Pulmonary hypertension is a type of high blood pressure that only affects the arteries in the lungs and the right side of the heart.
Pulmonary hypertension is a type of high blood pressure that only affects the arteries in the lungs and the right side of the heart.
Pulmonary hypertension begins when the pulmonary arteries and pulmonary capillaries are narrowed, blocked, or destroyed. This increases the pressure in the arteries in the lungs as blood flows through the lungs. As pressure builds, the ventricular chambers have to work harder to pump blood through the lungs, eventually, the heart muscle weakens and eventually fails completely.
Pulmonary hypertension is a serious disease that gets progressively worse and sometimes fatal. Although pulmonary hypertension cannot be cured, treatments are available that can help relieve symptoms and improve quality of life.
Signs and symptoms of pulmonary hypertension in the early stages may go unnoticed for months or even years. As the disease progresses, symptoms get worse.
Symptoms of pulmonary hypertension include:
Shortness of breath, initially during exercise and finally during rest.
Dizziness or fainting.
Chest pressure or chest pain.
Swelling (edema) in the ankles, legs, and eventually ascites.
Lips and skin pale green.
Fast pulse or heartbeat.
The heart has two upper and two lower parts. Each time blood flows through the heart, the lower right chambers pump blood to the lungs through a large blood vessel (pulmonary artery). In the lungs, the blood releases carbon dioxide and takes in oxygen. The oxygen-rich blood then flows through the blood vessels in the lungs (pulmonary arteries, capillaries, and veins) to the left side of the heart.
Normally, blood flows easily through the vessels in the lungs, so pulmonary artery pressure is usually much lower. With pulmonary hypertension, the increase in blood pressure is due to changes in the cells lining the pulmonary arteries. These changes cause further patterns, eventually narrowing or completely blocking the blood vessels, making the arteries stiff and narrow. This increases the pressure in the pulmonary arteries as blood circulates.
Primary pulmonary hypertension
When the cause of pulmonary hypertension cannot be found, the problem is called primary pulmonary hypertension (IPH).
Some people with IPH may have a gene, a risk factor for developing pulmonary hypertension. But in most people with idiopathic pulmonary hypertension, there is no recognized cause.
Secondary pulmonary hypertension
Pulmonary hypertension is caused by a medical problem called secondary pulmonary hypertension. This is the more common type of pulmonary hypertension than idiopathic pulmonary hypertension. Causes of secondary pulmonary hypertension include:
Blood clot in the lungs (pulmonary embolism).
Chronic obstructive pulmonary disease, such as emphysema.
Connective tissue disorders, such as scleroderma or lupus.
Sleep apnea and other sleep disorders.
Congenital heart disease.
Sickle cell anemia.
Chronic liver disease (cirrhosis).
Lung diseases, such as pulmonary fibrosis, cause scarring in the tissue between the alveoli (interstitium).
Lives at an altitude of more than 2438 meters.
Climbing or hiking to an altitude of more than 2,438 meters without acclimatization.
Using certain stimulants, such as cocaine.
Although anyone can develop either type of pulmonary hypertension, older adults are more likely to have secondary pulmonary hypertension, and younger people are more likely to have idiopathic pulmonary hypertension. Idiopathic pulmonary hypertension is also more common in women than in men.
One risk factor for pulmonary hypertension is a family history of the disease. Several genes may be linked to idiopathic pulmonary hypertension. These genes can cause an overgrowth of cells in the small arteries of the lungs, making them narrower.
If one of your family members develops idiopathic pulmonary hypertension and tests positive for a gene mutation, which may cause pulmonary hypertension, your doctor or genetic counselor may advise family members to check for mutations.
Pulmonary hypertension can lead to a number of complications, including:
Pulmonary heart disease (cor pulmonale). In chronic heart disease, the right ventricle becomes enlarged and must pump harder than usual to move blood through a narrowed or blocked pulmonary artery. At first, the heart tries to compensate by thickening and widening the right ventricular chamber to increase the amount of blood it can hold. But thickening and dilation are only temporary, and eventually the ventricles fail.
Blood clot. Coagulation helps stop bleeding after an injury. But sometimes blood clots form where they are not needed. Some small clots, or only a handful of large ones, separate from the vein wall and travel to the lungs, leading to pulmonary hypertension that is reversible with time and treatment. Pulmonary hypertension makes it more likely to develop blood clots in small arteries in the lungs, which is dangerous if there is already narrowing or blockage of a blood vessel.
