Disease: Pulmonary Hypertension

    What are pulmonary arteries?

    The human body has two major sets of blood vessels that distribute blood from the heart to the body. One set pumps blood from the right heart to the lungs and the other from the left heart to the rest of the body.

    • The portion of the circulation that distributes oxygen-rich blood from the left side of the heart, throughout the body, is referred to as the systemic circulation.
    • The blood then returns from the body to the right side of the heart and passes through the lungs to replenish oxygen.
    • It then returns to the left side of the heart for another round through the systemic circulation.
    • The portion of the circulation that distributes the blood from the right side of the heart to the lungs is referred to as the pulmonary (lung) circulation.
    • The pulmonary arteries are the major blood vessels that carry blood from the right side of the heart to the lungs. The pulmonary veins carry the blood with oxygen back to the left side of the heart for distribution to the rest of the body via the systemic circulation.

    The left ventricle of the heart pumps oxygenated blood (blood that has been reloaded with oxygen in the lungs) from the lungs into the systemic circulation. When a doctor or a nurse measures the blood pressure on a person's arm, he/she is measuring the pressures in the systemic circulation. When these pressures are abnormally high, the person is diagnosed as having high blood pressure (hypertension).

    What is pulmonary hypertension?

    The right ventricle pumps blood returning from the body into the pulmonary arteries to the lungs to receive oxygen. The pressures in the lung arteries (pulmonary arteries) are normally significantly lower than the pressures in the systemic circulation. When pressure in the pulmonary circulation becomes abnormally elevated, it is referred to as pulmonary hypertension. This most commonly occurs when the pulmonary venous pressure is elevated, so called pulmonary venous hypertension (PVH). This pressure is transmitted back to the right side of the heart and the pulmonary arteries. The result is elevated pulmonary pressure throughout the pulmonary circulation. Some of this is a direct pressure transmission from the venous system backward and some can result from a reactive constriction of the pulmonary arteries.

    Less commonly, pulmonary hypertension results from constriction, or stiffening, of the pulmonary arteries that supply blood to the lungs, so called pulmonary arterial hypertension (PAH).

    Whether it is PVH or PAH, it becomes more difficult for the heart to pump blood forward through the lungs. This stress on the heart leads to enlargement of the right heart and eventually fluid can build up in the liver and other tissues, such as the in the legs.

    What are primary and secondary pulmonary hypertension?

    In the conventional classification, pulmonary hypertension, which is also called pulmonary arterial hypertension, is divided into two main categories; 1) primary pulmonary hypertension (not caused by any other disease or condition); and 2) secondary pulmonary hypertension (caused by another underlying condition). Secondary pulmonary hypertension is much more common than primary pulmonary hypertension.

    A newer classification of this condition is based on the main underlying cause of pulmonary hypertension. This system classifies the condition based on whether it is due to:

    • Pulmonary hypertension from a variety of causes, some known and others unknown, such as:
      • drug-induced pulmonary artery hypertension,
      • pulmonary artery hypertension associated with collagen vascular diseases, HIV, and schistosomiasis (a parasitic infection), and
      • pulmonary arterial hypertension of unknown cause (idiopathic pulmonary arterial hypertension)
    • Pulmonary arterial hypertension from left-sided heart disease, often referred to as pulmonary venous hypertension, including thickening of the heart muscle, decrease heart pump function, and disorders of the heart valves
    • Pulmonary arterial hypertension associated with lung disease and or persistent drop in oxygen levels (hypoxia) including:
      • COPD (chronic obstructive pulmonary disease),
      • sleep apnea,
      • pulmonary fibrosis, and
      • living at high elevations
    • Pulmonary arterial hypertension from blood clotting disorders that deliver clots to the lung (pulmonary emboli) or are formed directly in the lung arteries (pulmonary thrombosis), also known as chronic thromboembolic pulmonary hypertension;
    • Pulmonary arterial hypertension from miscellaneous causes with unclear multifactorial mechanisms; including diseases such as:
      • sarcoidosis,
      • tumor obstruction,
      • metabolic disorders (glycogen storage disease), and
      • liver failure; so termed portopulmonary hypertension

    What causes pulmonary hypertension?

