Diagnosis And Treatment Of Pulmonary Hypertension
TRENTON D. NAUSER, M.D., and STEVEN W. STITES, M.D., University of Kansas Medical Center, Kansas City, Kansas
Am Fam Physician. 2001 May 1 63:1789-1799.
Pulmonary hypertension is a complex problem characterized by nonspecific signs and symptoms and having multiple potential causes. It may be defined as a pulmonary artery systolic pressure greater than 30 mm Hg or a pulmonary artery mean pressure greater than 20 mm Hg.
The etiology of primary pulmonary hypertension is unknown. Secondary pulmonary hypertension can be a complication of many pulmonary, cardiac and extrathoracic conditions. Cor pulmonale is enlargement of the right ventricle as a consequence of disorders of the respiratory system. Pulmonary hypertension invariably precedes cor pulmonale. Unrelieved pulmonary hypertension, regardless of the underlying cause, leads to right ventricular failure.
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Managing Pulmonary Arterial Hypertension
Being diagnosed with a chronic illness like PAH is life-changing. Patients will need to have a close relationship with a PAH expert as well as an excellent support system of family and friends.
Many pulmonary hypertension care centers require visits every few months and regular testing such as echocardiograms and 6-minute walk testing. Some centers will do right heart catheterizations every year to see how well treatment is working on pulmonary pressures and heart function. Additionally, it is essential to take your medications exactly as directed, being careful not to run out or change your schedule unless directed by your doctor. It is also important to get yearly flu and pneumonia vaccinations.
PAH patients should adopt or continue these lifestyle changes to maintain their health.
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What Causes Pulmonary Hypertension
Causes of pulmonary hypertension vary widely depending on the type of PH you have. They include a range of diseases and underlying conditions as well as environmental exposures .
Group 1 PH due to pulmonary arterial hypertension
There are many causes of pulmonary arterial hypertension. They include:
Some people develop PAH without any clear cause. These cases are known as idiopathic.
Group 2 PH due to left-sided heart disease
Heart problems are a common cause of pulmonary hypertension. Because the left and right sides of your heart work together, a problem with the left side also affects the right side. The right side of your heart is responsible for pumping blood into your pulmonary arteries. So, left-sided heart problems cause a chain reaction that affects the rest of your heart, your pulmonary arteries and your lungs.
Left-sided heart problems that can cause pulmonary hypertension include:
Group 3 PH due to lung disease or hypoxia
Lung problems are another common cause of pulmonary hypertension. Some people with left-sided heart disease also have lung disease or a lack of oxygen .
Lung issues that can cause pulmonary hypertension include:
Group 4 PH due to blockages in your lungs
This form of PH is usually caused by chronic thromboembolic pulmonary hypertension . CTEPH is a condition that involves blood clots and scarring in the arteries in your lungs.
Group 5 PH due to other disorders
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Epidemiology Of Pulmonary Hypertension
The outbreak of PH of secondary type depends on the prevalence of its causes in the community. For example, 20% to 30% of lung-disease patients can have degrees of pulmonary hypertension. In 30 to 40% of the patients, interstitial pulmonary hypertension has been reported .
Chest CT scan showing a dilated pulmonary artery in idiopathic pulmonary artery hypertension
The prevalence of pulmonary hypertension among liver patients has led them to transplant liver in 5% to 20% and only a very small percentage of these patients have pulmonary similar to idiopathic forms. In a rather vast statistical study on the patients suffering chronic liver diseases, 0.6 to 0.73% of the patients were suffering idiopathic pulmonary hypertension. 9% to 19% of rheumatic patients have degrees of pulmonary hypertension. Among patients with SLE, scleroderma and more of crest syndrome and mixed connective tissue are 15%, 60% and 67% respectively. And it has rarely been observed among rheumatoid arthritis.
Idiopathic hypertension is a rare disease, occurrence rate of which is reportedly one to two per million. Accordingly, there are about 100 people annually for Irans 70 million population. Considering the longevity of patients 2-3 years , there shall be about 200 to 300 patients suffering from pulmonary hypertension in Iran.
