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Introduction Many pulmonary hypertension patients are diagnosed with PH in association with one or more related conditions. Research shows that up to 30% of scleroderma patients, 20-40% of sickle cell patients and one out of every 200 HIV patients develop at least mild PH. Other conditions commonly associated with PH include mixed connective tissue disease, congenital heart disease (CHD), chronic obstructive pulmonary disease (COPD), hereditary haemorrhagic Telangiectasia (HHT), sleep apnoea, liver disease and lupus. PH also has been linked to the use of certain drugs and toxins, including fenfluramine, dexfenfluramine and methamphetamine. |
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Connect and find support
If you're juggling multiple condition's and the symptoms, specialists and medications that accompany each, you're not alone. Many patients and caregivers in our community understand the uncertainty and stress that go hand in hand with a diagnosis of PH related to another disease. Whether you're living with PH plus scleroderma, PH plus liver disease or PH plus another condition, you can share information and connect with people who understand. PHNA support group has many members who are juggling multiple conditions and PH so why not come along and have a chat to these amazing people so they can share their stories of strength and perseverance while tackling multiple chronic conditions. You can also go online to PHAssociation.org for booklets on individual conditions plus PH (PHNA stock a small amount of these).
Anna Bower (left) has pulmonary hypertension,
lupus and scleroderma, with her three nieces |
Symptoms of Pulmonary Hypertension
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The symptoms for all types of pulmonary hypertension (PH) may be similar, and symptoms are usually more severe as the disease progresses. Some of the symptoms of PH may include: (see right)
In advanced stages of pulmonary hypertension, minimal activity may produce some or all of these symptoms. Patients in advanced stages may experience irregular heartbeat, a racing pulse, passing out and difficulty breathing at rest. Sometimes these symptoms mean you have another condition, but sometimes, these symptoms mean you have PH. So ask your Doctor, "what if it's PH"? |
Breathlessness
Chest pain (also called angina pectoris) Dizziness Fainting (also called syncope) Loss of energy Swelling of the arms, legs, ankles, abdomen, face and hands (also called oedema) Dry cough Reynaud's phenomenon (chalky white or dusky blue fingers that may be painful and can sometimes be provoked by the cold). |
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Pulmonary veno-occlusive disease (PVOD) and or pulmonary capillary hemangiomatosis (PCH) & PH
My doctor says I have PVOD, what is this? As the name suggests PVOD involves the small veins of the lung circulation rather than the small arteries. The veins become blocked and the cause is generally unknown. |
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On rare occasions PVOD has occurred in families and a few cases are associated with mutations in BMPR2. PVOD occurs about 10 times less often than IPAH/HPAH, which makes it a really rare disease. PVOD is somewhat different than PAH in that more men are affected, smoking exposure is higher, and a lower carbon monoxide diffusing capacity of the lung (DLCO) is seen.
Also, changes can be detected on high resolution CT scans that are suggestive of PVOD. These are sometimes described as :presence of centrilobular ground glass opacities," although having this on your CT scan does not necessarily mean you have PVOD. A lung biopsy might possibly be necessary to distinguish PVOD from interstitial lung disease, for which there are treatments other than transplant. However, although a lung biopsy is the definitive diagnostic test for PVOD, such biopsies are dangers for patients with PH. Currently, the best treatment for PVOD is lung transplant. Although some of the available PAH therapies may be helpful, they must be used with extreme caution. The patient must be carefully observed to avoid the risk of developing pulmonary oedema (fluid in the lung. Clubbing of fingers might be present in PVOD. |
My doctor says I have PCH, what is this?
Pulmonary capillary hemangiomatosis (PCH) is a disease of the lung capillaries or tiny vessels that are found between arteries and veins. The capillaries tend to overgrow and extend in the lung tissue. This condition is extremely rare and some argue that it is not a separate disease but a different manifestation of PVOD or PAH. Chest x-rays and CT scans may show characteristic signs of disease (very similar to PVOD) and help in determining the best area of lung to biopsy for confirmation of the diagnosis. Unfortunately, open lung biopsy is required to accurately diagnose this condition also. These biopsies are dangerous for PH patients, but may be necessary in order to prevent endangering the patient with potentially harmful therapies. There is not yet an accepted drug treatment for this type of PH; Prostanoids (like Flolan) may be harmful; interferon has been tried, but still lacks a promising track record; lung transplantation is an option. |
Scleroderma & Pulmonary Hypertension
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I was just diagnosed with scleroderma, which I don't fully understand. My doctor has asked me if I get short of breath or feel fatigued or like I'm going to faint. Why am I being asked this?
