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Congenital Aortic Valve DiseaseAortic valves come in many different shapes and sizes; the most common congenital abnormality is the bicuspid aortic valve occurring in 1-2% of the population. Aortic valve morphology varies from one cusp (unicuspid) to two cusps (bicuspid) to three cusps (tricuspid) or four cusps (quadricuspid). The cusps may be equal in size, sometimes one cusp may be smaller than the others and cusps may be fused. These valves may be functionally normal but they may be severely obstructive and/or regurgitant and therefore, serial, accurate assessment is paramount.In addition, congenital aortic valve disease is strongly associated with other aortic disease, including? HYPERLINK "" \o "Coarctation" coarctation of the aorta?and a dilated ascending aorta. These may be present even if the valve is functionally normal.2D Echo / Colour DopplerNumber of valve cuspsEvidence of ‘doming’, thickening, calcification on 2D / eccentric closure line on M-ModeLV?dimensions – assess dilatation/hypertrophyColour Doppler to assess turbulence and presence / degree of ARAscending aortic dimensions – image from PLAX, high PLAX and SSN.N.B The ascending aorta can often be well visualised from the RSE with the patient on their right side.Image descending aorta for evidence of coarctation, assessing turbulence on CFMDoppler MeasurementsVelocity profile across the abnormal valve ; use the?Bernoulli equation, 4V2to determine peak pressure gradient and mean pressure gradient (VTI)EOAAR – PHT (loosely : <450msec significant AR, < 200 severe AR)??(*See pitfalls) and evidence of diastolic flow reversal in descending aorta (PW Doppler in the distal transverse arch)Common Pitfalls/LimitationsImage optimization is paramount to visualise individual cusps – think patient postion, sector width / frame rate, gain. Sub-costal SAX is useful if parasternal window is sub-optimal.Multiple imaging windows must be utilized to visualise the ascending aorta, right sternal windows with patient laying flat or on their right side are usefulAR PHT may be unreliable if the Doppler cursor and the direction of the jet on CFM are not well aligned ................
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