How Is Pulmonary Hypertension Diagnosed
Your provider will perform a physical exam and run tests to reach a pulmonary hypertension diagnosis.
First, youll have a physical exam to check for signs of pulmonary hypertension as well as other heart or lung issues. During this exam, your provider will:
- Ask you questions about your health and your medical history.
- Ask about your symptoms.
- Check the size of the veins in your neck. Bulging neck veins could be a sign of right-sided heart failure.
- Check the size of your liver by feeling the upper right area of your tummy.
- Listen to your heart and lungs with a stethoscope.
- Look at your belly, ankles and legs for edema.
- Measure your blood pressure.
- Measure the oxygen level in your blood using a pulse oximeter.
Pulmonary hypertension can be difficult to diagnose since many signs of PH are similar to those of other conditions. So, after your physical exam, your provider may run some tests to get more information.
How Can I Prevent Pulmonary Hypertension
Its not always possible to prevent pulmonary hypertension. Some risk factors are out of your control. If you have risk factors, your provider may recommend preventive screenings to check your heart and lung function.
Doing whatever you can to prevent or manage other medical conditions can help lower your risk of pulmonary hypertension. Steps you can take include:
- Create an exercise plan. Ask your provider what exercises are safe for you.
- Follow a heart-healthy diet. Avoid processed foods, fast food and other foods high in salt and saturated fat.
- Quit smoking and stop using tobacco. Smoking and tobacco use are top risk factors for heart and lung problems. Quitting isnt easy, especially if youve been smoking or using tobacco for a long time. But your provider can help provide resources. Support groups may also help.
- Take medications for blood pressure and other conditions as prescribed.
What Raises A Persons Risk Of Developing Pulmonary Hypertension
Risk factors for developing pulmonary hypertension include:
- Family history of blood clots.
- Family history of pulmonary hypertension.
- Living at high altitudes.
- Smoking and using tobacco products.
- Use of diet medications such as fen-phen .
- Use of some prescription medications that treat cancer and depression.
- Use of recreational drugs.
- Liver disease.
- Lung disease.
Talk with your provider about your risk factors and what you can do to lower your risk.
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Pathophysiology And Pathology Of Pulmonary Hypertension
According to Ohms law, factors affecting pulmonary pressure are pulmonary blood flow and the pulmonary vascular resistance. Accordingly, changes in right cardiac output , setting of pulmonary vascular resistance and venous pressure of pulmonary system are the major factors of pulmonary hypertension . This equation represents that pulmonary arterial hypertension depends on three factors:
First: right cardiac output
Second: pulmonary vascular resistance
Third: mean pulmonary artery pressure .
Accordingly, diseases such as atrial septal failure, ventricular septal failure and many congenital heart diseases such with the increase of blood flow , diseases like pulmonary embolism, rheumatic diseases and pulmonary interstitial diseases through the overall decrease of vascular setting and increase of the pulmonary hypertension resistance and diseases such as miteral stenosis, pulmonary venous occlusive and heart failure through the increase of venous pressure , all cause pulmonary hypertension . It shall be noted that in many cases, there are a couple of factors involved in PH.
How Pulmonary Hypertension Develops
Pulmonary hypertension begins when there is damage to the blood vessels of the lungs at a molecular level. Over time these problems build up and, if not corrected, can lead to permanent changes in the makeup of those blood vessels.Pulmonary hypertension is usually a progressive disease , which can be slowed with treatment. Part of the difficulty with treating people with pulmonary hypertension is the subtle and non-specific nature of their symptoms. This often leads to a delay in diagnosis.
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Standard Pulmonary Hypertension Treatments
Our team includes a nurse practitioner who specializes in caring for people with pulmonary hypertension. You can contact this specialist between appointments if you have any questions about your care plan.
Your personalized care plan may include:
- Healthy living recommendations: We give you helpful tips for getting the most out of treatment and lowering your risk of complications. These tips often include a low-sodium diet and mild exercise.
- Medications: Most people with pulmonary arterial hypertension take medications to help control their symptoms. If standard treatments are not effective, we may recommend new options available through clinical trials.
