As published in a recent edition of the Journal of Pediatric Surgery¹, physicians from McGill University (Montreal Children's Hospital) report on their long-term experience of pacing children with congential central apnea.

Breathing Pacemaker System

"The congenital central alveolar hypoventilation syndrome (CCAHS), also known as Ondine's curse, is characterized by the absence of adequate autonomic control of respiration.The purpose of our study is to review our 20-year experience with diaphragmatic pacing as a treatment modality for CCAHS. "

"Two surgical interventions are necessary in these children. Tracheostomy must be performed initially for control of the airway, usually within the first month of life. After the age of 18 months, when chest wall is less compliant, implantation of a phrenic nerve pacemaker may proceed electively. "

"After the initial recovery, patients start a phase of diaphragmatic conditioning, which involves progressive increments in the length of time the phrenic nerve is paced. Diaphragmatic conditioning is a process whereby the fast contracting fibers, which are anaerobic and highly glycolytic, and susceptible to fatigue, get progressively transformed into slow-contracting fatigue-resistant fibers, thereby making diaphragmatic pacing possible."

"Infants with CCAHS whose parents choose to continue treatment should be offered diaphragmatic pacing after the age of 1 year. It has a lower complication rate with each new generation of equipment and leads to a greatly enhanced quality of life for these unfortunate children. The group with less severe symptoms, who can breathe spontaneously during the day, benefit from being paced at night and therefore are totally free from mechanical ventilation."

¹ Ali A, Flageole H. "Diaphragmatic Pacing for the Treatment of Congenital Central Alveolar Hypoventilation Syndrome." J.Pediatr.Surg. 10(3), 108-110 (2007).

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