Pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare form of high blood pressure. It

occurs in the pulmonary arteries, which flow from your heart and throughout
your lungs.

Constricted and narrowed arteries prevent your heart from pumping adequate
blood. When constriction occurs, the heart will need to work harder to
compensate. This causes the blood pressure in the pulmonary arteries and in
the heart to increase dramatically.

As the condition worsens and the pressure becomes greater, you may begin
experiencing a variety of signs and symptoms.

A delay in symptoms
It may take months, even years, before the constrictions and narrowing in the
arteries become severe enough that noticeable pressure begins to build. For
that reason, PAH may progress for several years before symptoms become
noticeable.

The symptoms of PAH are also not immediately identifiable as being caused
by PAH. In other words, many of the symptoms are common to other
conditions. Even worse, you may easily dismiss them, as they typically grow
worse gradually, rather than quickly. This makes proper diagnosis more
difficult.

Recognizing the initial symptoms

The first symptoms of PAH, especially shortness of breath and fatigue, may
make you think you’re just out of shape. After all, it’s not uncommon to get out
of breath after climbing several sets of stairs, even if you’re physically active
every single day. For that reason, many people ignore the symptoms of PAH
and let the disease progress without treatment. This makes the condition
worse and potentially fatal.

Shortness of breath
One of the first symptoms of PAH you may notice is shortness of breath. The
arteries and blood vessels that carry blood into and through the lungs make
breathing possible. The inhale-exhale routine helps you quickly bring in
oxygen-rich air and expel oxygen-depleted air. PAH can make that well-
choreographed routine more difficult, even labored. Tasks that were once
easy — climbing stairs, walking the block, cleaning the house — may become
more difficult and leave you breathless quickly.

Fatigue and dizziness

When your lungs can’t get enough blood to properly function, that means your body
and your brain aren’t getting enough oxygen either. Your body needs oxygen to
perform all of its tasks. Without it, you can’t carry on with your normal routine. Your
legs will tire more quickly after a walk. Your brain and thought processing will seem
slower, more labored. In general, you’ll feel tired earlier and more easily.

Lack of oxygen to the brain also increases your risk

for dizziness or fainting (syncope).

Swelling in the limbs

PAH can cause swelling, or edema, in your ankles, legs, and feet. Swelling occurs
when your kidneys are unable to properly flush waste from your body. Fluid retention
becomes increasingly likely the longer you have PAH.

Blue lips
Your heart pumps oxygen-rich red blood cells through your body to help fuel
all the activities and functions you need. When the amount of oxygen in your
red blood cells is inadequate due to PAH, your body parts can’t get the
oxygen they need. Low oxygen levels in your skin and lips can cause a bluish
color. This condition is called cyanosis.