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1/108. Percutaneous fenestration of the aortic dissection membrane in malperfusion syndrome.

    We present two cases of malperfusion syndrome due to aortic dissection type-B. A supra-renal blind sac phenomenon resulted in renal failure and absent femoral pulses in both patients. Additionally, one patient suffered from spinal cord ischemia, the other from severe abdominal pain. By interventional techniques, catheter perforation of the blind sac was achieved. The resulting re-entries were enlarged with a balloon catheter. Distal perfusion without pressure gradients was restored by this technique in both patients and resulted in complete relief of symptoms. Percutaneous fenestration of the aortic dissection membrane may be an alternative to operative treatment in malperfusion syndrome.
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2/108. External iliac artery occlusion in posterior spinal surgery.

    STUDY DESIGN: Report of a case of external iliac artery occlusion occurring as a rare complication of lumbar posterior spinal surgery. OBJECTIVE: To clarify the cause of this rare complication and recommend methods for preventing its recurrence. SUMMARY OF BACKGROUND DATA: Several cases of central retinal artery occlusion after posterior spinal surgery have been reported, but there has been no reported case of external iliac artery occlusion after posterior spinal surgery. methods: A 65-year-old woman who had a 32-year history of systemic lupus erythematosus underwent posterior decompression and spinal fusion for degenerative scoliosis. She was supported on a Hall frame during the operation. Three hours after surgery, paralysis and sensory impairment of the left leg and cyanosis of the toes of the left foot were noted. The popliteal, dorsalis pedis, and posterior tibial pulses were not palpable. Angiography showed complete occlusion of the external iliac artery, and emergency removal of the thrombus was performed. RESULTS: One year later, neurologic symptoms were absent, and the leg vessels were normal. CONCLUSIONS: Prolonged direct pressure on the inguinal region during posterior spinal surgery on a Hall frame may cause external iliac artery occlusion. Early recognition and adequate treatment can prevent serious sequelae.
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3/108. Long-term effect of a new oral prostacyclin analogue, beraprost sodium, in isolated peripheral pulmonary artery stenosis in the adult.

    A 20-year-old woman was admitted with increasing breathlessness on exertion. A diagnosis of isolated peripheral pulmonary artery stenosis (PPS) was confirmed by right-sided cardiac catheterization and pulmonary angiography. We tried treatment with a newly developed oral prostacyclin analogue, beraprost sodium. Her symptoms gradually improved 1 month after treatment. After 1 year, we observed a decrease in pulmonary arterial pressure (from 118/16 [mean 59] to 87/13 [mean 40] mmHg) and systolic right ventricular pressure (from 125 to 90 mmHg). This is the first report to describe the chronic effect of oral prostacyclin analogue on isolated peripheral pulmonary artery stenosis.
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4/108. angioplasty and stenting of basilar artery stenosis: technical case report.

    OBJECTIVE AND IMPORTANCE: Symptomatic basilar artery stenosis has a poor prognosis. Treatment options are limited. Surgical bypasses are technically demanding and of no proven benefit. Percutaneous angioplasty is associated with a significant complication rate, because of intraplaque dissection, restenosis secondary to vessel recoil, and embolic phenomena. A new generation of intravascular stents that are flexible enough to navigate the tortuosities of the vertebral artery may provide a new therapeutic approach. We report a case of basilar artery stenosis that was treated using stent-assisted angioplasty. CLINICAL PRESENTATION: A 56-year-old woman experienced a vertebrobasilar ischemic stroke, from which she recovered. magnetic resonance angiography revealed severe proximal basilar artery stenosis. brain Neurolite-single-photon emission computed tomographic scans revealed significantly decreased perfusion of the brainstem. Endovascular intra-arterial pressure measurements revealed a 35-mm Hg gradient across the lesion. INTERVENTION: The patient underwent uncomplicated angioplasty and stenting of the proximal basilar artery, with excellent angiographic results. CONCLUSION: The availability of new flexible intravascular stents, allowing access to tortuous proximal intracranial vessels, provides a new therapeutic approach for patients with basilar artery stenosis. Long-term follow-up monitoring is required to assess the durability of this approach.
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5/108. Revascularization of femoropopliteal artery occlusion in Werner's syndrome. Report of a case and review of surgical literature in cardiovascular lesions.