    There are many causes of pulmonary hypertension. Often more than one mechanism is involved in a specific disease process. This can also change as the disease progresses.

    • Diseases that effect flow out of the heart to the rest of the body result in backflow of blood (stacking of blood) that raises pulmonary venous pressures leading to pulmonary hypertension.
    • Hypoxic pulmonary vasoconstriction is the process in which the lung vessels narrow in attempt to divert blood from poorly functioning segments of the lung. For instance, when pneumonia develops, a portion of lung becomes inflamed and works poorly in performing the functions of the lung (to add oxygen and remove carbon dioxide from the blood). This process diverts blood from these poorly working areas and sends it to better functioning lung tissue. However, a problem develops when all the blood has a low oxygen level (hypoxia). This causes constriction of the vessels on the pulmonary arterial side and hence raises the pressure.
    • Remodeling of blood vessels also occurs in some diseases whereby the inner lining (lumen) of the vessel becomes narrowed due to inappropriate growth of the tissue within and around the vessel. Masses and scarring from other diseases can compress and narrow vessels causing increased resistance to flow resulting in elevation of pressures.
    • In a fairly common parasitic infection in the Middle East (schistosomiasis), the blood vessels in the lung become blocked by the parasites causing pulmonary artery hypertension.
    • Some substances cause constriction of the blood vessels. Pulmonary hypertension has been rarely reported with the use of anti-obesity drugs, such as dexfenfluramine (Redux) and Fen/Phen. These medications have seen been removed from the market. Some street drugs such as, cocaine and methamphetamines can cause severe pulmonary hypertension.
    • Some diseases raise pulmonary pressures to cause pulmonary artery hypertension for unclear reasons. Perhaps an unknown toxin or chemical effects the blood vessels by causes constriction or inappropriate growth of the tissue within or around the vessel. For example, there is a condition known as portopulmonary hypertension that is result of liver failure. When these individuals receive a liver transplant, the pulmonary hypertension disappears suggesting that the failing liver is unable to clear some biochemical that leads to pulmonary artery hypertension.

    Learn more about: Redux | cocaine

    What causes primary pulmonary hypertension?

    Primary pulmonary hypertension has no identifiable underlying cause. Primary pulmonary hypertension is also referred to as idiopathic pulmonary hypertension. Primary pulmonary hypertension is more common in young people and more common in females than males.

    Primary pulmonary hypertension is an unusually aggressive and often fatal form of pulmonary hypertension. Whereas it is known that the arterial obstruction is caused by a building up of the smooth muscle cells that line the arteries, the underlying cause of the disease has long been a mystery.

    A genetic cause of the familial form of primary pulmonary hypertension has been discovered. It is caused by mutations in a gene called BMPR2. BMPR2 encodes a receptor (a transforming growth factor beta type II receptor) that sits on the surface of cells and binds molecules of the TGF-beta superfamily. Binding triggers conformational changes that are shunted down into the cell's interior where a series of biochemical reactions occur, ultimately affecting the cell's behavior. The mutations block this process. This discovery may provide a means of genetic diagnosis and a potential target for the therapy of people with familial (and possibly also sporadic) primary pulmonary hypertension.

    How common is pulmonary hypertension?

    There are no clear statistical data on the prevalence of pulmonary hypertension. Since pulmonary hypertension is seen in multiple different conditions, it is perhaps better to look at the prevalence in each condition. It is fairly common in patients with heart failure, COPD, and sleep apnea. It occurs in 7% to 12% of patients with collagen vascular disease and in less than 5% of HIV patients.

    What are the signs and symptoms of pulmonary hypertension?

    Many people with pulmonary hypertension may have no symptoms at all, especially if the disease is mild or in the early stages.

    Pulmonary hypertension symptoms may include:

    • Shortness of breath that worsens with activity
    • Other common complaints are cough, fatigue, dizziness, and lethargy.
    • With the advancement of the condition and ensuing right heart failure, shortness of breath may become worse and retention of fluid in the body may increase (due to failure of the heart to pump blood forward) resulting in swelling the legs.
    • People may also complain of chest pain and angina.
    • Depending on the underlying associated disease, pulmonary artery hypertension can have other manifestations. For example, characteristic skin changes seen in scleroderma, or the signs of liver disease seen in portopulmonary hypertension.

