"Pulmonary Hypertension which has unknown causes affects approximately two to three times as many women as it does men, and commonly appears in persons between the ages of twenty and sixty."
Defining Pulmonary Hypertension
Pulmonary Hypertension involves an increase in pressure within the pulmonary arteries, which are responsible for the flow of blood from a person's heart to their lungs in order to deliver oxygen. Pulmonary Hypertension can present symptoms including chest pain, shortness of breath during daily activities, a racing heartbeat, and tiredness. A person with Pulmonary Hypertension may face serious restrictions to their daily activities as the disease worsens.
The right ventricle of the heart; or lower-right chamber of the heart, pumps blood to the pulmonary arteries, and from there the blood moves to the lungs, picks up oxygen, and is pumped throughout the body. There are three changes which may affect pulmonary arteries, resulting in Pulmonary Hypertension. For example; blood clots may form in a person's arteries, or the walls of their arteries may tighten. At the time a person is born, their arteries may be stiff, or they may become stiff due to an overgrowth of cells.
The changes described can make it difficult for the heart to pump blood through the arteries to the lungs, and cause pressure in the arteries to rise. The result is that the right ventricle of the heart becomes weaker and more strained, and the heart itself works harder. Eventually, the heart can become weak enough that it cannot push sufficient quantities of blood to the lungs, and cause heart failure; one of the most common causes of death among persons with Pulmonary Hypertension.
There are five groups of Pulmonary Hypertension; depending on its cause. For every group of Pulmonary Hypertension, the accepted average pressure of the pulmonary artery when a person is resting is considered to be higher than twenty-five mmHg. When a person is physically active, this average pressure is considered to be thirty mmHg. For persons without Pulmonary Hypertension, resting pressure in the pulmonary artery is considered to be average at fifteen mmHg.
Groups of Pulmonary Hypertension
There are five groups of Pulmonary Hypertension, according to the World Health Organization (WHO). Organization of the groups is based on the cause of the disease.
Group One: Group One Pulmonary Hypertension may be inherited. There is a form that has an unknown cause, and another that may be caused by a variety of things such as an HIV infection, a congenital heart disease present at birth, a person's use of some specific diet medications or illegal drugs, or a Thyroid disease.
Group Two: Group Two Hypertension involves Pulmonary Hypertension accompanied with conditions affecting the left-side of the person's heart. These conditions include long-standing high-blood pressure, or Mitral Valve disease which may contribute to heart disease.
Group Three: Group Three Hypertension involves Pulmonary Hypertension accompanied with lung conditions. These lung conditions include Interstitial Lung disease, Sleep Apnea, and Chronic Obstructive Pulmonary Disease (COPD). Interstitial Lung diseases may cause scarring in a person's lung tissues.
Group Four: Group Four Hypertension involves Pulmonary Hypertension accompanied with Sickle Cell Anemia, blood clotting disorders, or blood clots in a person's lungs.
Group Five: Group Five Hypertension involves Pulmonary Hypertension accompanied with a variety of other conditions or diseases. These conditions or diseases may include Histiocytosis, Lymphangioleimyomatosis (LAM), Sarcoidosis, or the presence of a tumor which is pressing on a pulmonary blood vessel.
Doctors may refer to Group One Pulmonary Hypertension as, 'Idiopathic Hypertension,' or,'Primary Hypertension.' Groups two through five may be referred to by medical professionals as, 'Secondary Pulmonary Hypertension.'
Persons at Risk for Pulmonary Hypertension
Everyone has the potential to develop Pulmonary Hypertension, and the number of persons with the disease is unknown. Pulmonary Hypertension which has unknown causes affects approximately two to three times as many women as it does men, and commonly appears in persons between the ages of twenty and sixty; although it may occur at any age. Group One Pulmonary Hypertension, or, 'Idiopathic Hypertension,' which has no know cause is rare; there is usually another condition or disease associated with it.
