ABSTRACT
BACKGROUND: Chest wall deformities such as kyphoscoliosis, thoracoplasty, and fibrothorax cause ventilatory insufficiency that can lead to chronic respiratory failure, with recurrent fatal acute respiratory failure(ARF). This study evaluated the frequency and outcome of ARF, the physiologic status, and the long-term prognosis of these patients. METHODS: Twenty-nine patients with chest wall disorders, who experienced the first requirement of ventilatory support from ARF were examined. The mortality and recurrence rate of ARF, the pulmonary functions with arterial blood gas analysis, the efficacy of home oxygen therapy, and the long-term survival rate were investigated. RESULTS: 1) The mortality of the first ARF was 24.1%. ARF recurred more than once in 72.7% of the remaining 22 patients, and overall rate of successful weaning was 73.2%. 2) Twenty-two patients who recovered from the first ARF showed a restrictive ventilatory impairment with a mean FVC and TLC of 37.2% and 62.4 % of predicted value, respectively, and a mean PaCO2 of 57mmHg. Among the parameters of pulmonaty functions. the FVC(p=0.01) and VC(p=0.02) showed a significant correlation with the PaCO2 level. 3) There were no significant differences between the patients treated with conservative medical treatment only and those with additional home oxygen therapy due to significant hypoxemia in the patients with recurrent ARF and the mortality. 4) The 1, 3, 5-year survival rates were 75%, 66%, and 57%, respectively, in the 20 patients who had recovered from the first ARF, excluding the two patients managed by non-invasive nocturnal ventilatory support. CONCLUSION: These results suggest that active ventilatory support should be provided to patients with ARF and chest wall disorders. However, considering recurrent ARF and weak effect of home oxygen therapy, non-invasive domiciliary ventilation is recommended in those patients with these conditions to achieve a better long-term prognosis.