Scleroderma, which means "hard skin" in Latin, is a type of autoimmune disease that affects the connective tissues and blood vessels of the body. Connective tissue is mostly made of collagen and gives structure and support to the organs of the body. Collagen is a protein that the body uses to make scar tissue to repair itself. In scleroderma, the immune system attacks the body's organs and tissues. As a result, excess collagen is produced and scar tissue forms. The additional symptoms your doctor is mentioning (shortness of breath, fatigue) are those of pulmonary arterial hypertension (PAH) a disease of the small arteries of the lungs. PAH is a common complication of scleroderma. Between 8-12% of all scleroderma patients develop PAH. Scleroderma patients tend to have exercise limitations and it is important to report any changes in your current abilities. Unexplained shortness of breath or increasing fatigue, swelling of the ankles, legs, abdomen and arms, chest discomfort or pain, light headedness and fainting are all symptoms your doctor will want to know about, since these can also be signs of pulmonary arterial hypertension. What can happen to patients with these problems and why should I be concerned? It is absolutely essential that you as a scleroderma patient be aware of your condition, and that you stay in touch with your doctor concerning your symptoms. While many scleroderma patients go on to lead long and enjoyable lives, PAH is a serious and complex condition that requires immediate treatment. Pulmonary complications are the leading cause of death in people with scleroderma. In general, the medications that are used to treat regular PAH do not work as well when used on patients who suffer from PAH and scleroderma together. A possible explanation of this is that the combination of PAH and scleroderma occurs in older patients who might already have other forms of lung disease. It is also possible that patients with scleroderma have a more severe form of PAH of their pulmonary arterioles or that the right side of the heart is less able to handle the higher pressures caused by PAH. In all cases, the earlier a diagnosis is made, the sooner treatment can begin, and the better the outlook is for the patient. What tests can be done? Since patients with scleroderma are typically under a doctor's care for their scleroderma, annual screening for PAH and PH should be done. Screening should include a detailed history about the level of physical activity and if any problems are limiting those activities. The symptoms mentioned before (shortness of breath, swelling etc.) should always be brought to a doctor's attention. |
A pulmonary function test can help check for changes in the lungs associated with scleroderma. Echocardiography is a useful screening tool although it may not always be accurate if a patient is in the earlier or miler stages of PH. A blood test called serum BNP (brain Natriuretic peptide assay) is useful as well, and can help doctors identify several problems. Right heart catheterisation remains the most accurate diagnostic test and provides other information about the heart's condition as well.
If I have PAH as well as scleroderma, what can doctors do to help me? All patients benefit from simple basic measures including attention to fluid balance and therapies that improve the efficiency of the heart. These may include supplemental oxygen or the medication digoxin, as well as anticoagulants (blood thinners). Once a doctor and patient have been able to establish an effective medical approach, many patients show significant improvement, often in conjunction with doctor prescribed cardiopulmonary rehabilitation training (CPRT). Through the years, a variety of medications have been shown to slow the damage and relieve the symptoms caused by PAH. These medications are very complex, and choosing the correct initial treatment requires the knowledge and expertise of a physician who specialises in PAH. Professionals at pulmonary hypertension centres can help identify PH and choose the correct type of treatment. 
Scleroderma Australia (click)
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Detecting Pulmonary Hypertension in Systemic Sclerosis with an Algorithm
Pulmonary hypertension is often related to other diseases and conditions, one of them being scleroderma. Dr. Dinesh Khanna, from the University of Michigan, shares some thoughts on how to detect and diagnose PH in systemic sclerosis patients with the help of a two-step algorithm. ElsGlobalMedicalNews shared this video in 2013, where you can learn more about this subject. |
Congenital Heart Disease & Pulmonary Hypertension
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I have a congenital heart defect. How common is this?
Congenital heart defects affect slightly less than 1% of the population. Because of the development of successfully operative repairs and catheter based corrections performed at an early age, the number of adults with congenital heart disease (CHD) has gradually increased. There are now estimated to be more than a million adults with CHD (in the USA) and, for the first time in medical history, more adults than children with this problem. How is CHD connected to PH?
One of the most common and serious complications that CHD patients develop is pulmonary hypertension (PH). The patients most at risk for this complication are those with so called "shunt lesions." These result in blood flowing abnormally from the left side of the heart (the systemic circulation) to the right side of the heart (the pulmonary circulation). Why is it important to recognise PH in CHD?
