How To Diagnose Pulmonary Hypertension

Guidelines For Diagnosis And Treatment Of Pulmonary Hypertension

Galiè N, Humbert M, Vachiery JL, et al.

Citation:

2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension. Eur Heart J 2015 Aug 29:.

The following are key points to remember from the European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension :

PH is a pathophysiological disorder that may involve multiple clinical conditions and can complicate the majority of cardiovascular and respiratory diseases.

PH is defined as an increase in mean pulmonary artery 25 mm Hg at rest, as assessed by right heart catheterization. The normal mPA is 14 ± 3 mm Hg with an upper limit of normal of approximately 20 mm Hg. The clinical significance of an mPA 21-24 mm Hg is unclear. Patients with a pulmonary artery pressure in this range should be carefully followed because they are at risk for developing pulmonary arterial hypertension .

PAH describes a group of PH patients characterized hemodynamically by the presence of precapillary PH, defined by a pulmonary artery wedge pressure 15 mm Hg and a pulmonary vascular resistance > 3 Wood units in the absence of other causes of precapillary PH such as due to hypoxemia/lung diseases, or chronic thromboembolism. Post-capillary PH related to left heart and valve disease is defined as a PAWP > 15 mm Hg with a diastolic pressure gradient < 7 mm Hg and PVR 3 WU. Combined post- and precapillary PH is defined with PCWP > 15 mm Hg with DPG 7 mm Hg and PVR > 3 WU.

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

Do not smoke, vape or chew tobacco.

Avoid or reduce alcohol intake.

Pulmonary Hypertension And Covid

While theres no evidence that people with pulmonary hypertension are at a greater risk for developing COVID-19 , you are at a greater risk for developing serious illness if you get infected, according to the Pulmonary Hypertension Association.

For this reason, you should take precautions to reduce your risk of infection, such as staying home whenever possible, maintaining physical distance from other people, washing your hands frequently, and not touching your face.

Covering your face with a cloth mask or other covering while in public is also currently recommended by the Centers for Disease Control and Prevention, unless you have trouble breathing. You can talk to your doctor or use your own judgment to decide whether its safe for you to wear a cloth face covering.

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Yales Pulmonary Vascular Disease Program: One Of Just A Few Nationally

McGrath was referred to the Yale Medicine PVD Program because it is one of the few centers in the nation accredited by the Pulmonary Hypertension Association, and the only such program in the state. The PVD Program allows pulmonary specialists to collaborate with physicians in cardiology, rheumatology, hepatology, cardiothoracic surgery, diagnostic radiology, and pathology, making it a single destination to manage complex cases. Depending on how serious the condition is, treatment options include medications taken orally and those delivered intravenously through a continuous pump.

At the time, however, McGrath, a married mother of three and grandmother of five, had no idea how complicated her disease was or how much her life would change because of it.

There are four functional classes of PH disease with class 4 being the most severe, Dr. Singh explains. When he first met McGrath, she was already at class 4. Because of that severity, Dr. Singh put her on two oral medications and a continuous IV medication delivered through an external pump. Today, McGrath wears the pump, which is about the length of her smartphone, at all times on the side of her body. The pump has a 4-foot IV line that attaches to a catheter in her chest and delivers medication to a vein near her heart. McGrath receives the medications by mail and puts them in her pump.

Electrocardiogram Or Ekg Or Ecg

An EKG is a non-invasive test that measures the electricity of the heart. It involves placing what is called ‘leads’ on the chest, arms, and legs. The patient does not feel any electricity or discomfort during this test. The EKG provides information about how fast and regular the heart is beating and gives us a general sense about the global function of the different parts of the heart, which in turn need to be confirmed by an echocardiogram.

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Duration Of Pulmonary Hypertension

In many people, pulmonary hypertension develops over months or years. This can happen even without causing significant symptoms, so that by the time the condition is diagnosed, youve already had it for quite some time.

Unless your pulmonary hypertension is due to an underlying health condition that can be effectively treated, such as blood clots in your lungs, theres a good chance youll be living with it for the rest of your life. There are no defined stages of pulmonary hypertension, and your condition may get better or worse or remain somewhat stable over time.

For most people, treatment for pulmonary hypertension is aimed at improving your symptoms and preventing your condition from getting worse. By following treatment and lifestyle recommendations, you may be able to improve your health and quality of life.

