Understanding pulmonary hypertension

Hypertension or high blood pressure is the common condition of when blood pressure, the force of your blood pushing against the walls of your blood vessels, is consistently high.   Most of us are familiar with and have a basic understanding of it.  But there is another type of hypertension we may have heard of but are not clear on exactly what is means.  This hypertension is called pulmonary hypertension or PH and is when a person has high blood pressure in the lungs.  It can also be referred to as pulmonary arterial hypertension or PAH. 

What is PH?

Normally when we have our blood pressure taken it is measured by a cuff on your arm; but this type of blood pressure is not directly related to the pressure in your lungs.  In people with PH, the pressure in the lungs increases, while the blood vessels that supply the lungs constrict and narrow.  This narrowing can also lead to the artery walls thickening making it harder for the right side of the heart to get the blood flow through the lungs and then to the left side of the heart and to the rest of the body. 

This leads to the vessels being unable to carry as much blood and oxygen to the rest of the body, sort of like when a garden hose becomes kinked, and the pressure builds and backs up. This makes the heart have to work even harder as it tries to force the blood through.  If the pressure is high enough, eventually the heart can’t keep up resulting in less blood being able to circulate through the lungs to pick up oxygen.  This is when people with PH can get certain symptoms associated with this condition.

Symptoms of PH

It may take months or years before the constrictions and narrowing in the arteries become severe enough for noticeable symptoms to occur.  Often, the symptoms of PH are not immediately identifiable with PH since many of the symptoms are common to other conditions.  Unfortunately, some people with symptoms may dismiss them making a proper diagnosis more difficult.

Each person with PH will experience a different assortment of symptoms with the severity differing from person to person.  Just because two people may be diagnosed with PH does not mean they will experience the same path as another since the treatment options for PH are so individualized. 

Here are some common symptoms most individuals with PH may have:

·Breathlessness or shortness of breath

·Chest pain also known as angina pectoris

·Dizziness and fatigue

·Fainting also called syncope

·Loss of energy

·Swelling of the arms, legs, ankles, or abdomen also called edema

·Dry cough

·Low oxygen levels can lead to a bluish color to the skin and lips called cyanosis

Risk factors

PH can be diagnosed in people of all races, ages, and ethnic backgrounds but there are certain risk factors that can predispose some people more likely to get the disease:

·Family history

·Obesity and sleep apnea

·Gender – idiopathic PH are at least two and a half times more common in women than men.  Women of childbearing age are also more susceptible


·Altitude – living at a higher altitude increases PH

·Other diseases such as congenital heart disease, lung disease, liver disease and lupus

·Drugs and toxins such as methamphetamines and the diet drug “fen phen”

Diagnosis of PH

Diagnosing PH is not always easy.  Many of the symptoms associated with it are also common in other conditions making it difficult to decipher out what is the cause.  If your physician suspects PH, then there will be specialized tests which could include the following:

·Blood tests

·Chest x-rays

·Electrocardiogram (ECG)


·Pulmonary function tests

·Exercise Tolerance Test or a six-minute walk test

·Nuclear Scan

After conducting initial testing for PH and if the results are indicative of this condition, then a gold standard for a PH diagnosis is to have a right-heart catheterization done which is one of the most accurate and useful tests for diagnosing PH.  Another test that is often used once a patient does get a definitive diagnosis of PH is a vasodilator study.  This test can help determine which patients are suitable for calcium channel blockers and for determining the patient’s prognosis.

Treatment for PH

A diagnosis of PH can be very overwhelming leaving a patient with many questions and a feeling of what to do.  At this time, even though there is no cure for PH, there are treatment options available with more in the future to come.  Treatments include conventional medical therapies and oral, inhaled, intravenous and subcutaneous options. A possible heart or lung transplant is another option depending on the severity of the PH.

No two patients with PH will be treated the same since each case is different.  By working together with your doctor and healthcare team, this is the best way to come up with what treatment options are best for you.