Primary Pulmonary Hypertension

Primary Pulmonary Hypertension – This is a rare pathological condition of the lungs in which the blood vessels of the lungs become narrow. This narrowing progresses over time and results in an increase of blood pressure in these vessels. This condition is also known by the name of Unexplained Pulmonary Hypertension as in this disease there is an increase of blood pressure without any underlying heart or lung ailment.

Primary Pulmonary Hypertension

This article discusses about:

What Is Primary Pulmonary Hypertension?

Primary Pulmonary Hypertension as stated is a rare condition of the lungs in which there is narrowing of the blood vessels in the lungs which gradually worsens over a period of time and results in high blood pressure in these vessels. The symptoms of Primary Pulmonary Hypertension develop very slowly such that an individual may be suffering from Primary Pulmonary Hypertension but it may not be identified for many years. The main focus of treatment is to control symptoms and to keep the blood pressure in check. This can be done with medications, surgery, and various alterations in lifestyle. The exact cause of the narrowing of the vessels is still unknown.

How Does Primary Pulmonary Hypertension Affect The Body?

Primary Pulmonary Hypertension is caused due to changes in the cells that line the arteries of the lungs. There are basically four changes in the arteries which may eventually lead to Primary Pulmonary Hypertension and these changes are:

  • Tightening of the walls of the arteries
  • Stiffness of the wall of the arteries right at the time of birth
  • Stiffness of the arteries as a result of overgrowth of cells
  • Formation of blood clots in the arteries.

As a result of Primary Pulmonary Hypertension, the heart becomes weak and is unable to pump enough blood to lungs causing heart failure.

Who Is At Risk For Developing Primary Pulmonary Hypertension?

There is no age barrier for development of Primary Pulmonary Hypertension but it is usually found between the ages of 20-60. Studies have shown it tends to occur more in females than males.

Is Primary Pulmonary Hypertension A Serious Disease Condition?

At present, there is no definitive cure for Primary Pulmonary Hypertension. This condition worsens over time and makes performance of tasks difficult for the affected individual. It is seen that people who are above 40 years of age and do not have any underlying heart condition tend to survive longer than others. Quite a few of affected people make alterations in their lifestyle and go around performing their tasks without much of a problem. The treatment for Primary Pulmonary Hypertension is designed by the physician based on their ability to function in day to day activities. This functioning is classified into four different categories:

Class I: In this category, there is no limitation for the affected individual to perform any tasks without development of symptoms like chest pain or shortness of breath etc.

Class II: In this category, the individual has mild limitation of activities and the individual does not experience any symptoms at rest but any sort of excess physical activity causes development of symptoms like chest pain, shortness of breath, fatigue etc.

Class III: In this category, individual experience significant limitation in activities that they can do. They may be no problems at rest but any sort of physical exertion can cause development of symptoms.

Class IV: In this category, the individual is not able to perform any sort of activity without experiencing symptoms. There may also be signs of heart failure as well. The individual may experience symptoms at rest as well as with any activity.

What Are The Symptoms Of Primary Pulmonary Hypertension?

Some of the symptoms of Primary Pulmonary Hypertension are:

  • Extreme fatigue
  • Racing heartbeat
  • Chest pain
  • Shortness of breath with activities
  • Lightheadedness
  • Fainting spells
  • Edema of the lower extremities
  • Bluish discoloration of the skin.

How Is Primary Pulmonary Hypertension Diagnosed?

Primary Pulmonary Hypertension is basically a difficult disease to diagnose on routine examinations. Even in the advanced stages, the symptoms caused by Primary Pulmonary Hypertension are very similar to other heart or lung conditions.

In order to diagnose Primary Pulmonary Hypertension, the treating physician will first take a detailed history and inquire about the family history of the individual and may recommend the following tests:

  • EKG
  • Chest x-ray
  • Right heart catheterization
  • Pulmonary function tests
  • Overnight pulse oximetry
  • Lung perfusion scan
  • Blood draws to rule out other diseases causing symptoms like HIV, liver disease, or autoimmune disorders.
  • Exercise stress test.

What Are Treatments For Primary Pulmonary Hypertension?

Primary Pulmonary Hypertension as stated has no cure but treatments are formulated to relieve symptoms and slow the progression of the disease. The treatment formulated is dependent on the severity of the disease and the symptoms experienced. The treatment for Primary Pulmonary Hypertension is usually three-fold which includes:

  • Medication
  • Surgery
  • Lifestyle Modifications,

Medications For Primary Pulmonary Hypertension: The symptoms of Primary Pulmonary Hypertension can be treated using quite a few medications. The treating physician monitors closely the response to the medication: Some of the medications used are:

  • Diuretics: These medications reduce fluid buildup in the body and help with the edema in the lower extremities.
  • Blood Thinners: These medicines are used to prevent formation of blood clots in the arteries.
  • Digoxin: This is a medication which makes the heart pump more blood.
  • Oxygen Therapy: This is used to increase oxygenation of blood.

Surgical Procedures For Primary Pulmonary Hypertension: Some of the surgical procedures done for relief of symptoms are:

  • Transplantation: This is usually done for those people with extreme cases of Primary Pulmonary Hypertension.
  • Septostomy: This is a procedure in which the surgeon makes a hole between the top chambers of the heart so that blood is distributed in the body without going to the lungs. It reduces strain to the heart but at the same time significantly reduces the amount of oxygen that is delivered to the body.

Lifestyle Modifications For Primary Pulmonary Hypertension

Some of the alterations in lifestyle that can be done for relief of symptoms of Primary Pulmonary Hypertension are:

  • Nutrition: Maintain a balanced and healthy diet so that more nutrition goes into the body.
  • Physical Activity: Regular exercises improve the ability to be active
  • Smoking Cessation: This is only for smokers with Primary Pulmonary Hypertension, as it is important for them to stop smoking for them to stay healthy.

Written, Edited or Reviewed By:


Last Modified On: July 1, 2016

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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