Acute Management of a Cyanosed Infant with Systemic to Pulmonary Shunt (Black-Taussig or Sano Shunt) Presenting to Primary Care, ED or Acute Paediatrics
Flowchart
Introduction

Babies born with single ventricle anatomy are at high risk of mortality, morbidity and growth failure, between the Sano/Blalock-Taussig (BT) shunt surgery and Glenn procedure. Prior to 2017, these babies remained as in-patients during this inter-stage period to mitigate the risk following an initial palliative procedure. This practice adversely impacts on the family of the child and places additional strain on in-patient resources at the surgical centre.

The Home monitoring programme (HMP) aims to detect and act on subtle variances that may precede clinical deterioration. This approach has improved inter-stage survival in North America (Ghanayem 2003).

Like infants with Hypoplastic Left Heart Syndrome, other infants with systemic to pulmonary arterial shunts either using a Gortex tube graft or stenting of the arterial duct are at risk of sudden collapse. The major risk is thrombotic obstruction/occlusion resulting in either a major reduction or complete loss of pulmonary blood flow. This will result in rapid onset hypoxia, loss of shunt murmur, acidosis and secondary circulatory collapse.
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This situation can be mimicked by sepsis associated with significant drop in SVR (systemic vascular resistance), “stealing” blood from the pulmonary circulation.
Scope

This guideline has been developed alongside the ‘Home Monitoring Programme’ which facilitates infants staying at home during the interstage period.
Purpose
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  • To improve survival and reduce morbidity in infants and those infants with shunt dependent circulations. ​
  • Early identification of physiologic variances using a ‘red flag’ system, enabling timely response and intervention. Collaboration between parents, referring cardiologists, paediatricians and local nursing teams facilitating delivery of care for these children in their own homes, ensuring rapid access to appropriate levels of hospital care when needed.  ​
  • Guided management of an unwell child with systemic to pulmonary shunt (Blalock-Taussig or Sano). Emphasis on early discussion with the Paediatric cardiology registrar at (UHS) University hospital Southampton when community redflags breached or SORT (Southampton Oxford retrieval team) in acute deterioration.
Definitions
ABGs
Arterial Blood Gases
CBGs
Capillary Blood Gases
ED
Emergency Department
FiO2
Fraction of Inspired Oxygen
IO
Interosseous Access
IV
Intravenous Access
SaO2
Peripheral Oxygen Saturations
SORT
Southampton Oxford Retrieval Team
SVR
Systemic Vascular Resistance
U&Es
Urea & Electrolytes
Implementation

This guideline will be made available regionally on the PIER Website.  Local leads for critically ill children will disseminate guideline and raise awareness locally.
Process for Monitoring Effectiveness

The Wessex Paediatric Critical Care Network will review problems associated with a failure to comply with this guideline through its regional governance process.
References
  1. Ghanayem, N.., Hoffman, G.., Mussatto, K et al (2003) ‘Home surveillance program prevents interstage mortality after the Norwood procedure’, The Journal of Thoracic and Cardiovascular Surgery, 126(5), pp. 1367–1375. 
  2. Luise V. Marino,1,2 Mark J. Johnson,2,3 Nigel J. Hall,2,4,5 Natalie J. Davies,1 Catherine S. Kidd,1 M. LowriDaniels,1 Julia E. Robinson,1 Trevor Richens,5,6 Tara Bharucha,5,6 and Anne-Sophie E. Darlington7
    The development of a consensus-based nutritional pathway for infants with CHD before surgery using a modified Delphi process. . 2018 Jul; 28(7): 938–948.
  3. Resuscitation Council UK  https://www.resus.org.uk/resuscitation-guidelines/
  4. Frank Shann Drug doses 17th Ed. 2017-Dec 2020.
Document Version: 
2.0

Lead Author: 
Dr Trevor Richens, Consultant Paediatric Cardiologist, Southampton Children's Hospital

Additional Author:
Katy Stimson, Nurse Practitioner, Southampton Children's Hospital
Approving Network:
Southampton Oxford Cardiology Network

Date of Approval: 
June 2023

Review Date:
June 2026