Congenital Central Hypoventilation Syndrome Medication

Updated: Apr 21, 2021
  • Author: Amy Brown, MD, MS; Chief Editor: Girish D Sharma, MD, FCCP, FAAP  more...
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Medication Summary

Congenital central hypoventilation syndrome (CCHS) patients do not respond to pharmacological respiratory stimulants. Use of medications is restricted to the treatment of associated diseases. These patients frequently have problems with gastroesophageal reflux.


Prokinetic agents

Class Summary

These agents are useful in the management of gastroesophageal reflux, which is a frequent manifestation in patients with congenital central hypoventilation syndrome (CCHS), particularly during their younger years.

Metoclopramide (Reglan, Clopra, Maxolon)

Metoclopramide improves GI motility by releasing acetylcholine from the myenteric plexus, resulting in contraction of the smooth muscle. It is available in 5- and 10-mg tablets, 5-mg/mL syrup, and 5-mg/mL injection. Administer 30 minutes before eating.

Cisapride (Propulsid)

Cisapride indirectly improves GI motility by promoting acetylcholine release from postganglionic nerve endings in the myenteric plexus. It accelerates gastric emptying and enhances lower esophageal sphincter tone.

Cisapride was withdrawn from the US market on July 14, 2000. The manufacturer may make it available to certain patients who meet clinical eligibility criteria for a limited-access protocol only. It is available in 10- and 20-mg tablets and an oral suspension (1 mg/mL).


Agents to Reduce Apnea


Carbamazepine has been associated with decreased apneic events in CCHS. Though the mechanism is not fully understood, this is an area for future study.  [48]