الفهرس 
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Abstract
Congenital Aortic Stenosis (AS) encompass a series of stenotic lesions starting in the anatomic Left Ventricular Outflow Tract (LVOT) and stretches toward the ascending aorta. Obstruction may be subvalvar, valvar, or supravalvar. All of these lesions impose an increased afterload on the left ventricle (LV), which can result in LV hypertrophy, dilatation and eventually heart failure, when left untreated
Valvular aortic stenosis comprises the most comman cause of aortic stenosis.Variants range from a nearly trileaflet bicommissural valve with mild cusp inequality, bicuspid to a unicuspid unicommissural valve.BAV is the most common congenital malformations and has a prevalence of 1-2% in the general population
Presentations of aortic valve stenosis vary, ranging from critical aortic stenosis in neonates to no symptoms in children who have only a systolic ejection click upon physical examination, either with or without a murmur to suggest aortic stenosis
Patients with congenital AS with severe symptoms in infancy and childhood (angina, syncope, congestive heart failure) have a poor prognosis without intervention Sudden cardiac death may occur, especially in the setting of physical acitivity and exertion
Balloon valvotomy may be an attractive option in children, adolescents and young adults, who have pliable non calcified valves with fusion of the commissures, at the cost of re-stenosis or regurgitation
The main indication for aortic valve replacement is the presence of symptoms (i.e. angina, syncope, congestive heart failure) as this is associated with a worse outcome (i.e. overall cardiac mortality and sudden cardiac death)
The Ross procedure (pulmonary auto graft) is the treatment of choice in the pediatric population due to its growth potential, but the high reoperation rate and progressive auto graft dilatation renders it unsuitable in adults.