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An overview of Pulmonary Hypertension

What is Pulmonary Hypertension?

A form of high blood pressure known as pulmonary hypertension affects the arteries inthe lungs and the right side of the heart.

Blood arteries in the lungs are constricted, obstructed, or damaged in pulmonaryarterial hypertension (PAH), one kind of pulmonary hypertension. Due to the injury,blood pressure in the arteries of the lungs increases and blood flow through thelungs is slowed. Blood must be pumped through the lungs more forcefully by the heart. The added strain eventually results in the heart muscle failing due to weakness.

In some people, pulmonary hypertension slowly gets worse and can be life-threatening.Although there's no cure for some types of pulmonary hypertension, treatment canhelp reduce symptoms and improve quality of life

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What are the different types of pulmonary hypertension?

The World Health Organization (WHO) divides pulmonary hypertension into five groupsbased on its cause.

Group 1 PH due to pulmonary arterial hypertension (PAH) : PAH has manydifferent causes, ranging from underlying diseases to certain drugs. PAH makes yourpulmonary arteries become narrow, thick or stiff. Less blood can flow through, whichraises the pressure in your pulmonary arteries.

Group 2 PH due to left-sided heart disease : The left side of your heart pumpsout blood to your entire body. If there’s a problem on this side of your heart, itaffects the right side of your heart and your entire pulmonary circuit. Blood backsup in your heart, raising the pressure in your pulmonary arteries.

Group 3 PH due to lung disease or hypoxia : Certain lung problems cause thearteries in your lungs to tighten. Less blood can flow through your lungs, raisingthe pressure in your pulmonary arteries.

Group 4 PH due to blockages in your lungs : Blood clots or scars from bloodclots prevent your blood from flowing normally through your lungs. This puts morestress on the right side of your heart and raises pulmonary blood pressure.

Group 5 PH due to other disorders : PH occurs along with other conditions likeblood disorders and metabolic disorders. The exact mechanisms for how the conditiontriggers PH aren’t always clear.

What causes Pulmonary Hypertension?

Two top chambers (atria) and two lower chambers make up the average heart(ventricles). The lower right chamber of the heart (right ventricle) pumps blood tothe lungs via a sizable blood channel each time blood passes through the heart(pulmonary artery).

The blood releases carbon dioxide and absorbs oxygen in the lungs. Normally, theblood flows smoothly to the left side of the heart through the pulmonary arteries,capillaries, and veins.

The walls of the arteries, however, may stiffen, swell, and thicken due toalterations in the cells that line the pulmonary arteries. Pulmonary hypertensionmay result from these alterations that slow down or obstruct blood flow through thelungs.

Signs and symptoms of Pulmonary Hypertension

The signs and symptoms of pulmonary hypertension develop slowly. You may not noticethem for months or even years. Symptoms get worse as the disease progresses.Pulmonary hypertension signs and symptoms include:

1. Swelling (edema) in the ankles, legs and eventually the belly area (abdomen)

2. Shortness of breath (dyspnea), initially while exercising and eventually while atrest

3. Fatigue

4. Fast pulse or pounding heartbeat (palpitations)

5. Chest pressure or pain

6. Dizziness or fainting spells (syncope)

8. Blue lips and skin (cyanosis)

How can I prevent pulmonary hypertension?

It’s not always possible to prevent pulmonary hypertension. Some risk factors are outof your control. If you have risk factors, your provider may recommend preventivescreenings to check your heart and lung function.

Doing whatever you can to prevent or manage other medical conditions can help loweryour 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 foodshigh in salt and saturated fat.

Quit smoking and stop using tobacco : Smoking and tobacco use are top riskfactors for heart and lung problems. Quitting isn’t easy, especially if you’ve beensmoking or using tobacco for a long time. But your provider can help provideresources. Support groups may also help.

Take medications for blood pressure and other conditions as prescribed.

How is pulmonary hypertension diagnosed?

Your provider will perform a physical exam and run tests to reach a pulmonaryhypertension diagnosis. This tests include :

Echocardiogram : This ultrasound picture of the beating heart can check bloodpressure in the pulmonary arteries.

Ventilation-perfusion scan (V/Q scan) : This test can help find blood clotsthat can cause high blood pressure in the lungs.

Computed tomography (CT) scan : This can show enlarged pulmonary arteries. ACT scan can also spot other problems in the lungs that could cause shortness ofbreath.

Electrocardiogram (EKG or ECG) : An EKG traces the heart's activity and canshow whether the right side of the heart is under strain. That's a warning sign ofpulmonary hypertension.

Chest X-ray : An X-ray can show if your arteries or heart are enlarged. ChestX-rays can help find other lung or heart conditions that may be causing theproblems.

Exercise testing : Your doctor may want you to run on a treadmill or ride astationary bike while you are hooked up to a monitor, so they can see any changes inyour oxygen levels, heart function, lung pressure, or other things.

How is Pulmonary hypertension treated?

Your treatment approach will be personalized for you since pulmonary hypertensionmanifests itself differently in each patient. Find out your options and what toanticipate from your doctor.

Your doctor will first address the underlying cause of your illness. To improve yourpulmonary hypertension, for instance, you'll need to treat the emphysema that iscreating the issue.

The majority of patients also receive medication to help them breathe better, whichmakes it simpler to be active and perform daily duties. If you are having troublebreathing or have low blood oxygen levels, oxygen treatment, which involvesbreathing pure oxygen through prongs that go in your nose, can be helpful. Havingpulmonary hypertension makes you live longer. Your doctor will advise blood thinnersif you are at risk for blood clots. Some medications help your heart function betterand prevent fluid buildup in your body.

Calcium channel blockers are medicines that your doctor might recommend if you havesevere pulmonary hypertension. These drugs reduce blood pressure throughout thebody, including in the lungs.

A specialist treatment facility may be recommended by your doctor if calcium channelblockers are insufficient. You could require more specialized treatments to widenyour blood vessels. These could be oral pills, inhalable medications, or Intravenousmedications. Options consist of:

Pills : ambrisentan (Letairis), bocentan (Tracleer), macitentan (Opsumit),riociguat (Adempas), selexipag (Uptravi), sildenafil (Revatio), tadalafil (Adcirca),treprostinil (Orenitram)

Inhalers: : Iloprost tromethamine (Ventavis), treprostinil (Tyvaso)

IV drugs : epoprostenol sodium (Flolan, Veletri), treprostinil

In more severe cases, or if medicines don't help, your doctor may recommend a lungtransplant or a procedure called atrial septostomy. A surgeon creates an openingbetween the right and left sides of the heart. This surgery can have serious sideeffects.

Frequently Asked Questions About Pulmonary hypertension

Pulmonary hypertension can affect adults at any age.It commonly affects people who have heart or lung conditions.It’s also more common among people with other medical conditions

Some types of PH are rare, such as pulmonary arterialhypertension (PAH) and PH caused by blood clots. But other typesare much more common, especially PH caused by heart or lungproblems. We don’t know exactly how many people around the worldhave pulmonary hypertension. But some estimates show PH mayaffect 1 in 100 people. This means 50 million to 70 millionpeople are living with PH.

Without treatment, pulmonary hypertension leads toright-sided heart failure and, ultimately, death. Treatment canhelp you live longer and give you a better quality of life.

The life expectancy varies from person to person. Itdepends how quickly you’re diagnosed and what other medicalconditions you have. Talk with your provider about what you canexpect in your individual situation. Pulmonary hypertension is aprogressive disease. That means it gets worse over time. Itprogresses more quickly in some people than in others. Treatmentcan improve your chances of surviving pulmonary hypertension formany years.