Symptoms
Pulmonary arterial hypertension may initially be asymptomatic. It manifests as symptoms such as difficulty breathing that worsens with exertion, shortness of breath, fatigue, chest pain, brief loss of consciousness, cyanosis, and palpable heartbeats. Possible complications include cor pulmonale, blood clots, arrhythmias, and bleeding.
Causes
The condition is caused by an increase in pressure in the pulmonary arteries. Values are higher than 25 mm Hg at rest (normal: 15 mm Hg). The increased pressure is a result of narrowed blood vessels and capillaries. The disease is rare. Only about 15 cases per million adults are observed. Risk factors for the disease include:
- Heredity
- Intoxicants such as amphetamines and cocaine, toxins, slimming agents such as fenfluramine, benfluorex.
- Certain chronic diseases such as cardiovascular diseases, respiratory diseases and infectious diseases.
Diagnosis
The diagnosis is made in medical treatment. Numerous other conditions can cause similar symptoms. Right heart catheterization must be performed to confirm.
Drug treatment
The agents used for treatment are listed below. They have antithrombotic, antihypertensive, vasodilator, and antiproliferative properties.
- Anticoagulants: phenprocoumon (Marcoumar).
- Diuretics
- Oxygen
- Digoxin
- Calcium channel blocker
Endothelin receptor antagonists:
- Ambrisentan (Volibris)
- Bosentan (Tracleer)
- Macitentan (Opsumit)
Phosphodiesterase-5 inhibitor:
- Sildenafil (Revatio)
- Tadalafil (Adcirca)
Prostacyclin analogs:
- Epoprostenol (Flolan).
- Treprostinil (Remodulin)
- Iloprost (Ilomedin, Ventavis)
Selective prostacyclin IP receptor agonists:
- Selexipag (Uptravi).
sGC stimulators:
- Riociguat (Adempas)
