Pulmonary Hypertension: A Complete Guide (2020)

Do you know that if left untreated, the average life expectancy of a pulmonary hypertension patient is approximately 3 years from the occurrence of symptoms?

Another cause of worry is that 72% of the pulmonary hypertension patients are diagnosed with the disease in advanced stages.

In this article, I will talk about every important aspect of Pulmonary hypertension including its symptoms, diagnosis, stages, groups, treatment, and diet associated with pulmonary hypertension.

So, let’s begin with…

What is Pulmonary Hypertension?

Pulmonary Hypertension (also known as PH) is a condition or a type of high blood pressure in which blood pressure increases inside the blood vessels in the lungs.

PH vs PAH (Pulmonary Hypertension vs Pulmonary Arterial Hypertension)

Now, you must be thinking is PAH different from PH?

Yes, it is. PH is used as a general term to describe high blood pressure in lungs which can be due to any cause such as sleep apnea, blood clots in lungs, chronic lung disease, diseases of the left heart etc.

PAH or Pulmonary Arterial Hypertension, on the other hand, is a subgroup of PH. It is a chronic disease in which the arteries in the lungs are blocked, narrowed and destroyed. It occurs specifically when the pulmonary arteries get tightened or constricted that connects the right side of the heart to the lungs.

Due to this condition, blood flows slowly through your lungs, and your heart works hard to pump the blood so that it can easily go through your lungs. This results in the weakening of the cardiac muscle, and it makes the heart weak which might eventually result in heart failure.

Though PAH is a rare condition, it can affect both males and females of all ages and currently, there is no cure for this disease. Due to this, the early diagnosis of PAH is very important. If not treated on time, pulmonary arterial hypertension can slowly get worse and can be life-threatening.

Pulmonary Hypertension Symptoms

At starting you won’t be able to judge the symptoms. In some cases, you may have no symptoms at all. Generally, the pulmonary hypertension symptoms develop very slowly which you may not be able to notice for months or even a few years. Symptoms become more intense with time as the diseases progress.

Pulmonary hypertension symptoms

The symptoms of pulmonary hypertension include:

  • Short of breath or dyspnea
  • Dry coughing
  • Fatigue
  • Chest pain (Angina)
  • Swelling in legs and ankles
  • Bluish Lips
  • Abdominal Bloating
  • Loss of Consciousness
  • Rapid Breathing
  • Elevation of chest
  • Rapid Weight Gain
  • Dizziness and lethargy

Pulmonary Hypertension Diagnosis

To properly diagnose pulmonary hypertension, the patient needs a complete physical examination, medical history and detailed description of their symptoms to make the correct diagnosis.

The diagnosis process comprises of the following:

Physical Examination – While carrying out a physical examination, the doctor checks for abnormal heart sounds like a systolic murmur of tricuspid regurgitation, a loud pulmonic valve sound, or a gallop due to ventricular failure. The doctor examines the abdomen, legs, and ankles for fluid retention and the jugular vein in the neck for enlargement along with nail beds for the bluish tint. Doctors also try to look for underlying disease signs, which might be the cause of PH.

Blood Tests – Several blood tests can be used to determine the type of PH you are suffering from such as:

  • Complete Metabolic Panel test to examine liver and kidney function
  • Autoantibody Blood Tests like ANA, ESR that detects for collagen vascular diseases
  • Thyroid-stimulating hormone test to screen for thyroid problems
  • HIV test to screen for human immunodeficiency virus
  • Arterial blood gas test that determines oxygen level in arterial blood
  • Complete blood count test to detect infection, anaemia, and elevated haemoglobin
  • B-type Natriuretic Peptide test that is a marker for heart failure

Doppler Echocardiogram – Under Doppler Echocardiogram method, high-frequency sound waves are used to determine the functioning of the right ventricle, measure the flow of blood through the heart valves, and then calculate the systolic pulmonary artery pressure. The Doppler technology helps in determining the speed and direction of the blood flow by making use of the Doppler Effect.

PolysomnographyPolysomnography is a test which helps in determining whether the patient is suffering from sleep apnea or not. Polysomnography records brain waves, the oxygen level in the blood, breathing, and heart rate. Sleep apnea patients have low oxygen levels at night.

Electrocardiogram (ECG) – Electrocardiogram can show your heart’s electrical patterns, abnormal heartbeats, and signs of strain or enlargement of the right ventricle.

