Screening And Diagnostic Evaluation
There are no data to support screening of asymptomatic individuals for pulmonary hypertension, even in high-risk groups such as those with a family history of pulmonary arterial hypertension without known BMPR2 mutations.3,22 For patients with systemic sclerosis and related diseases, expert opinion suggests annual screening with laboratory testing , electrocardiography, and pulmonary function testing, followed by echocardiography.3,8 Expert consensus recommends annual echocardiography in patients with sickle cell disease and those with known BMPR2 mutations.3,14
Recognizing pulmonary hypertension in patients presenting with new signs or symptoms can be difficult because many symptoms are common and associated with an extensive differential diagnosis . Pulmonary hypertension should be considered in patients with chronic illness and symptoms that are disproportionate to the underlying disease or poorly responsive to treatment.
Findings Suggestive of Pulmonary Hypertension
History
Information from references 1 through 3, and 22.
Findings Suggestive of Pulmonary Hypertension
History
Information from references 1 through 3, and 22.
Evaluation of Suspected Pulmonary Hypertension in Primary Care
Figure 1.
Suggested algorithm for the evaluation of suspected pulmonary hypertension in primary care.
Evaluation of Suspected Pulmonary Hypertension in Primary Care
Figure 1.
Suggested algorithm for the evaluation of suspected pulmonary hypertension in primary care.
Pulmonary Hypertension Due To Left Heart Disease
The focus for these patients is optimizing the underlying heart disease and controlling comorbidities.3,9,10 This includes management of hypertension and heart failure, and addressing significant valvular disease when present. Control of fluid volume and diuretic therapy are essential, particularly in patients with a history of volume overload or right heart failure.10 Vasodilators are not recommended for the treatment of pulmonary hypertension due to left heart disease.10,26
Testing And Diagnosis Of Pulmonary Hypertension
Our involvement starts at the first phone call or Hospital visit, when we evaluate the history and symptoms to determine how urgently your child needs to be seen or treated. During your child’s evaluation for PH:
Because PH is a rare and complex disease and is difficult to diagnose, your child’s treatment team should have special expertise in the disease. At Children’s Hospital of Philadelphia, we provide in-hospital treatment to more than 20 children with PH every day, so PH is understood by every member of your CHOP team, from the emergency room to the intensive care.
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Other Medical Treatments For Pulmonary Arterial Hypertension
Other medical therapies for PAH may include:
- Oxygen inhaled by patients via a nasal cannula or face mask.
- Diuretics medicine that rids the body of excess fluid that puts pressure on the heart.
- Calcium channel blockers medicine that helps to decrease blood pressure.
- Warfarin medicine that thins blood and prevents it from clotting.
Surgery And Procedures For Pulmonary Hypertension
Some people with pulmonary hypertension may need surgery. The 3 types of surgery currently used are:
- pulmonary endarterectomy an operation to remove old blood clots from the pulmonary arteries in the lungs in people with chronic thromboembolic pulmonary hypertension
- balloon pulmonary angioplasty ;a new procedure where a;tiny balloon is;guided into the arteries and;inflated for a few seconds to push the blockage aside and restore blood flow to the lung; it may be considered if pulmonary endarterectomy is not suitable, and has been shown to lower blood pressure in the lung arteries, improve breathing, and increase the ability to exercise
- atrial septostomy a small hole is made in the wall between the left and right atria of the heart;using a cardiac catheter, a thin, flexible tube inserted into the heart’s chambers or blood vessels; it reduces the pressure in the right side of the heart, so the heart can pump more efficiently and the blood flow to the lungs can be improved
- transplant in;severe cases, a;lung transplant or a;heart-lung transplant;may be needed; this type of surgery is rarely used because effective medicine is available
The National Institute for Health and Care Excellence has;guidance on balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension.
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Diuretics Or Water Pills In The Treatment Of Pulmonary Hypertension
Diuretics are commonly used to help treat heart failure. Because the right ventricle of pulmonary arterial hypertension patients is straining to pump against the increased pressures in the pulmonary arteries, they have the same volume management problem. Diuretics help eliminate excess fluid in the body that would normally naturally be eliminated by an efficient heart and kidneys. There are many types of diuretics such as Lasix, Aladactone, Bumex, and Metolazone. It is important to take diuretics as they have been prescribed because excess amounts may cause a decrease in electrolytes such as potassium and damage to the kidneys. Routine blood work such as a BMP should be performed while taking diuretics. Close management of salt and water restrictions continue to apply even if a diuretic has been prescribed. Generally only one physician should be prescribing, monitoring, and changing a patients diuretic regime.
