Pulmonary arterial hypertension

Cause

What happens in the event of pulmonary hypertension (PH)? 

Pulmonary hypertension occurs when the mean arterial blood pressure in the pulmonary artery increases above 25 mmHg during rest (normal pressure: <20 mmHg).

Pulmonary hypertension means that there is an increase in pressure in the pulmonary circulation due to a narrowing of the blood vessels in the lungs. This places extra strain on the heart as it tries to continue pumping a sufficient amount of blood through the narrowed blood vessels of the lungs. These blood vessels will continue to narrow and become increasingly inelastic. If the arteries (the blood vessels that lead away from the right ventricle of the heart) are affected, then the disorder is called pulmonary arterial hypertension.

As the disease progresses, the right heart muscle mass will continue to increase in size to the point that the heart will finally lose the ability to pump enough blood into the lungs. The supply of oxygen to the body and heart will then deteriorate further. If the disease is left untreated, it will eventually lead to heart failure. 

Current classification of pulmonary hypertension

1. Pulmonary arterial hypertension

• Idiopathic
• Heritable
  • BMPR2
  • ALK1, Endoglin
  • Unknown
• Drug and toxin-induced
• Associated with PAH
  • Connective tissue diseases
  • HIV infection
  • Portal hypertension
  • Congenital heart disease
  • Schistosomiasis
  • Chronic hemolytic anemia
• 1'. Pulmonary Veno-occlusive disease and/or capillary hemangiomatosis
• 1". Persistent pulmonary hypertension of the newborn (PPHN)

2. Pulmonary hypertension due to left heart disease

• Left ventricular systolic dysfunction
• Left ventricular diastolic dysfunction
• Valvular disease
• Congenital/acquired left heart inflow/outflow tract obstruction an congenital cardiomyopathy

3. Pulmonary hypertension due to lung diseases and/or hypoxia

• Chronic obstructive pulmonary disease
• Interstitial lung disease
• Pulmonary diseases with restrictive and obstructive pattern
• Sleep-disordered breathing
• Alveolar hypoventilation disorders
• Chronic exposure to high altitude
• Developmental lung diseases

4. Chronic thromboembolic pulmonary hypertension (CTEPH)

• Complication of pulmonary embolism
• Large or proximal vessel disorders
• High blood pressure, blood clots
• Reduced compliance and luminal narrowing

5. Pulmonary hypertension with unclear and/or multi-factorial mechanisms

• Hematologic disorders (myeloproliferative disorders, splenectomy)
• Systemic disorders (Vasculitis sarcoidosis, pulmonary Langerhans cell, histiocytosis LAM, neurofibromatosis)
• Metabolic disorders (Glycogen storage disease, thyroid disorders)
• Congenital heart disease
• Cancer-related, renal failure on dialysis

Diagnosis

Patients suspected of having pulmonary hypertension (due to poor physical performance and impaired breathing), they will undergo a comprehensive physical examination and their medical history will be analyzed. Aside from the family medical history, it is also very important to provide details of any previous drug prescriptions and possible substance abuse.

Blood and imaging tests done to help diagnose pulmonary hypertension may include:

• Blood tests. Blood tests can help determine the cause of pulmonary hypertension or detect signs of complications.
• Chest X-ray. A chest X-ray creates pictures of the heart, lungs and chest. It can show enlargement of the right ventricle or the pulmonary arteries. A chest X-ray may also be used to check for other lung conditions that can cause pulmonary hypertension.
• Electrocardiogram (ECG). This noninvasive test shows the heart's electrical patterns and can detect abnormal heartbeats. An ECG may also reveal signs of right ventricle enlargement or strain.
• Echocardiogram. Sound waves are used to create moving images of the beating heart. An echocardiogram lets a doctor see how well the heart and its valves are working. It can show the size and thickness of the right ventricle and the pressure in the pulmonary arteries.
  
  Sometimes, an echocardiogram is done while exercising on a stationary bike or treadmill to understand how well the heart works during activity. If you have this test, you may be asked to wear a mask that checks how well the heart and lungs use oxygen and carbon dioxide.
  
  An echocardiogram may also be done after diagnosis to determine how well treatments are working.
• Right heart catheterization. If an echocardiogram reveals pulmonary hypertension, you'll likely have a right heart catheterization to confirm the diagnosis.
  