What Is Pulmonary Hypertension

Pulmonary hypertension is a rare lung disorder in which the arteries that carry blood from the heart to the lungs become narrowed, making it difficult for blood to flow through the vessels. As a result, the blood pressure in these arteries — called pulmonary arteries — rises far above normal levels. This abnormally high pressure strains the right ventricle of the heart, causing it to expand in size. Overworked and enlarged, the right ventricle gradually becomes weaker and loses its ability to pump enough blood to the lungs. This could lead to the development of right heart failure.

PH occurs in individuals of all ages, races, and ethnic backgrounds, although it is much more common in young adults and is approximately twice as common in women as in men.

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Medical History And Physical Exam

Your doctor may ask you about any symptoms you have been experiencing and any risk factors such as other medical conditions you have.

Your doctor will also perform a physical exam to look for signs that may help diagnose your condition. As part of this exam, your doctor may do the following:

• Check whether the oxygen levels in your blood are low. This may be done by pulse oximetry, in which a probe is placed on your finger to check your oxygen levels.
• Feel your liver to see if it is larger than normal.
• Listen to your heart to see if there are changes in how it sounds, and also to find out if your heartbeat is faster than normal or irregular or if you have a new heart murmur.
• Listen to your lungs for sounds that could be caused by heart failure or interstitial lung disease.
• Look at the veins in your neck to see if they are larger than normal.
• Look for swelling in your abdomen and legs that may be caused by fluid buildup.
• Measure your blood pressure.

Evaluation And Diagnosis Of Ph

There is a well-documented time lag between symptom onset and diagnosis of PH.According to the Registry to Evaluate Early and Long-Term PAH Disease Management, roughly 21% of patients experienced a 2-year delay in receiving adiagnosis of PAH.6 The lack ofimprovement in this lag over the past decades triggered the 6th WSPH taskforce onthe diagnosis of PH to modify the recommended diagnostic algorithm to facilitate amore efficient diagnostic process.7For example, patients with PH due to confounding medical conditions are typicallymedically complex and difficult to treat, prompting the recommendation that theyreceive early referral to specialized PH centers to guide their long-term management.6,8,9,10 In theUnited States, these centers are called Pulmonary Hypertension Care Centers and areaccredited by the Pulmonary Hypertension Association.11 Care received at PH care centers has been shown toimprove outcomes in patients with PAH.12

Algorithm for diagnosis of pulmonary hypertension and identifying itscause. Reproduced with permission of the © ERS 2020: DOI: 10.1183/13993003.01904-2018.7 PH: pulmonary hypertension V/Q:ventilation/perfusion CTEPH: chronic thromboembolic PH

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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

Why Do The Pulmonary Arteries Narrow

Scientists believe that the process starts with injury to the layer of cells that line the small blood vessels of the lungs. This injury, which occurs for unknown reasons, may cause changes in the way these cells interact with the smooth muscle cells in the vessel wall. As a result, the smooth muscle contracts and grows more than is normal and narrows the vessel.

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How Common Is Pulmonary Hypertension

There are many different types of pulmonary hypertension and some are associated with underlying health conditions, particularly conditions affecting the heart or lungs. Pulmonary hypertension may be more common in people with:

• sickle-cell anaemia
• chronic obstructive pulmonary disease

Generally, pulmonary hypertension can affect people of almost any age, although some types are more common in young women and some in people aged over 40.

One type of pulmonary hypertension, called pulmonary arterial hypertension , is more common in women and affects two to four people in every million each year.

Why Pah Is Hard To Diagnose

PAH is difficult to spot and diagnose, even when your doctor is looking for it during a physical exam. The symptoms of PAH mimic those of other similar conditions. Even as PAH progresses, your doctor may have trouble distinguishing it from other heart and lung conditions.

Your doctor will assess your medical history and use one or more tests to determine whats causing your symptoms.

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Sleep Study And/or Overnight Oximetry Study

One of the causes of pulmonary hypertension is poor quality breathing during sleep. If someone’s oxygen levels are low during sleep, this condition puts a lot of strain on the lungs and heart and may lead to pulmonary hypertension. Since a patient would be asleep when these abnormalities of breathing are occurring, she/he may not feel that their breathing is limited during sleep and may not be aware of this.

• A sleep study is a study done in a sleep center . The patients come to the sleep center in the evening, get hooked up to different monitors including an oxygen monitor and are asked to sleep . When they wake up in the morning, they go back home. If a patient indeed has trouble with her/his breathing when asleep, they may need to be on oxygen or use certain masks, such as a CPAP , at night to help with their breathing and to improve their pulmonary hypertension.
• An overnight oximetry study is a test in which a recording “pulse oximeter monitor” continuously measures a patient’s oxygen level and heart rate overnight. This test could be done in someone’s home. This is more convenient than the sleep study but does not give us all the information needed.

