ESPN 53rd Annual Meeting

ESPN 2021


 
Valvular abnormalities in children with Autosomal Dominant Polycystic Kidney Disease
ALEXANDRA SAVIS 1 JOHN SIMPSON 1 MANISH SINHA 1

1- EVELINA LONDON CHILDRENS HOSPITAL
 
Introduction:

 

The incidence of Mitral valve prolapse (MVP) in children with ADPKD has previously been reported as 12% and in adult populations between 26-33%. Our objective in this study was to establish the prevalence of valvular abnormalities in Children & Young People (C&YP) with Autosomal Dominant Polycystic Kidney Disease (ADPKD).

Material and methods:

 

Retrospective, single centre cross-sectional analysis of all children seen in a dedicated paediatric ADPKD clinic. Mitral valve prolapse (MVP) was diagnosed if one or both MV leaflets breaks the plane of the MV annulus in a non-symmetric manner and/or may demonstrate myxomatous degeneration. Mitral regurgitation, aortic valve abnormalities and variation of normal valvular anatomy, if any, were additionally evaluated.

Results:

 

The echocardiograms of 102 C&YP with ADPKD (range 0.25-18yrs) were analysed. Only 1 (0.98%), 12 year old boy had MVP. There was no associated mitral regurgitation. Five (4.9%) patients, 3 boys, aged 7-14 years had trivial/mild aortic regurgitation. Evaluating variations in normal valvular anatomy, 9 (8.82%) patients, aged 3 to 15.9 years, had minor buckling +/- visual elongation of either the anterior or posterior leaflet of the mitral valve, none of which fall within the criteria of true MVP, but may be early signs of developing MVP.  Of the 9 patients with minor bowing +/- leaflet elongation, only 4 had a trace of MR. None of the patients had any valvular abnormality that was of any clinical significance.

Conclusions:

 

We conclude that following careful evaluation in this contemporary cohort of C&YP with ADPKD, the incidence of mitral valve prolapse is significantly lower than previously reported in both children and in adults with ADPKD. Those with variants of normal anatomy may represent early signs of developing MVP in this population and longitudinal studies are needed.