How Do You Define And Diagnose Pulmonary Hypertension
Pulmonary hypertension is defined by either a mean pulmonary artery pressure at rest of 20 mm Hg or more as measured by a right heart catheterization or a right ventricular systolic pressure of 30 mm Hg or more as measured by an echocardiogram. An RHC is the diagnostic gold standard and an essential component in the evaluation of pulmonary hypertension. While an echocardiogram can be a useful screening tool for the presence of pulmonary hypertension, it only provides an estimate of RVSP. An RHC is absolutely required to confirm the diagnosis, assess the severity of hemodynamic impairment, provide risk assessment, and guide pulmonary hypertension-specific therapy.
The threshold to perform a left heart catheterization in addition to an RHC should be low in patients with risk factors for heart disease and when the measurement of left ventricular end-diastolic pressure is important to avoid potential misclassification of pulmonary hypertension.
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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When Should I Call My Doctor About Pulmonary Hypertension
- Weight gain â 2 pounds in one day or 5 pounds in one week
- Swelling in your ankles, legs, or abdomen that has become worse
- Shortness of breath that has become worse, especially if you wake up short of breath
- Extreme fatigue or decreased tolerance for activity
- A respiratory infection or a cough that has become worse
- Fast heart rate
- Episodes of chest pain or discomfort with exertion that are not relieved with rest
- Difficulty breathing with regular activities or at rest
- Restlessness, confusion
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What Is The Treatment
There is supportive therapy and specific drug therapy for pulmonary hypertension.
Supportive therapy includes:
- Oral anticoagulants, in select patients
- Diuretics, in patients with fluid overload
- Oxygen, in patients with correctable oxygen desaturation
- Digoxin, in patients with atrial tachyarrhythmias
- Iron substitution, in patients with iron deficiency
Pulmonary hypertension-specific therapy includes:
Risk Factors For Pulmonary Hypertension
Because the disease is so wide in scope, there are many potential risk factors for pulmonary hypertension. Along with the specific factors related to genetics, lifestyle, and medications, having any of the diseases or conditions known to cause pulmonary hypertension is a significant risk factor.
These are some of the risk factors that may increase your chances for developing pulmonary hypertension:
- A family history of pulmonary hypertension
- Being overweight
- Illegal drug use
- Prolonged exposure to high altitudes
- Having any of the diseases or conditions that can cause pulmonary hypertension
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Living With Pulmonary Hypertension: Everything You Need To Know
Feb 28, 2018 6:51:41 AM / byDuke Reeves
Pulmonary hypertension is a serious, chronic disease that affects the heart and the blood vessels in the lungs. Although it is somewhat rare, the disease can result from a large number of health conditions, genetics, or even occur spontaneously.
Unfortunately, most cases of pulmonary hypertension are chronic and incurable. But especially with early diagnosis and proper medical treatment, people with the disease are able to manage the symptoms of pulmonary hypertension and maintain a good quality of life.
In the early stages of the disease, people who have pulmonary hypertension tend to experience fatigue and shortness of breath during physical activity. But symptoms worsen and multiply as the disease progresses.
Life expectancy and treatment options depend mostly on how early the disease was caught and what cause, if any, doctors can find for the disease. In this guide, we will help you better understand pulmonary hypertension, what causes it, and what kind of treatments options are out there to help patients manage the disease.
Type : 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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Caring For Someone With Pulmonary Hypertension
Pulmonary hypertension usually progresses slowly, and eventually results in shortness of breath and a decreased ability to perform the activities of daily living at home. For most of the course of the illness you will not need any extra assistance at home. However, at the end stage of pulmonary hypertension, you may need services such as home oxygen, council cleaning, and meals on wheels. These can be arranged through your GP or local volunteer services.
Key Facts About Pulmonary Hypertension
Pulmonary hypertension is a rare and varied disease that has many different causes and can present itself in many different ways. In fact, there are five different groups of pulmonary hypertension, and within each of these groups there are multiple diseases and conditions that can cause a particular type of pulmonary hypertension.
It’s estimated that only 15-50 people out of every million adults develops the disease, but the incidence is much higher for people in at-risk groups .
Idiopathic pulmonary hypertension makes up more than 40% of total cases of pulmonary arterial hypertension. It is also, for unknown reasons, anywhere between 2 and 5 times more common in women than men, and is usually diagnosed around the age of 45 years.
In healthy people, the blood pressure in the lungs is significantly lower than the blood pressure throughout the whole-body, at about 8-20 mm/Hg. Unlike regular blood pressure measurement, testing the blood pressure in the pulmonary arteries requires special, more invasive techniques.
To test pulmonary artery pressure, doctors have to perform a procedure called right heart catheterization. It requires going under anesthesia and having a flexible tube, or catheter, inserted into a major blood vessel in your neck or groin.
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Causes Of Pulmonary Hypertension
Pulmonary hypertension is a complex disease that affects the entire body and has many known causes. In some cases doctors are unable to find a cause, but in many cases pulmonary hypertension results from another known disease or health condition.
Different forms of hypertension affect the body in different ways, and what kind of treatment will be effective largely depends on what is causing the disease. That’s why health researchers have divided up pulmonary hypertension into five main categories, each containing similar types of hypertension that are treated in similar ways.
The following sections describe the differences between all five categories of pulmonary hypertension and discusses the most common causes and conditions that lead to them.
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.
