Indtroduction

Although the disease “pulmonary hypertension” (PH) was described as long as 100 years ago, even today there is no comprehensive explanation of its pathogenesis, and nor are there currently any prospects of a cure for this disease. Despite growing awareness of this rare but serious disease, there is an average of two years between the initial consultation of a doctor and a diagnosis being made [1]. If untreated, the idiopathic form (iPAH) results in death within 3 years [2].