1/219. A case of aortitis syndrome and IgA nephropathy: possible role of human leukocyte antigens in both diseases. A 51-year-old woman, who had both aortitis syndrome (takayasu arteritis) and IgA nephropathy, presented with hypertension, fever, a high erythrocyte sedimentation rate, high c-reactive protein and serum IgG levels, proteinuria, and renal dysfunction. Renal arteriography showed stenosis and poststenotic dilatation at the origin of the right renal artery, as well as tortuosity of the left renal artery branches and marked atrophy of the left kidney. Renal biopsy showed IgA nephropathy with deposits of IgA, C3, and fibrinogen in the glomeruli and arteriolosclerosis. The present patient had human leukocyte antigen (HLA)-B 52, which is reported to be related to the aortitis syndrome, as well as HLA-DR 4, which is possibly related to IgA nephropathy, suggesting that HLA status may be involved in the pathogenesis of both diseases. ( info) |
2/219. Complete interruption of the aorta in Noonan's syndrome. A patient is presented who lived to age 60 with complete interruption of the aorta associated with Noonan's syndrome. On chest x-ray there were calcified mediastinal masses and bilateral rib notching. angiography demonstrated complete interruption of the aorta with markedly dilated tortuous intercostal arteries. The roentgenographic studies are correlated with the surgical and pathological findings. Noonan's syndrome is discussed. ( info) |
3/219. Interruption of the aortic arch in adults. The interruption of the aortic arch is a rare cardiac congenital disease; such patients may occasionally survive to adulthood without surgery. The associated intracardiac malformations may modify survival. Our aim is to report three young adults (18-19 years of age) with interruption of the aortic arch. Two had type C, and the other had type B with subpulmonic ventricular septal defect and pulmonary valve insufficiency. We review 106 cases collected from the medical literature into 3 groups: 1) the whole group of patients; 2) patients with isolated interruption of the aortic arch, and 3) patients with interruption of the aortic arch associated with ventricular septal defect. In the whole group we found 18 cases of interruption of the aortic arch type A, and 25 cases of interruption of the aortic arch type B; 37 cases of isolated interruption of the aortic arch and 43 cases associated with ventricular septal defect. Fifty percent of the patients died before 15 days of life (0.042 years). According to the cumulative frequency graphic, only 5% of the patients survived beyond the age of 5 years. We found no information to relate patient's survival rate and anatomic type of the interruption of the aortic arch. From adolescence, the survival of the patients with interruption of the aortic arch associated to septal ventricular defect was 7%, and a 14% survival was found in patients with isolated interruption of the aortic arch. No statistical difference was found between the means of the ages of these two groups (P > 0.25). ( info) |
4/219. Percutaneous transluminal angioplasty and stent placement for subclavian and brachiocephalic artery stenosis in aortitis syndrome. A 43-year-old man with progressive right common carotid, subclavian artery, and brachiocephalic artery stenoses due to aortitis syndrome is presented. The patient's right common carotid artery had been treated by percutaneous transluminal angioplasty (PTA) four times previously, but it was finally occluded. The right subclavian artery was treated by PTA once, which resulted in restenosis. The stenosis extended to the brachiocephalic artery. For this patient, PTA followed by stent placement was performed for the right subclavian and brachiocephalic artery stenosis. Because arterial stenosis is progressive in cases of aortitis syndrome, simple PTA alone does not appear to be sufficient for treatment. We suggest that PTA followed by stent placement may be an alternative treatment for recurrent stenosis in aortitis syndrome. ( info) |
5/219. Esophageal cancer associated with right aortic arch: report of two cases. We report herein two cases of thoracic esophageal cancer with a right aortic arch. Both cases were elderly males who complained of dysphagia. Computed tomography and aortography revealed a right aortic arch with a mirror-image branching and a diverticulum of the right descending aorta in both cases. They underwent an esophagectomy and a mediastinal lymph node dissection in a left thoracotomy, and we also added a median sternotomy in the second case for a lymph node dissection in the right mediastinum. The ductus arteriosus was divided in the first case, but he died of a rupture of the aortic diverticulum 13 months later. In conclusion, for a successful esophagectomy and mediastinal lymph node dissection, a median sternotomy should be added to the left thoracotomy, while careful attention should also be paid to the aortic diverticulum and the ductus arteriosus. ( info) |
