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1/21. Coarctation of the aorta, aortic valvar stenosis, and supravalvar aortic stenosis with left coronary artery ostial stenosis: management using a staged hybrid approach.

    In this report, we describe a case of coarctation of the aorta, congenital aortic stenosis with bicuspid valve, and supravalvar aortic stenosis with left coronary artery ostial stenosis. The child underwent staged treatment using a hybrid approach employing both surgical and interventional cardiology methods. A single pericardial patch repair technique for supravalvar aortic stenosis associated with left coronary ostial stenosis is also described. ( info)

2/21. Aortobiventricular fistulas associated with pseudoaneurysm of the ascending aorta 12 years after patch repair of supravalvular aortic stenosis.

    fistula formation between the aorta and cardiac chamber is a rare complication of the ascending aortic aneurysm. A 27 year-old man undergoing successful patch aortoplasty for supravalvular aortic stenosis 12 years before admission had a high fever, infectious signs in blood laboratory data, and congestive heart failure. Transthoracic and transesophageal echocardiography revealed a pseudoaneurysm of the aortic root and aortobiventricular fistulas. Detachment of the proximal end of the aortic patch was thought to be the cause of the pseudoaneurysm. debridement of necrotic tissue surrounding fistulas produced large defects in the anterior wall of the right ventricle, interventricular septum, and ascending aorta. A modified Konno operation effectively reconstructed the outflow tracts of both ventricles and the aorta. ( info)

3/21. Acquired supravalvar aortic stenosis following heart transplantation: report of 2 cases.

    We report 2 cases of acquired supravalvar aortic stenosis (SVAS) complicating orthotopic cardiac transplantation that contributed to the post-operative death of both patients. In the first patient the stenosis resulted from kinking of a too-long graft aortic component that was rendered into a fixed deformity by healed mediastinitis and constrictive pericarditis. Supra-aortic stenosis in the second patient occurred at a site of multiple aortic cannulations that was surrounded by dense fibrosis. Acquired SVAS contributed to the death of the first patient (who had graft arteriopathy) and was the proximate cause of death in the second patient. Specific echocardiography of the ascending aorta, which is not routinely performed, may detect SAVS. ( info)

4/21. Supravalvular aortic stenosis and peripheral pulmonary stenosis coexisting with a straight thoracic spine.

    Supravalvular aortic stenosis (SVAS) is recognized in cases of williams syndrome and in sporadic cases not associated with other features of the syndrome. It is also well recognized as associated with peripheral pulmonary stenosis (PPS). A male patient was diagnosed as having PPS at the age of 1 year and 8 months, and was found at the age of 18 years to have SVAS. cardiac catheterization showed that he had a localized type of SVAS and regression of the PPS. Chest X-ray showed that he did not have the normal thoracic curvature. His 19-year-old sister had also been diagnosed with PPS, and his 43-year-old mother was known to have a harsh systolic cardiac murmur of unknown etiology. Cardiac magnetic resonance imaging showed a localized type of SVAS in his mother also, though not in his sister, both of whom had a somewhat straight thoracic spine, most noticeably in the mother, though not to the degree observed in the patient. This case appears to be familial, though it is not clear whether this skeletal abnormality is an unknown phenotypic feature of this cardiovascular disease. ( info)

5/21. Three-dimensional reconstruction of heart defects associated with Williams-Beurer syndrome.

    Williams-Beurer syndrome is an unusual genetic alteration associated with congenital heart defects. We present the capabilities of three-dimensional echocardiography in the study of the cardiopathy associated with this syndrome. ( info)

6/21. Supravalvar aortic stenosis with supravalvar pulmonary stenosis and peripheral vascular stenoses.

    A non-dysmorphic 10 month old female was discovered at surgery to have severe vasculopathy of both the systemic and pulmonary arteries. These findings were confirmed by pathologic examination. Follow-up angiography has confirmed multiple sites of vascular obstruction which appear to be worsening. angioplasty has only partially relieved these obstructions. The pathology and possible etiology are reviewed. ( info)

7/21. Mitral regurgitation without supravalvular aortic stenosis in williams syndrome.

    Isolated mitral regurgitation without supravalvular aortic stenosis is rarely identified in williams syndrome. We describe the case of a 24-year-old man with isolated mitral regurgitation in williams syndrome. Severe regurgitation due to prolapse of the anterior leaflet was noted in an echocardiogram and color Doppler, and a left ventriculogram showed grade IV regurgitation. No pressure gradient between the left ventricle and the ascending aorta was found. Mitral regurgitation had been noted since his birth, and pediatricians suspected williams syndrome because of postnatal growth deficiency, mental deficiency, unusual personality, and unusual facial features in his childhood. The diagnosis was confirmed by demonstration of the hemizygous deletion of 7q11.23 in the karyotype by the fluorescent in situ hybridization technique after his admission to our department. The patient underwent mitral valve replacement, and microscopic examination of the excised valve revealed myxomatous degeneration. ( info)

8/21. williams syndrome associated with complete atrioventricular septal defect.

    williams syndrome is a genetic disorder associated with characteristic facies, supravalvar aortic stenosis, peripheral pulmonary stenosis, mental retardation, hypertension, premature aging of skin, and congenital cardiac defects. Many cardiac defects such as bicuspid aortic valve, mitral valve regurgitation, coarctation of the aorta, and ventricular or atrial septal defects are linked to the syndrome. Complete atrioventricular septal defect has rarely been associated with williams syndrome and only one necropsy case has been reported in the literature. The long term follow up of williams syndrome associated with complete atrioventricular septal defect is reported. During a 10 year follow up period, the pressure gradient in the ascending aorta did not increase despite narrowing of the ascending aorta as identified on an aortogram. ( info)

9/21. Neonatal williams syndrome presenting as an isolated supravalvular pulmonary stenosis.

    An infant with normal facies and none of the extracardiac anomalies usually associated with williams syndrome presented at birth with an echocardiographic pattern of supravalvular pulmonary stenosis and displastic pulmonary valve. A clinical reappraisal was planned at 3 months of age, but the girl died suddenly at home at 2 months of age. At autopsy, both ventricles were hypertrophic, and the valves showed mild dysplasia. The walls of the great arteries were thick, with a "washed leather" consistency, but there was no gross evidence of discrete stenosis. The histologic mosaic appearance of the media of the great arteries, due to elastosis and extreme disarray of the elastic lamellae, prompted a postmortem diagnosis of supravalvar aortic stenosis and suggested a diagnosis of williams syndrome, which was subsequently confirmed by fluorescence in situ hybridization. Pediatricians and pathologists should be alerted that williams syndrome in the newborn may present as an isolated supravalvular pulmonary stenosis, whereas supravalvular aortic stenosis becomes clinically significant only a few months later. ( info)

10/21. Progressive diffuse aortic stenosis after localized supravalvular aortic stenosis repair.

    A 6-year-old boy presented with diffuse stenosis of the aortic arch. He had undergone patch augmentation for localized supravalvular aortic stenosis at 2 years of age. Before the initial operation, the aortic arch and descending aorta were noticed to be hypoplastic with a diameter of 60 to 73% of that of the aortic annulus, without a pressure gradient. 4 years later, progressive diffuse stenosis of the aortic arch with a diameter of 42% of that of the aortic annulus was found. reoperation, consisting of patch augmentation of the aortic arch, was carried out. A pressure gradient of 40 mmHg distal to the repair was newly recognized, again. This is a rare case of supravalvular aortic stenosis, which required reoperation because of the progression of diffuse stenosis of the distal aorta. ( info)
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