- Pulmonary rehabilitation: This supervised exercise program is tailored to meet the unique needs of people with lung disease. Pulmonary rehabilitation can help you regain your strength and make a safe return to a more active lifestyle. Learn more about our pulmonary rehabilitation program.
- Monitoring: We develop a lasting relationship with you, including regular follow-up appointments. During these appointments, we determine whether treatments are working as they should. We may make adjustments to your care plan to help you feel your best.
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Epidemiology And Risk Factors
Reporting in the literature of PH incidence data at the global level is poor. In the UK, a prevalence of 97 cases per million with a female:male ratio of 1.8 has been reported. The age-standardized death rate in the USA ranges between 4.5 and 12.3 per 100,000 population. Comparative epidemiological data on the prevalence of the different groups of PH are not widely available, but it is clear that LHD is believed to be the most common cause of PH, although severe PH is relatively uncommon in this setting. Although patients belonging to groups 2 and 3 represent an important part of the clinical practice, there is disproportionately little information about the demographics and clinical course of this segment of the PH population, suggesting that registry database methodology may be useful for these groups. Globally, schistosomiasis-associated PAH and high altituderelated PH represent an important burden to mankind.
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Pulmonary Hypertension Caused By Blood Clots
If your pulmonary hypertension is caused by blood clots, your specialist will treat you with anticoagulant medicine. This medicine stops more blood clots forming.
You might be offered:
- warfarin, which is taken as a tablet
- a group of anticoagulation medicines called NOACs or DOACs . These are now used more often than warfarin, but warfarin is still needed in some cases.
If your blood clot has caused scar tissue in your pulmonary arteries, you may be offered a pulmonary endarterectomy . This is an operation to remove scar tissue from the inside layer of the pulmonary arteries. This improves the blood flow and reduces the pressure inside the arteries.
If a pulmonary endarterectomy isnt suitable, you may be offered a new procedure called balloon pulmonary angioplasty . In this procedure, a tiny balloon is guided into a narrowed lung artery and inflated for a few seconds to widen the artery. This can be done several times, and in different parts of the lung during one procedure. The aim is to improve blood flow through the lungs and reduce pressure on the heart. Royal Papworth Hospital, where BPA procedures are performed, have said that early use of this technique has showed BPA can lower blood pressure in the lung arteries, improve breathing and allow you to exercise more.
Pulmonary endarterectomy and balloon pulmonary angioplasty are specialist procedures currently only performed in the UK at the Royal Papworth Hospital.
Living With Pulmonary Hypertension
Because there is no cure for pulmonary hypertension, you will need to learn to manage your condition. The best way to do this is by following the treatment plan developed between you and your doctor. Call your doctor if your symptoms change or get worse. Know what symptoms could require emergency treatment, such as chest pain.
Making lifestyle changes can also help your condition. This could include:
- Quit smoking. Smoking makes symptoms of pulmonary hypertension worse.
- Follow a healthy diet. Eat a variety of fruits, vegetables, whole grains and lean protein. Talk to your doctor to see if you need to limit the amount of salt in your diet. Also ask if you should limit the amount of vitamin K in your diet. Vitamin K is found in green leafy vegetables and some oils. It can affect how well blood-thinning medicines work.
- Be physically active. Try to be as active as you can. Incorporate regular activity, such as walking, into your daily routine. Ask your doctor if there are any activities you should avoid. These could include lifting heavy objects, sitting in a hot tub, or traveling to high-altitude areas.
- Get support for emotional issues. Living with pulmonary hypertension can cause you worry, anxiety, stress, or depression. Talk about your feelings with your doctor. He or she will be able to direct you to the kind of help you need. This could include talking to a counselor, medicine for depression, or a patient support group.
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Medications For Pulmonary Arterial Hypertension
Being diagnosed with pulmonary arterial hypertension may be overwhelming. Working with your doctor to create a care plan can ease your symptoms and give you some peace of mind.
Treatment cant stop or reverse this aggressive disease, but medications can help slow the progression of PAH and improve your symptoms.
