Primary Pulmonary Hypertension

Definition of Primary Pulmonary Hypertension

Pulmonary hypertension is a kind of high blood pressure that affects the veins in the lungs and the right side of your heart.

Pulmonary hypertension is a fatal illness that worsens with its progress and sometimes it may become fatal also. Albeit pulmonary hypertensiondisease is not curable, treatments are available which can help alleviate symptoms.

Symptoms of Primary Pulmonary Hypertension

The symptoms of pulmonary hypertension in its primary stages may not be observed for several months or even years. With the progression of the disease, symptoms deteriorate. 
Pulmonary hypertension symptoms include:

  • Shortness of breath, initially during exercise and later at rest also
  • Tiredness
  • Dizziness or fainting episodes
  • Chest pain
  • Swelling in ankles, legs and ultimately in abdomen
  • Bluish lips and skin
  • Fast pulse or heart palpitations

Causes of Primary Pulmonary Hypertension

Idiopathic Pulmonary Hypertension

When the reason behind high blood pressure in the lungs can't be detected, the condition is calledidiopathic pulmonary hypertension (IPH).

In most people with idiopathic pulmonary hypertension, the exact cause of their pulmonary hypertension is not yet discovered.

Secondary Pulmonary Hypertension

Causes of secondary pulmonary hypertension are:

  • Blood clots in the lungs
  • Connective tissue disorders
  • Congenital heart disease
  • Sleep apnea and other sleeping disorders
  • Chronic obstructive pulmonary diseases like emphysema
  • Sickle cell anemia
  • Chronic liver disease (cirrhosis)
  • Left-sided heart failure
  • AIDS
  • Hiking to altitudes higher than 8,000 feet without acclimating first
  • Lung diseases viz., pulmonary fibrosis, a medical condition causing scarring in the tissue between the lungs' airways
  • Use of certain stimulant drugs, such as cocaine


Pulmonary hypertension can result in various complications, including:

  • Right-sided heart failure
  • Arrhythmia
  • Blood clots
  • Bleeding

Tests and Diagnosis

The first tests to diagnose pulmonary hypertension can be:

  • Chest X-ray
  • Echocardiogram
  • Transesophageal echocardiogram
  • Right heart catheterization

Your doctor may prescribe additional tests to monitor your lungs and heart condition which may include:

  • Perfusion lung scan
  • Pulmonary function test
  • Open-lung biopsy
  • Computerized tomography (CT) scans.
  • Magnetic resonance imaging (MRI)

Treatments and Medication

When pulmonary hypertension is a result of some other condition, the physician may diagnose underlying reasons.


  • Blood vessel dilators: This medication is regularly injected through an intravenous (IV) catheter using a small pump that you wear on your belt or shoulder.
  • Endothelin receptor antagonists: This drug reverses the influence of endothelin, a substance in the walls of blood veins that narrows them.
  • Sildenafil: Revatio (sildenafil) is often administered to treat pulmonary hypertension. It opens the blood vessels in the lungs to enable blood to flow through more easily.
  • High-dose calcium channel blockers: These drugs enable the muscles to relax in the walls of your blood vessels.  
  • Ambrisentan: This is yet another drug that prevents the narrowing of your blood vessels.
  • Anticoagulants: Your doctor is likely to recommend the anticoagulant warfarin (Coumadin) to prevent the creation of blood clots within the small pulmonary veines.
  • Diuretics: Generally known as water pills, these medications enable eliminate excess fluid from your body.
  • Oxygen: Pure oxygen may be recommended by your doctor to breathe to help treatpulmonary hypertension.


  • Atrial SeptostomyIn this surgery the surgeon develops an opening between the left and right chambers of your heart to release the pressure on the right side of your heart.
  • Transplantation: In some cases, a lung or heart-lung transplant may be recommended, especially for younger individuals who have idiopathic pulmonary hypertension.

Lifestyle and Home Remedies

Nevertheless medical science can't cure pulmonary hypertension, it can alleviate the symptoms. Lifestyle changes, which can improve the condition, are:

  • Take plenty of rest: Resting can alleviate the tiredness that may be felt because ofpulmonary hypertension.
  • Stay active: Even the slightest forms of activity may be too tiring for some individual withpulmonary hypertension.
  • Quit smoking: If you smoke, the most necessary thing you should do for your lung and heart is to quit smoking.
  • Avoid being pregnant or using birth control pills: Pregnancy can be life-threatening for both you and your child. Also discard birth control pills which are risky and can enhance blood clots.
  • Avoid going to or living at high altitudes: High altitudes can deteriorate the symptoms ofpulmonary hypertension.
  • Avoid getting into low blood pressure: Avoid enjoying a sauna or hot tub or taking long warm baths or showers. It will lower your blood pressure and cause fainting or even prove to be fatal.
  • Find ways to reduce stress: These can span from meditation, yoga, and biofeedback to music, warm baths, or a good book.
  • Follow a nutritious diet and maintain a healthy weight: It's likely that you will be advised to limit the amount of salt in your diet to alleviate swelling of your body's tissues (edema).