Examples of shunts include holes between the upper heart chambers (artrial septal defects) and holes through the lower heart chambers (ventricular septal defects). Usually such shunts are present from birth, but in other cases they have been surgically created as a way to temporarily improve blood flow to the lungs. In either case the presence of excessive blood flow to the pulmonary arteries may trigger the development of increased resistance in the arterial vessels and thus PH. Repairing these lesions early, before they have the chance to cause PH, is important, However, many of these lesions are not detected until PH has already developed. Up to 40% of CHD patients have structures in the heart that predispose them to the development of PH, and up to 10% of patients will actually develop PH. If PH is unrecognised and untreated, it can result in what has traditionally been recognised as "irreversible" damage to the pulmonary vessels and the shunt flow reverses (now traveling from the pulmonary to the systemic circulation). This results in cyanosis (low oxygen delivery to the tissues), a condition that is referred to as Eisenmenger Syndrome. It is now recognised that Eisenmenger Syndrome is not truly irreversible and that medical therapy may improve exercise capacity and possibly even increase survival. Fortunately the incidence of Eisenmenger Syndrome is now rare because of improved detection of CHD in children and the availability of simplified and perfected surgical and catheter based corrections. However, in parts of the world with limited access to healthcare, only 2-15% undergo appropriate surgery and up to 30% of unrepaired patients develop Eisenmenger Syndrome. This makes CHD-PH a major public health concern. Even in the absence of Eisenmenger, PH can have a substantial negative health impact. A recent study showed that PH more than doubled the risk of death and heart complications in CHD patients, including heart failure and arrhythmias. In addition, hospitalisations and length of stay in the hospital and in the ICU were almost three times higher when PH was present. The presence of PH can be detected by performing an echocardiogram. Because PH can be due to an intrinsic problem in the lung vessels (PAH) or from backflow from problems on the systemic side of the heart (pulmonary venous hypertension), a heart catheterisation is often required to differentiate these two very different problems, as therapy varies greatly depending on the diagnosis. What can be done for me? CHD-PH patients should be closely monitored at regular intervals by physicians experienced in the management of their condition. Although separate guidelines now exist for the treatment of CHD patients and for patients with other forms of PH, treatment strategies are for the most part, still based more on clinical experience than on study evidence. Eisenmenger patients, in particular should be managed in centres with experience in the treatment of this syndrome. In general, patients with CHD-PH benefit from a team based approach to care with input from the CHD specialist and the PH specialist. There are several facets of care that need to be addressed in the CHD-PH patient: Oxygen supplementation: Many CHD patients, depending on the degree of shunting, have chronic hypoxia (low oxygen in the blood). Some also have varying degrees of lung disease. Oxygen therapy has not been demonstrated to improve exercise tolerance or survival in patients with CHD-PH, but may improve symptoms. Care should be taken not to over oxygenate, as improvements in saturation are limited and administration can lead to problems such as nose bleeding when given by nasal cannula. Aerobic exercise: It is important to maintain good exercise tolerance, but strenuous exercise generally should be avoided. |
Cardiovascular disease (CVD) is a very common and serious disease in Australia with about 3.5 million people reporting having the condition in 2007-08. Despite significant advances in the treatment of CVD and for some of its risk factors, it remains the cause of more deaths than any other disease - about 50,000 in 2008 - and the most expensive, costing about $5.9 billion in 2004-05. And not all sectors of Australian society are affected equally by CVD with people in lower socioeconomic groups, Aboriginal and Torres Strait Islander people and those living in the remote areas of Australia often more likely to be hospitalised with, or to die from CVD than other members of the population.
Anticoagulation: Although many patients with PH are anticogulated, this practice in the CHD patient is controversial. Patients with CHD are prone to bleeding, particular lung bleeding or haemoptysis (coughing up blood). When anticoagulation is used, levels of blood thinning should be monitored very carefully to prevent such complications.
Diuretics: Diuretics should be used sparingly in order to avoid dehydration, which can provoke hyperviscosity (abnormal denseness of the blood that can limit the blood flow) and can also lead to drops in systemic blood pressure. Treatment of iron deficiency: Despite having higher haematocrits (red blood cell counts) due to low blood oxygen levels (cyanosis), patients with Eisenmenger Syndrome are actually at higher risk for iron deficiency. The latter can increase their risk for hyperviscosity. Phlebotomy ( practice of removing blood to decrease the red blood cell count) should generally be avoided, as this can worsen iron deficiency. When phlebotomy is absolutely necessary, like when neurological problems result, fluid should always be administered whenever blood is removed. Avoidance of pregnancy: CHD-PH is associated with a substantially increased risk for both mother and child during pregnancy. With Eisenmenger Syndrome, there is up to 50% risk of maternal death and 40% risk of spontaneous abortion. Patients should avoid pregnancy and discuss contraceptive options with their doctors. Advanced medical therapy: Randomised controlled trials demonstrating benefits of advanced medical therapy in PH patients have included a small number of patients with CHD-PH (most of whom had undergone a repair of the lesion). There are now reports of use of all three PH drug classes (endothelin antagonists, phosphodiesterase-5 inhibitors and Prostanoids) in patients with CHD-PH. In the randomised BREATHE-5 study, Bosentan (an endothelin antagonist) improved distance walked and did not appreciably change oxygen saturations in Eisenmenger patients. Recent studies suggest that advanced therapy in CHD-PH may significantly improve quality of life and may even improve survival. Transplantation: When no other treatments are successful and quality of life is severely impaired, patients with CHD-PH can undergo heart and lung transplantation. Another option is repair of the heart defect along with lung transplantation. The latter, however, is rarely possible or successful. The early death rate after transplantation appears slightly higher than for patients without CHD; but after this early period, most patients experience dramatic improvements in symptoms and quality of life. |
Liver Disease & Pulmonary Hypertension
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What is liver disease?
To understand liver disease, it is first essential to know what the liver does. The liver is the largest glandular organ in the body, and it has many jobs. It provides a substance that breaks down fats, produces several important compounds, stores certain vitamins, makes specific amino acids, converts glucose to glycogen, and makes sure there are certain levels of glucose in the blood. Most importantly for this discussion, however, the liver filters the blood and removes harmful substances from it. Because it has so many jobs to do, the liver is an extremely important organ in keeping the body healthy. Therefore, if the liver becomes damaged or diseased, it creates problems throughout the body. Liver disease can take the form of cirrhosis (damage and scarring of the liver tissues that results in blocked blood flow and impaired liver function), or obstruction of blood flow to the liver without cirrhosis. How does liver disease relate to pulmonary hypertension?