    Signs of pulmonary hypertension may include:

    • Rapid breathing, hypoxia (low oxygen level in the blood), and swelling in the legs.
    • In severe pulmonary hypertension, the health care professional may hear louder than normal components of heart sounds when he or she listens to the heart with a stethoscope (auscultation).
    • The doctor may also feel elevation of the chest wall when the heart pumps and this may indicate enlargement of the right side of the heart suggestive of pulmonary hypertension (right ventricular heave).

    How is pulmonary hypertension diagnosed?

    The first step in diagnosis of pulmonary hypertension is to clinically suspect it. This may be done as part of an evaluation of another disease that can lead to pulmonary hypertension (such as scleroderma or chronic obstructive pulmonary disease), or based on patients and signs and symptoms as described above.

    Many tests, such as echocardiogram, may be performed that may give clues to the possibility of pulmonary hypertension. It is important that a Doppler study be performed with the echocardiogram, which enables the doctor to approximate the pulmonary artery pressures. These values are calculated based on the sound quality of the wave approaching and leaving the echocardiogram machine sensor/probe. This is based on the principle that explains why the sound of an approaching and then passing train varies.

    But the gold standard (the best test available) is right heart catheterization. This test entails inserting a catheter through the groin into the femoral vein, a large vein in the lower body (or under the collar bone or in the upper arm or neck into a large vein in the upper body) and advancing it to the right side of the heart. The catheter is connected to a device that can monitor and measure blood pressure in the right side of the heart and pulmonary arteries.

    During right heart catheterization, oxygen levels are measured during various positions of the catheter in the pulmonary circulation. This can help determine if a congenital or acquired hole has formed in the heart contributing to the elevated pulmonary pressures. Certain medications or activities can be administered or performed during the procedure to help with the assessment. Nitric oxide can be inhaled and in certain forms of PAH a dramatic improvement in pressures can occur suggesting specific treatment options such as the use of calcium channel blocker medication. Some patients have an exaggerated pulmonary pressure response to exercise and this too can be measured by utilizing arm exercise during the procedure. Titration of medications for pulmonary hypertension while the he catheter is in place can help monitor the response to treatment and assist in optimizing therapy. This can assist in providing individualized therapy for patients with this complex disease.

    Pulmonary hypertension is defined as the mean pulmonary artery blood pressure greater than 25 millimeter of mercury (mmHg) measured by right heart catheterization. The pressures can be much higher than 25 mmHg in some people. Therefore, the pulmonary hypertension can be labeled as mild, moderate, or severe based on the pressures. Mean arterial pressure is two-thirds of the difference between systolic and diastolic blood pressure (systolic is the upper number and diastolic is the lower number in measuring blood pressure). Normally, the pulmonary blood pressure is much lower pressure system than the systemic blood pressure (which is usually measured with a blood pressure cuff).

    What tests other than right heart catheterization may be used in diagnosing pulmonary hypertension?

    Other tests available for diagnosing pulmonary hypertension include electrocardiogram (ECG, EKG), chest X-ray, and echocardiogram. An ECG may show some abnormalities that may be suggestive of right heart failure. Chest x-ray may also show enlargement of the chambers of the right heart. And echocardiogram (ultrasound of the heart) shows ultrasound images of the heart and can detect evidence of right heart failure and with the use of Doppler (as described previously) can estimate pressures in the pulmonary artery. These tests, in the right clinical setting, are very useful in diagnosing and managing pulmonary hypertension.

    Other tests may be useful in evaluating the conditions leading to secondary pulmonary hypertension. For example, a ventilation-perfusion scan (V/Q scan) can suggest blood clots in the pulmonary arteries or sometimes a CT scan of the chest can be used. The chest CT scan can detect pulmonary arterial clots, but also can show abnormalities of the lung tissue and surrounding structures that can contribute to pulmonary hypertension. A pulmonary function testing can be useful in diagnosing chronic obstructive pulmonary disease (COPD) and monitor disease progression. This test can be used to detect many aspects of lung function including airflow and evidence of obstruction, lung volumes, and the capacity for the lung to extract oxygen from the air.

