Anaesthesia for a patient affected by Central Hypoventilation Syndrome

Is it possible to perform general anaesthesia in a patient with CHS?
Yes, an individual with CHS can have a general or a local anaesthetic. Specific supervision will be needed from the anaesthetist in order to plan care before, during and after the procedure. It is especially important to monitor the adequacy of breathing before and after anaesthesia.

Is it possible to perform local anaesthesia for an individual with CHS?
Local anaesthesia can be performed normally, as would be done, for example, in dental procedures and minor procedures in the emergency department. The doctor, dentist or nurse must be informed about CHS in order to avoid the use of certain anaesthetic drugs like nitrous oxide (an anaesthetic gas) or sedatives.

Are there any drugs that must be avoided?
The medical literature reports harmful events with the use of propofol for anaesthetic procedures for patients with CHS.  Propofol is an intravenous anaesthetic that is commonly used in the operating room for anaesthesia.  This drug can cause problems with the cardiac rhythm, particularly causing excessive slowing of the heart rate and should therefore be avoided.

Are there specific tests that must be performed before anaesthesia?
CHS patients should follow all the procedures needed by other patients, including a  detailed pre-anaesthetic assessment. This should help clarify for the anaesthetist the method and settings for the mechanical ventilation in use, the type of tracheostomy tube or the model of the mask.  The anaesthetist should also learn whether other aspects of the autonomic system might be a problem, including the heart rhythm, blood pressure, circulation and body temperature. It may be necessary to have an ECG or 48-72 hour ECG recording (Holter) performed in order to find out about the presence of disturbances of heart rhythm.
Is it necessary to choose a specific hospital for anaesthesia in CHS patients?
General anaesthesia (where the patient is put to sleep)  should be performed by surgeons and anaesthetists who are used to these special situations, that is, disorders of the control of breathing and the body’s autonomic nervous system. In addition, it is essential that there is a recovery room, where the patient can be  closely monitored as the patient  gradually wakens from the anaesthetic. During this period, the patient will have to return to using their own kind of mechanical ventilation and to discontinue it only after completely awakening. So it would be better to chose a hospital already used to treating patients with CHS.

What kind of care should be considered for the period after surgery / anaesthesia?
After anaesthesia, patients affected by CHS should be monitored in a recovery room with pulse oximetry (oxygen saturation or SpO2) and electrocardiogram (ECG – heart rhythm). Regular observations should be made of the SpO2, heart rate, respiratory rate, blood pressure, body temperature and blood sugar. Ideally, carbon dioxide levels (CO2) should be monitored as well.
The anaesthetist will decide on the duration  needed before the patient can be discharged from the recovery room. This may extend to a few hours – longer periods will usually mean transfer to a high dependency or intensive care unit.

Is it possible to discharge home a patient on the same day as the procedure performed with general anaesthesia?
Whilst this is possible if they have fully woken and all observations have been repeatedly normal, it may be better to observe the patient in the hospital for at least 24 hours.

What kind of monitoring should be performed after pre-medication?
Pre-medication is the medicine given to patients before the surgery / anaesthesia. Sedative medication should never be used unless the patient is fully ventilated. If premedication is given, the patient should not be left alone. A trained nurse or the anaesthetist should stay close to the patient and monitoring of SpO2 and CO2 should begin. The patient’s own mechanical ventilator must be available and may be started if oxygen and carbon dioxide levels begin to change from normal values.

And during general anaesthesia?
Some specific monitoring should always be available for CHS patients. Other monitoring can be utilized according level of invasiveness of the planned procedure. The standard monitoring will be: SpO2 (oximeter); ECG (electrocardiogram); CO2 (end-tidal or transcutaneous); non-invasive systemic blood pressure (BP); and body temperature (T°)

And after general anaesthesia?
All the monitoring will have to be applied also during the recovery period. The need for other monitoring will be assessed by the anaesthetist according to the condition of the patient.

Is it possible to use the patient’s own ventilator during pre-medication and during recovery from general anaesthesia?
Patients affected by CHS will be asked to bring their equipment with them in order to have it available. It is possible that during the change over from anaesthesia to complete recovery, the equipment in use at home could be useful and needed. During the operation / procedure, a different type of ventilator is used.

Is it possible that the tracheostomy tube could be changed during the procedure?
Often uncuffed tracheostomy tubes do not fit well and make mechanical ventilation more difficult during general anaesthesia. It is possible that the anaesthetist will need to change the tube and use a cuffed tube during general anaesthesia. The uncuffed one can then be re-inserted as the patient wakes.