Interventional procedure overview of phrenic nerve pacing for congenital central hypoventilation syndrome
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Indications and current treatment
CCHS is a rare genetic condition, with around 1,000 cases identified worldwide. CCHS affects how the autonomic nervous system manages or controls breathing. Normally, when breathing is shallow while asleep, the levels of carbon dioxide in the blood increase, which stimulates breathing. In CCHS, this stimulus does not happen, and breathing can stop. Common symptoms include difficulty breathing (especially during sleep), hypercapnia and hypoxemia. So, lifelong ventilatory support is needed during sleep or all the time.
There is no cure for CCHS, but the symptoms can be managed. As CCHS can affect several systems in the body, it needs to be managed by several medical teams (multidisciplinary approach). For respiratory insufficiency, the common treatment includes PPV by tracheostomy or mask to assist with breathing.
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