Liver disease can cause what is known as “portal hypertension,” meaning increased blood pressure in the veins that enter the liver. This increased pressure causes blood to bypass the liver; as a result, the blood is not subject to the liver’s work, which includes the removal or detoxification of chemicals and poisons in the body. As a result, the blood vessels of the lung are exposed to possible toxic substances and this can damage the small arteries of the lungs, causing pulmonary arterial hypertension (PAH). What is POPH?
Portopulmonary hypertension, or POPH, is a type of PAH that occurs as a result of advanced liver disease. This disease has the same characteristic symptoms as those found in cases of PAH that are not associated with liver disease. Those characteristic symptoms of POPH include blood clots and other changes in the blood vessels of the lung. Symptoms the patient may notice are similar to those associated with classic PH, and include shortness of breath and limited ability to tolerate exercise.
Why does POPH develop?
The reason that POPH develops is unclear, but research has given us some clues. It is known that patients with POPH have a deficiency of prostacyclin (a substance that causes the blood vessels to relax), and an excess of endothelin I (an inflammatory mediator that causes the blood vessels to constrict, and also causes the inner and middle layers of the arteries of the lung to grow). In addition, it appears that women and those with autoimmune-related cirrhosis may be predisposed to POPH. Most patients diagnosed with POPH are adults; few are children. Fatty Liver non- alcoholic (click)
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What makes POPH so serious?
In addition to the fact that POPH is a complication of liver disease (which is in itself very serious), POPH is a serious risk factor if a patient is being considered for a liver transplant. The risk of complications and death increase in a transplant patient with moderate or severe POPH. How is POPH diagnosed? A specialist can diagnose POPH by identifying portal hypertension (high pressure in the veins of the liver), in conjunction with the usual signs of PAH. Patients who are being screened to be liver transplant candidates are given an echocardiogram as part of established practice guidelines. If the echocardiogram shows that the pressure in the right side of the heart is abnormally high, that patient will need to have another test, known as a right heart catheterization, to rule out POPH. Any patient with chronic liver disease and shortness of breath (even if he or she is not considering a transplant) should discuss with a specialist the possibility of having echocardiography to be screened for PH. Can patients with POPH be candidates for a liver transplant?
There is not yet an easy answer to this question, again because studies are so limited. The outcome for patients with POPH when liver transplant is attempted continues to be unpredictable. If the pressure is high in the lungs during transplant, mortality during and after surgery is more likely. However, limited but encouraging data suggests that patients who respond to 24-hour continuous intravenous epoprostenol, treprostinil, oral bosentan or sildenafil and who then undergo liver transplantation have excellent survival and in some cases complete resolution of POPH. In all cases, the sooner a patient is tested, the sooner problems like POPH can be ruled out or addressed, generally leading to a better outcome for the patient. How is POPH treated?
Treatment of patients with POPH is an area that is still being studied. Because patients with POPH have not been included in many of the regular PAH drug studies, our knowledge is limited in terms of which drugs are most beneficial in caring for POPH patients. However, there is hope on the horizon. Small studies done in the U.S. with epoprostenol have been encouraging. In both Europe and the U.S., improvement in POPH patients has also been seen in small drug studies with bosentan, ambrisentan, or sildenafil. |
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Pulmonary hypertension (PH) is a severe lung condition that causes shortness of breath, chest pain, fatigue, and dizziness among other symptoms. It’s a disease that can affect anyone, at any age, although women are more prone to developing the condition.
Find out who is more at risk of developing pulmonary hypertension in this article. But pulmonary hypertension can also be related to other diseases, such as scleroderma, lupus, pulmonary fibrosis and, in this case, liver disease. Is there an explanation for the association of pulmonary arterial hypertension with intravenous drug use or chronic liver disease? Does the risk of developing PAH increases with IV drug use? impactID.com clears up doubt with this short interview with Dr. Harrison Farber, where some of the potential causes of PAH are discussed, as well as the influence of intravenous drug and chronic liver disease. Harrison W. Farber, MD, is the director of Pulmonary Hypertension Center Boston University/Boston Medical Center. Learn more about him. |
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Sleep Apnoea and Pulmonary Hypertension
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What is the connection between sleep apnoea and PH? Obstructive sleep apnoea (OSA) has been identified as a significant cause of and/or contributor to cardiovascular disease. OSA has been shown to increase the risk for hypertension, pulmonary vascular disease, ischemic heart disease, stroke, congestive heart failure, and arrhythmia's. The true relationship remains controversial despite the growing body of evidence that links OSA to the development of cardiovascular disease. Many risk factors for OSA are also known risk factors for cardiovascular disease, such as age, male gender, obesity, diabetes mellitus and hypertension. Therefore, it is difficult to prove whether OSA independently causes cardiovascular disease.
