Endobronchial valve deployment in severe α-1 antitrypsin deficiency emphysema: a case series


  • Authorship and contributorship

  • JJ Egan designed the study/protocol; Kasper Remund and JJ Egan performed study; MM Tuohy and Kasper Remund collected data; R. Hilfiker, Dara T Murphy and John E Murray analysed data; MM Tuohy and Kasper Remund wrote paper.

  • Ethics

  • The protocol has been approved by the Audit and Research Ethics Committee of the Mater Misericordiae University Hospital and was performed with the ethical standards laid down in the 2000 Declaration of Helsinki. All persons gave their informed consent prior to inclusion in the study.

  • Conflict of interest

  • The authors have stated explicitly that there are no conflicts of interest in connection with this article.

Jim John Egan, MD, Advanced Lung Disease and Lung Transplant Programme Medicine, Mater Misericordiae University Hospital, University College Dublin, Eccles Street, Dublin 7, Ireland. Tel: +353 1 8034296, Fax: +353 1 8034773, email: jegan@mater.ie


Introduction:  Patients with end-stage emphysema because of α-1 antitrypsin (AAT) deficiency represent a challenging clinical management problem, and studies of volume reduction therapy to date have largely excluded these patients. We report the outcome of bronchoscopic volume reduction with the insertion of Emphasys endobronchial valves (Emphasys Medical, Redwood City, CA, USA) in six patients with end-stage emphysema because of AAT deficiency.

Case Series:  Of 51 patients with end stage emphysema referred for transplantation, we studied six patients with AAT deficiency and utilized the BODE index and lung allocation score for survival estimation.

Measurements and Main Results:  The forced expiratory volume in 1 s improved from a median of 0.575 L to 0.905 L (P = 0.028). There was a median reduction in total lung capacity (TLC) of 0.61 L. The residual volume /TLC fell from 74.0% to 58.4%. Before treatment, four patients had a BODE index of greater than eight units, which correlates with a 4-year survival of 18%. After treatment, two patients improved their BODE index to below seven units, which correlates with an estimated 4-year survival of over 50%.

Conclusions:  The data from this case series suggest that this intervention may provide bridging therapy to subsequent transplantation for younger AAT patients with end-stage emphysema.

Please cite this paper as: Tuohy MM, Remund KF, Hilfiker R, Murphy DT, Murray JG and Egan JJ. Endobronchial valve deployment in severe α-1 antitrypsin deficiency emphysema: a case series. Clin Respir J 2013; 7: 45–52.