congenitalAS

It occurs much more frequently in males, with a sex ratio of 4:1.

in upto 20% of patients.

PDA and coarctation of the aorta occur most frequently with aortic valve stenosis; (all three of these lesions may coexist.)

There is thickening of valve tissue with various degrees of commissural fusion -> fusion of leaflet. Despite fusion of the leaflet, in most cases three sinuses are still present.

bicuspid valve occures due to fusion of two leaflets rather than actual absence of one of the leaflets.

the two coronary sinuses or the right and noncoronary sinuses.

There is either no attachment or one lateral attachment to the aorta at the level of the orifice.

the aortic valve annulus

The relevance is that this population invariably does not have isolated aortic valve pathology. It is common for them to have associated endocardial fibroelastosis, as well as abnormalities of their mitral valves.

These patients are often initially seen in heart failure, are generally managed with balloon dilation at that time, and invariably have ongoing aortic valve issues in the form of residual stenosis and/or regurgitation. Many require reintervention in their younger years in the form of further balloon dilation or aortic valve replacement. These patients are surviving into adolescence and young adulthood and will have more ongoing issues than those initially encountered at a later date.

the diagnosis is usually made following detection of a murmur; symptomatic functional decline, presyncope, and syncope are rarely the initial features.

They showed that more rapid progression of aortic valve stenosis is more likely to take place within the first 2 years of life, following which the rate of progressive obstruction is more uniform.

In general, affected children are asymptomatic, with normal peripheral pulses if the stenosis is less severe and low-volume, slowly rising pulses when it progresses. Exercise-related fatigue and chest pain are rare complaints and occur only when the stenosis is severe.

With severe stenosis there is a systolic thrill in the same area that can also be felt in the suprasternal notch and carotid arteries. Beyond the newborn period, an ejection click usually occurs at the apex and precedes the murmur. The second heart sound is generally normal in children. An ejection systolic murmur is heard along the left sternal border, with radiation into the right infraclavicular area. Associated AR may be heard.

LVH with or without strain is the hallmark feature.

Overall heart size is normal unless left ventricular remodeling is severe or important associated valvar regurgitation is present.

Two-dimensional echocardiography provides detailed information about the morphology of the valve, left ventricular function, and the presence of associated left-sided lesions. Doppler echocardiography can be used to determine the severity of stenosis and the presence or absence of associated aortic regurgitation.

The importance of this lies in the fact that natural history studies and clinical decision making have thus far been based on peak-to-peak catheterization gradients in infants, children, and adolescents. Valve areas are not usually calculated in this age group because of the lack of good data supporting their use in pediatric patients. Mean gradients as derived from Doppler examination and catheterization correlate closely, but again, no data support their use in clinical decision making. Some data can be used to convert the Doppler-derived mean gradients to peak-to-peak gradients, with the addition of pulse pressure as obtained from blood pressure measurements.

Whatever absolute number is chosen to work with, the additional finding of left ventricular hypertrophy on electrocardiography and echocardiography provides supportive data regarding timing for intervention. Pediatric cardiologists generally agree that a peak-to-peak gradient of 60 mm Hg or greater probably warrants intervention even in the absence of symptoms, although clearly the thresholds for intervention are different from those in adults.

Cardiac catheterization is now rarely used to establish the site and severity of obstruction to left ventricular outflow. Instead, catheterization is undertaken when therapeutic interventional balloon aortic valvuloplasty is indicated.

In this era, balloon dilation has almost completely replaced primary surgical valvotomy in children (Video 62-88). Balloon valvuloplasty retains a place in the management of adolescents and young adults, but with increasing age it becomes a less attractive option and is rarely successful in those with sclerotic and calcified valves at any age. A 2012 paper compared the results of surgical and balloon therapy for congenital aortic stenosis.113 Another described the midterm outcomes of the Ross procedure in infants.114

They indicate that aortic valvotomy is a safe and effective means of palliative treatment that affords excellent relief of symptoms. Aortic insufficiency can occasionally be progressive and require valve replacement. Moreover, after commissurotomy the valve leaflets remain somewhat deformed, and further degenerative changes, including calcification, will probably lead to significant stenosis in later years. Thus prosthetic aortic valve replacement is required in approximately 35% of patients within 15 to 20 years of the original operation.

surgical options include replacement with a mechanical aortic valve, an aortic homograft, or a pulmonary autograft in the aortic position. Accumulating evidence is showing that a pulmonary autograft may ultimately be preferable to an aortic homograft.

It is a pulmonary autograft operation. The patient’s pulmonary valve is removed and used to replace the diseased aortic valve, and the RVOT is reconstructed with a pulmonary valve homograft. This approach appears to confer a survival advantage in the younger age group, in whom repeated mechanical valve replacement is associated with increased mortality.

Because of the associated aortic root dilation inherent with this lesion it can complicate the long-term durability of the Ross procedure.

This surgical approach can be applied from neonatal through adult life. Neither homografts nor autografts require anticoagulation.