Ph Associated With Left Ventricular Diastolic Dysfunction

The heart cycle is divided into two phases, systole and diastole. Systole is when the heart is pumping blood out to the lungs and body. After the ventricles eject the blood they must relax and refill with blood to prepare again for pumping. This relaxation phase is known as diastole. The ventricles can become stiff over time not allowing the ventricles to fill completely with blood during diastole. The stiffness of the ventricles and inability to relax causes the pressure to rise on the left side of the heart, and eventually the pressure in the lungs rises. This condition is known as diastolic dysfunction. Diastolic dysfunction may occur with normal aging but is also associated with chronic high blood pressure, coronary artery disease and many forms of cardiomyopathies .

Diastolic dysfunction can only be diagnosed by heart catheterization. The data that the doctor obtains during the heart catheterization will help identify this type of heart disease. Although diastolic dysfunction is a form of pulmonary hypertension the treatment is different and the goal is to relax the left ventricle using specific drugs such as nitrates. It is also important to control the bodys blood pressure, treat coronary artery disease and control lung congestion with diuretics.

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Treatment And Medication Options For Pulmonary Hypertension

Your doctor may prescribe drugs for your pulmonary hypertension, to address any known underlying causes or to deal with its symptoms and potential complications. Pulmonary hypertension treatment may also involve lifestyle changes or other treatments to improve your heart function and physical endurance, and to improve your symptoms or prevent them from getting worse.

Persistent Pulmonary Hypertension Of The Newborn

This is a type of pulmonary hypertension estimated to affect two in every 1,000 newborn babies.

It has been linked to the mother taking non-steroidal anti-inflammatory drugs during the third trimester of the pregnancy .

Conditions that may also be associated with persistent pulmonary hypertension of the newborn include:

• severe pulmonary hypoplasia when your babys lungs are seriously underdeveloped
• hypoglycaemia an abnormally low level of glucose in the babys blood
• a life-threatening illness caused by the babys body overreacting to an infection
• meconium aspiration syndrome when a newborn baby breathes in a mixture of amniotic fluid and meconium , which can block their airways

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Adjusting To An Iv Pump

Life soon became more manageable, McGrath says. I went from being very sick and very anxious about everything I was feeling to suddenly feeling better, she says. I didnt have to worry about being out of breath. At work, I spend eight hours a day talking on the phone, and I was able to do it. I went from hating my pump to considering it my friend. I even dress it up in designer bags.

In order to shower, she has to cover her catheter site with tape, which also means she can no longer go swimming. But she says its worth it for the increased energy she now has. I feel 80% better than I did before, she says.

Chere McGrath relaxes at home after treatment for her pulmonary arterial hypertension. “I feel 80% better than I did before,” she says.

Initially, McGrath had check-ups with Dr. Singh monthly, but as her condition improved, they were able to move to every three months. Her condition has improved to the point where it is now considered functional class 1, Dr. Singh says.

For Chere, getting from severe disease to now having no limitations is quite a turnaround, he says. And from first being told what this disease even is to being a patient advocate herself is very rewarding to see.

These days, whenever a Yale PVD Program patient is apprehensive about starting IV therapy, Dr. Singh and his team reach out to McGrath and ask if shed be willing to talk to the patient.

Without hesitation, McGrath always agrees.

How Is It Different From Systemic Blood Pressure

Systemic blood pressure is a measure of how forcefully blood flows through other arteries in the body.

The Centers for Disease Control and Prevention note that normal systemic blood pressure is below 120/80 mm Hg, where the top number represents systolic blood pressure and the bottom diastolic.

A person has hypertension if their blood pressure is 130/80 mm Hg or higher, and doctors may recommend treatment when a persons blood pressure is consistently 140/90 mm Hg or higher.

Systemic hypertension is more common than PAH. Although both are risk factors for heart disease, PAH is more likely to cause heart failure. Over time, systemic hypertension may damage the heart, causing PAH.

Pulmonary hypertension is an area of intense research, particularly with respect to:

• the correct type of medication to use in the different groups of pulmonary hypertension, and in which combinations
• management of right heart failure, and possible new treatment targets or therapies
• treatments at the molecular level, such as monoclonal antibodies, which may be useful tools to slow disease progression
• further genetic markers that may identify people at high-risk of developing pulmonary hypertension.

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