What is Pulmonary Hypertension?
Pulmonary Hypertension is a condition where the blood pressure in the blood vessels in the lungs or the right side of the heart is higher than normal.
Most patients are familiar with blood pressure measurements taken in the arm which normally is less than 120/80. However, our body has another circulation system of blood vessels which takes blood from the heart to the lungs where the blood receives oxygen from the inhaled air and takes it back to the heart to spread through the rest of the body. This circulation is called the Pulmonary Circulation.
In some conditions, the blood vessels in the lungs lose their elasticity and become stiff leading to increased resistance and pressure within these vessels. This condition is called Pulmonary Hypertension.
Higher pulmonary pressure can cause shortness of breath, dizzy spells, near-fainting, chest pressure, low oxygen levels, heart rhythm problems and also cause changes in the heart over time which can lead to heart failure.
Pulmonary hypertension rarely may occur as an independent problem with no known cause. In most other cases, Pulmonary Hypertension is a result of other conditions that affect the lungs or blood vessels causing either low oxygen, inflammatory disorders of blood vessels, blood clots or heart problems such as leaky valves which can cause increased pressure in the pulmonary blood vessels. In some cases, more than one cause of Pulmonary Hypertension maybe present in the same patient.
What are the symptoms of Pulmonary Hypertension?
- Shortness of breath/difficulty breathing
- Tiredness
- Chest pressure or pain
- Near-fainting episodes
- Heart rhythm disturbances
- Leg swelling
- Heart failure
These symptoms may develop slowly over time or may progress rapidly depending on the underlying cause of the pulmonary hypertension.
What are the causes of Pulmonary Hypertension?
There are various classifications of Pulmonary Hypertension based on the underlying cause.
Some of the causes of pulmonary hypertension are:
- Breathing disorders like emphysema (COPD), Interstitial lung disease, Obstructive Sleep Apnea; longstanding asthma or bronchiectasis
- Heart problems-like Valvular Heart Disease with leaky valves, Congestive Heart failure
- Blood clots in the lungs
- Liver disorders like Cirrhosis
- Certain drugs can also cause pulmonary hypertension
- Connective Tissue Disorders like Scleroderma, lupus, rheumatoid arthritis infections like Hepatitis C, HIV Blood disorders like sickle cell anemia, some bone marrow disorders
How is Pulmonary Hypertension diagnosed?
After a detailed history and clinical examination. Your doctor will order some tests:
Complete Lung Function Tests as well as Exercise Oximetry, 6-Minute Walk Distance
ECG and Echocardiogram The echocardiogram is especially important as it gives an estimate of the pressure in the pulmonary blood vessels However, this is only an estimate and not an accurate measurement. It is the commonest, readily available. non-invasive test which helps in detecting and also in monitoring Pulmonary Hypertension
Chest X-ray, CT scan of the chest withcontrast/CT pulmonary angiogram where a contrast dye is injected to examine for possible blood clots in the lungs, High Resolution CT scan of the chest
Right Heart Catheterisation -The most important and specific test for the diagnosis of pulmonary hypertension is a Right Heart Cathererisation. This is a specialized procedure where a catheter is introduced into the pulmonary artery via one of the larger veins in the neck or the leg to directly measure the pressure. This is the most accurate way of assessing pulmonary hypertension. Specialists can also determine the responsiveness to certain medications
How is Pulmonary Hypertension managed?
The goal of treatment of pulmonary hypertension is to relieve symptoms, improve ability to tolerate exercise/stress, slow down progression and prolong survival. The main treatment is aimed at the underlying cause whether it is connective tissue disorder or lung disorder etc
Supportive treatment – This consists of Oxygen therapy, use of diuretics to get rid of excess fluid in the body, blood thinners to minimize the chances of development of blood clots in the lungs.
Targeted treatment – This is aimed at directly decreasing the pressure in the pulmonary blood vessels. Calcium channel blockers- this is a class of blood pressure medications which act on the pulmonary blood vessels and help lower the pressure, Phosphodiesterase inhibitors – This is a class of drugs which help to relax the blood vessels and therefore decrease the pressure (similar to Viagra). e.g. Sildenafil, Tadalafil Endothelin Receptor antagonists-Drugs like Bosentan/Ambrisentan also help improve the elasticity of the blood vessels and lower the pressure.
Prostanoids – This is a class of medications which also lower pressure, however they also have significant side effects and need to be very closely monitored at specialty pulmonary hypertension centers. These medications are not easily available in India.
Surgery Surgical removal is used for Chronic Thromboembolic Pulmonary Hypertension (CTEPH). In the more rare cases of Idiopathic or Primary Pulmonary Hypertension, heart-lung transplant is suggested for selected patients.
What lifestyle changes will I have to make?
Diet & Fluid Restriction As the pressure in the pulmonary vessels increases and they lose their elasticity or ability to stretch, it becomes increasingly difficult for the heart and lungs to handle any extra fluid in the body. The excess fluid tends to back up into the lungs making it difficult to breathe. Patients with pulmonary hypertension have to be extremely careful about their salt and fluid intake as this can make all the difference in being able to breathe comfortably.
Your doctor will tell you how much fluid is allowed in a day and it is extremely important to never exceed this amount.
Patients on blood thinners have to avoid green leafy vegetables and certain vegetables (your doctor/dietitian will give you a list).
Medications-Supportive medications include:
- Diuretics/water pills, which make one pass more urine and eliminate excess water. It is important to not miss medication doses.
- Blood Thinner - Blood thinners are prescribed to some patients to prevent formation of blood clots in the lungs. These doses must be taken at the prescribed time daily.
- Regular Checkups - Patients on blood thinners need to go for regular blood tests to make sure that their blood is neither too thin (which can cause bleeding) or too thick (which can allow clot formation).
- Patients will also require certain other blood tests such as liver function tests done periodically, Echocardiogram may also be repeated at intervals to monitor progress on treatment.
- Oxygen Patients with Pulmonary Hypertension benefit from home oxygen therapy. This may be at night or all through the day as per your doctor's advice.
- Travel - Patients should consult their doctors before making travel plans as they may require oxygen on board flights.
- Pregnancy Pregnancy places additional stress on the heart and lungs and requires close monitoring. Patients with pulmonary hypertension must consult their doctors if they want to plan a family.
With the collaborative efforts of patients and the health care providers, patients with pulmonary hypertension can lead more active and
healthy lives.