The National Institute for Clinical Excellence is examining radiofrequency valvotomy for pulmonary atresia with intact interventricular septum and will publish guidance on its safety and efficacy to the NHS in England, Wales and Scotland. The Institute's Interventional Procedures Advisory Committee has considered the available evidence and the views of Specialist Advisors, who are consultants with knowledge of the procedure. The Advisory Committee has made provisional recommendations about radiofrequency valvotomy for pulmonary atresia with intact interventricular septum.

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 recommendation
• 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• The Advisory Committee will then prepare draft guidance, which will be the basis for the Institute's guidance on the use of the procedure in the NHS in England, Wales and Scotland.

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

Closing date for comments: 22 June 2004

Target date for publication of guidance: September 2004

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

Current evidence on the safety and efficacy of radiofrequency valvotomy for pulmonary atresia with intact interventricular septum is limited due to the rarity of the condition, but appears adequate to support the use of the procedure for the treatment of seriously ill neonates, provided that normal arrangements are in place for consent, audit and clinical governance.

Radiofrequency valvotomy for pulmonary atresia with intact interventricular septum should be performed in a specialist unit where paediatric cardiac surgery is available.

The Department of Health runs the UK Central Cardiac Audit Database (UKCCAD) and clinicians are encouraged to enter all patients undergoing paediatric cardiovascular interventions onto this database (www.ccad.org.uk)

Radiofrequency valvotomy is used to treat pulmonary atresia, a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs. Babies with this type of cyanotic congenital heart disease survive only for the first few days of life while the normal fetal shunts between left and right circulations remain patent. Without an operation in that period to open the pulmonary valve or to make a shunt between the aorta and the pulmonary arteries, the condition is fatal.

The standard treatment for pulmonary atresia is open heart surgery which includes the Fontan procedure (the surgical creation of a right ventricular bypass by directly connecting either the right atrium or the superior or inferior vena cava and the pulmonary artery) and the Blalock-Taussig shunt (a palliative procedure where a shunt is created to allow blood to pass from the aorta to the pulmonary artery by dividing the left subclavian artery and connecting it to the left pulmonary artery). Further open heart surgery may include open surgical valvotomy.

Radiofrequency valvotomy is a minimally invasive cardiac catheterisation procedure which involves creating an opening in the blocked pulmonary valve followed by dilation using balloon angioplasty. It avoids open surgery but some children will later need a permanent shunt procedure.

The evidence was limited to one small non-randomised comparative study and four small uncontrolled studies. The success rate of the procedure varied between 75% (9/12) and 93% (14/15). In a recent case study of 30 patients, 53% (16/30) had a biventricular circulation (the follow-up time was 1-87 months). The success rate of surgical valvotomy was not reported in the comparative study. For more details, refer to the sources of evidence (see Appendix).

One Specialist Advisor commented that proper patient selection was important in order to achieve good clinical outcomes.

In the comparative study, the mortality rate for patients who underwent radiofrequency valvotomy was 16% (3/19), compared with 29% (4/14) for patients who underwent surgical valvotomy. The largest non-comparative study (of 30 patients) reported three postoperative deaths and two late deaths. Other complications reported in the studies included perforation of the pulmonary artery in between 3% (1/30) and 33% (4/12) of patients, and perforation of the right ventricular outflow tract in 17% (3/18) of patients. For more details, refer to the sources of evidence (see Appendix).

The Specialist Advisors considered the main risks of the procedure to be death, perforation of the heart, cardiac tamponade, cardiac or pulmonary artery perforation/rupture, arrhythmias, infection and multi-organ failure.

In making its recommendations the Committee was influenced by the specialist advice that the procedure is established treatment for severely ill neonates who may otherwise die.

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

• Interventional procedure overview of radiofrequency valvotomy for pulmonary atresia with intact interventricular septum, March 2003

Available from: www.nice.org.uk/ip147boverview