    A patient with Werner's syndrome suffering from a chronic ulcer on the right ankle joint underwent femoropopliteal bypass and patch angioplasty combined with endarterectomy of the distal popliteal artery. Postoperative angiography showed satisfactory graft patency and distal run-off, and the ulcer improved. Femoropopliteal occlusive disease in Werner's syndrome tends to have poor run-off and the internal diameter of the popliteal artery with diffuse arteriosclerotic lesion is often too small to facilitate distal anastomosis. Therefore, a conventional bypass procedure is not always effective and an aggressive attitude is essential to obtain sufficient ankle blood pressure and improve the leg ulcer in Werner' syndrome.
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6/108. Dilated phase of hypertrophic cardiomyopathy with mid-ventricular obstruction after 20-year follow-up.

    This paper reports a case of dilated phase in hypertrophic cardiomyopathy with mid-ventricular obstruction. Following the first cardiac catheterization and endomyocardial biopsy, the patient was diagnosed as having hypertrophic cardiomyopathy (HCM) with mid-ventricular obstruction. He had been first diagnosed at the age of 38 years and was subsequently followed for 20 years. Echocardiogram revealed gradually progressive dilatation of the left ventricle, associated with disappearance of the mid-ventricular obstruction. The second cardiac catheterization and endomyocardial biopsy performed at the age of 58 disclosed that the patient was in the dilated phase of HCM with a dip-and-plateau pattern diastolic pressure trace.
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7/108. Variant angina pectoris.

    A patient with variant angina pectoris due to a pedunculated calcific mass extending from the aortic valve and resulting in intermittent obstruction of the left coronary ostia is reported. No atherosclerotic disease was demonstrated by coronary angiography. During attacks, marked ST segment elevation and episodes of tachycardia were associated with a moderate rise in pulmonary artery pressure. Replacement of the calcified aortic valve resulted in total relief of symptomatology.
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8/108. A case of pulmonary arteritis with stenosis of the main pulmonary arteries with positive myeloperoxidase-antineutrophil cytoplasmic autoantibodies.

    A 53-year-old woman was referred to our hospital with the main symptoms of productive cough, fever and exertional dyspnoea. Chest X-ray revealed enlargement of the left hilar shadow and cavitary infiltration in the right upper lobe. 99mTechnetium-macroaggregated albumin (99mTc-MAA) perfusion scintigram showed complete hypoperfusion through the entire right lung. A pulmonary angiogram revealed stenotic lesions in the right and left main pulmonary arteries. Right cardiac catheterization showed an elevated right ventricular systolic pressure. There was no evidence of systemic arterial lesions nor vasculitis. The patient was positive for myeloperoxidase (MPO)-antineutrophil cytoplasmic autoantibodies (ANCA) (168 EU). The mycobacterium avium complex sputum culture was positive. The pulmonary stenotic lesions were surgically resected. The resected pulmonary arterial lesions were pathologically diagnosed as non-specific vasculitis. The cavitary lesion disappeared 6 months after the surgery. Two years after the surgery, although the MPO-ANCA level had decreased to 12 EU, stenosis of the pulmonary arteries reappeared. It is suggested that the patient became positive for MPO-ANCA in association with the mycobacterium avium complex infection, and that the presence of MPO-ANCA may not be related to the development of pulmonary stenosis of the main pulmonary arteries.
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9/108. hypertension due to renal artery occlusion in a patient with antiphospholipid syndrome.

    We report an unusual case of renovascular hypertension in a 16-year-old boy with primary antiphospholipid syndrome (PAPS), admitted to our clinic for severe drug-resistant hypertension and hypokalemia. Hormonal investigation revealed secondary aldosteronism and positive captopril test for renovascular disease. aortography confirmed the occlusion of the left renal artery. After nephrectomy, normalization of blood pressure and secondary aldosteronism occurred. Presently the patient remains in good health, receiving warfarin anticoagulant therapy. PAPS is defined by the presence of antiphospholipid antibodies and recurrent thrombosis. Arterial thrombosis (29%) appears to be less prevalent than venous thrombosis. Thrombotic microangiopathy of the kidney is frequently observed but renal artery occlusion, as seen in our patient, is unusual.
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10/108. Severe arterial occlusive disorder and brachysyndactyly in a boy: a further case of Grange syndrome?

    We report on a 15-year-old boy with stenosis and occlusion of multiple cranial, renal, and celiac arteries, aneurysm of the basilar artery, bilateral cutaneous syndactyly between fingers IV-V, partial cutaneous syndactyly between fingers III-IV on the right hand, brachydactyly, and borderline mental retardation. The clinical course was characterized by recurrent abdominal pain, gastritis, and high blood pressure. The pattern of the clinical and radiological findings is different from fibromuscular dysplasia (FMD) and moyamoya disease, and highly suggestive of a syndrome described by Grange in four siblings (MIM#602531) Grange et al. [1998: Am J Med Genet 75: 469-480].
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