Supravalvar aortic stenosis: unexpected findings at surgery

Abstract

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.

Total Aortic Arch Replacement for the Treatment of Kommerell's Diverticulum in a Jehovah's Witness

“…Total arch replacement of a right-sided aortic arch and aberrant left subclavian aneurysm has been previously reported. 8 Our operative approach differed, however, as we decided to utilize a bilateral thoracosternotomy that facilitated exposure of the proximal descending aorta and proximal left subclavian artery greatly. Total arch replacement along with proximal descending replacement with left subclavian ligation was integral to the management of this aortic pathology.…”

Williams Syndrome in an Adult

“…Both appear to be caused by mutations involving the elastin gene on chromosome 7q11.23. Grahame‐Clarke et al 8 reported a case of supravalvular aortic stenosis in an otherwise well 51‐year‐old male diagnosed during cardiac surgery. However, there were no other features of Williams syndrome.…”