Introduction

Emphysema and chronic obstructive pulmonary disease (COPD), of which emphysema is a part, are major causes of disability and death in the developed world. After a brief discussion of definitions and pathophysiology, the epidemiology of COPD will be reviewed to highlight the magnitude and causes of the problem. The clinical aspects of usual and alpha-1-antitrypsin (AAT) deficiency COPD will be reviewed (see also Chapter 6). The history and worldwide experience with lung transplantation will be summarized and the outcome of transplantation for COPD and other lung conditions will be compared. The limited cost-effectiveness information that is available for lung, liver and heart transplantation will be summarized. Finally, some health care policy implications of lung transplantation as a therapeutic initiative for relieving the suffering and prolonging the life of patients with end-stage emphysema will be discussed.

Definitions and pathophysiology

Emphysema is defined as abnormal permanent enlargement of the air spaces distal to the terminal bronchioles accompanied by destruction of their walls and without obvious fibrosis. COPD is defined as the presence of emphysema or chronic bronchitis associated with chronic airflow obstruction. Emphysema occurs most commonly in association with chronic bronchitis [1].

The major cause of impaired blood gas exchange, dyspnoea, disability and death in COPD is chronic airflow obstruction. Airflow obstruction occurs in emphysema because of loss of elastic recoil of the lung and rupture of alveolar attachments to airways of < 2 mm diameter or bronchioles. As a result of these changes, the small, poorly supported airways collapse and narrow at much larger lung volumes than normal, thus causing structural and irreversible airflow obstruction.