The Use Of Blood Thinners In The Treatment Of Pulmonary Hypertension
Blood thinners such as coumadin are commonly used to prevent or treat blood clots and are useful in the treatment of chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension as the narrowed pulmonary arteries are prone to blood clots. The risks and benefits of any medication should be discussed before placing a patient on therapy and this is very important before initiating blood thinners as there are serious risks associated with these medications. It is very important to perform regular blood tests to measure how thin the blood is to avoid serious complications. If the blood is too thin the patient is at risk for bleeding complications and if is too thick they remain at risk of blood clots.
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Whats The Best Treatment For Pulmonary Hypertension
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Pulmonary Hypertension Caused By Left Heart Disease Or Lung Conditions
In both groups, pulmonary hypertension is a secondary condition. This means its caused by a heart or another lung condition – the primary condition. Examples of primary lung conditions are COPD, interstitial lung disease such as pulmonary fibrosis, and OSA.
If your pulmonary hypertension is a secondary condition, your health care professional will focus on treating the primary condition. If the primary condition improves with treatment, it should improve your pulmonary hypertension too. Its very unlikely that youll be treated with pulmonary vasodilators if pulmonary hypertension is not your primary condition.
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Can Pulmonary Hypertension Be Cured
Most cases of pulmonary hypertension cant be cured. Your provider may prescribe medications to:
- Ease your symptoms.
- Improve your quality of life.
- Slow down the progression of the disease.
Your provider may also recommend lifestyle changes.
However, surgery can cure some people with chronic thromboembolic pulmonary hypertension .
Genetics Epidemiology And Risk Factors Of Pulmonary Hypertension
Comparative epidemiological data on the prevalence of the different groups of PH are not available. In a survey performed in an echocardiography laboratory , the prevalence of PH systolic pressure > 40mmHg) among 4579 patients was 10.5%. Among the 483 cases with PH 78.7% had left heart disease , 9.7% had lung diseases and hypoxia , 4.2% had PAH , 0.6% had CTEPH and in 6.8% it was not possible to define a diagnosis.
Group 1, PAH: recent registries have described the epidemiology of PAH , . The lowest estimates of the prevalence of PAH and IPAH are 15 cases and 5.9 cases per million adult population, respectively. The lowest estimate of PAH incidence is 2.4 cases per million adult population·yr1. Recent data from Scotland and other countries have confirmed that PAH prevalence is in the range 1550 subjects per million population in Europe . In the French registry, 39.2% of patients had IPAH and 3.9% had family history of PAH. In the subgroup of APAH, 15.3% had connective tissue diseases , 11.3% had CHD, 10.4% had portal hypertension, 9.5% had anorexigen-associated PAH and 6.2% had HIV infection .
Group 5, PH with unclear and/or multifactorial mechanisms: the heterogeneity of this group prevents an appropriate description of genetics, epidemiology and risk factors in these guidelines.
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Pathobiology Of Pulmonary Hypertension
Different pathobiological features characterise the diverse clinical PH groups.
Group 2, PH due to left heart disease: the mechanisms responsible for the increase in PAP are multiple and include the passive backward transmission of the pressure elevation . In these cases the transpulmonary pressure gradient and PVR are within the normal range. In other circumstances the elevation of PAP is greater than that of Ppcw and an increase in PVR is also observed . The elevation of PVR is due to an increase in the vasomotor tone of the pulmonary arteries and/or to fixed structural obstructive remodelling of the pulmonary artery resistance vessels : the former component of reactive PH is reversible under acute pharmacological testing while the latter, characterised by medial hypertrophy and intimal proliferation of the pulmonary arteriole, does not respond to the acute challenge . Which factors lead to reactive PH and why some patients develop the acutely reversible vasoconstrictive or the fixed obstructive components or both is poorly understood. Pathophysiological mechanisms may include vasoconstrictive reflexes arising from stretch receptors localised in the left atrium and pulmonary veins, and endothelial dysfunction of pulmonary arteries that may favour vasoconstriction and proliferation of vessel wall cells.
Group 5, PH with unclear and/or multifactorial mechanisms: the pathobiology in this group is unclear or multifactorial.