Idiopathic pulmonary arterial hypertension (IPAH) is a rare form of pulmonary arterial hypertension characterized by increased vascular resistance and thus increased blood pressure in the pulmonary circulation.
If the middle arterial blood pressure in the pulmonary artery is higher than 25 mmHg, a manifest of pulmonary hypertension is made. The symptoms are initially very unspecific. The focus is on exercise dyspnoea, tiredness and fatigue, chest pain, syncope, edema and increase in the abdominal circumference. Symptoms at rest usually occur only in advanced cases.
Increased obliteration of the pulmonary circulation causes a progressive right heart strain, which can ultimately result in right heart failure.
IPAH is the primary form of pulmonary arterial hypertension, which means that, unlike secondary pulmonary hypertension, it does not occur as a result of another underlying disease.
The exact cause of their development is unknown, however, an increased release of vasoconstrictors, such as thromboxane and endothelin, plus a concurrent diminished production of vasodilators, such as nitric oxide (NO) and prostacyclin, is observed in those affected.
Other factors include increased serotonin levels and increased production of cytokines. The treatment of isolated pulmonary hypertension is demanding and the prognosis is usually poor.
On average, without treatment, a life expectancy of about 3 years after diagnosis. The cause of death is the most commonly occurring right-cardiac decompensation with malignant cardiac arrhythmias.