  During this procedure, a cardiologist places a thin, flexible tube (catheter) into a vein, usually in the groin. The catheter is gently guided into the right ventricle and pulmonary artery.
  
  Right heart catheterization allows a doctor to directly measure the pressure in the main pulmonary arteries and right ventricle of the heart. The test may also be used to determine how well pulmonary hypertension medications are working.

Everyday life

Patients diagnosed with pulmonary arterial hypertension, they must learn how to adapt to the new condition.

Strenuous physical activity should be avoided since the lungs cannot provide the body with the necessary oxygen requirements any longer. Breathing difficulties are a clear sign that the strenuous activity should be stopped. However, it is very important that the patient does light exercise and walks regularly in order to maintain muscle strength and to encourage circulation.

Bodyweight needs to be inspected at regular intervals, preferably always at the same time of the day. Sudden increases in bodyweight can be a signal of a deterioration in the heart and kidneys.

Both active and passive smoking should be avoided.

If a female patient wants to have a child it is of utmost importance for her to first consult a PAH specialist given the increased risk for expectant mothers and their unborn children caused by PAH.

Dietary recommendations for pulmonary hypertension patients

A diagnosis of pulmonary hypertension causes a change in lifestyle for many people. Many patients often ask themselves what they should eat. There is no special “high-pressure lung diet”, but there are nutritional measures that can make everyday life easier, especially when coping with with diarrhea and both loss of appetite and weight.

The patient's diet should be rich in micronutrients, fiber, and vitamins. However, the intake of cooking salt should be reduced.

Alcohol should only be enjoyed in small amounts.

What can you do if you have diarrhea?

Diarrhea is when three or more loose or watery bowel movements occur within 24 hours. The stool consistency is unshaped, which means mushy or thin. Persistent diarrhea results in significant fluid and electrolyte loss. To compensate for these losses, isotonic drinks (electrolyte drinks) are recommended because the body can absorb this liquid better than pure water or tea.

Drinking is particularly important when having diarrhea and the recommended amount is at least 1 to 1.5 liters per day!

Drinks at room temperature are optimal and carbonated drinks will cause flatulence.

Avoid drinking large amounts at once, instead drink regularly throughout the day.

Suitable teas:

• Always let teas infuse for 10-15 minutes!
• Black tea: Leave to infuse for 10-15 minutes so that the tannic acid (which can irritate the bowel) is dissolved
• Green tea
• Chamomile tea (antibacterial)
• Strawberry or blackberry leaf tea (contain tannins, can be obtained from a pharmacy)
• Fennel tea or mixed tea of anise, fennel and caraway seeds in equal parts
• Tea made from dried blueberries (available in the pharmacy): 8 tablespoons dried blueberries + 35 g glucose + knife tip salt + 1 liter of water

Less suitable drinks:

These drinks are not suitable for low-viscosity stools because they contain too little sugar and minerals:

• Pure drinking and mineral water
• Unsweetened tea
• Light drinks like Gröbi, Cola light / zero, Schartner diet etc.

Recommended foodstuffs during the diarrhea phase:

When digestion is intensive, easily digestible food is a much better option. In this phase, you should rather refrain from hearty home-cooked food, especially if it is greasy, fatty and stimulatesstool.

Spices such as herb salt, parsley, dill, ground nutmeg, caraway and ground vegetables (carrots, celery) and also pumpkin, courgette/ zucchini and fennel are generally well tolerated.

Pectins support intestine-function.

They help to remove harmful decomposition products from the intestine and provide food for the healthy intestinal flora. They also increase water absorption from the intestine into the blood.

Pectins are contained in apple with peel, banana, carrot, blueberries (especially dried), and jams.

Foodstuffs that slow down intestine function:

• White flour products (white bread, toasted bread, rusks, bread from the day before, Soletti, ...)
• Potatoes, puree/ mashed (with water or lactose-free milk, no butter!)
• White rice, pasta - soft-cooked
• Thick-textured/ soupy food (rice, oatmeal, semolina)
• Carrot soup, carrot porridge - cooked for a long time!
• Mashed or steamed vegetables (celeriac, spinach, pumpkin, zucchini, salads, carrots)
• Blueberry mousse or compote
• Banana, grated apple (grate very finely with the peel and definitely let it brown!). In addition, with crushed biscuits this makes a delicious dessert!
• Sponge cake, sponge roll
• Cooked lean poultry or veal, lean pork
• Steamed lean fish (pike-perch, plaice, cod)
• Low-fat cream cheese, cottage cheese - preferably lactose-free
• Cocoa, dark chocolate

