Skip to main content

Main menu

  • Home
  • Our journals
    • Clinical Medicine
    • Future Healthcare Journal
  • Subject collections
  • About the RCP
  • Contact us

Clinical Medicine Journal

  • ClinMed Home
  • Content
    • Current
    • Ahead of print
    • Archive
  • Author guidance
    • Instructions for authors
    • Submit online
  • About ClinMed
    • Scope
    • Editorial board
    • Policies
    • Information for reviewers
    • Advertising

User menu

  • Log in

Search

  • Advanced search
RCP Journals
Home
  • Log in
  • Home
  • Our journals
    • Clinical Medicine
    • Future Healthcare Journal
  • Subject collections
  • About the RCP
  • Contact us
Advanced

Clinical Medicine Journal

clinmedicine Logo
  • ClinMed Home
  • Content
    • Current
    • Ahead of print
    • Archive
  • Author guidance
    • Instructions for authors
    • Submit online
  • About ClinMed
    • Scope
    • Editorial board
    • Policies
    • Information for reviewers
    • Advertising

Definition and delivery of an aortopathy bundle of care (ABC): a tool for improving diagnosis and management of Marfan syndrome and related conditions

Emma Burkitt Wright, Sasha Henriques, Catherine Houghton, Bernard Clarke, Bernard Keavney and Luigi Venetucci
Download PDF
DOI: https://doi.org/10.7861/clinmedicine.16-3-s30
Clin Med June 2016
Emma Burkitt Wright
1Manchester Centre for Genomic Medicine, Manchester, UK
2University of Manchester, Manchester, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Sasha Henriques
1Manchester Centre for Genomic Medicine, Manchester, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Catherine Houghton
1Manchester Centre for Genomic Medicine, Manchester, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Bernard Clarke
2University of Manchester, Manchester, UK
3Manchester Heart Centre, Manchester, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Bernard Keavney
2University of Manchester, Manchester, UK
3Manchester Heart Centre, Manchester, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Luigi Venetucci
2University of Manchester, Manchester, UK
3Manchester Heart Centre, Manchester, UK
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Info & Metrics
Loading

Aims

To accelerate diagnosis and improve management of Marfan syndrome and related conditions, a multidisciplinary improvement science project was developed between cardiology and genomic medicine.

Methods

Local experience suggested potential for improvement in the delivery of care for patients with Marfan syndrome and related conditions. Under the prevailing system, patients required separate clinical genetic and cardiological assessments. Delays in diagnosis were common and timely delivery of elements of follow-up, such as clinic appointments and imaging, was not always achieved. Current guidelines were reviewed to define key indices of effective management: the aortopathy bundle of care (ABC). A monthly genetic aortopathy team clinic (GATC) was established, permitting multidisciplinary consultations with a cardiologist, clinical geneticist and genetic counsellor. To optimise the utility of this new, resource-intensive clinic, plan-do-study-act cycles were embedded in each clinic, and increasing numbers of patients were seen each month. ABC measures were used to evaluate delivery of care using this model. Semi-structured questionnaires pre and post clinic were used to elicit patient feedback.

Results

Six elements to the ABC were defined: documentation of diagnosis according to revised Ghent criteria (and molecular genetic testing, where performed), clinical follow-up, genetic counselling provision, appropriate imaging, blood pressure management and appropriate referral for cardiac surgical opinion. Prior to the clinic's inception, concordance with the ABC was exceptionally low: no patients met all elements of this on retrospective review. Patients seen in the GATC received more appropriate and timely care in accordance with the ABC. The proportion of patients whose diagnosis was confirmed and documented according to revised Ghent criteria rose from 25% to 100%. 100% of patients received appropriate imaging, compared to 25% previously. Improvements were also seen in hypertension management. Conclusions cannot yet be drawn on the ability of the GATC to provide reliably timed follow-up and appropriate cardiac surgical referral (due to the lead-time issue), and formal cost-effectiveness analysis will require these data. Patients reported high levels of satisfaction with the multidisciplinary approach. Clarification of diagnosis was possible in 11 patients from seven families, who were discharged from clinic as a result.

Conclusions

The ABC appears useful for measuring the effectiveness of care for patients with genetic aortopathies such as Marfan syndrome. The multidisciplinary GATC was shown to be effective in achieving a diagnosis in fewer clinic visits than previously, and provides a means of delivery of appropriate and comprehensive care to this patient group.

  • © Royal College of Physicians 2016. All rights reserved.
Back to top
Previous articleNext article

Article Tools

Download PDF
Article Alerts
Sign In to Email Alerts with your Email Address
Citation Tools
Definition and delivery of an aortopathy bundle of care (ABC): a tool for improving diagnosis and management of Marfan syndrome and related conditions
Emma Burkitt Wright, Sasha Henriques, Catherine Houghton, Bernard Clarke, Bernard Keavney, Luigi Venetucci
Clinical Medicine Jun 2016, 16 (Suppl 3) s30; DOI: 10.7861/clinmedicine.16-3-s30

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Share
Definition and delivery of an aortopathy bundle of care (ABC): a tool for improving diagnosis and management of Marfan syndrome and related conditions
Emma Burkitt Wright, Sasha Henriques, Catherine Houghton, Bernard Clarke, Bernard Keavney, Luigi Venetucci
Clinical Medicine Jun 2016, 16 (Suppl 3) s30; DOI: 10.7861/clinmedicine.16-3-s30
del.icio.us logo Digg logo Reddit logo Twitter logo CiteULike logo Facebook logo Google logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One

Jump to section

  • Article
    • Aims
    • Methods
    • Results
    • Conclusions
  • Info & Metrics

Related Articles

  • No related articles found.
  • Google Scholar

Cited By...

  • No citing articles found.
  • Google Scholar

More in this TOC Section

  • Inaccessible back: frustration and misery of lonely patients
  • Methylation of somatostatin receptor 2 gene in neuroendocrine tumours as a predictor of tumour response to peptide receptor radionuclide therapy
  • Viral and other infections in community and primary hypothyroidism – cause or coincidence?
Show more Research and innovation

Similar Articles

Navigate this Journal

  • Journal Home
  • Current Issue
  • Ahead of Print
  • Archive

Related Links

  • ClinMed - Home
  • FHJ - Home
clinmedicine Footer Logo
  • Home
  • Journals
  • Contact us
  • Advertise
HighWire Press, Inc.

Follow Us:

  • Follow HighWire Origins on Twitter
  • Visit HighWire Origins on Facebook

Copyright © 2021 by the Royal College of Physicians