There are people who are more at risk of developing Pulmonary Hypertension than others. These people include those who have heart and lung diseases, an HIV infection, or liver disease. People who have a family history of Pulmonary Hypertension are a greater risk of developing it. There are some diet medications which may contribute to the development of Pulmonary Hypertension. Persons who use Cocaine or other forms of illegal drugs are at great risk of developing Pulmonary Hypertension.
Symptoms and Signs of Pulmonary Hypertension
There are some different symptoms, or signs of potential Pulmonary Hypertension. For example; if you experience a shortness of breath while you are doing everyday activities such as climbing stairs, you may have Pulmonary Hypertension. Chest pains and a racing heartbeat are other signs of Pulmonary Hypertension, as well as tiredness.
Worsening Pulmonary Hypertension may lead to increased difficulty in performing any physical activity. Signs of worsening Pulmonary Hypertension include light-headiness or even fainting, notably during physical activity. There may be swelling present in the ankles or legs, or a blue tint to the skin or lips.
Diagnosing Pulmonary Hypertension
A doctor diagnoses Pulmonary Hypertension based on a person's family and medical history, a physical examination, as well as test results and procedures. There are no early warning symptoms of Pulmonary Hypertension, and people often have the disease for years without knowing it; Pulmonary Hypertension can progress slowly. Diagnosis of Pulmonary Hypertension is made more difficult because when symptoms do develop they often resemble other lung and heart conditions; Asthma for example.
Pulmonary Hypertension Tests and Procedures
A doctor may order procedures and testing in order to confirm a diagnosis of Pulmonary Hypertension, as well as to define any underlying causes of it. They will use any results they find to determine the severity of the Pulmonary Hypertension the person is experiencing. There are several procedures and tests a doctor may order.
Echocardiography: Echocardiography involves the use of sound waves in order to create a moving picture of the person's heart. The image can illustrate the thickness and size of the person's right ventricle, how well it is working, and provide an estimate of the pressure in their pulmonary arteries.
Chest X-ray: Chest X-ray images of the person's lungs and heart may show if their heart's right ventricle or pulmonary arteries have become enlarged. The images may also show any signs of developing lung diseases that can contribute to Pulmonary Hypertension.
Electrocardiogram (EKG): The EKG test shows the rate at which a person's heart beats, and whether their heartbeat is consistent or irregular. The test might also show if the right ventricle of the heart has become strained, or enlarged.
Right Heart Catheterization: Right Heart Catheterization involves the insertion of a thin and flexible tube called a, 'Catheter,' into a blood vessel located in either the person's neck or groin, which is then moved into the pulmonary artery. Through this catheter, a doctor can perform diagnostic testing which will enable them to determine how well blood is being pumped throughout the person's body, as well as the pressure in their pulmonary arteries. A doctor can also determine if there is any leakage between the left and right sides of the person's heart during this procedure.
Lung Function Tests: Lung Function Testing involves measurement of the capacity of a person's lungs, the pace at which they can exhale air, and the concentration of oxygen being delivered to the blood through the lungs. The tests may also help to find any lung diseases which may be present and causing Pulmonary Hypertension.
Overnight Oximetry: Overnight Oximetry involves the measurement of a person's oxygen level in their bloodstream overnight. Many people with Pulmonary Hypertension have a low oxygen level during sleep, causing the condition to worsen. A small device called a, 'Pulse-Ox,' is used during this test, which may be done either at home or in a sleep lab. The device has a small light, and can be attached to the person's fingertip, toe, or to their earlobe.
Lung Ventilation/Perfusion Scan (VQ): A VQ test involves measurement of the blood and air flows in a person's lungs, and can show if there is any blood clotting in the blood vessels in their lungs.
Blood Testing: Blood Testing is used by health care professionals in order to rule out other diseases such as Liver disease, autoimmune diseases such as Rheumatoid Arthritis, or HIV infection.
