Restrictive lung disease encompasses a large and diverse group of disorders
characterized by a diminished lung volume. These disorders exhibit common
pathophysiologic features including abnormal gas exchange caused by loss of
functioning alveolar-capillary unit, abnormal respiratory muscle energetic
s caused by altered mechanical ventilatory function, and secondary hemodyna
mic and cardiac dysfunction. Impaired gas exchange is the most prominent ex
ercise abnormality in interstitial lung disease and eventually develops in
other causes of lung restriction as well. Measurements of diffusing capacit
y (DL,,) and alveolar-arterial oxygen tension gradient during exercise are
more sensitive detectors of disease than measurements at rest. Excessive de
ad space ventilation is common in pulmonary parenchymal, pleural, and thora
cic diseases, leading to a higher minute ventilation and ventilatory work d
uring exercise. The associated increase in the metabolic energy requirement
of respiratory muscles may exceed 50% of available total body oxygen deliv
ery and result in insufficient energy delivery to nonrespiratory muscles th
at sustain locomotion. Pulmonary arterial hypertension develops secondarily
to an increased pulmonary vascular resistance. In addition, diastolic fill
ing of the ventricles during exercise may be restricted by pulmonary fibros
is or anatomical restriction of the pleura and thorax, contributing to seco
ndary cardiac dysfunction. Examples of heart-lung interaction are illustrat
ed by the patient after unilateral pneumonectomy. These pathophysiologic ch
anges help explain why functional disability in these patients is often out
proportion to the impairment in lung function.