Management of Pulmonary Hypertension

The human body has two defining sets of blood vessels which distribute blood from the heart to the body. One set pumps blood from the right side of the heart to the lungs. This is the pulmonary circulation. The other set pumps blood from the left side of the heart to the rest of the body via the aorta. This called the systemic circulation. When a health care professional checks your blood pressure on your arm they are checking for your systemic blood pressure.

What is Pulmonary Hypertension?

Pulmonary hypertension is defined as high blood pressure that directly affects the arteries in your lungs and right side of your heart; the pulmonary circulation. The right ventricle in the heart pumps blood returning from the body into pulmonary arteries that flow through the lungs to receive oxygen. The pressure in the pulmonary arteries/veins is considerably lower than pressures in the systemic arteries/veins. This is the case because blood in the systemic circulatory system must be pumped at a much great distance compared to blood in the pulmonary circulatory system. When pressures in the pulmonary circulation become significantly higher, it is referred to as pulmonary hypertension. Management of pulmonary hypertension is important in keeping a healthy cardiovascular system.

Persistent Pulmonary Hypertension

Persistent pulmonary hypertension most commonly occurs when there is elevated pulmonary venous pressures, called pulmonary venous hypertension (PVH.) The high pressure in the veins transmit back to the pulmonary arteries and the right side of the heart. This causes and elevated pressure throughout the pulmonary circulation and without management of the pulmonary hypertension many complications can occur. Persistent pulmonary hypertension can also be the result of stiffening and constrictions of the pulmonary arteries which supply blood to the lungs. This is called pulmonary arterial hypertension (PAH.) Typically, medication can be taken in the management of arterial pulmonary hypertension. Whether a patient has pulmonary arterial or pulmonary venous hypertension, it becomes very difficult for the heart to pump blood forward into the lungs.

Pulmonary Vasoconstriction

Your heart has two upper chambers called atria, and two lower chambers called ventricles. During every cycle of the heart blood passes through the right ventricle into your lungs through a large blood vessel called the pulmonary artery. While the blood circulates throughout your lungs it releases carbon dioxide and picks up fresh oxygen. The oxygen rich blood then flows through the blood vessels in your lungs into the left side of your heart where it can be pumped throughout the rest of your body. The high pressure in patients with pulmonary hypertension causes changes in the cells which line your pulmonary arteries. These changes can cause the cellular walls to become thick, and extra tissues may form. The blood vessels may also become inflamed and tight. These changes in the pulmonary arteries can reduce or completely block blood flow through certain vessels. This makes it much harder for blood to flow through the pulmonary circulation and raises the blood pressure in the pulmonary arteries. Proper management of pulmonary hypertension can help reduce risk of getting blood clots and pulmonary vasoconstriction which slowly deteriorate your pulmonary circulation. Proper management will also help the overall prognosis of your pulmonary hypertension.

As of 2013, pulmonary hypertension can be classified into five groups depending on the underlying cause of the condition.

Group 1 – pulmonary arterial hypertension

• Cause unknown, typically known as idiopathic pulmonary arterial hypertension
• A certain gene mutation which causes pulmonary hypertension in families, called heritable pulmonary arterial hypertension
• Certain drugs, either prescription or illicit drugs (methamphetamines)
• Congenital heart disease (present at birth)
• Connective tissue disorders (e.g. lupus, scleroderma), HIV infection or liver disease (cirrhosis)

Group 2 – pulmonary hypertension caused by left sided heart disease

• Any left sided valvular heart defect including mitral valve disease or aortic valve disease
• Left ventricle failure

Group 3 – Pulmonary hypertension caused by lung disease

• Patients with chronic obstructive pulmonary disease (COPD)
• Lung diseases caused by tissue scarring (interstitium)
• Sleep apnea and other sleep disorders
• Long term exposure to high altitude

Group 4 – pulmonary hypertension caused by chronic blood clots

• Chronic blood clots in the lungs (pulmonary emboli)

Group 5 – Pulmonary vasoconstriction/hypertension

• Caused by conditions which have unclear reason to why the hypertension occurs
• Metabolic disorders
• Tumors pressing against pulmonary arteries or veins
• Blood disorders

Persistent Pulmonary hypertension

Persistent pulmonary hypertension is a common and well documented complication of chronic obstructive pulmonary disease. Management of COPD is important so patients can potentially avoid getting pulmonary hypertension. Pulmonary hypertension usually worsens during sleep, exercise and exacerbation. Pulmonary vasoconstriction in COPD patients and combined effects of hypoxia (inadequate oxygen), inflammation and loss of capillaries in severe emphysema are thought to be the main cause of increase pulmonary artery pressure. This is why pulmonary hypertension is frequently an added complication in COPD patients and why management of this condition is so important.

Your risk factors of developing pulmonary hypertension are greater if you are overweight or have a family history of the disease. You are also at a greater risk if you have various conditions which can increase the risk of developing pulmonary hypertension, including COPD. An unhealthy diet including foods that raise you LDL cholesterol (“bad” cholesterol) count will also put you at a higher risk of getting pulmonary hypertension. It is also extremely important to stay away from illicit drugs especially cocaine and amphetamines as these drugs can be detrimental to your circulatory system. Proper management of your pulmonary hypertension will help your cardiovascular system which and help you live a healthier and fuller life.

Persistent pulmonary hypertension can also lead to a number of complications. This can include:

Right sided heart enlargement and heart failure: your hearts right ventricle becomes thickened and enlarged to pump more blood because of narrowed or blocked pulmonary vasoconstriction. Only works temporarily and can cause heart failure.

Blood clots:patients with pulmonary hypertension are more likely to develop clots which can be dangerous in patients who already experience pulmonary vasoconstriction. These blood clots are typically reversible with treatment and time.

Arrythmia: irregular heartbeats from the upper or lower chambers of the heart are complications of hypertension. Can lead to palpitations, dizziness or fainting and can be fatal.

Diagnosis of Pulmonary Hypertension

The diagnosis of pulmonary hypertension is somewhat hard to diagnose early because it is difficult to detect hypertension or pulmonary vasoconstriction during a regular routine physical exam. Even when the symptoms and signs are more advanced they are very similar to other lung and heart conditions. To diagnose your pulmonary hypertension your health care professional will review medical and family history, discuss symptoms and conduct a physical examination. The main test that your health care professional may use to determine your diagnosis of pulmonary hypertension will typically be an echocardiogram. This is used to create moving images of the beating heart in real time. Your health care professional will check the size and functionality of the right ventricle and thickness of the right ventricular wall in the diagnosis of your pulmonary hypertension. Other tests that are used in the diagnosis of pulmonary hypertension include chest x-ray, electrocardiogram (ECG), right heart catheterization and blood tests. Your health care professional may also order additional tests to check the condition of your pulmonary arteries and lungs to determine cause(s) of your condition. These tests can include CT scan, MRI scan, pulmonary function test, polysomnogram and ventilation/perfusion scan. Proper tests and management of your pulmonary hypertension is vital so that health care professionals know the underlying cause and can treat it.

Prognosis of Pulmonary Hypertension

Typically the prognosis of pulmonary hypertension differs depending on the person. Prognosis of pulmonary hypertension can have a great outlook or poor outlook completely depending on the underlying cause of the disease. Regular check up at the doctor will help the prognosis of pulmonary hypertension as patients who are treated early are more likely to survive and get better. Typically 1 year survival rate is around 90% and 5 year survival rate is around 60%. Management of pulmonary hypertension is important so you can get proper treatment and medication. This will aid in your overall health and help in having a better prognosis of your pulmonary hypertension.