Prevalence of cardiac valvar abnormalities in children and young people with autosomal dominant polycystic kidney disease.


Journal

Pediatric nephrology (Berlin, Germany)
ISSN: 1432-198X
Titre abrégé: Pediatr Nephrol
Pays: Germany
ID NLM: 8708728

Informations de publication

Date de publication:
03 2023
Historique:
received: 20 10 2021
accepted: 07 02 2022
revised: 06 02 2022
pubmed: 29 6 2022
medline: 19 1 2023
entrez: 28 6 2022
Statut: ppublish

Résumé

Valvar abnormalities in children and adults with autosomal dominant polycystic kidney disease (ADPKD) have previously been reported as a frequent occurrence. Mitral valve prolapse (MVP), in particular, has been reported in almost one-third of adult patients and nearly 12% of children with ADPKD. Our objective in this study was to establish the prevalence of valvar abnormalities in a large, contemporary series of children and young people (CYP) with ADPKD. A retrospective, single centre, cross-sectional analysis of the echocardiograms performed on all consecutive children seen in a dedicated paediatric ADPKD clinic. Full anatomical and functional echocardiograms were performed and analysed for valvar abnormalities. The echocardiograms of 102 CYP with ADPKD (range 0.25-18 years, mean age 10.3 years, SD ± 5.3 years) were analysed. One (0.98%), 3-year-old boy, had MVP. There was no associated mitral regurgitation. Evaluating variations in normal valvar anatomy, 9 (8.8%) patients, aged 7.1 to 18 years, had minor bowing ± visual elongation of either the anterior or posterior leaflet of the mitral valve, none of which fell within the criteria of true MVP. Three (1.9%) patients, 2 boys and 1 girl aged between 7 and 14 years, had trivial or mild aortic regurgitation. No patients had echocardiographic evidence of tricuspid valve prolapse (TVP). In this contemporary cohort of CYP with ADPKD, the incidence of MVP and other valvar lesions is significantly lower than previously reported. A higher resolution version of the Graphical abstract is available as Supplementary information.

Sections du résumé

BACKGROUND
Valvar abnormalities in children and adults with autosomal dominant polycystic kidney disease (ADPKD) have previously been reported as a frequent occurrence. Mitral valve prolapse (MVP), in particular, has been reported in almost one-third of adult patients and nearly 12% of children with ADPKD. Our objective in this study was to establish the prevalence of valvar abnormalities in a large, contemporary series of children and young people (CYP) with ADPKD.
METHODS
A retrospective, single centre, cross-sectional analysis of the echocardiograms performed on all consecutive children seen in a dedicated paediatric ADPKD clinic. Full anatomical and functional echocardiograms were performed and analysed for valvar abnormalities.
RESULTS
The echocardiograms of 102 CYP with ADPKD (range 0.25-18 years, mean age 10.3 years, SD ± 5.3 years) were analysed. One (0.98%), 3-year-old boy, had MVP. There was no associated mitral regurgitation. Evaluating variations in normal valvar anatomy, 9 (8.8%) patients, aged 7.1 to 18 years, had minor bowing ± visual elongation of either the anterior or posterior leaflet of the mitral valve, none of which fell within the criteria of true MVP. Three (1.9%) patients, 2 boys and 1 girl aged between 7 and 14 years, had trivial or mild aortic regurgitation. No patients had echocardiographic evidence of tricuspid valve prolapse (TVP).
CONCLUSION
In this contemporary cohort of CYP with ADPKD, the incidence of MVP and other valvar lesions is significantly lower than previously reported. A higher resolution version of the Graphical abstract is available as Supplementary information.

Identifiants

pubmed: 35763085
doi: 10.1007/s00467-022-05500-w
pii: 10.1007/s00467-022-05500-w
doi:

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

705-709

Subventions

Organisme : British Heart Foundation
ID : TA/F/20/210021
Pays : United Kingdom

Informations de copyright

© 2022. Crown.

Références

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Auteurs

Alexandra Savis (A)

Department of Paediatric Cardiology, Evelina London Children's Hospital, Guys & St Thomas NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH, UK.

John M Simpson (JM)

Department of Paediatric Cardiology, Evelina London Children's Hospital, Guys & St Thomas NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH, UK.

Saleha Kabir (S)

Department of Paediatric Cardiology, Evelina London Children's Hospital, Guys & St Thomas NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH, UK.

Kelly Peacock (K)

Department of Paediatric Cardiology, Evelina London Children's Hospital, Guys & St Thomas NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH, UK.

Hayley Beardsley (H)

Department of Paediatric Cardiology, Evelina London Children's Hospital, Guys & St Thomas NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH, UK.

Manish D Sinha (MD)

Department of Paediatric Nephrology, Evelina London Children's Hospital, Guys & St Thomas NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH, UK. manish.1.sinha@kcl.ac.uk.
Kings College London, London, UK. manish.1.sinha@kcl.ac.uk.

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