Hybrid procedure for interim management of hypoplastic left heart syndrome (HLHS) in neonates (interventional procedures consultation)

NATIONAL INSTITUTE FOR HEALTH AND CLINICAL EXCELLENCE

Interventional Procedure Consultation Document

Hybrid procedure for interim management of hypoplastic left heart syndrome (HLHS) in neonates

Some babies are born with a poorly developed left side of their heart. The hybrid procedure is a combination of surgical and 'endovascular' techniques to help establish blood flow to and from the left side of the heart. It is performed soon after birth with the aim of delaying more major surgery until the baby is older and better able to withstand it.


The National Institute for Health and Clinical Excellence is examining the hybrid procedure for interim management of hypoplastic left heart syndrome (HLHS) in neonates and will publish guidance on its safety and efficacy to the NHS in England, Wales, Scotland and Northern Ireland. The Institute's Interventional Procedures Advisory Committee has considered the available evidence and the views of Specialist Advisers, who are consultants with knowledge of the procedure. The Advisory Committee has made provisional recommendations about the hybrid procedure for interim management of hypoplastic left heart syndrome (HLHS) in neonates .

This document summarises the procedure and sets out the provisional recommendations made by the Advisory Committee. It has been prepared for public consultation. The Advisory Committee particularly welcomes:

  • comments on the preliminary recommendations
  • the identification of factual inaccuracies
  • additional relevant evidence.

Note that this document is not the Institute's formal guidance on this procedure. The recommendations are provisional and may change after consultation.

The process that the Institute will follow after the consultation period ends is as follows.

  • The Advisory Committee will meet again to consider the original evidence and its provisional recommendations in the light of the comments received during consultation.
  • The Advisory Committee will then prepare draft guidance which will be the basis for the Institute's guidance on the use of the procedure in the NHS in England, Wales, Scotland and Northern Ireland.

For further details, see the Interventional Procedures Programme manual, which is available from the Institute's website (www.nice.org.uk/ipprogrammemanual).

Closing date for comments: 25 September 2007
Target date for publication of guidance: December 2007


Note that this document is not the Institute's guidance on this procedure. The recommendations are provisional and may change after consultation.


1 Provisional recommendations
 
The hybrid procedure to which these recommendations apply consists of pulmonary artery banding, stenting of the ductus arteriosus and, if necessary, atrial septostomy.
1.1 Current evidence on the safety and efficacy of the hybrid procedure for interim management of hypoplastic left heart syndrome (HLHS) in neonates does not cover sufficiently all the elements of the procedure when used in combination. The procedure should therefore only be used with special arrangements for consent, audit or research, and clinical governance.
1.2

Clinicians wishing to undertake the hybrid procedure for interim management of HLHS in neonates should take the following actions.

  • Inform the clinical governance leads in their Trusts.
  • Ensure that parents or carers understand the uncertainty about the procedure?s safety and efficacy, and understand that the child will require other treatments in the future. They should provide parents or carers with clear written information. Use of the Institute?s information for patients (?Understanding NICE guidance?) is recommended (available from www.nice.org.uk/IPG XXX publicinfo). [[details to be completed at publication]]
  • Audit and review clinical outcomes of all patients having the hybrid procedure for interim management of HLHS in neonates.
1.3 The procedure should only be undertaken in paediatric cardiology centres specialising in the treatment of HLHS.
1.4 Clinicians undertaking this procedure should enter all patients onto the Department of Health's UK Central Cardiac Audit Database (www.ccad.org.uk).
1.5 Publication of more data about criteria for patient selection and on the particular combination of techniques used in the hybrid procedure would be useful. The Institute may review the procedure upon publication of further evidence.
 
 
2 The procedure
 
2.1 Indications
 
2.1.1 HLHS is a combination of congenital abnormalities resulting in an underdeveloped left side of the heart. Without surgical intervention it is fatal in the first weeks of life.
2.1.2 The standard treatment for HLHS is three-stage reconstruction requiring up to three complex open heart operations over three or more years. 'Stage 1' involves attaching the pulmonary artery to the aorta, inserting a synthetic shunt between the pulmonary artery and the right ventricle or the aorta, and an atrial septectomy. 'Stage 2' involves connecting the superior vena cava to the pulmonary artery. 'Stage 3' involves connecting the inferior vena cava to the pulmonary artery.
2.1.3 Some children who survive staged reconstruction will reach adulthood in good health. However, some may need other cardiac procedures or a heart transplant at a later stage.
 