Exercise Testing: Exercise Testing involves either a form of cardiopulmonary exercise test or a six-minute walk test. The tests can assist in determining whether or not another disease is responsible for causing the person's symptoms. A cardiopulmonary test may involve either exercising on a treadmill, or riding a stationary bicycle. A six-minute walk test involves measuring the distance a person is able to quickly walk in a six-minute period of time.
The purpose of exercise testing is to determine the severity of the Pulmonary Hypertension a person is experiencing. A doctor rates the person's activity level during the testing process, and links their performance to a rating system which ranges from Class One to Class Four.
A doctor may repeat exercise testing for persons with Pulmonary Hypertension in order to measure the progression of the disease, as well as to determine how treatment is working. They will compare previous activity levels with current ones in order to guide their treatment of the individual.
Treatment of Pulmonary Hypertension
There is currently no cure for Pulmonary Hypertension; however, there are forms of treatment which can help to relieve the symptoms of it, as well as to slow the progression of the disease. Pulmonary Hypertension can be treated through therapies, medications, and procedures. The treatment of the disease depends on the type of Pulmonary Hypertension the person has, and its severity.
Group One Treatment
Treatment for Group One Pulmonary Hypertension involves medications and medical procedures. The medications prescribed for this group reduce excess cell growth in the person's blood vessels and relax the blood vessels in their lungs. Medications such as Prostanoids, Endothelin Receptor Antagonists, Phosphodiesterase-5 Inhibitors, and calcium blockers are used.
A doctor will probably have a, 'Vasoreactivity test,' done in order to determine which medication works best for the person. The test demonstrates how pressure in the pulmonary arteries reacts to medications, and is performed through right-heart catheterization. A doctor may also perform one or more other procedures for persons in Group One.
Arterial Septostomy: Arterial Septostomy involves insertion of a catheter into a blood vessel in the person's leg, which is then positioned into their heart. The catheter is then placed through the wall which separates the left and right upper chambers of the person's heart, called the, 'Septum.' A doctor inflates a tiny balloon on the tip of the catheter, creating an opening between the chambers of the heart and relieving pressure, while increasing the flow of blood. This procedure is rarely done in America.
Lung Transplant: A Lung Transplant is a surgical procedure which replaces a person's diseased lung with a healthy one from a donor. Persons with severe lung disease causing Pulmonary Hypertension may receive this procedure.
Heart-Lung Transplant: A Heart-Lung Transplant is a surgical procedure where the person's heart and lungs are replaced with those from a donor or donors.
Group Two Treatment
Conditions affecting persons with Pulmonary Hypertension in Group two include symptoms affecting the left-side of their heart such as Mitral Valve disease. Treatments involve the use of medications, surgery, and changes in the person's lifestyle.
Group Three Treatment
Conditions affecting persons with Pulmonary Hypertension in Group Three include Interstitial Lung disease, Sleep Apnea, and COPD. Treatments for Group Three may include Oxygen Therapy to raise levels of oxygen in the person's blood. Oxygen Therapy is applied through soft, plastic prongs which fit in the person's nose and plastic tubing attached to an oxygen tank, and can be received either in a hospital, or at home.
Group Four Treatment
Conditions affecting persons with Pulmonary Hypertension in Group Four include blood clotting disorders, or blood clots in the lungs. Some persons in Group Four have Sickle Cell Anemia. Treatment for persons in Group Four can involve prescription blood thinners which prevent clots from either growing larger or forming in the first place. Surgical procedures may be used to remove scarring that may have occurred in a person's pulmonary arteries, caused by blood clots.
Group Five Treatment
Conditions affecting persons with Pulmonary Hypertension in Group Five include Histiocytosis, Lymphangioleimyomatosis (LAM), and Sarcoidosis. There may also be a tumor present in the person which is pressing on a pulmonary vessel. Treatment for persons in Group Five depends on the cause of the Hypertension.
Treatment Related to All Types of Pulmonary Hypertension
There are a number of treatments which can be used for every type of Pulmonary Hypertension. These treatments include:
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