How does sleep-disordered breathing affect the body? Episodes of sleep-disordered breathing cause blood vessel changes that can cause and contribute to cardiovascular disease. The adverse effects that OSA imposes on cardiovascular function are thought to arise from recurrent cycles of not breathing, intermittent hypoxia and the resulting arousal's. This cycle of nocturnal desaturation results in an increase in sympathetic tone, impaired nitric oxide synthesis, endothelial dysfunction and ultimately leads to vascular and cardiac dysfunction, atherosclerosis and hypertension. What is the relationship between sleep apnoea and pulmonary hypertension? The 2004 American College of Chest Physicians (ACCP) consensus panel found that pulmonary hypertension occurred in 17% to 53% of individuals with OSA, whereas a review from Johns Hopkins found that 82% of patients with pulmonary arterial hypertension had underlying sleep-disordered breathing. The nocturnal drop in oxygen impairs nitric oxide synthesis and causes vascular remodelling which can lead to the development of pulmonary vascular disease. However, whether OSA independently causes clinically significant pulmonary hypertension remains controversial. Thus, the current ACCP guidelines do not recommend evaluating patients with OSA for pulmonary hypertension unless it is clinically suspected. However, patients with pulmonary arterial hypertension should be evaluated for OSA. What treatments are available? CPAP (continuous positive airway pressure) has been shown to be effective in patients with heart failure. Controlled trials have shown that treatment of OSA with CPAP is associated with significant improvements in cardiac function, sympathetic activity and quality of life. In the Canadian Positive Airway Pressure study, CPAP improved nocturnal oxygenation, increased left ventricular function, lowered norepinephrine levels and improved functional capacity among patients with sleep apnea and heart failure. Several recent studies have shown reductions in pulmonary artery pressure in patients with OSA after nocturnal CPAP treatment. |
PHNA sponsor Respiratory Supplies can help you with not just your oxygen needs but also CPAP machines and more so check out their range or give them a call, ask for Bernard & remember they give PHNA support group members a discount.
www.respiratorysupplies.com.au/ Phone:1300 738 003 |
Lupus and Pulmonary Hypertension
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What do I need to know about lupus-associated PH? First of all, it is essential to know that PH and lupus are two separate conditions and that PH does not cause lupus. However, some lupus patients develop PH. As was discussed in the introduction, there are a number of reasons why blood pressure in the lung may become elevated — almost all of which can occur in lupus. For this reason, it is very important that patients with lupus and suspected PH be looked at very carefully to understand exactly what might be contributing to PH.
Lupus-associated PH may be due to more than just one problem. Other causes of PH include left-heart dysfunction, vasculitis (an inflammation or irrita- tion of small blood vessels in the lung), pulmonary embolism (blood clots in the lungs) or pulmonary parenchymal disease (scarring or irritation of the area surrounding the air sacs and blood vessels in the lungs). Each of these situations would be treated differently. Since this is much more complex than PAH alone, receiving care from a physician who is experienced in the management of lupus and PAH is important. One of the most serious kinds of PH is PAH. This remains a relatively uncommon complication of lupus, but because PAH can be very problematic, it is important to find and treat this as soon as possible. Current estimates suggest that somewhere between 0.5 and 9 percent of patients with lupus may have PAH. Lupus remains a very complex process that can affect one or many organs. In particular, lupus can cause kidney, heart or lung dysfunction in some people, which can lead to shortness of breath, fatigue or fluid retention — all of which can also occur in PAH. These additional factors particular to lupus can complicate management of PAH and may require additional testing to ensure the best treatment of lupus-associated PAH. Why do PH and lupus seem to occasionally occur together? This is another question that medical science is searching to answer. Medical researchers have not yet been able to say for sure how lupus leads to the develop- ment of PAH in some patients. It is not yet known, for example, whether lupus itself can directly cause PH, or whether lupus is simply a trigger for the development of PAH in susceptible individuals. Lupus remains a complicated disease that can cause many problems that weaken blood vessels, and these, too, might play into the development of PAH. Who is most susceptible to a lupus-associated PAH? Lupus-associated PAH occurs more frequently in females and in a slightly younger age group than idiopathic PAH (IPAH) or scleroderma-associated PAH. There also appear to be racial differences in lupus-associated PAH with increased frequency of non-Caucasian patients compared to IPAH or scleroderma-associated PAH. There are no blood tests able to accurately predict if a lupus patient has PAH or might develop it. Should a lupus patient be tested for PH? Lupus patients know that medical supervision is critical to their best possible long-term health and that any change in their condition should be brought to the attention of their doctors. Changes that may have to do with PH might include difficulty when attempting physical activity; swelling of the feet, ankles, legs or abdomen; increased shortness of breath; worsening fatigue; chest discomfort or pain; lightheadedness or fainting. These changes should be communicated to your doctor promptly.
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How is testing for PH carried out? A visit to your doctor and a thorough exam is a good first step for evaluation of potential PH. An echocardiogram is a very useful test that looks at the size and functions of the heart among other things and can suggest whether PH may exist. Unfortunately, it alone is not accurate enough to make the diagnosis of lupus-associated PAH. A test known as right-heart catheterization remains the most accurate way of diagnosing lupus- associated PAH. Additional testing is also typically done to see how much exercise you can do and to check lung function or x-rays to be sure that additional problems in the lungs are not present.