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How Is Pulmonary Hypertension Treated
Appropriate diagnosis and analysis of the problem is necessary before starting any treatment. Treatment varies by patient, based on the different underlying causes, but generally includes:
- Taking medications
Listed below are medication, lifestyle and dietary suggestions, and surgical treatment approaches.
Medications
Many different types of medications are available to treat pulmonary hypertension. Treatment choices, such as those listed below, depend on the underlying cause of pulmonary hypertension, how severe the pulmonary hypertension is, how likely it is to progress, and a patientâs drug tolerance.
Medication guidelines
- Know the names of your medications and how they work. Keep a list with you.
- Take your medications at the same time every day. If you forget a dose, do not take two doses to make up for the dose you missed.
- Do not take any over-the-counter drugs unless you ask your doctor or pharmacist first. Some drugs such as decongestants and nonsteroidal anti-inflammatory agents can cause problems in people who have heart failure. Also avoid any over-the-counter medications whose labels state that caution is to be used if you have high blood pressure.
- Do not stop taking or change your medications unless you first talk with your doctor.
- Avoid herbal products because of their uncertain effects when combined with medications used for pulmonary hypertension.
Dietary changes
Lifestyle changes
Less Invasive Approaches To Chronic Thromboembolic Pulmonary Hypertension Treatment
Zachary N. Kon, MD, surgical director of the lung transplantation program and assistant professor in the Department of Cardiothoracic Surgery, leads a program to treat chronic thromboembolic pulmonary hypertension . In 2017, NYU Langone joined the short list of select centers around the world to offer the technically challenging and potentially curative surgery, pulmonary thromboendarterectomy. Only an estimated 300 procedures are carried out nationwide each year. Dr. Kon is one of a small number of surgeons trained to perform this delicate surgery, which is the definitive treatment for CTEPH.
Typically, removing chronic blood clots and scar-like blockages that narrow the pulmonary arteries of CTEPH patients requires a complete dissection of the entire pulmonary vascular tree, with patients under hypothermic circulatory arrest. Dr. Kon has begun performing the procedure using a less invasive incision that avoids a full median sternotomy. It translates into faster recovery time, less postoperative pain, and improved cosmetic resultswithout sacrificing outcome, says Dr. Kon. This is something our patients at NYU Langone really appreciate.
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How Do You Risk Stratify And Provide A Risk Assessment
Regular risk assessment of patients with pulmonary hypertension is strongly recommended. A comprehensive risk assessment is required since there is no single determinant that provides enough prognostic information. The most important question to be addressed at every clinic visit, is the patient compatible with a good long-term prognosis ? To answer this question, a structured approach is needed, and all pulmonary hypertension specialists should have a checklist to help provide a comprehensive risk assessment:
Achieving low clinical risk also functions as the primary treatment goal, thus treatment should be tailored to achieve and maintain a low-risk status.
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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Chronic Thromboembolic Pulmonary Hypertension
A pulmonary endarterectomy performed at a center of excellence can be curative and is first-line therapy in patients with chronic thromboembolic pulmonary hypertension who are surgical candidates. Patients who are not surgical candidates can be considered for targeted medical therapies also used to treat pulmonary arterial hypertension. Regardless of the treatment, these patients should receive lifelong anticoagulation therapy in the absence of contraindications.25
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
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Key Points About Pulmonary Hypertension
- Primary pulmonary hypertension is a rare lung disorder that causes high blood pressure in the lungs.
- The cause of PPH is unknown.
- Symptoms of PPH can develop so slowly that you can have PPH for years without knowing it. And symptoms get worse as the disease progresses.
- There is no cure for PPH. Treatment is aimed at managing symptoms.
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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Investigation Into Pulmonary Hypertension
The series of tests required to confirm a diagnosis of pulmonary hypertension can be quite extensive. Your first appointment with your specialist might just be spent organising these tests, which may include:
If your doctor asks you to have any tests before the appointment, please do so because it will speed up the process of determining what the nature of your pulmonary hypertension may be, and how to treat it.
What Are The Symptoms Of Primary Pulmonary Hypertension
The following are the most common symptoms for primary pulmonary hypertension . Symptoms can develop so slowly that you can have it for years without knowing it. However, each person may experience symptoms differently. Symptoms may include:
- Extreme tiredness
- Trouble getting enough air
- You feel like your heart is fluttering, or beating fast or hard
These symptoms get worse as the disease progresses. More severe symptoms are a sign of more advanced disease. In advanced stages, you may:
- Have symptoms even when resting
- May become bedridden
The symptoms of PPH look like other conditions or medical problems, and PPH can be hard to diagnose. Consult a health care provider for a diagnosis.
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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.
How Does Yale Medicine Treat Pulmonary Hypertension
Yale Medicines pulmonary specialists collaborate with physicians in cardiology, rheumatology, hepatology, cardiothoracic surgery, diagnostic radiology, and pathology to provide one of the most comprehensive pulmonary hypertension care programs in the Northeast region. The pulmonary hypertension program provides a one-stop shop for diagnostic tests, treatment, and patient education. We are one of the few centers in the nation that is accredited by the Pulmonary Hypertension Association, a comprehensive care center for patients with PH. The PH Care Centers initiative of the Pulmonary Hypertension Association accredits centers with expertise in pulmonary hypertension that have demonstrated an ability to properly diagnose the disease and have the capability to manage these complex patients.
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