Pulmonary hypertension

Pulmonary hypertension includes those disorders that are characterized by an increase in arterial blood pressure in the lung. As a consequence, the oxygen supply to the body is inadequate. Therapy has, however, made a great deal of progress in recent years, so that the prognosis for pulmonary hypertension has improved considerably. Since pulmonary hypertension can have different causes, development of personalized treatment procedures is of particular importance.

According to Simonneau et al., PH can be divided into five subgroups:

• pulmonary arterial hypertension (PAH): this form includes idiopathic PAH (IPAH), better known as primary pulmonary hypertension due to a mutation as the basic pathomechanism,
• patients with PH due to left heart failure,
• classification of PH patients with alveolar hypoxia and/or disorders of the respiratory system,
• all (thrombo)embolic diseases connected with PH and
• various diseases directly affecting the pulmonary vessels.

Together, all PH variations affect an estimated 100 million people worldwide.

Vascular pathology is characterized by pulmonary vasoconstriction as well as abnormal (pseudomalignant) alteration processes. According to Rabinovitch et al., possibly all vascular layers (intima, media and adventitia) are affected.