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Definition of Pulmonary Hypertensive Disease

The most common type of hypertension occurs when the blood flows through the left side of the heart with too much force. Pulmonary hypertensive disease is another type of hypertension that affects the blood flow through the right side of the heart and the pulmonary (lung) arteries. Pulmonary hypertension may occur as the result of another disorder or on its own. There is no cure for pulmonary hypertension, but it can be controlled with treatment.

  1. What Causes Pulmonary Hypertension?

    • Pulmonary hypertension begins when the arteries and capillaries in the lungs either become narrower, become blocked or are destroyed by the formation of extra lung cells. Thus, blood flow through the lungs becomes strained and increases the blood pressure in the pulmonary arteries. This in turn causes the right ventricle of the heart to work harder and weaken over time.

    Idiopathic Pulmonary Hypertension

    • Idiopathic pulmonary hypertension (also known as primary pulmonary hypertension) occurs when there is no underlying medical issue causing pulmonary hypertension. Some individuals may be genetically predisposed to suffer from pulmonary hypertension.

    Secondary Pulmonary Hypertension

    • Sometimes pulmonary hypertension is the direct result of another underlying medical condition. Secondary pulmonary hypertension occurs more often that idiopathic pulmonary hypertension. Conditions that may cause secondary pulmonary hypertension are emphysema, pulmonary embolisms, sclerderma, congenital heart disease, sickle cell anemia, cirrhosis of the liver, AIDS, lupus, pulmonary fibrosis and even sleep apnea. Some drugs such as fenfluramine and cocaine may also cause secondary pulmonary hypertension.

    Symptoms of Pulmonary Hypertensive Disease

    • Pulmonary hypertensive disease many exist months or years without noticeable symptoms. Over time the symptoms will worsen. Symptoms include trouble breathing, chest pressure and pain, fatigue, ankle and leg swelling, dizziness, bluish colored skin and lips and fast heartbeat.

    Diagnosis and Classifications

    • An echocardiogram is used to diagnosis pulmonary hypertension. There are four classes of pulmonary hypertension. Individuals diagnosed with Class I pulmonary hypertension have the disease, but do not have any symptoms yet. Those with Class II pulmonary hypertension only exhibit symptoms with physical exertion. Individuals with Class III pulmonary hypertension exhibit symptoms with very little exertion and those with Class IV have symptoms both at rest and with exertion.

    Treatment

    • Treatments may include medications that help to dilate the blood vessels such as Flolan, Ventavis, Tracleer, Revatio, Ambrisentan and calcium channel blockers. Also, anticoagulants such as Warfarin may be prescribed to prevent dangerous blood clots. Diuretics may be used to remove extra fluid from the body so that the heart does not have to work as hard. Some patients may also receive oxygen therapy and in the most severe cases a heart and/or lung transplant may be needed.

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