit after the surgery in the operative survivors. No patient had a stroke (0%). Temporary paraplegia and paraparesis occurred in 1 and 2 patients, respectively (7.7%); all 3 patients were able to walk prior to their discharge from hospital. Mortality in this series was 5.0% (2 of 40 patients); the cause of death was rupture of an esophageal ulcer and cardiogenic shock possibly due to myocardial infarction. CONCLUSIONS: The AAC and EEP, in addition to deep hypothermia and DHCA, minimized the risk of cerebral embolism after distal arch aortic replacement by the left lateral approach.

Successful surgical treatment for dilated cardiomyopathy with cardiac sarcoidosis.

We report a surgical case of severe left ventricular dysfunction due to cardiac sarcoidosis. A 45-year-old man who underwent pacemaker implantation for complete atrioventricular block was admitted to the hospital because of dyspnea due to congestive heart failure. Echocardiography revealed a left ventricular ejection fraction of 11%, with severe mitral insufficiency and thinning of the ventricular septum. He was successfully treated by anteroseptal ventricular exclusion, mitral and tricuspid annuloplasty, and bi-ventricular pacing. Postoperative pathologic study revealed noncaseating granulomas. The patient was referred to a cardiologist for further treatment with prednisone.