 Pulmonary Hypertension
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Pulmonary hypertension is a rare blood vessel disorder that results in high blood pressure in the lungs.
When the heart and lungs are functioning normally, a "tree" of blood vessels called the pulmonary artery carries oxygen-poor blood from the heart to the lungs, where it is replenished with fresh oxygen.
See About the Heart and Blood Vessels and Anatomy and Function of the Heart Valves for more information on how the heart and arteries normally work.
Pulmonary hypertension starts when the walls of the blood vessels that supply blood to the lungs constrict, or tighten up. This makes it more difficult for blood to get through to the lungs, and the heart must pump harder to overcome the resistance.
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Pulmonary hypertension can be divided into five categories, based on what can be determined about the cause.
Pulmonary arterial hypertension (PAH)
This type of pulmonary hypertension includes:
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- Idiopathic pulmonary hypertension -- When pulmonary hypertension occurs and there is no known cause, it is referred to as idiopathic pulmonary hypertension.
- Familial pulmonary hypertension -- When pulmonary hypertension is inherited, it is referred to as familial pulmonary hypertension. It is estimated that at least 10 percent of pulmonary hypertension cases occur in families where at least one other person had it.
- PAH related to other causes -- When pulmonary hypertension occurs as a result of exposure to toxins or the effects of other diseases, it is referred to as associated pulmonary hypertension. Causes include:
- diseases such as scleroderma, sickle cell disease and lupus
- heart diseases that cause shunting of extra blood through the lungs, such as patent ductus, ventricular septal defect and atrial septal defect
- diet drugs like fenfluramine and dexfenfluramine ("Fen-phen")
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Pulmonary venous hypertension
This type of pulmonary hypertension is caused by diseases of the left side of the heart, including mitral valve disease and pulmonary vein stenosis.
Pulmonary hypertension associated with disorders of the respiratory system or hypoxemia
This type of pulmonary hypertension is caused by an inadequate amount of oxygen in the bloodstream as a result of either lung disease, exposure to high altitude or breathing disorders such as chronic lung disease, sleep apnea, emphysema and bronchitis.
Pulmonary hypertension caused by chronic thrombotic or embolic disease
This type of pulmonary hypertension is caused by blood clots in the pulmonary arteries, pulmonary embolism or sickle cell disease.
Pulmonary hypertension caused by disorders directly affecting the pulmonary blood vessels
This type of pulmonary hypertension is caused by inflammatory diseases such as schistosomiasis, sarcoidosis or pulmonary capillary disease.
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Pulmonary hypertension starts when the walls of the blood vessels that supply blood to the lungs constrict, or tighten up. This makes it more difficult for blood to get through to the lungs, and the heart must pump harder to overcome the resistance.
Eventually, the extra stress causes the heart to enlarge and become less efficient. Therefore, it is more difficult for blood to flow out of the heart, through the lungs and into the body.
Pulmonary hypertension is a chronic condition and can be life-threatening, however, new treatments are available that have significantly improved the prognosis.
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The following are the most common symptoms of pulmonary hypertension. However, each child and young adult may experience symptoms differently. Symptoms may include:
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- shortness of breath with mild exertion
- fatigue
- chest pain or pressure
- dizzy spells and fainting
- fast heartbeat
- ankle or leg swelling (edema)
- bloating
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The symptoms of pulmonary hypertension may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.
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A pediatric cardiologist or other pediatric physician will perform a physical examination, listening to your child's heart and lungs, and make other observations that help in the diagnosis. Tests that are needed to help in the diagnosis and potential management may include the following:
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- Blood Testing -- This may help identify familial pulmonary hypertension or associated causes of pulmonary hypertension.
- Radiographic Exams (e.g. CT scans) -- Can detail the extent of the disease and possibly identify associated causes of pulmonary hypertension.
- Chest X-ray -- A diagnostic test that uses invisible energy beams to produce images of internal tissues, bones and organs onto film.
- Electrocardiogram (ECG or EKG) -- A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle stress or enlargement.
- Echocardiogram (echo) -- A procedure that evaluates the structure and function of the heart by using sound waves, recorded by an electronic sensor, that produce a moving picture of the heart and heart valves. Estimates of pressures within the heart may be achieved in this fashion.
- Pulmonary Function Testing -- A series of tests to measure your child's breathing and lung capacity, and a diagnostic test that uses radioactive injected and inhaled nuclear lung scan to determine various aspects of blood and air flow through the lungs.
- Cardiac Catheterization -- A procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the heart, as well as in the pulmonary artery and aorta. Contrast dye may be injected to more clearly visualize the structures inside the heart or lungs. Medications may be administered to attempt to better understand causes or treatment options.
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Pulmonary hypertension is a serious condition that can be life-threatening. Although there is no cure, there are several treatments available.
For primary pulmonary hypertension (PPH), treatments may include:
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- inhaled oxygen to help raise the levels of oxygen in the bloodstream
- nitric oxide (NO) to help reduce the resistance in the lung blood vessels and improve heart function
- calcium-channel blockers to relieve constriction in the pulmonary arteries and improve the heart's ability to pump blood
- intravenous prostacyclin, "Flolan," to help open up constricted lung blood vessels and reduce high blood pressure in the lungs
- endothelin antagonists, prostacylin analogs, and phosphodiesterase inhibitors to reduce high blood pressure in the lungs
- anticoagulants to prevent blood clots in the lungs
- diuretics to help kidneys eliminate water
- digoxin to help support the ability of the heart to pump the blood
- lung transplantation for patients who do not respond to medication -- a single-lung, double-lung or heart-lung transplant may be recommended
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For secondary pulmonary hypertension, treating the underlying disease or defect may have significant benefit. Use of many of the treatments listed above, in conjunction with treatment for the associated cause of the disease, may help ease the effects of pulmonary hypertension.
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Children's Hospital Boston is a leader in the use of nitric oxide, prostacyclin analogs, endothelin antagonists and other investigational therapies to treat both secondary and primary pulmonary hypertension in children. Innovations such as these are making primary pulmonary hypertension, once presumed irreversible, a treatable and potentially curable disease. Research in this area is ongoing.
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Contact Children's Hospital Boston Cardiovascular Program physicians for a second opinion.
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