Interventional procedure overview of phrenic nerve pacing for congenital central hypoventilation syndrome
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Existing assessments of this procedure
In 2009 the American Thoracic Society (ATS) published an official clinical policy statement on CCHS (Weese-Mayer 2010). ATS stated that diaphragm pacing can be one of the options to provide chronic artificial ventilation for children with CCHS at home as they do not usually have lung disease. ATS also mentioned that bilateral implantation of PN electrodes and diaphragm pacer receivers is recommended to achieve optimal ventilation in children. For people with CCHS who may need cardiac pacemakers in addition to diaphragm pacers, both can be used together in the same person without interference as long as the cardiac pacemaker is bipolar, thereby minimising the potential for electromagnetic interference with the bilateral monopolar PN electrodes.
In 2020, the European CCHS Consortium Project guidelines for diagnosis and management of congenial central hypoventilation syndrome were published (Trang 2020). The guidelines covered infants, children and adults with CCHS. The guideline development group recommended PNP to be one of the options for ventilatory support, stating:
Mask ventilation requires a cooperative person with normal airway and either adequate spontaneous ventilation or PNP during wakefulness.
PNP offers freedom from the ventilator during daytime in people ventilated 24 hours per day, thus increasing mobility and allowing sporting and professional activities.
To avoid interference with a PH pacer, the use of bipolar cardiac pacing electrode is preferred.
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