Long-Term Survival on Noninvasive Ventilation in AdultsWith Thoracic Scoliosis

BACKGROUND: Subjects with thoracic scoliosis were an important group in early studies of noninvasive ventilation (NIV). The aim of this study was to describe current rates of initiation of NIV and survival after initiation in this population. METHODS: This study included patients identified as having thoracic scoliosis and established between 1993 and 2018 on home NIV. Patients with scoliosis secondary to neuromuscular disease (other than poliomyelitis) were excluded. Survival rates were calculated for various time intervals up to 25 y. RESULTS: A total of 53 subjects with thoracic scoliosis were successfully established on NIV. P-aCO2 levels prior to starting NIV were 55 +/- 23 mm Hg. FVC was 0.5 +/- 0.1 L, 18.5 +/- 9% of predicted, with a Cobb angle of 101 +/- 3.5 degrees. The 5-, 10-, 15-, 20-, and 25-y survival rates were 96%, 88%, 61%, 46%, and 39%, respectively. At the time of death, subjects had been on home NIV for 9.2 +/- 5.1 y and were 75.5 +/- 9.2 y old. There was no significant correlation between mortality and age at time of commencing home NIV, initial arterial blood gas results, FVC, or Cobb angle. There was no significant difference in survival between those with and without poliomyelitis. In 8 of 10 of the most recent years of this survey, subjects with scoliosis have been commenced on home NIV. CONCLUSIONS: Small numbers of subjects with scoliosis continued to present with respiratory failure. Once established on home NIV, around 40% survived >= 25 y. Long-term care will be needed for many years to come for this patient population.