Chest X-ray – Chest x-ray can show enlargement of pulmonary arteries and enlargement of the right ventricle of the heart which can be usually seen in pulmonary hypertension. Other lung conditions can also be checked using a chest X-ray which can cause pulmonary hypertension.

6-Minute Walk Test (aka Exercise Tolerance Test)– The 6-minute walk test can be used to analyze patients’ exercise tolerance level and their blood oxygen saturation level while exercising.

Pulmonary Function Tests – Pulmonary Function Tests are tests which are carried out to determine how well you are able to breathe or how effectively your lungs are working. It can help find lung conditions such as chronic obstructive pulmonary disease, and idiopathic pulmonary fibrosis, among others. The test measures the lung volume, capacity, gas exchange etc.

Pulmonary Angiogram – Pulmonary Angiogram test is done to find whether there is a blockage due to blood clots in the pulmonary arteries or not. It is a medical fluoroscopic procedure which is used to visualize the pulmonary arteries or pulmonary veins (rarely) using a special contrast dye injected into the blood vessels.

Right Heart Catheterization – Your doctor might suggest you to go for right-heart catheterization if your initial diagnosis points towards PH. It is considered as one of the most accurate tests for the diagnosis of pulmonary hypertension. In this test, a catheter (a thin rubber tube) is inserted in the patient’s groin or neck through a large vein. The catheter is then passed in the heart to measure the patient’s blood pressure on the right side of the heart and lungs.

Ventilation Perfusions Scan – Ventilation Perfusions Scan, also known as VQ lung scan is carried out to find traces of blood clots in the lungs, called pulmonary embolism (PE). VQ lung scan uses radioactive material for examining the airflow or ventilation and blood flow or perfusion in the lungs. It helps in determining the ventilation/perfusion ratio.

Stages of Pulmonary Hypertension

World Health Organization (WHO) and the New York Heart Association have classified pulmonary hypertension into four stages which include:

Stage 1: Patients who have been diagnosed with the diseases but don’t show any symptoms, fall under Stage 1 category. It can only be identified through medical check-up. Under Stage 1, normal physical activity doesn’t cause any discomfort such as chest pain, fatigue, dyspnea etc.

Stage 2: Under Stage 2 pulmonary hypertension, some symptoms can be seen when you do any physical activity, but there are no symptoms when you are at rest.

Stage 3: In Stage 3 pulmonary hypertension, symptoms can be easily seen when you do physical activities, and mild symptoms can be seen when you are at rest.

Stage 4: In Stage 4, symptoms are clearly visible while you are resting and you will be facing regular problems and discomfort such as dyspnea, fatigue or right-sided heart failure if indulged in any physical activity.

Pulmonary Hypertension Causes

So what causes pulmonary hypertension?

There are many causes of pulmonary hypertension. Broadly, the causes are clubbed into five groups by the World Health Organization (WHO).

WHO’s Classification of Pulmonary Hypertension

Pulmonary-Hypertension-WHO-Groups

WHO has classified pulmonary hypertension in five groups named as: –

WHO Group 1 – Pulmonary arterial hypertension (PAH)

WHO Group 2 – Pulmonary hypertension due to left heart disease

WHO Group 3 – Pulmonary hypertension due to lung disease and/or chronic hypoxia

WHO Group 4 – Pulmonary hypertension due to blood clots in the lungs

WHO Group 5 – Pulmonary hypertension due to blood and other disorders

Let’s discuss each group in detail –

WHO Group 1 – Pulmonary arterial hypertension (PAH)

WHO Group 1 refers to pulmonary arterial hypertension (PAH) which is caused when the arteries present in the lungs get narrowed or blocked.

There are several types of PAH including:

  • Idiopathic PAH (IPAH)
  • Heritable PAH or Familial PAH (Linked to genes inherited from family members)
  • PAH caused by drugs such as methamphetamine or toxins and diet pills
  • PAH associated with medical conditions such as liver disease, HIV, congenital heart disease, connective tissue diseases such as Lupus or Scleroderma and infections such as sickle cell anaemia

Is targeted treatment available for PAH? – Yes

WHO Group 2: Pulmonary hypertension due to left heart disease

WHO Group 2 refers to pulmonary hypertension caused due to left heart disease. WHO Group 2 is the most common form of pulmonary hypertension and is caused due to prolonged problems with the valves on the left side of the heart. The left heart is not able to keep up with the blood returning from the lungs which causes a backup of blood and as a result raises the pressure in the lungs.

