Pulmonary hypertension is a rare condition that affects the lungs. The pulmonary arteries are involved in this condition. If left untreated, the condition can lead to heart failure.
What are Pulmonary Arteries?
The groups of arteries that supply blood to the lungs are called as pulmonary arteries. These arteries are present on the right side of the heart. These major blood vessels carry blood from the right side of the heart towards the lungs. When blood pressure within these arteries is high, it leads to pulmonary hypertension.
What is Pulmonary Hypertension?
The increase in pressure in the pulmonary arteries leads to pulmonary hypertension. The heart is divided into upper and lower chambers and two sides left and right by a muscular wall called the septum. The lower right chamber, called the right ventricle helps in pumping the blood into the pulmonary arteries. These arteries carry the blood to the lungs, where it becomes oxygen rich. In a similar fashion, the left atrium receives the oxygenated blood from the lungs. This blood is pumped in the lower left chamber, the left ventricle.
Now, when the pulmonary artery walls starts tightening, narrowing or form blockage due to presence of blood clots, it makes it very difficult for the heart to pump blood into the lungs.
The difficulty to pump blood causes increase in pressure in the arteries. This leads to development of pulmonary hypertension.
As mentioned above, the blood flows through the arteries on the right side of the heart into the lungs. When certain changes occur within the arteries, it leads to increase in pressure. These changes include narrowing, stiffening or blockage of the pulmonary arteries.
The cause of these changes is mostly idiopathic. This means the exact cause or factor leading to pulmonary hypertension is unknown. In such a case, it is called as idiopathic pulmonary hypertension. It is suspected, there is a gene involved that makes certain people susceptible to pulmonary hypertension. Idiopathic pulmonary hypertension is a rare condition.
When pulmonary hypertension is caused by underlying medical conditions, it is called as secondary pulmonary hypertension. This is the most common type of pulmonary hypertension. The medical conditions that can lead to secondary pulmonary hypertension include:
- Pulmonary emboli
- Congenital heart defects
- Sickle cell anemia
- Hemolytic anemia
- Systolic or diastolic dysfunction
- Valvular disease
- Chronic obstructive pulmonary disease (COPD)
- Interstitial lung disease
- Living in high altitudes for a long time
- Sleep disorders like sleep apnea
- Gaucher disease
- Thyroid disorders
- Chronic renal failure
- Splenectomy, etc.
Signs and symptoms of pulmonary hypertension are not observed during the initial stages of the conditions. The symptoms usually crop up when the condition worsens. Some of the symptoms include:
- Shortness of breath
- Angina pectoris
- Edema around the ankles and feet
- Fainting and syncope
Pulmonary hypertension can lead to different complications. These include:
- Internal bleeding into the lungs. One should immediately seek medical attention the minute they cough or vomit blood as this can be a fatal complication.
- Heart palpitations, dizziness, fainting are signs of arrhythmia. This is another complication of pulmonary hypertension.
- Formation of blood clots in the arteries, leading to increase in pulmonary artery pressure.
- The right ventricle becomes enlarged as it has to work harder to pump blood into the lungs. Soon, the right chamber gives in to the strain and leads to right-sided heart failure.
The increase in pressure makes it harder for the heart to pump blood. It leads to stiffening of the affected arteries leading to fibrosis. This leads to right-sided heart failure (cor pulmonale). Over time, the blood flow to the lung decreases. This leads to decrease in the oxygen carrying capacity of the blood. Thus, one tends to suffer from shortness of breath and fainting spells, due to low supply of oxygen to the body. Under strenuous physical activity, this could lead to heart attack that is often fatal.
It is very difficult to diagnose pulmonary hypertension during the initial stages. Only when the patient starts showing symptoms that points towards heart or lung ailment, does the doctor conduct tests to rule out pulmonary hypertension.
First, a physical examination will help in pointing towards the typical signs of pulmonary hypertension. These include abnormal heart sounds, pulmonary regurgitation, increase in pressure of the jugular veins, peripheral edema, swelling of abdomen, clubbing, etc.
Diagnostic tests to confirm pulmonary hypertension include:
- Blood tests to rule out autoimmune diseases, HIV or liver diseases.
- Arterial blood gas measurements
- Pulmonary function test
- Chest X ray
- CT scan
- Transesophageal echocardiogram
- Right heart catheterization
- Open-lung biopsy
Pulmonary hypertension has no specific cure, but treatments aim to control the symptoms and curb the disease from progressing. Treatment depends on the severity of individual condition. It includes:
- Medications like diuretics, anticoagulants, blood vessel dilators, calcium channel blockers, etc. Drugs like tadafil and sildenafil help in treatment of pulmonary hypertension.
- Surgery is suggested in some cases of severe pulmonary hypertension. Atrial septostomy helps in opening the heart chambers and relieving the pressure on the heart.
One can control the symptoms by making changes in their lifestyle. These changes include
- Avoid exertions and give yourself plenty of rest. This will reduce the fatigue and episodes of shortness of breath
- Quit smoking as it exerts extra pressure on the already exerted heart and lungs.
- Eat healthy and reduce the salt intake in your diet.
- Yoga and meditation will help you stay calm, reduce stress and improve the quality of your life.
- Avoid travelling to places at high altitudes. Those living in such areas should try and move to regions of lower elevation,
Pulmonary hypertension, if left untreated can lead to death within 3 years of diagnosis. However, today with better treatment options life expectancy has dramatically increased. Women of child bearing age with pulmonary hypertension are asked to avoid pregnancy. This is because they have high levels of mortality as well as it affects the life of their fetus. Thus, such women should take the advice of their doctor, before they plan a pregnancy.
Pulmonary hypertension is easy to control today. Medications and other therapies help in slowing down the progress of the disease. Speak to a healthcare provider for more details.
Written by: Saptakee sengupta
Date last updated: February 02, 2015