Can PH be treated the same way in lupus patients as in those without lupus? Through the years, a variety of medications have been shown to slow the damage and relieve the symptoms caused by PAH. These medications are very complex, and choosing the correct initial treatment requires the knowledge and expertise of a physician who specializes in PAH. Professionals at PH centres can help identify PH and choose the correct type of treatment. In addition to medications approved for the treatment of PAH, patients may benefit from simple basic measures including attention to fluid balance and therapies that improve the efficiency of the heart. These may include supplemen- tal oxygen or the medication digoxin, as well as anticoagulants (blood thinners). Once a doctor and patient have been able to establish an effective medical approach, many patients show significant improvement, often in conjunction with doctor-prescribed cardiopulmonary rehabilitation training (CPRT). A few patients with lupus-associated PH may also benefit from a group of drugs called immunosuppressive medications. These medications treat irritation or inflammation in the body, which can occur in lupus and occasionally can cause additional damage in the blood vessels of the lungs. It is difficult to know in all cases which patient may benefit from these kinds of medications. Does having lupus make PH worse? The answer is yes and no. In general, patients who have lupus-associated PAH tend to respond to therapy similarly to IPAH patients who do not have lupus. As was noted above, however, patients with lupus may have other problems such as left-heart dysfunction, blood clots in the lungs, inflammation of the lungs or its blood vessels. All of these could make PH worse for that person. |
Hereditary Haemorrhagic Telangiectasia (HHT) & Pulmonary Hypertension
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My doctor says I have Hereditary Haemorrhagic Telangiectasia. What exactly does that mean? Hereditary Haemorrhagic Telangiectasia (HHT) is a genetic disorder that causes abnormalities of blood vessels, usually resulting in excessive bleeding (known as haemorrhaging). In order to understand HHT, you must first understand the way blood is pumped to and from the heart. Blood vessels are hollow structures that transport blood throughout the body.
There are two types of blood vessels: arteries and veins. Arteries carry blood under high pressure away from the heart to all other areas of the body. Blood then passes through capillaries, where oxygen, nutrients and waste products can be exchanged. Veins carry blood that should be under low pressure back to the heart. An artery does not usually connect directly to a vein. A person with HHT has a tendency to form blood vessels that lack capillaries between the arteries and veins, meaning that arterial blood under high pressure flows directly into a vein without first going through the very small capillaries. The site of this abnormal connection tends to be fragile and can rupture and result in bleeding. This type of abnormal blood vessel is called a telangiectasia if it involves small blood vessels. It is called an arteriovenous malformation (AVM) if it involves large blood vessels. Telangiectases usually occur on the surface of the body, such as the skin and the mucous membranes that line the nose and the gastrointestinal tract. AVMs are found in the lungs, liver and central nervous system. My doctor has asked me whether I feel lightheaded, out of breath or faint, and has said these might be symptoms of pulmonary hypertension. How does this relate to my HHT? About 15 to 20% of people with HHT have at least mildly elevated pulmonary artery pressures, which means they either have or are developing PH. HHT patients can develop PH in two ways and the differences are significant.
In HHT-associated pulmonary arterial hypertension, abnormal blood flow through the blood vessels in the lungs causes elevated blood pressure. While it is not known exactly how many people have HHT-associated PAH, research has identified one affected member in 15% of families with a form of HHT known as HHT2. In another type of HHT, known as HHT1, HHT-associated PAH appears to be much less common. |
More commonly, PH can develop in HHT patients as a result of increased blood flow from the heart, a condition called high cardiac output state. A high cardiac output state may be the result of several things, and a doctor may be able to determine the cause. Treatment will vary depending on what type of PH you have.
How do doctors test for this disease? It is often difficult to detect PAH in HHT patients. In fact, some of the symptoms of PAH (fatigue, difficulty breathing and difficulty during physical exertion) are already found in people with HHT. To make things even more confusing, these symptoms are often seen in HHT patients as side effects of other health problems including heart failure, anemia and liver problems. Therefore, in many cases, a doctor must strongly suspect PAH, or it may go overlooked. An echocardiogram can test for abnormally high blood flow in the lungs of HHT patients, and also can screen for PH. In addition, the echocardiogram provides important information about the heart, including its size and function. This test can also help uncover underlying problems the patient may be facing. If PH is suspected in an HHT patient or diagnosed through an echocardiogram, another test, a right heart catheterization, can help doctors confirm it, and can also help to diagnose the exact type of PH the patient is suffering from. What can be done for me? Treatment is available; however, in order for a doctor to accurately treat HHT-associated PH, he or she must first diagnose the specific type of PH the patient has. If HHT-associated PAH is diagnosed, PAH specific therapies can be carefully prescribed, but these medications can also aggravate bleeding tendencies that are already present in HHT patients. As a result, HHT patients with PAH should seek out a specialist at a PH treatment centre. |
Methamphetamine Use & Pulmonary Hypertension
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My doctor recently told me that I have pulmonary hypertension, and also asked me if I had ever used stimulants like methamphetamines (speed). Why am I being asked this? Research continues into the causes and development of pulmonary hypertension (PH). In particular, scientists are trying to identify factors that lead to what we currently call idiopathic pulmonary arterial hypertension (IPAH), or PAH where the cause of the condition is not yet known.
