There are many possible causes of pulmonary hypertension (secondary PHT), such as a pre-existing heart disease, a congenital malformation, or other primary lung disease:
- Blood clots in the lungs
- COPD/emphysema
- Scleroderma
- Obstructive Sleep Apnea
- Sickle cell anemia
- Chronic liver disease
- AIDs
- Lupus
- Pulmonary fibrosis
- Left-sided heart failure
Or, there may be no identifiable cause. This is called "idiopathic pulmonary hypertension" or primary pulmonary hypertension (PPH). The blood vessels that supply oxygen to the lungs will, for some reason, become constricted or thickened to the point that the oxygen-rich blood cannot get through to the lungs. This causes an abnormal amount of pressure to build up, forcing the heart to work harder and – in some cases – lead to congestive heart failure.
Signs and Symptoms of Pulmonary Hypertension
Signs of pulmonary hypertension should be the signal to see your physician as soon as possible:
- Shortness of breath (dyspnea) while exerting oneself or at rest
- Unusual fatigue
- Fainting or lightheadedness
- Chest pain
- Heart palpitations (racing heart)
- Bluish coloring in fingertips, ears, lips and skin
- Swelling in the extremities (edema)
The rise in blood pressure in the lungs may eventually result in scarring of the lungs, aggravating the difficulty for blood to flow and oxygen to be exchanged in the lungs.
Treatment of Pulmonary Hypertension
Although the prognosis for PHT is limited because there is no cure for pulmonary hypertension, which gets progressively worse, there are treatments which can lessen the symptoms and make life more comfortable.
- Blood Vessel Dilators-vasodilators- open narrowed blood vessels. One of the most commonly prescribed is Flolan (epoprostenol) which must be continuously administered through an intravenous pump that you wear on your belt or shoulder, as its effects last only a few minutes. Another, newer form of the drug, Ventavis (iloprost) is inhaled every 3 hours with a nebulizer which is more convenient and easier to use.
- Endothelin receptor antagonists – reverses the effects of endothelin, the substance that causes the blood vessel walls to thicken. One of these, Tracleer (bosentan) may improve stamina and relieve symptoms.
- Sildenafil – The same active ingredient that is in Viagra, the medication Revatio is sometimes used, because it opens the blood vessels in the lungs.
- Calcium Channel Blockers – in high doses, these help relax muscles in the walls of the blood vessels. These include Norvasc, Cardizem, Procardia, and others.
- Ambrosentan (Letairis) is another drug that is used but can only be prescribed through the Letairis Education and Access Program (LEAP) because of its high toxicity to the liver.
- Anticoagulants, such as warfarin (Coumadin) help prevent blood clots in the lungs which can be fatal.
- Diuretics, such as Lasix, eliminate excess fluid from the body which lessens the work load on the heart. They also limit the fluid build up in the lungs, and relieve any edema in the ankles, feet and abdomen.
- Oxygen – particularly if sleep apnea is a co-morbidity – is usually prescribed to assist the lungs to function and relieve stress on the heart.
- Transplantation, the last resort, may be an option.
One's own course of action for the best quality of life, is to get plenty of rest, stay as active as possible, quit smoking, avoid pregnancy or using birth control pills, maintain a healthy weight, and adopt a low-sodium diet. Most experts agree that people should consume no more than 1500mg to 2400mg (1 tsp.) of sodium a day.
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