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4a-1. Aortic valvular stenosis. (Legend.)

A. There is marked cardiomegaly with the left ventricle apex displaced to the left chest wall. The left atrium displaces the esophagus to the right. The pulmonary vascular markings are normal.

B. Right anterior oblique view demonstrates posterior displacement of the esophagus by the dilated left atrium.  The differential is cardiomyopathy or an obstructive left heart lesion.

Aortic valvular stenosis.

Aortic stenosis may be classified as subvalvular, valvular or supravalvular. Valvular aortic stenosis accounts for 60-75% of cases. There is a 4:1 male:female ratio. Up to 20% of patients have associated cardiac lesions including ventricular septal defect, patent ductus arteriosus, and coarctation. Bicuspid aortic valve is the commonest malformation of the aortic valve, although the valve may also be unicuspid when all three cusps are fused. Generally the fewer the number of cusps, the more severe the degree of valvular stenosis. Less common causes of aortic valvular stenosis include myxoid dysplasia and annular hypoplasia.

Presentation: The majority of patients are asymptomatic and diagnosis is following incidental detection of a murmur. Symptoms may manifest in the newborn period with cardiac failure, and occasionally with cyanosis and shock, particularly when associated with hypoplastic left heart syndrome.

Later presenting symptoms typically include dyspnea, chestpain and rarely syncope from acute reduction in cardiac output. Increasing fatigue and decreasing exercise tolerance however is probably the commonest presentation. Sudden death is a risk with severe outflow obstruction and competitive exercise is contraindicated. Physical findings include pulsus planus (plateau pulse), narrow pulse pressure, a left ventricular impulse, a suprasternal thrill, and on auscultation an ejection click (which does not vary with respiration), followed by a harsh ejection systolic murmur which radiates to the carotids and the apex of the heart. The greater the severity of stenosis, the harsher the murmur, the closer the ejection click to the first heart sound, and the more narrowly split is the second heart sound with paradoxical splitting in severe stenosis.

Severity: Trivial 0-25mmHg; Mild 25-50mmHg; Moderate 50-75mmHg: Severe >75mmHg.

Treatment: Patients with gradients > 50mmHg should undergo either transcatheter balloon dilation or surgical valvotomy. Aortic regurgitation often develops post valvuloplasty, although improvement in balloon technology has significantly reduced this complication.