Pulmonary Arterial Hypertension is otherwise called as Primary Pulmonary Hypertension and these medical terms are shortened as PAH and PPH respectively. Even though, this condition was earlier called by the latter name, the name was changed to the former in the year 1998 as it was determined to be more descriptive.
What is it?
This is a disorder that is associated with an abnormal increase in the pressure of pulmonary artery, pulmonary Capillary Wedge and Pulmonary Vascular Resistance. A gradual increase in these pressure levels in pulmonary veins can lead to eventual heart failure. Increased pressure is an indication of PAH.
Imaging tests are generally used for looking inside the body for diagnosis of this condition. When the health care provider identifies that there is an enlargement of arteries or damage to the heart, it is a sure sign of PAH. However, the doctor will prescribe medications for preventing heart damage and for dealing with the symptoms.
Reports state that about a third of people die within three years from the diagnosis of this condition. But, new treatments options are now available and the number of deaths has been brought down. Thanks to the development of medical technology.
Risk factors associated with PAH:
Even though, facts about this condition is not fully understood, it is found that certain diseases can make people more prone to the development of PAH:
• HIV Infection and even though the reason for this is not known, it is found that the rate of development of PAH in patients with HIV is 6 times higher than normal people.
• Patients with Portal Hypertension are more likely to develop PAH.
• In addition, patients with chronic inherited and unrepaired heart disease are more likely to develop PAH.
What causes PAH?
The causes can be either idiopathic PAH or Familial PAH. The former occurs spontaneously without any specific cause, the later is genetically passed down through families. Reports state that most cases of FPAH have acquired this condition due to mutation in the BMPR2 gene. Patients with severe FPAH are generally linked to the presence of mutation. Even though, this will be present at birth itself, it will take years or months together for developing. IPAH can be caused due to some medications and environmental factors. For instance, some appetite suppressants and hormone therapy can lead to IPAH.
Symptoms of PAH:
The characteristic symptom associated with this condition is an increase in the pulmonary vascular resistance. This leads to an increase in the pressure on the right ventricle of the heart and this in turn leads to issue in filling the left part of the heart. The result is eventual heart failure. Common symptoms include:
• Difficulty in breathing or exertion
• Tiredness that is caused by the heart as it will have to pump harder and harder
• Near fainting
Actually diagnosis of this condition cannot be done easily. The reason is that damage inside the heart cannot be diagnosed during general physical examination. However, an ECG, Chest X-Ray, CT Scan, transthoracic echocardiography or lung biopsy might also be suggested.