 
 
2.2 Outline of the procedure
 
2.2.1

The hybrid procedure is performed under general anaesthesia as soon as possible after birth, aiming to delay the need for high-risk open heart surgery reconstruction until the patient is older. It involves:

  • banding of the right and left branches of the pulmonary artery at open surgery, via a median sternotomy
  • endovascular insertion of a stent into the ductus arteriosus, via a percutaneous approach
  • atrial balloon septostomy (if blood flow across the atrial septum is inadequate) by a percutaneous endovascular technique.
2.2.2 The various components of the procedure may occasionally not be carried out synchronously.
 
 
 
2.3 Efficacy
 
2.3.1 In three case series including HLHS patients treated with the hybrid procedure, 93% (54/58), 57% (8/14) and 62% (18/29) of patients progressed to stage 2 reconstruction, and for those patients survival after the stage 2 operation was 85% (46/54) in the first and 75% (6/8) in the second series.
 
2.3.2 Fifteen of 17 (88%) patients treated with the hybrid procedure in a case series of 40 survived the procedure, of whom four subsequently died before further surgical management was possible, and 10 were treated with a heart transplant.
2.3.3 A study of 22 patients compared 5 high-risk HLHS patients treated with the hybrid procedure with 17 standard- and high-risk patients treated with stage 1 reconstruction. All 5 patients treated with the hybrid procedure and 65% (11/17) of patients treated with stage 1 reconstruction survived their respective operations. Two of the 5 hybrid procedure survivors died before undergoing a stage 2 operation. One of the 11 (9%) stage 1 reconstruction survivors died before being treated with a stage 2 operation.
2.3.4 The case series of 14 patients reported two deaths (14%) in the period between the hybrid procedure and the stage 2 operation. For more details, refer to the sources of evidence (see appendix).
2.3.5 The Specialist Advisers stated that the efficacy of the hybrid procedure is uncertain because only small numbers of patients have been reported. One stated that the long-term efficacy, specifically survival following the hybrid procedure compared with the standard open heart procedure without the hybrid procedure, is not clear in the literature.
 
 
2.4 Safety
 
2.4.1 The case series of 58 patients reported 30-day mortality of 3% (2/58). The case series of 29 patients reported five hospital deaths (17%) after the hybrid procedure. In the case series of 14 and 40 patients, 21% (3/14) and 6% (1/17) of patients died during the hybrid procedure.
2.4.2 In the case series of 40 patients, it was reported that 35% (6/17) of patients had complications relating to pulmonary artery banding. These were due to an oversized device (2 patients), acute device occlusion (2 patients) and unbalanced atrioventricular septal defect (1 patient) (which led to the death of the patient; 1 complication not described). For more details, refer to the sources of evidence (see appendix).
2.4.3 The Specialist Advisers stated potential theoretical events to include death, brain damage, bleeding, infection, heart failure, damage to pulmonary arteries, stent migration, stent stenosis, stent thrombosis, migration of the pulmonary artery bands and perforation of the ductus arteriosus. One Specialist Adviser suggested that the procedure may shift mortality from the neonatal period to the infant period.
 
3 Further information
3.1

The Institute has issued the following guidance.

  • 'Balloon dilatation of systemic to pulmonary arterial shunts in children' (ipg77)
  • 'Balloon dilatation with or without stenting for pulmonary artery or non-valvar right ventricular outflow tract obstruction in children' (ipg76)
  • 'Balloon angioplasty of pulmonary vein stenosis in infants' (ipg75)
  • 'Endovascular atrial septostomy' (ipg86)
  • 'Endovascular closure of patent duct arteriosus' (ipg97)
  • 'Percutaneous fetal balloon valvuloplasty for aortic stenosis' (ipg175)
 
 

Bruce Campbell
Chairman, Interventional Procedures Advisory Committee
August 2007

Appendix: Sources of evidence
 

The following document, which summarises the evidence, was considered by the Interventional Procedures Advisory Committee when making its provisional recommendations.

  • ' Interventional procedure overview of hybrid procedure for interim management of hypoplastic left heart syndrome (HLHS) in neonates', June 2007.

Available from: www.nice.org.uk/ip405overview.

This page was last updated: 30 March 2010