    What is the treatment for pulmonary hypertension?

    The treatment for pulmonary hypertension depends on the underlying cause.

    If left sided heart failure is the primary problem, then adequate treatment of the left heart failure by a cardiologist is the main stray of treatment.

    In cases where hypoxia (low oxygen levels) due to any chronic lung disease, such as COPD, is the cause, then providing oxygen and appropriately treating the underlying lung disease by a lung doctor (pulmonologist) is the first step in treatment.

    In some patients, the elevated pressure may be related to obstructive sleep apnea syndrome (OSAS), and can be reduced with the use of a CPAP, BIPAP (a device that delivers positive airway pressure during sleep).

    In conditions, such as scleroderma, which often can cause pulmonary hypertension, a rheumatologist is involved in the treatment program.

    Anticoagulation (thinning the blood) may be a treatment option if the main underlying cause is thought to be recurrent blood clot (chronic thromboembolic pulmonary hypertension). As indicated in previous section, referral to a specialty center may be warranted for a possible surgical removal of blood clot (thromboendarterectomy).

    For patients with primary pulmonary hypertension (those with no underlying cause), more advanced therapy may be attempted. These drugs have complex mechanisms, but in general they work by dilating (opening up) the pulmonary arteries and, therefore, by reducing the pressure in these blood vessels and some help prevent the excessive overgrowth of tissue in the blood vessels (that decrease remodeling of the vessels, as described previously). Besides constriction, PAH can result in an actual structural change of the pulmonary arteries, whether it is due to chronically elevated pressures or the disease itself, or a combination of the two is not completely clear. These drugs also can delay and in some cases reduce the degree of remodeling of the pulmonary arteries.

    There are three major classes of drugs used to treat idiopathic pulmonary hypertension and pulmonary hypertension associated with collagen vascular diseases: 1) prostaglandins; 2) phosphodiesterase type 5 inhibitor; and 3) endothelium antagonists.

    1. Prostaglandins such as epoprostenol (Flolan), treprostinil (Remodulin, Tyvaso), iloprost (Ventavis). These drugs are very short-acting and often must be given intravenously or inhaled on a very frequent or continuous basis.
    2. Phosphodiesterase type 5 inhibitors such as sildenafil (Revatio, Viagra) and tadalafil (Adcirca, Cialis) are somewhat less effective than the prostaglandins, but are easily administered one to three times per day by mouth. (The dosing is much different when these drugs are used for erectile dysfunction.)
    3. Endothelium antagonists are the newest medications used for this condition. These include bosentan (Tracleer) and Ambrisentan (Letairis). These medications are also given by mouth one to two times per day.

    In rare cases, calcium channel blockers may be of benefit.

    Currently, research is investigating the best ways to combine these medications for the optimal clinical outcomes.

    It should be noted that these medications are extremely expensive, costing thousands of thousands of dollars per year. The companies that manufacture these medications often have programs to assist in funding.

    These more advanced therapies have also been used for other forms of pulmonary hypertension, however, no clinical studies have yet confirmed benefits in these situations.

    It is worth mentioning that regardless of the cause of pulmonary hypertension, supplemental oxygen and diuretics (water pills) may play an important role in relieving the symptoms of pulmonary hypertension of any cause. Low oxygen in the atmosphere causes low blood oxygen levels and aggravates pulmonary hypertension. Therefore, patients with pulmonary hypertension may benefit from breathing supplemental oxygen, especially during air travel or traveling to high altitude destinations.

    Despite advances in various treatments, there is no cure for pulmonary hypertension.

    What are primary and secondary pulmonary hypertension?

    In the conventional classification, pulmonary hypertension, which is also called pulmonary arterial hypertension, is divided into two main categories; 1) primary pulmonary hypertension (not caused by any other disease or condition); and 2) secondary pulmonary hypertension (caused by another underlying condition). Secondary pulmonary hypertension is much more common than primary pulmonary hypertension.

