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.

Update on alpha-1 antitrypsin deficiency: New therapies

“…The remaining 12 individuals were followed up for at least 1 year: FEV 1 increased by a mean of 54%, quality of life was much improved and 2 individuals were taken off oxygen therapy. There was no significant deterioration in lung function during the 4-year follow-up [72].…”

A Long-Term Follow-Up Investigation of Endobronchial Valves in Emphysema (the LIVE Study): Study Protocol and Six-Month Interim Analysis Results of a Prospective Five-Year Observational Study

“…While clinical evidence from RCTs, controlled trials and case series [13,14,15,16,17,18,19,20,21,22,23] demonstrates that by achieving lobar occlusion in the absence of CV, significant lung volume reduction can be obtained with associated significant clinical responses in severe emphysema patients, the efficacy/safety profile of the Zephyr® valve has not been reported in a large-scale observational study under conditions of daily clinical practice. We now report the study design and interim clinical results of the LIVE Study which was designed to gather safety and effectiveness data on EBV therapy in the real-world setting.…”

Successful lung volume reduction surgery after coronary artery bypass grafting: a case report

“…Small randomized series showed also some yield for the surgical intervention. [6,7] This procedure has been recently suggested by Trotter et al [8] in 552 randomized patients and the authors demonstrated that the choice of LVRS techniques did not affect the outcomes. Due to an extremely limited respiratory reserve, this is the safest technique which minimizes possible air leaks.…”

“…[6] Unilateral resection can be planned for the treatment of hemodynamic instability in selected cases. [7] Herein, we report lung volume reduction in a case with hemodynamic instability early after coronary artery bypass graft (CABG) surgery. Our approach resulted in satisfactory surgical outcomes without any harmful complications in such a difficult case.…”