Hereditary Emphysema (Alpha 1-antitrypsin Deficiency)
Conditions
Keywords
Emphysema, alpha1 antitrypsin deficiency, endoscopic lung volume reduction
Brief summary
Patients with advanced heterogeneous emphysema due to alpha1 antitrypsin deficiency might benefit from endoscopic implantation of intrabronchial valves.
Detailed description
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.
Interventions
DEVICEImplantation 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.
Sponsors
Heidelberg University
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
30 Years to No maximum
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Improvement in pulmonary function (FEV1 (forced expiratory volume in 1 second) and RV/TLC (residual volume/total lung capacity) | 3 months |
Secondary
| Measure | Time frame |
|---|---|
| Average changes in pulmonary function (FEV1, VC (vital capacity), RV, TLC, RV/TLC) | 3 months |
| Average changes in 6-minute-walk-distance | 3 months |
| Number of major complications | 3 months |
| Evaluation of IBV migration rate | 3 months |
Countries
Germany
Outcome results
None listed