    A newer classification of this condition is based on the main underlying cause of pulmonary hypertension. This system classifies the condition based on whether it is due to:

    • Pulmonary hypertension from a variety of causes, some known and others unknown, such as:
      • drug-induced pulmonary artery hypertension,
      • pulmonary artery hypertension associated with collagen vascular diseases, HIV, and schistosomiasis (a parasitic infection), and
      • pulmonary arterial hypertension of unknown cause (idiopathic pulmonary arterial hypertension)
    • Pulmonary arterial hypertension from left-sided heart disease, often referred to as pulmonary venous hypertension, including thickening of the heart muscle, decrease heart pump function, and disorders of the heart valves
    • Pulmonary arterial hypertension associated with lung disease and or persistent drop in oxygen levels (hypoxia) including:
      • COPD (chronic obstructive pulmonary disease),
      • sleep apnea,
      • pulmonary fibrosis, and
      • living at high elevations
    • Pulmonary arterial hypertension from blood clotting disorders that deliver clots to the lung (pulmonary emboli) or are formed directly in the lung arteries (pulmonary thrombosis), also known as chronic thromboembolic pulmonary hypertension;
    • Pulmonary arterial hypertension from miscellaneous causes with unclear multifactorial mechanisms; including diseases such as:
      • sarcoidosis,
      • tumor obstruction,
      • metabolic disorders (glycogen storage disease), and
      • liver failure; so termed portopulmonary hypertension

    What causes pulmonary hypertension?

    There are many causes of pulmonary hypertension. Often more than one mechanism is involved in a specific disease process. This can also change as the disease progresses.

    • Diseases that effect flow out of the heart to the rest of the body result in backflow of blood (stacking of blood) that raises pulmonary venous pressures leading to pulmonary hypertension.
    • Hypoxic pulmonary vasoconstriction is the process in which the lung vessels narrow in attempt to divert blood from poorly functioning segments of the lung. For instance, when pneumonia develops, a portion of lung becomes inflamed and works poorly in performing the functions of the lung (to add oxygen and remove carbon dioxide from the blood). This process diverts blood from these poorly working areas and sends it to better functioning lung tissue. However, a problem develops when all the blood has a low oxygen level (hypoxia). This causes constriction of the vessels on the pulmonary arterial side and hence raises the pressure.
    • Remodeling of blood vessels also occurs in some diseases whereby the inner lining (lumen) of the vessel becomes narrowed due to inappropriate growth of the tissue within and around the vessel. Masses and scarring from other diseases can compress and narrow vessels causing increased resistance to flow resulting in elevation of pressures.
    • In a fairly common parasitic infection in the Middle East (schistosomiasis), the blood vessels in the lung become blocked by the parasites causing pulmonary artery hypertension.
    • Some substances cause constriction of the blood vessels. Pulmonary hypertension has been rarely reported with the use of anti-obesity drugs, such as dexfenfluramine (Redux) and Fen/Phen. These medications have seen been removed from the market. Some street drugs such as, cocaine and methamphetamines can cause severe pulmonary hypertension.
    • Some diseases raise pulmonary pressures to cause pulmonary artery hypertension for unclear reasons. Perhaps an unknown toxin or chemical effects the blood vessels by causes constriction or inappropriate growth of the tissue within or around the vessel. For example, there is a condition known as portopulmonary hypertension that is result of liver failure. When these individuals receive a liver transplant, the pulmonary hypertension disappears suggesting that the failing liver is unable to clear some biochemical that leads to pulmonary artery hypertension.

    Learn more about: Redux | cocaine

    What causes primary pulmonary hypertension?

    Primary pulmonary hypertension has no identifiable underlying cause. Primary pulmonary hypertension is also referred to as idiopathic pulmonary hypertension. Primary pulmonary hypertension is more common in young people and more common in females than males.

    Primary pulmonary hypertension is an unusually aggressive and often fatal form of pulmonary hypertension. Whereas it is known that the arterial obstruction is caused by a building up of the smooth muscle cells that line the arteries, the underlying cause of the disease has long been a mystery.

    A genetic cause of the familial form of primary pulmonary hypertension has been discovered. It is caused by mutations in a gene called BMPR2. BMPR2 encodes a receptor (a transforming growth factor beta type II receptor) that sits on the surface of cells and binds molecules of the TGF-beta superfamily. Binding triggers conformational changes that are shunted down into the cell's interior where a series of biochemical reactions occur, ultimately affecting the cell's behavior. The mutations block this process. This discovery may provide a means of genetic diagnosis and a potential target for the therapy of people with familial (and possibly also sporadic) primary pulmonary hypertension.

