Pulmonary hypertension (PH), is a chronic progressive disease characterized by an increase in pressure in the pulmonary circulation.
According to the cause, PH is divided into 5 subgroups according to the Nice Classification. Pulmonary hypertension is a relatively common complication of pulmonary and cardiac diseases, e.g., COPD or heart failure. Another subgroup pulmonary arterial hypertension (PAH) subsumes pathological changes in the pulmonary arterial circulation due to specific causes, e.g., heredity, in certain underlying diseases (e.g., connective tissue diseases, HIV infection, portal hypertension, congenital systemic pulmonary shunts, or the worm disease schistosomiasis) or without a known cause. PAH is a very rare disease: in Europe, approximately 5-10 new cases of PAH per 1 million population are diagnosed annually. The overall PAH disease rate is reported to be 15-60 per million population. The risk of developing PAH is known to be higher for women.
PH is often diagnosed only at an advanced stage. In the majority of patients, the symptom of dyspnoea is prominent. Other symptoms are often nonspecific and include fatigue, physical weakness, dry cough, angina, dizziness, and syncope. When PH is suspected, patients should be referred to specialized centers where specific investigations are performed for the diagnosis of PH. In these specialized centers, an interdisciplinary network of cardiologists/pneumologists, specialized clinical nurses, radiologists, and psychologists is further established for the comprehensive treatment of PH. Various drugs are available for the therapy of PAH: Endothelin receptor antagonists, PDE-5 inhibitors (e.g., Pulmolan®), a soluble guanylate cyclase stimulator, prostacyclins, and a selective IP receptor agonist.1
1 Diestelmaier K & Lang I. Pulmonary hypertension – state of the art of modern therapies. DFP Literature Clinicum 4/2017,p14-8.