Cases reported "Aortic Arch Syndromes"

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1/10. 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).
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2/10. Persistence of hypoplastic and recoarcted fifth aortic arch associated with type A aortic arch interruption: surgical and balloon angioplasty results in an infant.

    A rare aortic malformation, the persistence of the fifth aortic arch (PFAA) associated with coarctation of the aorta, is reported in a 3-month-old male infant who underwent an emergency surgical intervention at 10 days of life, to relieve a severe aortic coarctation by pericardial patch technique. A successful balloon angioplasty was performed 2 months later eliminating a recoarctation. The gradient fell from 77 mmHg to 0, but a definitive surgical intervention for recoarctation was necessary 14 months after angioplasty.
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3/10. Coronary arterial narrowing in Takayasu's aortitis.

    A patient with Takayasu's aortitis and angina pectoris due to severe narrowing of the right and left coronary arterial ostia is described. Takayasu's arteritis produces a panaortitis, with thickening of the adventitia predominating, and an inflammatory cell infiltrate involving the adventitia, outer media and vasa vasorum. Narrowing of the coronary arteries in this disease is due to extension into these arteries of the processes of proliferation of the intima and contraction of the fibrotic media and adventitia that occur in the aorta. The distal coronary arteries usually do not manifest arteritis and are normal in caliber. angina pectoris may be the first symptom of the disease if the coronary arteries are the initial site of severe arterial narrowing. The coronary arterial bypass graft operation is effective therapy for treating coronary arterial narrowing due to Takayasu's arteritis.
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4/10. A case of Takayasu's arteritis associated with membranoproliferative glomerulonephritis and nephrotic syndrome.

    We describe a 57-year-old Japanese female who had Takayasu's arteritis associated with nephrotic syndrome due to membranoproliferative glomerulonephritis. Although a focal and segmental mesangial proliferative glomerulonephritis associated with Takayasu's arteritis has been described, membranoproliferative glomerulonephritis has not been reported previously in this condition.
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5/10. glomerulonephritis associated with Takayasu's arteritis: report of three cases and review of literature.

    Clinical features and pathologic findings of the kidney in three patients with Takayasu's arteritis and associated nephritis are described. Clinical evidence of renal disease included proteinuria, hematuria, and a reduction of glomerular filtration rate. Renal histology revealed mesangial proliferative glomerulonephritis in one patient and minor glomerular abnormalities in the other two. The clinical course of the glomerulopathy is slow, yet progressive, and the corticosteroid and immunosuppressive therapy appear to delay the progression of the glomerular damage. In one patient in whom repeated examination of the kidney after corticosteroid and immunosuppressive treatment was possible, the glomerular pathology remained unchanged. This report of three patients with Takayasu's arteritis and concomitant nephritis supports the previous speculation of the association between these two conditions. The glomerular, vasculitic, and arteriopathic involvements in these patients may suggest a common immunologic mechanism.
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6/10. Surgical treatment of Takayasu's disease.

    Thirty-nine patients (mean age: 33 years) with Takayasu's disease were observed over the past 8 years. Among these patients, 33 had surgical intervention with a mean follow-up of 4 years. Lesions of the aortic arch were the most common (29 patients, 69 lesions) and frequently were associated with lesions in another site as well. However, in this group of patients, the infrequent presence of signs of cerebral vascular insufficiency limited the number of suitable surgical candidates to 14 patients. Lesions of the renal arteries were noted in 25 patients (37 lesions) and were associated with coarctation of the thoracoabdominal aorta in 12 patients. In contrast to lesions of the aortic arch, the presence of severe hypertension was a frequent indication for surgery in Takayasu's disease. Twenty-one patients had operation. Twenty of 21 patients were considered cured or improved of hypertension. The one patient who received no benefit was the only operative death of the study. In conclusion, although Takayasu's disease is progressive and the life expectancy is foreshortened, hypertension secondary to aortic and renal artery lesions is a frequent and important indication for aggressive operative treatment.
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7/10. Membranoproliferative glomerulonephritis in Takayasu's arteritis.

    The occurrence of glomerulonephritis (GN) in patients with Takayasu's arteritis (TA) is rare. We describe a 28-year-old man with TA who presented with mesangial proliferative GN and subsequently developed membranoproliferative GN with nephrotic syndrome and deterioration of the renal function. The former is the most common type of GN complicating TA, while the latter has not been adequately reported in association with this disease. Although the mechanisms for the initiation and progression of these glomerular injuries are not clear, the present case suggests that a wider spectrum of GN can be seen in patients with TA and that cases of TA with urinary abnormalities should be followed carefully.
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8/10. Coronary ostial stenosis in Takayasu's arteritis.

    A patient with Takayasu's arteritis with left coronary ostial narrowing is presented. The dramatic clinical and pathologic findings are discussed in detail. Emphasis is placed on making the diagnosis as soon as possible, in order to expedite bypass surgery to prolong life.
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9/10. Renal histological studies in patients with Takayasu's arteritis. Report of 3 cases.

    Clinical findings and structural alterations of the kidney in 3 patients with Takayasu's arteritis (TA) and associated glomerulonephritis are described. Clinical evidence of renal disease included persistent proteinuria and microscopic hematuria in all patients. Renal histology showed proliferative glomerulonephritis in 2 of the 3 patients. In 1 patient in whom sequential examination of the kidney was possible, glomerular changes had progressed in severity, in parallel with the expansion of arterial damage of TA. prednisolone therapy induced a complete disappearance of systemic symptoms of TA and an improvement of proteinuria and hematuria. These findings suggest that TA, which quite possibly results from an immune response to disseminated antigen(s), may occasionally induced glomerulonephritis as a part of its histological expression.
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10/10. Traumatically injured aortic arch repaired under profound hypothermia and circulatory arrest.

    A case report has been presented to illustrate that profound hypothermia with complete circulatory arrest may be used as an effective method to control massive hemorrhage and to repair lacerations resulting from trauma to the aortic arch. This method should be considered for control of bleeding in intrathoracic life-threatening situations.
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