Effect of long-term non-invasive ventilation on quality of life and cardiac function of children's neuromuscular disorders with chronic respiratory failure: a clinical trial

Background: Use of long-term non-invasive positive pressure ventilation is increasing greatly worldwide in children with chronic respiratory failure (CRF) of all ages. This treatment requires delivery of ventilation through a non-invasive interface. Cardiac function in majority of these children is impaired. The aim of this study was to assess the effect of institution of non-invasive ventilation (NIV) on quality of life (QOL) and cardiac function in children with CRF related to neuromuscular disorders. Methods: Information obtained from all of the children under 16 years old with CRF due to neuromuscular disorders who were on NIV for at least six months and that were referred to Mofid children's hospital, Tehran, Iran between September 1, 2013, to September 1, 2017.Based on previous studies they were assessed from the year prior to starting NIV and annually thereafter. Data obtained included diagnosis, pulmonary function test, echocardiographic data, length of hospitalizations, and health care costs. Patients and parents completed questionnaires assessing QOL with NIV and recalling QOL one year before commencing NIV. All results were recorded in information forms and data were analyzed with chi square and entered in SPSS 21. Results: Follow-up ranged from 6 to 36 months (median 18). Before and after NIV hospitalization rates (P<0.001), PICU admission (P<0.001) and health care costs decreased respectively. QOL remained stable after NIV despite disease progression (P<0.001). Systolic pulmonary arterial pressure (P=0.009) is diminished. Symptoms of daytime sleepiness (P<0.001) and headache (P<0.001) improved after initiation of NIV. Conclusions: This study revealed that use of NIV results in a reduction in PAH without adverse effects on quality of life and pulmonary function.