    How common is pulmonary hypertension?

    There are no clear statistical data on the prevalence of pulmonary hypertension. Since pulmonary hypertension is seen in multiple different conditions, it is perhaps better to look at the prevalence in each condition. It is fairly common in patients with heart failure, COPD, and sleep apnea. It occurs in 7% to 12% of patients with collagen vascular disease and in less than 5% of HIV patients.

    What are the signs and symptoms of pulmonary hypertension?

    Many people with pulmonary hypertension may have no symptoms at all, especially if the disease is mild or in the early stages.

    Pulmonary hypertension symptoms may include:

    • Shortness of breath that worsens with activity
    • Other common complaints are cough, fatigue, dizziness, and lethargy.
    • With the advancement of the condition and ensuing right heart failure, shortness of breath may become worse and retention of fluid in the body may increase (due to failure of the heart to pump blood forward) resulting in swelling the legs.
    • People may also complain of chest pain and angina.
    • Depending on the underlying associated disease, pulmonary artery hypertension can have other manifestations. For example, characteristic skin changes seen in scleroderma, or the signs of liver disease seen in portopulmonary hypertension.

    Signs of pulmonary hypertension may include:

    • Rapid breathing, hypoxia (low oxygen level in the blood), and swelling in the legs.
    • In severe pulmonary hypertension, the health care professional may hear louder than normal components of heart sounds when he or she listens to the heart with a stethoscope (auscultation).
    • The doctor may also feel elevation of the chest wall when the heart pumps and this may indicate enlargement of the right side of the heart suggestive of pulmonary hypertension (right ventricular heave).

    How is pulmonary hypertension diagnosed?

    The first step in diagnosis of pulmonary hypertension is to clinically suspect it. This may be done as part of an evaluation of another disease that can lead to pulmonary hypertension (such as scleroderma or chronic obstructive pulmonary disease), or based on patients and signs and symptoms as described above.

    Many tests, such as echocardiogram, may be performed that may give clues to the possibility of pulmonary hypertension. It is important that a Doppler study be performed with the echocardiogram, which enables the doctor to approximate the pulmonary artery pressures. These values are calculated based on the sound quality of the wave approaching and leaving the echocardiogram machine sensor/probe. This is based on the principle that explains why the sound of an approaching and then passing train varies.

    But the gold standard (the best test available) is right heart catheterization. This test entails inserting a catheter through the groin into the femoral vein, a large vein in the lower body (or under the collar bone or in the upper arm or neck into a large vein in the upper body) and advancing it to the right side of the heart. The catheter is connected to a device that can monitor and measure blood pressure in the right side of the heart and pulmonary arteries.

    During right heart catheterization, oxygen levels are measured during various positions of the catheter in the pulmonary circulation. This can help determine if a congenital or acquired hole has formed in the heart contributing to the elevated pulmonary pressures. Certain medications or activities can be administered or performed during the procedure to help with the assessment. Nitric oxide can be inhaled and in certain forms of PAH a dramatic improvement in pressures can occur suggesting specific treatment options such as the use of calcium channel blocker medication. Some patients have an exaggerated pulmonary pressure response to exercise and this too can be measured by utilizing arm exercise during the procedure. Titration of medications for pulmonary hypertension while the he catheter is in place can help monitor the response to treatment and assist in optimizing therapy. This can assist in providing individualized therapy for patients with this complex disease.

    Pulmonary hypertension is defined as the mean pulmonary artery blood pressure greater than 25 millimeter of mercury (mmHg) measured by right heart catheterization. The pressures can be much higher than 25 mmHg in some people. Therefore, the pulmonary hypertension can be labeled as mild, moderate, or severe based on the pressures. Mean arterial pressure is two-thirds of the difference between systolic and diastolic blood pressure (systolic is the upper number and diastolic is the lower number in measuring blood pressure). Normally, the pulmonary blood pressure is much lower pressure system than the systemic blood pressure (which is usually measured with a blood pressure cuff).