COVID-19 and PAH

Having PAH does not increase the risk of catching the coronavirus, which is also referred to as COVID-19. Because COVID-19 is a new virus, there is no immunity in society, meaning the entire human population is prone to infection. After an infection with COVID-19 the severity of symptoms can range from very mild (or even no symptoms at all) to severe. People who are older or have existing chronic medical conditions, such as heart or lung diseases or diabetes, may be at higher risk of serious illness. Of course, PAH patients tend to be at a higher risk. We therefore urge PAH patients to please STAY at HOME. PAH patients should be cautious and try to avoid any risk of infection! COVID-19 is primarily spread via respiratory droplets when people cough or sneeze. Normally, symptoms of COVID-19 begin 2 to 14 days after exposure. These symptoms include, but are not limited to, fever, tiredness, dry cough, shortness of breath or difficulty breathing.

General recommendations:

• Comply with and keep up with your usual treatments, as recommended by your treating physician.
• Focus on a healthy diet and keep well hydrated according the guidance of your treating physician.
• Stay at home as far as possible, avoiding any unnecessary contacts with other people.
• It is not recommended that healthy people wear a face mask to protect themselves from respiratory illnesses, including COVID-19. However, it is highly advisable to wear a mask if you are infected in order to protect others.

What general preventive measures should people take?

The following simple preventive measures can help minimize the spread of COVID-19:

• Wash your hands often and with soap, lathering both the front and the back of the hands and fingers for at least 20 seconds - in particular after being in public places.
• If soap is not available, use a hand sanitizer that contains at least 60% alcohol.
• Make sure everyone around you is practicing good hand washing techniques.
• Avoid close contact with anyone with respiratory symptoms like coughs, colds or ongoing chest infections.
• Stay at home if you are sick or have even the slightest flu-like symptoms.
• Maintain a distance of at least 1 meter to other persons.
• Use a tissue to cover your mouth and nose if you cough or sneeze and dispose of it promptly afterwards, alternatively you can sneeze into your sleeve/elbow.
• Try to avoid touching your eyes, nose, and mouth with your hands as much as possible.
• Avoid places where there is likely to be an increased risk of exposure such as crowds, especially in poorly ventilated areas.

What extra precautions should pulmonary hypertension patients take?

Individuals with respiratory diseases, such as pulmonary hypertension, do not appear to be more at risk of contracting COVID-19 than the general public. However, if they catch COVID-19 they are more likely to suffer from severe symptoms. 

Patients with respiratory diseases should, therefore, take extra precautions to minimize the risk of getting infected with COVID-19. In addition to the general preventive measures listed above, they should:

• Make sure that they are up to date with repeat prescriptions
• Stock up on necessary medications and supplies that can last for a few weeks
• Avoid crowds and non-essential travel
• STAY AT HOME!
• Those patients with existing respiratory problems should only wear face masks when necessary, as they can make breathing more difficult.

Advice for family members and caregivers

Family members and caregivers of people with chronic diseases like pulmonary hypertension should take appropriate precautions and take extra care to avoid bringing COVID-19 home. They should constantly monitor patients and stock medicines and other necessary supplies that can last for several weeks. Storing extra non-perishable food can help minimize trips to the grocery store. People who show symptoms of COVID-19 or who have been exposed to COVID-19 (e.g. having been in physical contact with COVID-19 infected people) should have to immediately stop visiting their PAH relatives/patients until the self-isolation period is complete.

What should sick individuals do?

If symptoms are present and a COVID-19 diagnosis is confirmed, patients should follow these steps to prevent the spread of the infection:

• Stay at home, preferably in a separate room not shared with others, and isolate yourself, with the exception of getting medical care.
• Avoid public areas and public transportation.
• Limit contact with pets and animals.
• Avoid sharing personal items.
• Cover coughs and sneezes with tissues and dispose of such tissues properly.
• Sanitize hands regularly.
• Disinfect surfaces such as phones, keyboards, toilets, and tables.
• People should call ahead before visiting the hospital for an appointment. This way, the hospital can take the necessary steps to prevent the spread of the infection.
• Patients who have confirmed COVID-19 should wear face masks when going out.

With joint support, we will get through this crisis!