The National Institute for Clinical Excellence is examining balloon angioplasty of pulmonary vein stenosis in infancy 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 balloon angioplasty of pulmonary vein stenosis in infancy.

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• The Advisory Committee will then prepare draft guidance which will be the basis for the Institute's guidance on the use of the procedure in the NHS in England, Wales and Scotland.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: 23 March, 2004

Target date for publication of guidance: June 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 balloon angioplasty of pulmonary vein stenosis in infancy does not appear adequate for this procedure to be used without special arrangements for consent and for audit or research. Such evidence as there is suggests that the procedure is not efficacious. However, there are no special concerns about the safety of the procedure, especially in the context of very ill infants for whom it is used.

Clinicians wishing to undertake balloon angioplasty of pulmonary vein stenosis in infancy should take the following action.

• Inform the clinical governance leads in their Trusts.
• Ensure that the parents of patients understand that the limited available evidence indicates a lack of efficacy. Parents should be given clear written information. Use of the Institute's Information for the Public is recommended.
• Audit and review clinical outcomes of all patients having balloon angioplasty of pulmonary vein stenosis in infancy. Publication of safety and efficacy outcomes will be useful in reducing the current uncertainty. The Institute may review the procedure upon publication of further evidence.

This procedure should only be offered to gravely ill infants with a very poor prognosis, after all other treatment has failed, and in the setting of a specialist neonatal cardiac unit.

Pulmonary vein stenosis (narrowing) may be congenital, or may be acquired after surgery to correct other congenital cardiac anomalies. It is rare and often associated with other cardiac abnormalities. Untreated, it leads to severe lung damage.

There is currently no reliable alternative treatment.

Balloon angioplasty of pulmonary vein stenosis, sometimes combined with stenting, is a palliative treatment for children with a very poor prognosis, or sometimes a temporary measure for children awaiting further interventions. The procedure involves inserting a catheter into a large blood vessel, and passing it into the narrowed area under X-ray control. A balloon is then inflated to relieve the narrowing. A stent may be inserted following dilatation to maintain patency.

Efficacy

The evidence was limited to four very small, poor-quality case-series studies, the largest including only five patients. The two largest studies found no benefit from the procedure in any patients. Another study found an immediate reduction in pulmonary vein pressure in all three patients, as well as angiographic evidence of relief of stenosis in 33% (1/3) of patients. However, this patient died of infection within 36 hours of surgery. For more details, refer to the sources of evidence (see Appendix).

The Specialist Advisors considered that this procedure may have only short-term efficacy (if any at all), and that recurrence rates may be high. They also noted, however, that there was almost no role for surgery in this condition, and that even a partial result from this procedure may offer important palliation in this group of patients.

Some of the main adverse events reported in the studies were: venous tear leading to mediastinal haemorrhage in 20% (1/5) of patients; haemoptysis in 20% (1/5) of patients; death due to infection in 33% (1/3) of patients; and puncture of the distal vein in 33% (1/3) of patients. For more details, refer to the sources of evidence (see Appendix).

The Specialist Advisors considered the main potential adverse events to be death, rupture of myocardium, rupture of pulmonary vein, cerebral or other systemic embolism, arrhythmias, and sepsis.

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

• Interventional procedure overview of balloon angioplasty of pulmonary vein stenosis in infancy, March 2003

Available from: www.nice.org.uk/ip153overview