One- to Four-Year Follow-Up of Endobronchial Lung Volume Reduction in Alpha-1-Antitrypsin Deficiency Patients: A Case Series

Abstract

Background: Lung volume reduction surgery can improve lung function and working capacity in severe heterogeneous emphysema. Endobronchial lung volume reduction (ELVR) performed by one-way valves inserted via a flexible bronchoscope can result in a moderate but significant improvement in lung function and exercise tolerance, eliminating the surgical risks. Objectives: Most studies of this method have excluded patients with α₁-antitrypsin (AAT) deficiency, but small series of cases with positive short-term outcome have been reported. The sustainability of results has been questioned and we here present our experience in AAT-deficient patients treated with ELVR followed up for up to 4 years. Methods: From August 2008 to January 2012, 15 patients were treated with ELVR. Inclusion criteria were homozygotic AAT deficiency, age <80 years, residual volume of 140% or more, forced expiratory volume in 1 s (FEV₁) 15-45% of predicted, severe heterogeneous emphysema, symptoms severely restricting daily life, informed consent and absence of other serious diseases. Results: One patient coughed up valves after 2 months, 1 developed pneumothorax and had valve displacement and subsequent removal, and 1 improved from an FEV₁ of 0.62 to 0.84 liters, but after 4 months developed repeated and severe pneumonia and the valves had to be removed. Thus, 12 patients remained and were followed up for at least 1 year. In these patients, FEV₁ increased (mean: 54%), the quality of life was much improved, and 2 patients could be taken off oxygen therapy. During the 4-year follow-up, patients demonstrated no significant deterioration in lung function. Conclusion: In carefully selected AAT deficiency patients with severe emphysema, ELVR can be safely performed with encouraging long-lasting results.