Repair of supravalvar aortic stenosis: Cardiovascular morphometric and hemodynamic results

Left Ventricular Outflow Obstruction

“…86 Fatigue stress and shear forces are increased on the aortic valve leaflets in the setting of a poorly distensible sinotubular junction and result in leaflet thickening and damage. 87 Resultant aortic valve dysfunction, stenosis, regurgitation, or a combination of both is the most frequent reason for reoperation and may predict decreased long-term survival. 88,89 Subaortic stenosis occurs in 16% of cases and may contribute to aortic valve damage.…”

Left Ventricular Apical Aneurysm as a Consequence of Diffuse Type Congenital Nonfamilial Supravalvular Aortic Stenosis in a 30-Year-old Female

“…20,21) The operative results obtained by Brown, et al in diffuse SVAS were encouraging, 17) while those of Braunstein, et al in their 2 diffuse type cases were excellent. 18) To the best of our knowledge, LV apical aneurysm secondary to SVAS has not been reported before. LV aneurysm-related complications such as sudden death and arrhythmia may complicate the clinical status of SVAS and may be one of the causes of sudden death in these patients.…”

Left Ventricular Outflow Tract Obstruction

“…In most patients, they are of mild or moderate severity. These findings often will persist after relief of the supravalvar stenosis and in some surgical series, 17-40% of patients operated on for supravalvar stenosis will require reoperation for persistent stenosis [175][176][177]. Concomitant untreated aortic valve disease has been identified as a risk factor for reoperation and mortality.…”

“…Additionally, an endarterectomy procedure can be performed to excise a stenotic intimal ridge. The longitudinal aortotomy is closed using a teardrop-shaped single-sinus Dacron or polytetrafluoroethylene patch to create an aortoplasty including the entire stenotic segment as far in the aortic arch as is necessary [175,176]. This technique of patch enlargement was first independently reported by McGoon, Starr, and their associates in 1961 [196].…”

“…This operation provides a more symmetric reconstruction of the aortic root. Even if it may be difficult to achieve completely accurate restoration of the dimensions at the sinotubular junction in comparison with the aortic annulus, excellent hemodynamic results have been reported with this inverted bifurcated patch technique [175][176][177]198,199]. It remains our procedure of choice for surgical treatment of localized supravalvar aortic stenosis.…”