862 Volume-Assured Pressure Support improves outcomes in a patient with Congenital Central Hypoventilation Syndrome

Abstract Introduction Congenital Central Hypoventilation Syndrome (CCHS) is a condition caused by a mutation of the PHOX2B gene and an incidence of 1 in 50,000 live births. Clinically the condition is characterized by autonomic nervous system dysfunction, the most prominent feature of which is the failure of respiratory homeostasis during sleep. In patients severely affected, life-long ventilatory support is required. This might start as early as the newborn period. Subsequent adjustments are required due to their growth and development. The role of Volume-Assured Pressure Support (VAPS) ventilation in treatment of CCHS was only described in a couple reports before. Report of case(s) A 17-year-old female patient born at term and diagnosed with CCHS at birth at our center with a PHOX2B mutation confirmed. Her daytime ventilatory support was weaned at age 18 months and the tracheostomy was removed at age 10 years old. She relocated to another state, was lost to follow-up, and returned this year for adjustments of her ventilator whose settings had not been adjusted for several years. She was on a Trilogy 100 ventilator, in pressure-controlled mode with settings of EPAP 5, IPAP 20, and a rate of 22/min without supplemental oxygen. Her measured weight is 69kg, body mass index (BMI) 27. The patient complained of difficulty breathing while on those settings and reported decreased desire to use the machine. The patient was empirically switched to VAPS due to titration availability limitations during the COVID-19 pandemic. Initial AVAPS settings were: EPAP 4–7, PS 3–12, breath rate 16/min, TV 350mL. Upon implementing the changes, the patient reported improved comfort and increased usage. Average minute ventilation decreased from 10.5 to 5.8 L/min, patient triggered breaths increased from 1.1% to 12.3%, average breaths per minute decreased from 22.0/min to 16.2/min, the average peak flow 31.8 to 26.2L/min, tidal volume decreased from 463 to 355mL. Conclusion AVAPS ventilation can be successfully used in managing patients with CCHS, and it might be superior to pressure-controlled mode in certain cases, improving patient comfort and compliance. Support (if any) Department of Internal Medicine, University of New Mexico School of Medicine, Albuquerque, NM.