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Other Names:
Pulmonary Arterial Hypertension, Sporadic Primary Pulmonary Hypertension, Familial Primary Pulmonary Hypertension
Pulmonary Hypertension

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Definition

Pulmonary hypertension is a condition when the blood pressure in the arteries of the lungs is abnormally high due to narrowing or blockage.

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Types

  • Primary or idiopathic pulmonary hypertension - In this condition a definitive cause for the high blood pressure in the lungs can not be found.
  • Secondary hypertension - When the pulmonary hypertension results from another condition.

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Causes

  • Primary pulmonary hypertension
    • Definitive cause is unknown
    • Inherited predisposition may be due to a genetic defect.
    • Other medical conditions like chronic liver disease (cirrhosis), AIDS, sickle cell anemia and connective tissue diseases such as scleroderma and lupus seem to trigger this condition
  • Secondary pulmonary hypertension
    • Pulmonary embolism.
    • Chronic obstructive pulmonary disease
    • Emphysema.
    • Scleroderma.
    • Sleep apnea.
    • Congenital heart disease.
    • Pulmonary fibrosis
    • Left ventricular failure

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Symptoms

  • Breathing difficulty especially during activity
  • Chest pain
  • Weakness and fatigue
  • Fainting
  • Dizziness and fainting spells
  • Swelling of leg, ankles and abdomen
  • Cyanosis
  • Rapid pulse
  • Palpitations

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Implications

Pulmonary hypertension is the result of greater resistance to blood flow. As a result of the increased workload caused by this resistance, the right side of the heart becomes enlarged. Eventually, progressive heart failure may develop. Blood clots may develop in the narrowed pulmonary arteries, thus further aggravating the condition. The clots may dislodge from the pulmonary arteries and travel to to other parts of the body like brain, heart, leg veins, kidney etc and cause serious life threatening complications like stroke. Fluid might accumulate in the lungs due to high pressure of blood in the pulmonary arteries. This is called pulmonary edema and is a potentially life threatening condition. Presence of fluid in the lungs makes it difficult for gaseous exchange to take place.

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What You Can Do

  • Medications
    • Vasodilators - Drugs such as Prostacyclin (Epoprostenol) acts as a powerful blood vessel dilator and anti-clotting agent. This drug must be continuously infused through an intravenous (IV) catheter via a small pump that you wear in a pack on your belt or shoulder.
    • Endothelin receptor antagonists - These medications are present in the form of pills such as Bosentan (Tracleer). This drug helps to relax the muscles in the blood vessel wall.
    • Calcium channel blockers - These drugs help relax the muscles in the walls of the blood vessel and are also used to treat systemic hypertension. They include medications such as amlodipine (Norvasc), diltiazem (Cardizem, Tiazac) and nifedipine (Adalat, Procardia).
    • Anticoagulants - These are also called blood thinners and include drugs such as warfarin (Coumadin). These medications help to prevent the formation of blood clots. Since the anti-coagulants prevent normal blood clotting, they increase your risk of bleeding complications such as bleeding from your gums and intestinal tract.
    • Diuretics - Also known as water pills, these medications help remove excess fluid from your body, which reduces the amount of work your heart has to do to pump the blood.
  • Constant oxygen therapy might be needed.
  • Consult your physician if you suddenly develop breathing difficulty which progressively worsens and there is chest pain that has recently increased in intensity.
  • Lung transplantation - Single-lung transplant is the most common lung transplant procedure used for people with primary pulmonary hypertension.
  • General measures
    • Get plenty of rest. Getting ample rest can help combat the fatigue that may accompany pulmonary hypertension.
    • Eat a nutritious and well balanced diet mainly comprising of lean meat, fish, whole grains, fresh fruits and vegetables.
    • Maintain a healthy weight.
    • Quit smoking
    • Stay as active as possible by doing low impact physical exercises.
    • Avoid living or traveling to places at high elevation as it may aggravate the symptoms of pulmonary hypertension.
    • Reduce stress by practicing techniques like yoga, meditation, biofeedback, warm baths, music or a good book. This can dramatically relieve the symptoms and improve your quality of life.

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Pulmonary Hypertension