1 Recommendations

Guidance

1.1

Use phrenic nerve pacing as an option to treat congenital central hypoventilation syndrome (CCHS) with standard arrangements in place for clinical governance, consent and audit.

1.2

For auditing the outcomes of this procedure, the main efficacy and safety outcomes identified in this guidance can be entered into NICE's interventional procedure outcomes audit tool (for use at local discretion).

1.3

Patient selection should be done by a multidisciplinary team experienced in managing the condition in specialist centres.

1.4

This procedure should only be done in specialist centres by clinicians with specific training and experience in the procedure. Patients should be followed up by clinicians experienced in managing the condition.

Why the committee made these recommendations

Because CCHS is a rare genetic condition, there is limited evidence for the procedure in this population. But the available evidence shows benefits such as an increase in ventilator-free time and tracheostomy tube removal. The evidence does not raise any major safety concerns. So, phrenic nerve pacing is recommended.