Pulmonary Arterial Hypertension

What is PAH (Pulmonary Arterial Hypertension)?

A subgroup of a broader spectrum of diseases called Pulmonary Hypertension, PAH is a progressive disease that might result in heart failure and death if not treated. Early diagnosis and treatment is vital because it impedes patients’ daily life functions and has high mortality rates.

What is the prevalence of PAH?

Although the data on worldwide incidence rate of Pulmonary Hypertension is insufficient, its average prevalence is 50 people per million. The prevalence of its subgroup, Pulmonary Arterial Hypertension, ranges from 5 to 15 per million. The largest group of these cases are the one with unknown causes. Although these groups of cases were diagnosed late in previous years, the average age of diagnosis today is mid-30s for PAH of unknown cause, and around 50 to 60 overall.

What are the diagnosis and treatment methods?

Even though using the basic diagnosis methods such as ECG, Echocardiography, Pulmonary Function Tests and Imaging methods (X-ray, CT, MRI, etc.) are administered, it is necessary to measure arterial pressure with cardiac catheterization and run certain drug reaction tests to determine the groups of high technology and complex medications for prescription. If these tests enable a diagnosis, the function grade of patients should be determined considering their specific effort capacity, and treatment should be started with one or more of the medication groups currently acting on the three pathways. 

Severe cases, which are non-responsive to treatment, might require organ transplantation as a last resort.
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