    What tests other than right heart catheterization may be used in diagnosing pulmonary hypertension?

    Other tests available for diagnosing pulmonary hypertension include electrocardiogram (ECG, EKG), chest X-ray, and echocardiogram. An ECG may show some abnormalities that may be suggestive of right heart failure. Chest x-ray may also show enlargement of the chambers of the right heart. And echocardiogram (ultrasound of the heart) shows ultrasound images of the heart and can detect evidence of right heart failure and with the use of Doppler (as described previously) can estimate pressures in the pulmonary artery. These tests, in the right clinical setting, are very useful in diagnosing and managing pulmonary hypertension.

    Other tests may be useful in evaluating the conditions leading to secondary pulmonary hypertension. For example, a ventilation-perfusion scan (V/Q scan) can suggest blood clots in the pulmonary arteries or sometimes a CT scan of the chest can be used. The chest CT scan can detect pulmonary arterial clots, but also can show abnormalities of the lung tissue and surrounding structures that can contribute to pulmonary hypertension. A pulmonary function testing can be useful in diagnosing chronic obstructive pulmonary disease (COPD) and monitor disease progression. This test can be used to detect many aspects of lung function including airflow and evidence of obstruction, lung volumes, and the capacity for the lung to extract oxygen from the air.

    What is the treatment for pulmonary hypertension?

    The treatment for pulmonary hypertension depends on the underlying cause.

    If left sided heart failure is the primary problem, then adequate treatment of the left heart failure by a cardiologist is the main stray of treatment.

    In cases where hypoxia (low oxygen levels) due to any chronic lung disease, such as COPD, is the cause, then providing oxygen and appropriately treating the underlying lung disease by a lung doctor (pulmonologist) is the first step in treatment.

    In some patients, the elevated pressure may be related to obstructive sleep apnea syndrome (OSAS), and can be reduced with the use of a CPAP, BIPAP (a device that delivers positive airway pressure during sleep).

    In conditions, such as scleroderma, which often can cause pulmonary hypertension, a rheumatologist is involved in the treatment program.

    Anticoagulation (thinning the blood) may be a treatment option if the main underlying cause is thought to be recurrent blood clot (chronic thromboembolic pulmonary hypertension). As indicated in previous section, referral to a specialty center may be warranted for a possible surgical removal of blood clot (thromboendarterectomy).

    For patients with primary pulmonary hypertension (those with no underlying cause), more advanced therapy may be attempted. These drugs have complex mechanisms, but in general they work by dilating (opening up) the pulmonary arteries and, therefore, by reducing the pressure in these blood vessels and some help prevent the excessive overgrowth of tissue in the blood vessels (that decrease remodeling of the vessels, as described previously). Besides constriction, PAH can result in an actual structural change of the pulmonary arteries, whether it is due to chronically elevated pressures or the disease itself, or a combination of the two is not completely clear. These drugs also can delay and in some cases reduce the degree of remodeling of the pulmonary arteries.

    There are three major classes of drugs used to treat idiopathic pulmonary hypertension and pulmonary hypertension associated with collagen vascular diseases: 1) prostaglandins; 2) phosphodiesterase type 5 inhibitor; and 3) endothelium antagonists.

    1. Prostaglandins such as epoprostenol (Flolan), treprostinil (Remodulin, Tyvaso), iloprost (Ventavis). These drugs are very short-acting and often must be given intravenously or inhaled on a very frequent or continuous basis.
    2. Phosphodiesterase type 5 inhibitors such as sildenafil (Revatio, Viagra) and tadalafil (Adcirca, Cialis) are somewhat less effective than the prostaglandins, but are easily administered one to three times per day by mouth. (The dosing is much different when these drugs are used for erectile dysfunction.)
    3. Endothelium antagonists are the newest medications used for this condition. These include bosentan (Tracleer) and Ambrisentan (Letairis). These medications are also given by mouth one to two times per day.

    In rare cases, calcium channel blockers may be of benefit.

    Currently, research is investigating the best ways to combine these medications for the optimal clinical outcomes.

    It should be noted that these medications are extremely expensive, costing thousands of thousands of dollars per year. The companies that manufacture these medications often have programs to assist in funding.

