Endoscopic Lung Volume Reduction in Patients With Advanced Emphysema Due to alpha1 Antitrypsin Deficiency

Study Overview
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Brief Summary
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Patients with advanced heterogeneous emphysema due to alpha1 antitrypsin deficiency might benefit from endoscopic implantation of intrabronchial valves.

Detailed Description
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Patient enrollment and data acquisition is to be carried out on a prospective basis. It is planned to enroll a total of 25 patients with advanced heterogeneous emphysema due to alpha1 antitrypsin deficiency. All patients will undergo treatment at one study centre in Heidelberg. Therapy of all patients consists of implantation of intrabronchial valves (IBV, Spiration, Olympus) in the most emphysematous destroyed lobe.

Official Title
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Endoscopic Lung Volume Reduction in Patients With Advanced Emphysema Due to alpha1 Antitrypsin Deficiency

Conditions
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Hereditary Emphysema (Alpha 1-antitrypsin Deficiency)

Intervention / Treatment
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* Device: Implantation of intrabronchial valves (IBV) (Spiration IBV)


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: pulmonary function: FEV1 < 45 %, RV > 150 %, TLC > 100 % alpha1 antitrypsin deficiency (< 80 mg/dl), genotype: PiZS, PiZZ, Pi0/0 heterogenous emphysema Exclusion Criteria: homogenous emphysema significant bronchiectasis severe concomitant diseases pregnancy

Ages Eligible for Study
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Minimum Age: 30 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Intervention/Treatment |
| --- |
|Device: Implantation of intrabronchial valves (IBV) (Spiration IBV)|In advanced emphysema due to alpha1 antitrypsin deficiency even optimum treatment including drugs, physical training and possibly oxygen therapy is unable to influence exercise dyspnoea and exercise capacity.Implantation of intrabronchial valves in the most destroyed and hyperinflated lung lobe can improve the elastic recoil of the small airways by a reduction in lung volume and thus leading to more ergonomic breathing mechanics and diaphragm function.The one-way mechanism of these valves allows air to escape from the downstream lung segment without any influx of new air during inspiration.|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Improvement in pulmonary function (FEV1 (forced expiratory volume in 1 second) and RV/TLC (residual volume/total lung capacity) |  | 3 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Number of major complications |  | 3 months | 
| Evaluation of IBV migration rate |  | 3 months | 
| Average changes in pulmonary function (FEV1, VC (vital capacity), RV, TLC, RV/TLC) |  | 3 months | 
| Average changes in 6-minute-walk-distance |  | 3 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Emphysema, alpha1 antitrypsin deficiency, endoscopic lung volume reduction