How often is PAH diagnosed?


The incidence (i.e., the number of new disease cases reported in a population over a certain period of time) of PAH is not well documented.


The different forms of PAH have a different incidence. Moreover, the low frequency of the disease, the often non-specific presentation and the fact that it is difficult to diagnose, mean that PAH is likely to be under-diagnosed and under-reported, making it difficult to obtain an accurate estimate of the incidence.

Pulmonary arterial hypertension (PAH) is a type of pulmonary hypertension (PH) characterized by narrowing of the vessels that carry blood from the heart to the lungs to provide oxygen. Due to the narrowing of these blood vessels, the heart has to work harder, which ultimately results in right-sided heart failure and premature death.

What are the causes?


PAH is a complex condition with a wide variety of underlying causes. In all cases it results in the obstruction of the lung circulation.


The result is that the pressure in the pulmonary artery increases. If the pressure (tension) in the blood vessels of the lungs (pulmonary) exceeds a certain value (hyper), it is called pulmonary arterial hypertension.


Common causes for pulmonary arterial hypertension include: congenital heart defects and connective tissue diseases (including systemic sclerosis). However, the cause is unknown for a large number of patients, this is called idiopathic pulmonary arterial hypertension.



The diagnosis of PAH is an intensive procedure, because the health complaints are not specific and often occur in other diseases.


In order to diagnose the patient, the functioning of the heart and the lungs will be examined. In any case, a patient suspected of having PAH or being diagnosed with PAH will undergo many different tests.


Important examinations for the diagnosis of PAH are echo, ECG and pulmonary function tests. If these exams give suspicion of PAH, additional exams, such as an MRI, V/Q and / or CT scan, will often be performed. If all results indicate PAH, the diagnosis can be confirmed using a blood pressure measurement in the pulmonary artery (right heart catheterization).

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