How To Treat Pulmonary Hypertension: Groups 2 And 3
Group 3 pulmonary hypertension includes cases of the disease caused by left-side diseases such as mitral valve disease. In order to treat patients in this group, physicians will focus on the treatment of the underlying condition with;lifestyle changes, medicines, and surgery.
Similarly, cases in the group 3 pulmonary hypertension are caused by lung conditions like chronic obstructive pulmonary disease and interstitial lung disease, or sleep disorders like sleep apnea. In addition to treating the underlying cause of the disease, physicians may also recommend oxygen therapy, a resource administrated through soft, plastic prongs that fir into the nose at home or in a hospital, to increase the level of oxygen in the blood.
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Symptoms Of Pulmonary Hypertension
Pulmonary hypertension may not cause any symptoms at first. Often, shortness of breath or lightheadedness during activity is the first symptom. As the disease gets worse, symptoms can include the following:
- Increased shortness of breath, with or without activity
- Fatigue
- Pain in the upper right side of the abdomen
- Swelling of the ankles, legs and abdomen
- Bluish tint or shading to your skin or lips
Symptoms of pulmonary hypertension usually limit a persons ability to exercise and do other activities.
When Should I Go To The Emergency Department
Go to the emergency department or call 9-1-1 if you are experiencing:
- Hickman catheter complications with intravenous prostacyclins, such as infection, catheter displacement/leakage of solution or blood, IV pump malfunction
- New chest pain or discomfort that is severe, unexpected, and is accompanied by shortness of breath, sweating, nausea, or weakness
- Fast, sustained heart rate â especially if you are short of breath
- Shortness of breath that is NOT relieved by rest
- Sudden weakness or paralysis in your arms or legs
- Sudden onset of severe headache
- Fainting spells with loss of consciousness
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Treatment For Severe Cases
The treatment options are different for each type of pulmonary hypertension. In severe cases of pulmonary hypertension, your doctor may consider administering drugs to help control your high blood pressure, such as ACE inhibitors or beta-blockers.
In mild cases, your doctor may recommend lifestyle changes and medications. If you have pulmonary hypertension that is not severe, your doctor may recommend nutritional or lifestyle changes, such as exercising and losing weight if youre overweight. If you have severe pulmonary hypertension, your doctor may prescribe drugs to help reduce your blood pressure.
Improving Health With Current Research
Learn about the following ways the NHLBI continues to translate current research into improved health for people with pulmonary hypertension. Research on this topic is part of the NHLBIs broader commitment to advancing lung disease scientific discovery.
Learn about the pioneering research contributions we have made that improved clinical care.
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What Causes Pulmonary Hypertension
The following are some known causes of pulmonary hypertension:
- The diet drug “fen-phen.” Although the appetite suppressant “fen-phen” has been taken off the market, former fen-phen users have a 23-fold increase risk of developing pulmonary hypertension, possibly years later.
- Liver diseases, rheumatic disorders, lung conditions. Pulmonary hypertension also can occur as a result of other medical conditions, such as chronic liver disease and liver cirrhosis; rheumatic disorders such as scleroderma or systemic lupus erythematosus ; and lung conditions, including tumors, emphysema, chronic obstructive pulmonary disease , and pulmonary fibrosis.
- Certain heart diseases. Heart diseases, including aortic valve disease, left heart failure, mitral valve disease, and congenital heart disease, can also cause pulmonary hypertension.
- Thromboembolic disease. A blood clot in a large pulmonary artery can result in the development of pulmonary hypertension.
- Low-oxygen conditions. High altitude living, obesity, and sleep apnea can also lead to the development of pulmonary hypertension.
- Genetics. Pulmonary hypertension is inherited in a small number of cases. Knowing that someone in the family had or has pulmonary hypertension should prompt you to seek early evaluation should symptoms occur.
Pulmonary hypertension may also be caused by other conditions, and in some cases, the cause is unknown.
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.
;See patient information handout on pulmonary hypertension, written by the authors of this article.
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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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.
Type 4: 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 new group of medicines called NOACs or DOACs
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. Early use of this technique has showed it can lower blood pressure in the lung arteries, improve breathing and allow you to exercise more.
