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Despite significant technical and management advancements improving the survival of children born with Hypoplastic Left Heart Syndrome (HLHS), patients with this condition remain at high risk of morbidity and mortality. The existing management strategy for HLHS typically includes an initial stage of palliation (the Norwood Procedure or Hybrid Procedure) which is followed by a second stage of surgical intervention (creation of the Bi-Directional Glenn). The time between these interventions - known as the inter-stage period - is a period of risk for children with HLHS. It carries a high risk of mortality.
The Home Monitoring Programme (HMP) was pioneered by the Children’s Hospital of Wisconsin because of this concern of high mortality between hospital discharge and the second stage of surgical management. Implementation of the HMP was based on the hypothesis that a drop in oxygen saturation from baseline, poor weight gain or weight loss may predict the presence of serious anatomic lesions or evolving illness and allow for life saving intervention. The introduction of this programme has been proven to reduce inter-stage mortality. This practice has been adopted in many centres with HLHS programmes in the USA, and increasingly the United Kingdom.
The Royal Hospital for Children (RHC) in Glasgow introduced a HMP for children with HLHS in 2011, with the shared aim of existing programmes in improving survival and reducing morbidity of infants with HLHS between first and second stage surgery. The discharge of infants with HLHS will be conditional upon fulfilling the discharge criteria dictated by the HMP; including optimised medication, optimised nutritional status and the demonstration of parental understanding and program compliance.
Principally this service will be delivered by the CNS Team and Paediatric Cardiology Dietetics, and supported where appropriate by:
This protocol is applicable to every infant having undergone Stage 1 Norwood or Hybrid Procedure for HLHS with shunt or ductal stent-dependent pulmonary blood flow.
Any patient on the HMP who turns one year of age, will ordinarily cease being on the programme unless they remain shunt or stent dependent, and to remain on the programme, the named Cardiologist will need to request this.
The HMP criteria may no longer be applicable and suitable for patients over one year of age, and so this will need to be considered.
It must be remembered that each child will have an individually-prescribed set of normal parameters. It is critical that deviation from the norm for a particular patient be associated with a low threshold for early medical review.
In the first instance this may be at GP (if appropriate), Local Hospital, or RHC.
The Cardiologist, CNS Team and Paediatric Cardiology Dietitian are to be notified of A&E attendance immediately.
CNS to update CNS team and Dietician on a weekly basis, which includes each HMP patient’s weekly data. Any concerns are highlighted.
The HMP MDT Team meet on a monthly basis as a channel of communication surrounding the HMP patients.
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Last reviewed: 22 December 2023
Next review: 30 September 2026
Author(s): Lorraine Mulholland; Dr Ben Smith; Laura Flannagan
Approved By: Scottish Paediatric Cardiac Service