| Obstruction of the right coronary ostial anastomosis is a rare late complication after composite graft replacement of the ascending aorta and the aortic valve with separate Dacron coronary grafts (Cabrol method). Occlusion at the right coronary ostial anastomosis in a 36-year-old woman with aortitis syndrome who underwent a composite graft with a Dacron coronary graft is described. She underwent a third successful operation for right coronary reconstruction by minimally invasive direct coronary artery bypass grafting technique using the right gastroepiploic artery. This approach is likely to be extremely useful in avoiding resternotomy and cardiopulmonary bypass in patients requiring coronary reoperation. ( info) |
7/219. Total aortic replacement for aortitis syndrome: report of a case. We report herein the case of a 35-year-old woman who was administered steroid therapy based on a diagnosis of aortitis syndrome. Despite this treatment, the symptoms of cardiac failure gradually progressed, and she was referred to our hospital to undergo surgery. Preoperative evaluations revealed a large aneurysm extending from the ascending aorta to the thoracoabdominal aorta. A two-staged total aortic replacement was carried out and postoperative digital subtraction angiography showed excellent reconstruction of the entire aorta. ( info) |
| We performed surgery on a 61-year-old woman who had increasingly severe right shoulder pain and paresthesia in her right upper extremity as a result of a large right subclavian artery aneurysm. She had suffered from aortitis syndrome for 10 years for which she was treated with steroids and had multiple arterial lesions, including bilateral subclavian artery aneurysms, abdominal aortic aneurysm and obstruction of bilateral superficial femoral arteries. The right subclavian artery aneurysm measured 4 cm in diameter and rupture appeared imminent, prompting surgical therapy. Via the supraclavicular incision approach and additional partial sternotomy, the aneurysm was excluded and the brachiocephalic to right axillar arterial bypass was set up using an extended polytetrafluoroethylene graft. The patient recovered without complications and a subclavian artery aneurysm demonstrated by computed tomography was thrombosed 1 month after surgery. In conclusion, we recommend the exclusion technique to treat subclavian artery aneurysms in cases in which aneurysmectomy is likely to injure adjacent veins and nerves. ( info) |
9/219. Atherosclerotic disruption of the aortic arch during coronary artery bypass operation. A 70-year-old-man presented with a symptomatic three vessel coronary artery disease and was scheduled for myocardial revascularization. During extracorporeal circulation an intrathoracal bleeding occurred and aortic rupture was suspected. An iatrogenic plaque rupture in the concavity of the aortic arch was found due to cannulation attempts. The aortic arch was grafted in the so-called elephant trunk technique. Thereafter bypass grafts were anastomosed to the stenosed coronary arteries. The patient was discharged from hospital after 2 weeks in good condition. ( info) |
10/219. Evidence for early vessel involvement in the dysfunctional myocardium of Takayasu's arteritis. A 15-year-old girl presented with persistent fevers, night sweats, leukocytosis, an elevated erythrocyte sedimentation rate, and a 13-pound weight loss over 2 months. Duplex Doppler scans, computed tomographic scan, and magnetic resonance imaging studies were suggestive of Takayasu's arteritis. Left ventricular dysfunction occurred during the episode of active disease, and an endomyocardial biopsy demonstrated increased HLA-DR (human leukocyte antigen-DR) on the endothelium and evidence of immune complex deposition in the walls of small vessels. One year later, after treatment with corticosteroids and resolution of clinical symptoms, repeat endomyocardial biopsy revealed focal interstitial fibrosis and persistent immune complex deposition. These results indicate that the inflammatory, vasculitic process affecting the large vessels in Takayasu's arteritis may also involve the endomyocardium and its small vessels resulting in ventricular dysfunction. ( info) |