Both are specialist procedures currently performed in the UK only at Papworth Hospital.
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Treatments For Other Conditions
Your doctor may recommend medicines or procedures to treat the condition that is causing your pulmonary hypertension.
- Blood pressure medicines such as angiotensin-converting enzymes inhibitors, beta blockers, or calcium channel blockers when left heart disease is the cause
- Blood transfusions or hydroxyurea to treat sickle cell disease
- Heart valve repair
- Ironsupplements to increase blood iron levels and improve anemia
Research For Your Health
The NHLBI is part of the U.S. Department of Health and Human Services National Institutes of Health the Nations biomedical research agency that makes important scientific discovery to improve health and save lives. We are committed to advancing science and translating discoveries into clinical practice to promote the prevention and treatment of heart, lung, blood, and sleep disorders, including pulmonary hypertension. Learn about the current and future NHLBI efforts to improve health through research and scientific discovery.
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Prevent Complications Over Your Lifetime
To help prevent some of the complications of pulmonary hypertension, your doctor may recommend the following.
- Make heart-healthy lifestyle changes such as heart-healthy eating if your pulmonary hypertension is due to heart failure from ischemic heart disease or high blood pressure.
- Engage in regular physical activity. Before starting any exercise program, ask your doctor about what level of physical activity is right for you.
- Avoid high altitudes when possible and discuss with your doctor any plans for air travel or visits to places at high altitude.
- Talk to your doctor if you are planning to get pregnant, as there is an increased risk of pregnancy complications.
- Treat other medical conditions, such as COPD, heart conditions, and sleep apnea.
Surgical Management For Pulmonary Hypertension
Atrial Septostomy- If medical management of pulmonary hypertension fails or is non- responsive patient, surgical management is the next option available for these patients. Atrial septostomy is an open heart surgery. Atrial septostomy is a surgical procedure for the treatment of Pulmonary Hypertension in which the surgeon makes an opening between the upper chambers of heart i.e. between right and left atrium to relieve the pressure within the right chamber. There is shunting of blood from right Atrium to left Atrium, therefore there is mixing of blood in the Chambers causing a decrease in the level of oxygen to the tissues. Cardiac arrhythmias are the associated complication of atrial septostomy.
Thromboendarterectomy is a surgical treatment of choice in the cases of thromboembolic pulmonary hypertension. In this procedure, the thrombus is removed from within the lumen of pulmonary vessels through sophisticated technology. As this is a new technique so it is performed only a few selected centers.
Transplantation- Lung transplantation treatment is done in those patients where above procedures cannot be performed. Transplantation of the lung can cure pulmonary hypertension but the complications of transplantation are the main concern for the doctors. Organ rejection, graft versus host reaction, Anaphylactic shock are some of the complications of transplantation. This also compiles the patient to take steroid throughout the life.
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Curriculum Topic: Primary Pulmonary Hypertension
Pulmonary hypertension is a descriptive name for abnormally elevated pressures in the pulmonary vasculature. PH has been defined as a mean pulmonary arterial pressure 25mmHg at rest, measured by right heart catheterisation .1 It can be caused by an increase in pulmonary vascular resistance , pulmonary blood flow, pulmonary venous pressure or a combination of these factors. PH leads to right ventricular overload and finally RV failure and death.2 Commonly, PH is diagnosed at a late stage of the disease, and is associated with poor survival.3 This underscores the importance of early recognition and treatment of PH in order to improve the outcome in this patient population.4
PH is characterised by different pathological lesions in the pulmonary vasculature, depending on the underlying cause. The 5th World Symposium on Pulmonary Hypertension in Nice in 2013 classified PH according to these underlying aetiologies, creating five groups: pulmonary arterial hypertension ; PH due to left sided disease; PH due to lung disease; chronic thromboembolic PH ; PH with unclear or multifactorial mechanisms .5
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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Mental Health And Patient Education
An ethnographic study of patients with pulmonary hypertension found that many described uncertainty surrounding their prognosis and expressed feelings of isolation.35 Patients may benefit from resources available through the Pulmonary Hypertension Association .
Data Sources: A PubMed search was completed using the terms pulmonary hypertension and pulmonary arterial hypertension. We also searched the National Guideline Clearinghouse. Our search included consensus guidelines, systematic reviews, meta-analyses, randomized controlled trials, and large retrospective or cohort studies. Search dates: May to December 2015.
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