Is targeted treatment available for PH due to left heart disease? – No

WHO Group 3: Pulmonary hypertension due to lung disease and/or chronic hypoxia

WHO Group 3 refers to pulmonary hypertension caused due to lung diseases. The primary causes for Group 3 pulmonary hypertension are lung diseases or due to the shortage of oxygen (Hypoxia).

The common lung diseases associated with WHO Group 3 include:

  • Chronic obstructive pulmonary disease (COPD) or Emphysema
  • Interstitial lung disease or pulmonary fibrosis
  • Obstructive Sleep apnea
  • High-altitude exposure for long
  • Alveolar hypoventilation disorders
  • Lung development abnormalities

In this, the arteries present in the lungs tighten which forces the blood to go to those areas of the lungs that receive more air. This tightening of arteries leads to high blood pressure in the lungs.

Is targeted treatment available for PH due to lung disease or Hypoxia? – No

WHO Group 4: Pulmonary hypertension due to blood clots in the lungs

WHO Group 4 refers to pulmonary hypertension caused due to blood clots in the lungs which obstructs pulmonary arteries. WHO Group 4 is also referred to as chronic thromboembolic pulmonary hypertension (CTEPH).

Clots are formed when the body suffers any bleeding or injuries, but if they are formed without an apparent cause, it can harm the lungs and heart. Pulmonary thrombosis is a condition in which the body is not able to dissolve the clots that are formed in the lungs that can block the pulmonary arteries. Due to this blockage, the right side of the heart has to work harder which increases the blood pressure in the lungs.

Is targeted treatment available for chronic thromboembolic pulmonary hypertension (CTEPH)? – Yes

WHO Group 5: Pulmonary hypertension due to blood and other disorders (Multifactorial pulmonary hypertension)

WHO Group 5 refers to pulmonary hypertension caused due to blood disorders or certain metabolic disorders. The main causes for WHO Group 5 PH are

  • Blood disorders such as anaemia
  • Systemic disorders such as sarcoidosis and histiocytosis
  • Metabolic disorders such as glycogen storage diseases and thyroid issues
  • Chronic kidney failure and tumours obstructing pulmonary arteries

These are rare causes which are not commonly seen among pulmonary hypertension patients.

Is targeted treatment available for Multifactorial pulmonary hypertension? – No

Pulmonary Hypertension Treatment

Though pulmonary hypertension can’t be cured, proper treatment can reduce your symptoms and condition significantly.

Here are several treatment options available for pulmonary hypertension the use of which might vary from cases to cases depending on the symptoms of the patient.

Pulmonary-Hypertension-Treatment

Medications –

Oxygen Therapy – In oxygen therapy, the patient inhales pure oxygen from the prong fit in the nose which increases the oxygen level in the blood. Oxygen therapy is popularly used among patients who live at a high altitude or have sleep apnea. According to a study, oxygen therapy should be used for long term among patients whose arterial oxygen tension is lower than 55 mmHg at rest.

Vasodilators – Vasodilators relaxes and opens the narrowed blood vessels, and also improves the blood flow. Vasodilators are sent by an intravenous (IV) line attached to a small pump. Some of the most commonly prescribed vasodilators are epoprostenol (Flolan, Veletri), nifedipine (Procardia®), diltiazem (Cardizem®), and treprostinil (Tyvaso, Remodulin, Orenitram). Vasodilators are associated with some side effects including chest pain, jaw pain, headache, nausea, and diarrhoea.

Anticoagulants / Blood Thinners – Anticoagulants such as warfarin (Coumadin, Jantoven) are used to prevent the blood clots in arteries present in the lungs so that blood can flow freely through blood vessels. It is important to consider the bleeding complications in mind as this drug can put you at risk of bleeding, so consult regularly with the doctor while you are on anticoagulants.

Diuretics – Diuretics, also known as water pills, such as furosemide (Lasix), torsemide (Demadex), and spironolactone (Aldactone) are used to remove the excess amount of water from the body through urine which is known as diuresis. This reduces the extra load from the heart, reduces swelling, and eases the breathing process. Diuretics are also used to limit the fluid retention in the lungs, legs and abdomen.

