P456Adult parachute mitral valve - poorly recognised rather than extremely rare?


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Abstract

Introduction: Adult parachute mitral valve (APMV) - a form of congenital mitral stenosis -occurs when all the chordae tendineae are inserted into a solitary papillary muscle. It is usually seen as part of Shone's complex which can also include aortic coarctation and bicuspid aortic valve (BAV). APMV is said to be very rare with a recent systematic literature review in 2010 documenting only 9 published cases.Methods: Positive identification of two papillary muscles can be challenging on transthoracic echo (TTE), so we have concentrated on searching for:Anterior chordal redundancy (ACR): the “offset” nature of APMV means that the anterior chords are often redundant and folded in systole and can even produce left ventricular outflow tract obstruction (LVOTO). A “pear-shaped” appearance of the left atrium and mitral valve in diastole, formed by the left atrium and funnel-shaped valve leaflets. “Domed” appearance of the valve - this is also due to ACR - a dome is seen in 2 chamber view rather than a valve orifice.Figure 1 shows: solid arrow- anterior chordal redundancy. hollow arrow - solitary papillary muscleResults: In the last 3 years, we have identified 4 adult patients (3 cases published so far) with history of coarctation or BAV in whom we have positively identified APMV - 2 have required surgery for LVOTO from ACR. Images are included in this presentation. Our published cases alone represent a 33% increase in the total published “world series”!Conclusions: We believe that APMV is really much more common than the literature suggests. Confirmation of a solitary papillary muscle can be difficult on TTE so other distinctive patterns as outlined above should be carefully sought in any patient with a history of coarctation or BAV.

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