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Everything you wanted to know about Primary (Idiopathic) Pulmonary Arterial Hypertension

Apr 08, 2019 | 10 months ago | Read Time: 3 minutes | By iKnowledge Team

Here’s what you should know about Primary (Idiopathic) Pulmonary Arterial Hypertension. You will also gain insight into its causes, symptoms and treatment.

Idiopathic pulmonary arterial hypertension, or IPAH, is a rare lung disorder that is characterised by elevated pressure in the pulmonary arteries. There is no apparent cause for this event to occur, and hence the name ‘idiopathic’. It is unfortunately a life-threatening condition and can worsen over time. However, it is entirely possible to manage the condition so that a person diagnosed with IPAH is still able to lead a quality life as before.


Simply put, when you have IPAH, the fine, small arteries in your lungs become narrow or get blocked. The average blood pressure in your pulmonary arteries is higher than 25 mmHg (millimetres of mercury) while resting, and higher than 30 mmHg while you’re at rest, when you have IPAH.


This makes it harder for blood to flow through them, which in turn causes the blood pressure in your lungs to rise. Consequently, this puts pressure on your heart, because the organ now must pump harder to get blood flowing through these blocked arteries. Over a period, this can weaken the heart muscle, eventually leading even to heart failure. This makes it doubly important for the condition to be managed well, once diagnosed.


What, then, could be the cause of this disease? As the name suggests, a cause for IPAH isn’t ascertained, but the theory is that it may just be a genetic disorder. Other reasons could include heart valve disorders, congenital heart conditions, a tumour pressing down on the pulmonary artery, or even the presence of a lung disease.


You are at a higher risk of developing this condition if you are already suffering from:

  • Liver cirrhosis
  • HIV and AIDS
  • A history of having smoked tobacco, used drugs like cocaine, taken appetite suppressants
  • A connective tissue disorder such as systemic lupus erythematosus

At the smallest sign of a problem with your heart or lungs, do get yourself checked for IPAH. It is sometimes hard to diagnose since it doesn’t always present symptoms in those suffering from it. What’s worse, the symptoms for IPAH may be confused with those of other heart and lung disorders.


There are tests that can be carried out to screen you for this disease. These include:

  • 12-lead electrocardiogram or EKG
  • Pulmonary function test
  • Studies to check your lungs, heart, chest such as an x-ray, CT scan, MRI or even an echocardiogram
  • A walking test for six minutes under supervision of a doctor is also carried out to ascertain the presence of symptoms such as shortness of breath

While the disease can be identified through timely checks as indicated, there is no cure available for IPAH. It is a condition that can be managed to reduce the progression of the disease. Typically, it is recommended to take the below mentioned medications:

  • Diuretics to flush out excess bodily fluids and reduce swelling
  • A blood thinner to prevent clots
  • A calcium channel blocker to relax the muscles in the blood vessels
  • A therapy such as endothelin receptor antagonist, to slow the progression of IPAH

Making healthy lifestyle changes can also help keep the condition in check.


However, do keep in mind that IPAH requires constant medical supervision owing to the medical complications that can quickly arise. This may even cause irreparable damage to your heart or lungs.


Health insurance plans will see you through the illness and a possible hospital stay. In today’s times of rapidly spiralling costs, being unwell is almost unaffordable and having adequate insurance cover can make all the difference. But, just how much term insurance do you require?


Here’s how you can ascertain what the perfect life cover is for you. This handy guide tells you just how much term insurance cover you require for you and your family. We often tend to overlook basic aspects such as planning for our financial future. Unfortunately, what we don’t realise is that it’s not just the distant future, but even the more immediate one that needs attention. Illness can strike you or your loved ones at any time, and it’s best to be prepared to tackle any contingency. This is where term insurance such as the iTerm Plus Insurance Plan from Aegon Life comes in. Given that this plan covers up to 36 critical illnesses in addition to providing life cover, you don’t need to look elsewhere for a critical illness policy.


Ensure the financial future of your loved ones today.

II/Mar 2019/4885

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