It is essential that you be honest and open with your doctor about all activities in your life, including past or present drug use, since establishing these connections can help your doctor with your treatment plan. It also helps researchers understand the risk factors that apply to patients like you, and to others. What is the connection between stimulants and PH? Information from a new study suggests that methamphetamine (speed) use is associated with the occurrence of IPAH. In that study, individuals with IPAH were 10 times more likely to have used stimulants than individuals with PAH associated with other known risk factors. Interestingly, that risk figure was similar to one found in studies of individuals who developed PAH and had used appetite stimulants (also known as anorexigens). While these are not controlled studies, they do make medical professionals aware of a strong association between stimulant use and PAH. How the two are associated, however (in terms of exactly how a specific stimulant would cause or contribute to the development of PAH), remains unknown. In addition, we know that amphetamines are similar to diet pills like fenfluramine, which is most well known as one component of the once-popular anti-obesity drug known as Fen-Phen. Fen-Phen has since been withdrawn from the market because of a potential link between use of that drug and development of PH and heart valve problems. A high rate of stimulant use (including methamphetamine or cocaine depending on the region of the country) is also common among individuals who are both HIV-infected and who have PAH. Scientists are not sure how infection with HIV increases the risk of PAH, but it is possible that stimulant use has played a role in some of those patients. |
What kinds of symptoms should I tell my doctor about? As mentioned previously, it is important to be completely honest with your health care providers about all aspects of your life. In addition, always report symptoms like fatigue or shortness of breath on exertion, or swelling of the hands or feet. If these symptoms are already present, be sure to notify your doctor if they worsen or become more pronounced.
How can a doctor be sure it’s PH? Several different types of screening tests are available, and some are more effective than others. An echocardiogram is a good screening tool but may not always be accurate. A right heart catheterization remains the most accurate diagnostic test. How is PH treated in patients who have a history of drug use? Despite the fact that individuals with IPAH were 10 times more likely to have used stimulants than individuals with PAH associated with other known risk factors, there is still a relatively small number of cases where patients have admitted to stimulant use. Therefore, very little data exists on the treatment of this group of patients. Healthcare professionals believe that those who have used stimulants and who now have PAH can be treated the same way as other patients, with similar results. |
Sickle Cell Disease & Pulmonary Hypertension
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I have sickle cell disease. My doctor wants to screen me for pulmonary hypertension. Why is this? Sickle cell disease (SCD), a type of anemia (shortage of red blood cells), is characterized by abnormally shaped red blood cells. Sickle cells (named for the sickle, or oblong, slender shape of the malformed blood cells) have decreased flexibility, meaning they break easily. This results in a number of life-long complications.
PH is an increasingly recognized complication of SCD. Studies show that approximately 30% of screened adult patients with SCD develop mild PH in adulthood. In 10% of patients, the PH is more severe. Recent autopsy studies also suggest that up to 75% of sickle cell patients show changes in the lung tissue at the time of death, indicating the existence of pulmonary arterial hypertension (PAH). One possible reason sickle cell patients seem to be at risk for developing PH is that increased breakdown of red blood cells within the blood vessels causes inflammation and a decrease of nitric oxide (a substance that causes relaxation of the blood vessels). This causes constriction of the small arteries of the lungs which may lead to PH. What are some of the symptoms of pulmonary hypertension? Because they are somewhat general symptoms, the characteristics of PH felt by the patient often are mistakenly attributed to other problems. These symptoms include shortness of breath, feeling fatigued, being unable to tolerate increased levels of exercise, unexplained swelling of the ankles, legs, abdomen or arms, chest discomfort or pain, light-headedness and fainting. Your doctor wants to make sure you do not have symptoms that are being overlooked. Why is it so important to screen for PH now? Individuals who suffer from SCD are more likely to develop PH, and those who suffer from PH in addition to SCD are at a higher risk of death than that of SCD patients without PH. Therefore, your doctor wants to test your pulmonary pressure to make sure you’re not currently affected by PH. If PH is present, he or she will want to begin treatment immediately to control both your SCD and your PH. |
Studies from the National Heart, Lung and Blood Institute suggest that PH is the greatest risk factor for death in the aging population of patients with SCD. An echocardiogram is an important screening tool to check for the development of PH in patients with problems like SCD. The echocardiogram can also help to determine the cause of the high pulmonary pressures.
If a doctor determines that I have PH, what’s the next step? Because SCD is a complex disorder with many complications, no specific clinical guidelines have been written for patients who have both SCD and PH. Researchers do not know exactly what role PH plays in the worsening of disease. The National Institute of Health recommends that each case of PH be evaluated by a specialist, and assigned a value, from mild to severe. The therapy used to treat various gradations of the disease will likewise increase or decrease, depending on the severity. A number of different treatments are available, and have been shown to be very effective. Your doctor will help determine the best treatment for you, and will continue to monitor your progress throughout your treatment. Specialists at a PH centre can also help guide you through the process and answer your questions. Brochure PH & Sickle Cell for Medical Professionals Brochure (PDF) PH & Sickle Cell Order copy of PH & Sickle Cell (or contact PHNA for copy) 
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Sarcoidosis & Pulmonary Hypertension
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Pulmonary hypertension (PH) is a rare but life-threatening lung disorder that damages the vessels responsible for transporting blood from the heart to the lungs, known as the pulmonary arteries.
