Multiplane transoesophageal echocardiography is the only definitive ultrasound approach in adult supravalvular aortic stenosis

Schürger, D.; Bartel, Thomas; Müller, Silvana; Gliech, V.; Baumann, Gert

doi:10.1016/0167-5273(96)02563-6

Cited by 3 publications

(1 citation statement)

References 8 publications

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“…Recently, a new noninvasive approach, multiplane transesophageal echocardiography (TEE), has been used to identify the SVAS lesion with clarity and determine pressure gradients with the same efficacy as cardiac catheterization. 34 Multiplane TEE appears to be more reliable than two-dimensional echocardiography and doppler techniques.…”

Section: Cardiovascular Problemsmentioning

confidence: 95%

Williams-Beuren Syndrome: An Update and Review for the Primary Physician

Lashkari¹,

Smith²,

Graham

1999

Clin Pediatr (Phila)

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Williams-Beuren syndrome is an autosomal dominant disorder resulting from a submicroscopic deletion of contiguous genes on the long arm of chromosome 7. It consists of a variety of hallmark physical features, which include distinctive facial characteristics, cardiac anomalies (of which the most common is supravalvular aortic stenosis), and occasional idiopathic hypercalcemia. The condition also includes a unique cognitive profile, with relative sparing of language and facial recognition skills against a background of mental retardation. This paper reviews the early history and clinical experience with this syndrome, how it unfolds from infancy through adulthood, and how it manifests in different organ systems. Evidence-based recommendations are then offered for the treatment of the specific developmental and medical issues that arise in patients with Williams syndrome.

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Section: Cardiovascular Problemsmentioning

confidence: 95%

Williams-Beuren Syndrome: An Update and Review for the Primary Physician

Lashkari¹,

Smith²,

Graham

1999

Clin Pediatr (Phila)

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Häufige Befunde und ihre Differenzialdiagnosen

Bartel¹

2007

Echokardiographie

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Supravalvar aortic stenosis: unexpected findings at surgery

Grahame-Clarke¹,

Pugsley

Swanton

1998

Heart

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A 51 year old man was referred for investigation of presumed valvar aortic stenosis. A murmur had been noted as a child but he had developed normally and was entirely asymptomatic. He had had two episodes of infective endocarditis successfully treated medically in 1968 and 1977. He was currently on no medication. Annual echocardiography had shown a possible bicuspid aortic valve with a pressure gradient of 30 mm Hg and a hypertrophied left ventricle with good systolic function.Examination revealed a fit man with a blood pressure of 140/90 mm Hg in the right arm but only 110 mm Hg systolic in the left, suggesting a preferential streaming up the innominate artery. His pulse was regular at 68 beats/min, which was slow rising in quality. His jugular venous pressure was low and his apex beat was not displaced. He had a long ejection systolic murmur heard all over the precordium radiating to the carotids with no ejection sound.Electrocardiography showed sinus rhythm with T wave inversion inferolaterally indicative of left ventricular hypertrophy. Chest radiography showed a normal cardiothoracic ratio and aortic arch.Cardiac catheterisation revealed typical pressure tracings of supravalvar aortic stenosis with a pressure gradient of 50 mm Hg (fig 1). There were giant patent coronaries (fig 2), a dilated innominate artery, and a stenosis at the origin of the left common carotid artery. Right sided pressures were normal with no pulmonary artery aneurysms on pulmonary angiography.At surgery the aorta was found to be stenotic just above the coronary ostia, which were greatly enlarged. The aortic valve, although tricuspid in architecture, was friable and regurgitant. It was excised and replaced with a 25 mm St Jude's prosthesis. The aortic root wall was unexpectedly rigid, being up to 0.5 cm thick, which caused problems when enlarging the aortic root. Three separate pieces of pericardium were used to construct a gusset, which enlarged the root orifice from 3 mm to 1 cm in diameter. The patient made a satisfactory recovery and was fit and well at one year follow up. Histology of the aortic wall showed medial necrosis with focal calcification and disruption of the elastic lamina in keeping with a diagnosis of atheroma.

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Multiplane transoesophageal echocardiography is the only definitive ultrasound approach in adult supravalvular aortic stenosis

Cited by 3 publications

References 8 publications

Williams-Beuren Syndrome: An Update and Review for the Primary Physician

Williams-Beuren Syndrome: An Update and Review for the Primary Physician

Häufige Befunde und ihre Differenzialdiagnosen

Supravalvar aortic stenosis: unexpected findings at surgery

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