Diaphragmatic Pacing in Infants and Children with Congenital Central Hypoventilation Syndrome

Artificially supported ventilation is a mainstay of care for children with congenital central hypoventilation syndrome (CCHS). Determining the optimal type and duration of ventilatory support for each individual should proceed with careful consideration. Diaphragm pacing offers a number of advantages over positive pressure ventilation as an alternative method for providing artificial ventilatory support to children with chronic respiratory failure. With diaphragm pacing, the pacer sends electrical current directly to the phrenic nerves. Negative pressure ventilation is created by the child’s diaphragm rather than by an external piston or blower of the mechanical ventilator. These benefits can substantially improve the quality of life of these patients and potentially optimize both behavioral and neurocognitive development in these children, especially in toddlers. This chapter explores important considerations including the pros and cons of diaphragmatic pacing.