1 Guidance

1 Guidance

1.1 Current evidence on external aortic root support in Marfan syndrome is based on small numbers of patients. The evidence on safety shows occasional serious adverse events and the evidence on efficacy is limited to the short term. Therefore this procedure should only be used with special arrangements for clinical governance, consent and audit or research.

1.2 Clinicians wishing to undertake external aortic root support in Marfan syndrome should take the following actions.

  • Inform the clinical governance leads in their Trusts.

  • Ensure that patients and their carers understand the uncertainty about the procedure's safety and efficacy and provide them with clear written information. In addition, the use of NICE's information for patients ('Understanding NICE guidance') is recommended (available from www.nice.org.uk/guidance/IPG304/publicinfo).

  • Audit and review clinical outcomes of all patients having external aortic root support in Marfan syndrome (see section 3.1).

1.3 Further research should report on long-term outcomes, particularly the occurrence of dissection and aortic dilatation, and the need for further procedures.

  • National Institute for Health and Care Excellence (NICE)