[Pathophysiology of respiratory muscle weakness]

The respiratory system consists of two parts which can be impaired independently from each other, the lungs and the respiratory pump. The latter is a complex system covering different anatomic structures: the breathing centre, the peripheral nervous system, the respiratory muscles, and the thorax. According to this complexity several underlying conditions can cause insufficiency of the respiratory pump, i. e. ventilatory failure. Disturbances of the breathing centre, different neuromuscular disorders, impairments of the mechanics, such as thoracic deformities or hyperinflation, and airway obstruction are example conditions responsible for ventilatory failure. Main characteristic of ventilatory failure is the occurrence of hypercapnia which is in contrast to pulmonary failure where diffusion disturbances typically not cause hypercapnia. Both acute and chronic ventilatory failure presenting with hypercapnia can develop. In acute ventilatory failure respiratory acidosis develops, but in chronic respiratory failure pH is normalized as a consequence of metabolic retention of bicarbonate. However, acute on chronic ventilatory failure can present with a combined picture, i. e. elevated bicarbonate levels, acidosis, and often severe hypercapnia. Clinical signs such as tachypnea, features of the underlying disease or hypercapnia are important diagnostic tools in addition to the measurement of pressures generated by the respiratory muscles. Non-invasive and widely available techniques, such as the assessment of the maximal ins- and expiratory mouth pressures (PImax, PEmax), should be used as screening instruments, but the reliability of these measurements is reduced due to the volitional character of the tests and due to the impossibility to define normal values. Inspiratory pressures can be assessed more accurately and independently from the patients' effort: with or without the insertion of oesophageal and gastric balloon catheters. However, this technique is more invasive and very complex. It is therefore restricted to centres with scientific aims.