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Valvar aortic stenosis in children

Author
David W Brown, MD
Section Editor
David R Fulton, MD
Deputy Editor
Carrie Armsby, MD, MPH

INTRODUCTION

Left ventricular outflow tract (LVOT) obstructive lesions account for approximately 6 percent of cases of congenital heart disease in children; in one series, the incidence was estimated to be 3.8 in 10,000 live births [1,2]. Obstruction can occur at valvar, subvalvar, and supravalvar levels.

The most common form of LVOT obstruction in children is valvar aortic stenosis (AS), accounting for as many as 71 to 86 percent of patients [2-4]. More than three-quarters of affected patients are male [3].

Valvar AS will be reviewed here. Subvalvar and supravalvar AS are discussed separately. (See "Subvalvar aortic stenosis (subaortic stenosis)" and "Supravalvar aortic stenosis".)

ETIOLOGY

Among children with valvar AS, the most common cause is a bicommissural or bicuspid aortic valve, that is, only two leaflets are present rather than the normal three. Bicommissural aortic valve is associated with both congenital and acquired aortic stenosis. It is also the most common cause of acquired AS in adults. As an example, a review of 932 adults who underwent operative excision of an aortic stenotic valve reported that two-thirds of the patients had a bicommissural aortic valve [5]. The severity of AS progressed with age, with the majority of patients requiring surgical intervention after 50 years of age. (See "Clinical manifestations and diagnosis of bicuspid aortic valve in adults".)

Based upon large retrospective autopsy studies, bicommissural aortic valve had been estimated to occur in 1 to 2 percent of the general population [5-8]. This is likely to be an overestimation as demonstrated by two echocardiographic screening studies, which reported a lower prevalence of 0.5 percent in both neonates [9] and school-aged children [10]. In each of these studies, bicommissural aortic valves were more common among males than females (0.7 versus 0.2 percent) [9,10]. Familial clustering has also been observed, as approximately 35 percent of patients with a bicommissural aortic valve have at least one additional family member with a bicommissural aortic valve [11].

                            

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Literature review current through: Nov 2016. | This topic last updated: Wed Mar 16 00:00:00 GMT 2016.
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