Diaphragm Pacing as a Treatment for Congenital Central Hypoventilation Syndrome

As published in a recent edition of Expert Review of Medical Devices (1), physicians from the University of Southern California (Children's Hospital of Los Angeles) report on their long-term experience of pacing children with congential central apnea.



Thoracoscopic implantation of a breathing pacemaker.

"Congenital central hypoventilation syndrome is a rare syndrome present from birth, and is defined as the failure of automatic control of breathing."

"Diaphragm pacers offer a modality of ventilatory support that affords CCHS patients with maximal mobility for full-time ventilatory patients, and the may allow for a more normal lifestyle in the appropriate patient."

"There is currently only one manufactured system (Avery Biomedical Devices, Inc.) that is approved by the US Food and Drug Administration (FDA)...For the rest of this review, the authors will focus on the Avery system, as this is the only system that the authors' patients have had implanted."

"At the authors' institution, nine patients have had open thoracotomies, all occurring before 1998, and the other ten have had thoracoscopic placement (2) of their pacers."

"Diaphragm pacing in children with CCHS has been established as an effective method of mechanically assisted ventilation, and allows for maximal mobility. With time, the authors hope that diaphragm pacing becomes more commonplace with more centers and physicians gaining familiarity in their placement and continued care."


(1) Chen ML, Tablizo MA, Kun S, Keens TG. "Diaphragm pacers as a treatment for congenital central hypoventilation syndrome." Expert Rev. Med. Devices 2(5), 577-585 (2005).

(2) Shaul DB, Danielson PD, McComb JG, Keens TG. "Thorascopic Placement of Phrenic Nerve Electrodes for Diaphragm Pacing in Children." Journal of Pediatric Surgery. July 2002, Vol.37, No.7, pp.974-978.

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