Digoxin – Digoxin is generally prescribed by the doctor to pulmonary arterial hypertension patients who have atrial tachyarrhythmia. It strengthens the heart and improves its pumping ability so that heart can pump more blood easily.

Surgery & Procedures

If your pulmonary hypertension can’t be controlled through medications, your doctor might advise you to go for surgical procedures. Here are some of the common surgeries available to treat pulmonary hypertension.

Atrial Septostomy – Atrial Septostomy is an open heart surgery in which the surgeon creates a small hole between the left and the right chamber of the heart using a cardiac catheter (a thin and flexible pipe) to reduce the pressure on the right side of the heart.

Pulmonary Endarterectomy – It is an operation in which old blood clots are removed from the pulmonary arteries in the lungs. It is used in people with chronic thromboembolic pulmonary hypertension.

Transplantation – In some of the extreme cases, the doctor advises to go for a lung transplant or heart-lung transplant, especially in the case of young people who have idiopathic pulmonary arterial hypertension. But transplantation is rare and kept as the last option when other options for treating pulmonary hypertension are of no help.

How to Treat Pulmonary Hypertension Naturally?

1) Pulmonary hypertension patients tend to retain water so they need to prevent their body from holding too much fluid. Too much fluid in the body gets accumulated in the legs, abdomen, and patients become uncomfortable to exercise or even move. Hence, it is better to avoid having more than 8 cups of fluid in a day.

2) Consume a limited amount of salt. Do not take above 2,000 mg of salt in a day as the body cannot get rid of the excess. Also, stay away from foods having a high amount of sodium such as chips, canned foods, and frozen foods.

3) Pulmonary hypertension patients should have a vegetarian or vegan diet, as these will make them feel better.

4) Pulmonary hypertension patients should practice some light exercises which can help build stamina and manage their breathing. However, patients need to consult with the doctor if they wish to start swimming. Patients with the right kind of exercise can walk more, breathe better, and feel better about handling their pulmonary hypertension.

Note: Patients should avoid lifting weights of more than 15 pounds.

5) Pulmonary hypertension patients should stay away from caffeine and alcohol. They should avoid tea and coffee.

6) Pulmonary hypertension patients should eat smaller meals and cut back on high-fat food items to avoid nausea problem.

7) Sleep issues are common for people with pulmonary arterial hypertension (PAH), especially in conditions such as sleep apnea which makes the breathing worse. Try to get adequate sleep to keep yourself energized.

Pulmonary Hypertension Diet

  • Reduce the intake of salt or sodium-rich foods
  • Reduce the intake of water
  • Eat more garlic
  • Take more Vitamin K in your diet which is found in green leafy vegetables, cauliflower cabbage, cereals, eggs, fish, pomegranate, dried figs, tomatoes etc.
  • Take proper sleep on a daily basis
  • Eat smaller meals at a time
  • Limit the intake of coffee and alcohol
  • Avoid fatty foods and foods which are high in carbs
  • Include vitamin C in your diet such as oranges, tomatoes, broccoli

FAQs

Q1) What is the life expectancy of pulmonary hypertension patients?

Ans) Earlier it was observed that people having pulmonary hypertension have a life expectancy of up to 2-5 years but with proper care, diet, medication and lifestyle, you can survive up to 10 more years.

Q2) Does high altitude affect pulmonary hypertension patients?

Ans) Yes, the pulmonary pressure increases at high altitude areas which can result in worsening of the condition.

Q3) Does cold weather affect pulmonary hypertension?

Ans) Yes, you need to take extra care and precautions during winter as cold and flu generally aggravates in winter. It may elevate the blood vessels in the lungs which is not a good sign.

Q4) Which foods to avoid in pulmonary hypertension?

Ans) Avoid or limit the intake of salt, coffee, and alcohol. Also, stay away from oily foods and high carb foods.

Q5) Why does Chronic Obstructive Pulmonary Disease (COPD) cause pulmonary hypertension?

Ans) It has been observed that pulmonary vascular remodelling in COPD is the primary cause of the increase in pulmonary artery pressure and is resulted from the combined effects of inflammation, hypoxia, and loss of capillaries in emphysema.

Feel free to ask your queries related to pulmonary hypertension below and if you liked the article, share it in your close groups.

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