There is currently no cure for pulmonary hypertension, and if not treated it can lead to death. However, there are treatments to help cope with the symptoms. Pulmonary hypertension can be both caused by, or cause, other diseases. Want to learn more about 11 pulmonary hypertension related diseases? Among these diseases is sarcoidosis, a multi system inflammatory disease characterized by noncaseating granulomas, particularly in the lungs and intrathoracic lymph nodes. But how do pulmonary hypertension and sarcoidosis relate? Pulmonary hypertension is a serious complication of sarcoidosis. The combination of the two diseases can occur in any patient, but it is more common among patients in an advanced stage. |
PH and HIV
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What do I need to know about pulmonary hypertension in connection with HIV? Although pulmonary hypertension and HIV are two separate conditions, some HIV patients have developed pulmonary hypertension, a condition called HIV-associated PAH. Scientists are continuing to study this to see if there is a connection, and what that connection might be.
The first case of pulmonary hypertension in an HIV-infected individual was reported in 1987, and since that time research has been ongoing. About 150 patients with HIV infection and pulmonary arterial hypertension have been reported since that time. It is possible that there are other unreported cases. Because the first several HIV-PAH cases that were reported had occurred in patients with haemophilia (a congenital tendency to have uncontrolled bleeding), researchers originally (and incorrectly) thought that the cause of HIV-PAH was the haemophilia itself or perhaps had to do with problems associated with the haemophilia. However, since that time, more HIV-PAH cases have been found in patients who do not have haemophilia at all. Therefore, scientists have decided that the development of pulmonary hypertension in HIV patients is in some way related to the HIV infection itself. How common is the combination of HIV and PAH? PAH is still an uncommon complication of HIV infection. As research into HIV continues and as new and better therapies are developed to combat the disease, HIV patients are living longer lives. As a result, the chance that an HIV-positive individual may develop PAH may increase as time goes on. We can still safely say that PH occurs more frequently in the HIV-infected population than it does in those who do not have HIV. Thus, idiopathic pulmonary arterial hypertension (pulmonary hypertension that results from unknown causes, in this case an HIV-negative group of people), occurs less frequently than it does in those who are HIV infected. Those who are infected with HIV have roughly six to 12 times the occurrence of PAH than the uninfected general population. Who is most susceptible to a combination of HIV and PH? HIV-PAH occurs more frequently in males, and in a slightly older individual than is usually seen in IPAH. In studies of HIV-PAH, the most frequent risk factors for HIV were intravenous drug use (45%), homosexual contact (25%), haemophilia (12%) and heterosexual contact (10%). Smaller numbers of cases have been identified in which the risk for HIV infection is blood transfusion, HIV passed from mother to child during pregnancy, or a combination of homosexual contact and intravenous drug use. A high rate of stimulant use (methamphetamine or cocaine, depending on the region of the country) is common among individuals who are both HIV-infected and afflicted with PAH. (As a side note, intravenous drug use is also associated with development of pulmonary hypertension, even without HIV infection). Does having HIV make PH worse? There doesn’t seem to be any data to indicate that HIV makes PH worse. The usual symptoms of HIV-PAH are similar to those in other individuals with PAH, shortness of breath being the most common. Does PH make HIV worse? It’s hard to say. When tested, some HIV patients who also suffer from PH have lower pulmonary artery pressures than those who have PH alone; despite this, their survival rate appears worse. Medical professionals are not sure whether this has to do with a combination of the two conditions, or whether other problems associated with HIV contribute to the lower survival rate. |
Why do PH and HIV seem to occasionally occur together, then? This is another question that medical science is searching for. We do know that many HIV-infected individuals have other problems and other risk factors that can lead to PH. A direct cause and effect relation between HIV infection and pulmonary hypertension has not yet been found, and proteins associated with HIV have not yet been found in the lining of the blood vessels of the lungs.
Medical researchers have not yet been able to say for sure what role HIV infection plays in the development of PAH. It is not yet known, for example, whether HIV itself can directly cause pulmonary hypertension, or whether HIV is simply a trigger for the development of PAH in susceptible individuals. HIV remains a complicated disease that can cause many problems that weaken blood vessels, and these, too, can play into the development of PAH. Should an HIV patient be tested for pulmonary hypertension? HIV patients know that medical supervision is critical to their best possible long-term health, and that any change in their condition should be brought to the attention of their doctors. Changes that may have to do with PH might include difficulty when attempting physical activity, swelling of the feet, ankles, legs or abdomen, increased shortness of breath, worsening fatigue, chest discomfort or pain, light-headedness or fainting. However, because these symptoms are general and may have to do with several types of problems, the patient’s doctor should always be contacted. How is testing for PH carried out? An echocardiogram is a useful screening tool; unfortunately, it alone is not accurate enough to make the diagnosis of HIV-PAH. A test known as right heart catheterization remains the most accurate way of diagnosing HIV-PAH. Can pulmonary hypertension be treated the same way in HIV patients as in those without HIV? Researchers are working to find the answer to this question, but to date, there have been few studies done with HIV patients who have PAH. The fact that the population of HIV-PAH patients is very small has been a problem for researchers. Another problem has been that the long-term survival of HIV-PAH patients is lower than those who have PH without the addition of HIV. In all cases, however, the sooner PH (or any other complicating problem) is diagnosed in an HIV patient, the better the outlook is for the patient 
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