    These more advanced therapies have also been used for other forms of pulmonary hypertension, however, no clinical studies have yet confirmed benefits in these situations.

    It is worth mentioning that regardless of the cause of pulmonary hypertension, supplemental oxygen and diuretics (water pills) may play an important role in relieving the symptoms of pulmonary hypertension of any cause. Low oxygen in the atmosphere causes low blood oxygen levels and aggravates pulmonary hypertension. Therefore, patients with pulmonary hypertension may benefit from breathing supplemental oxygen, especially during air travel or traveling to high altitude destinations.

    Despite advances in various treatments, there is no cure for pulmonary hypertension.

    Source: http://www.rxlist.com

    The treatment for pulmonary hypertension depends on the underlying cause.

    If left sided heart failure is the primary problem, then adequate treatment of the left heart failure by a cardiologist is the main stray of treatment.

    In cases where hypoxia (low oxygen levels) due to any chronic lung disease, such as COPD, is the cause, then providing oxygen and appropriately treating the underlying lung disease by a lung doctor (pulmonologist) is the first step in treatment.

    In some patients, the elevated pressure may be related to obstructive sleep apnea syndrome (OSAS), and can be reduced with the use of a CPAP, BIPAP (a device that delivers positive airway pressure during sleep).

    In conditions, such as scleroderma, which often can cause pulmonary hypertension, a rheumatologist is involved in the treatment program.

    Anticoagulation (thinning the blood) may be a treatment option if the main underlying cause is thought to be recurrent blood clot (chronic thromboembolic pulmonary hypertension). As indicated in previous section, referral to a specialty center may be warranted for a possible surgical removal of blood clot (thromboendarterectomy).

    For patients with primary pulmonary hypertension (those with no underlying cause), more advanced therapy may be attempted. These drugs have complex mechanisms, but in general they work by dilating (opening up) the pulmonary arteries and, therefore, by reducing the pressure in these blood vessels and some help prevent the excessive overgrowth of tissue in the blood vessels (that decrease remodeling of the vessels, as described previously). Besides constriction, PAH can result in an actual structural change of the pulmonary arteries, whether it is due to chronically elevated pressures or the disease itself, or a combination of the two is not completely clear. These drugs also can delay and in some cases reduce the degree of remodeling of the pulmonary arteries.

    There are three major classes of drugs used to treat idiopathic pulmonary hypertension and pulmonary hypertension associated with collagen vascular diseases: 1) prostaglandins; 2) phosphodiesterase type 5 inhibitor; and 3) endothelium antagonists.

    1. Prostaglandins such as epoprostenol (Flolan), treprostinil (Remodulin, Tyvaso), iloprost (Ventavis). These drugs are very short-acting and often must be given intravenously or inhaled on a very frequent or continuous basis.
    2. Phosphodiesterase type 5 inhibitors such as sildenafil (Revatio, Viagra) and tadalafil (Adcirca, Cialis) are somewhat less effective than the prostaglandins, but are easily administered one to three times per day by mouth. (The dosing is much different when these drugs are used for erectile dysfunction.)
    3. Endothelium antagonists are the newest medications used for this condition. These include bosentan (Tracleer) and Ambrisentan (Letairis). These medications are also given by mouth one to two times per day.

    In rare cases, calcium channel blockers may be of benefit.

    Currently, research is investigating the best ways to combine these medications for the optimal clinical outcomes.

    It should be noted that these medications are extremely expensive, costing thousands of thousands of dollars per year. The companies that manufacture these medications often have programs to assist in funding.

    These more advanced therapies have also been used for other forms of pulmonary hypertension, however, no clinical studies have yet confirmed benefits in these situations.

    It is worth mentioning that regardless of the cause of pulmonary hypertension, supplemental oxygen and diuretics (water pills) may play an important role in relieving the symptoms of pulmonary hypertension of any cause. Low oxygen in the atmosphere causes low blood oxygen levels and aggravates pulmonary hypertension. Therefore, patients with pulmonary hypertension may benefit from breathing supplemental oxygen, especially during air travel or traveling to high altitude destinations.

    Despite advances in various treatments, there is no cure for pulmonary hypertension.

    Source: http://www.rxlist.com

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