Long Term Non-invasive Ventilation in Children With Central Hypoventilation

Abstract

Central hypoventilation (CH) is a quite rare disorder caused by some congenital or acquired conditions. It is featured by increased arterial concentration of serum carbon dioxide related to an impairment of respiratory drive. Patients affected by CH need to be treated by mechanical ventilation in order to achieve appropriate ventilation and oxygenation both in sleep and wakefulness. In fact, in severe form of Congenital Central Hypoventilation Syndrome (CCHS) hypercarbia can be present even during the day. Positive pressure ventilation via tracheostomy is the first therapeutic option in this clinical condition, especially in congenital forms. Non-Invasive ventilation is a an option that must be reserved for more stable clinical situations and that requires careful monitoring over time.

Genetic identification of novel medullary neurons underlying congenital central hypoventilation syndrome

“…In humans and mice, pulmonary ventilation rapidly increases in response to elevated levels of atmospheric CO2 (37-39). We next evaluated whether CNO-dB2-Silence mice fail to respond to a hypercarbic challenge (high CO2 in the air; 21% O2, 8% CO2, balanced N2), a well-known respiratory deficit observed in CCHS patients (9,10,40,41). Of note, healthy humans and mice respond to hypercarbic challenges by increasing their minute ventilation and tidal volumes, while decreasing respiratory cycle length.…”

Strategy of changing from tracheostomy and non‐invasive mechanical ventilation to diaphragm pacing in children with congenital central hypoventilation syndrome

“…In Sweden, non‐invasive ventilation (NIV) has been the first‐line ventilator support for patients with CCHS 3 . However, disadvantages such as midface hypoplasia and unintentional leakage have required assessment over time 4 …”

“…3 However, disadvantages such as midface hypoplasia and unintentional leakage have required assessment over time. 4 …”

Hipoventilación alveolar crónica en pacientes pediátricos a altitud moderada

“…Sin VNI, n (%) o mascarilla y en casos en los que se requiere ventilación continua es de elección realizar traqueostomía. 31 Destaca en este estudio el uso de VNI en tan sólo 41.2% de los casos, a diferencia de lo que ocurre en otros centros en países desarrollados, en los que existe mayor accesibilidad a los dispositivos superando el 90% de los casos 21 y como se expuso previamente, la mayoría de los dispositivos de VNI fueron donaciones debido a la dificultad para adquirir los equipos en nuestro medio, más aún los de soporte vital por su alto costo, y muchas veces los tiempos de inicio de ventilación se prolongan hasta obtener los equipos con lapsos de hasta 24 meses. En relación al uso de la ventilación, la literatura demuestra una ventaja de supervivencia ligeramente mayor con uso de más de cuatro horas/día exponiendo el beneficio clínico de la VNI sobre otras formas de tratamiento.…”