Home » Patient
Resource Center » Primary Pulmonary Hypertension
Primary Pulmonary Hypertension
Pulmonary Hypertension or PH is a rare lung disorder
caused due to increase in blood pressure in the pulmonary arteries,
pulmonary vein or pulmonary capillaries. This condition leads to shortness
of breath, dizziness, fainting and other symptoms. Pulmonary hypertension is
a severe disease marked by decreased exercise tolerance and heart failure.
As the disease reaches advance stage, its symptoms may even limit all the
physical activities.
Causes of Pulmonary hypertension
Pulmonary hypertension is caused due to change in the cells that line the
lung's artery. The changes affect the pulmonary artery and veins in three
different ways.
- The walls of the arteries tighten.
- The walls of the arteries are stiff at birth or become stiff from an
overgrowth of cells.
- Blood clots form in the arteries.
The changes make it difficult to push the blood through the arteries into
lung. The result in the rise of pressure causing pulmonary hypertension. A
number of other factors may contribute to the process that leads to the
different types of PH.
Primary pulmonary arterial hypertension (PAH) may have no known cause, or it
may be inherited (passed from parents to children through genes). Certain
diseases and conditions, such as congenital heart disease, HIV, and thyroid
disease, can also cause this type of PH.
Signs & Symptoms
Symptoms of primary pulmonary hypertension may not occur until the
condition progresses. However, some of the common symptoms are as follows:
- Shortness of breath during routine activity, such as climbing two
flights of stairs.
- Tiredness.
- Chest pain.
- A racing heartbeat.
In the advanced stage of the disease, even minimal activity will produce
some or the other symptoms. You may find it difficult to carry any physical
activity. Symptoms at advanced stage are:
- Feeling lightheaded, especially during physical activity.
- Fainting at times.
- Swelling in your legs and ankles.
- A bluish color on your lips and skin.
Who is at Risk
Any one can develop Pulmonary Hypertension. The condition generally
develops between the age of 20 to 60, but it can occur at any age. People
who are considered prone to the disorder include:
- Those who have a family history of the condition.
- Those who have certain diseases or conditions, such as heart and lung
diseases, liver disease, HIV infection, or blood clots in the pulmonary
arteries.
- Those who use certain diet medicines or street drugs (such as
cocaine).
Diagnosis & Treatment
Because pulmonary hypertension may be caused by different medical
conditions, a complete medical history, physical exam, and description of
your symptoms are necessary to identify the other diseases and make the
correct diagnosis. During the physical exam, your health care provider will:
- Listen for abnormal heart sounds such as a loud pulmonic valve sound,
a systolic murmur of tricuspid regurgitation, or a gallop due to
ventricular failure.
- Examine the jugular vein in the neck for engorgement.
- Examine the abdomen, legs, and ankles for fluid retention.
- Examine nail beds for bluish tint.
- Look for signs of other underlying diseases that might be causing
pulmonary hypertension.
- Conduct different types of tests including blood, doppler, chest
X-ray and more.
Appropriate diagnosis and analysis is the prerequisite for starting any
treatment. Treatment varies per individual based on the different underlying
causes. However the treatment includes taking medications; making lifestyle
and dietary changes; having surgery, if necessary; and seeing your doctor
regularly.