Arrhythmia. Irregular heartbeat (arrhythmia) of atrial or ventricular origin is a complication of pulmonary hypertension. These can lead to dizziness, palpitations, or fainting and can be fatal.
Bleed. Pulmonary hypertension can lead to bleeding into the lungs and coughing up blood. This is a potentially fatal complication.
Tests and diagnostics
Pulmonary hypertension is difficult to diagnose early because it often goes undetected during a routine exam. Even if the disease is more severe, its signs and symptoms are similar to those of other heart and lung problems. Your doctor may do one or more tests to rule out other possible causes of the condition. The first tests to diagnose pulmonary hypertension include:
X-ray. The test can check pulmonary artery pressure if the lungs or right ventricle of the heart are enlarged. Radiographs will be normal in about one-third of people with pulmonary hypertension.
Echocardiography. Your doctor may suspect pulmonary hypertension based on the results of this test. This test is non-invasive, uses harmless sound waves, and allows your doctor to view your heart without surgery. During the procedure, the transducer is placed on the chest. It collects reflected sound waves (echoes) from the heart and transmits them to a machine that uses sound wave patterns to create images of the heart on a screen.
These images show how the heart is working, and the images taken allow the doctor to measure the size and thickness of the heart muscle. Sometimes your doctor will recommend a stress echocardiogram to help determine how hard your heart is working.
Transesophageal echocardiography. If it is difficult to get a clear picture of the heart and lungs with standard echocardiography, your doctor may recommend a transesophageal echocardiogram. During this procedure, a catheter containing an adapter is guided down the throat and into the esophagus, simply using an anesthetic spray at the back of the throat. From here, the transducer can get detailed images of the heart.
Cardiac catheterization. After an echocardiogram, if your doctor thinks you have pulmonary hypertension, a catheter may be inserted. This procedure is often the most reliable way of diagnosing pulmonary hypertension. During the procedure, the cardiologist places a catheter into a vein in the neck or groin. This catheter is then threaded into the right ventricle and pulmonary artery. Cardiac catheterization allows the doctor to directly measure the pressure in the main pulmonary artery and right ventricle. It is also used to see what else you may have about pulmonary hypertension.
Cardiac catheterization is usually done under local anesthesia and analgesia in a hospital, but it is usually possible to go home immediately after the procedure. Will need someone to drive home after the test.
Your doctor may order further tests to check the condition of your lungs and pulmonary arteries, including:
Pulmonary function test. The noninvasive test shows how much air the lungs can hold, and how much air flows in and out of the lungs. During the test, you will blow into an instrument called a spirometer.
Pulmonary angiography. This test uses small amounts of radioactive substances (radioactive isotopes) to study blood flow in the lungs. Radioisotopes are injected into a blood vessel in the arm. Immediately after, a special machine (gamma camera) records images of the blood flow in the pulmonary vessels. A pulmonary angiogram is then used to determine if a blood clot is causing the symptoms of pulmonary hypertension.
Pulmonary angiography is usually performed with ventilation measurement. In this test, a small amount of radioactive gamma is inhaled while the machine records the movement of air into the lungs.
Computed tomography (CT scan). CT scans allow doctors to view body parts in two-dimensional "slices." In this test, lying in a machine the doctor can see a cross-sectional image of the lungs. Medicine may also be given to make the images of the lungs appear clearer.
Magnetic resonance imaging (MRI). This test, which does not use X-rays, is sometimes used to take pictures of the blood vessels in the lungs. The computer creates tissue "slices" from data generated by a strong magnetic field and radio waves. However, an MRI may not measure arterial pressure.
Lung biopsy. In rare situations, your doctor may recommend an open lung biopsy. An open lung biopsy is a type of surgery in which a small sample of tissue is removed from the lung under anesthesia to check for a possible primary cause of pulmonary hypertension. It will be done just to see if certain treatments might be effective, or to allow discontinuation of certain medications.
Genetic testing. If a family member already has pulmonary hypertension, your doctor can see if genes are linked to pulmonary hypertension. If the test is positive, your doctor may recommend that other family members be tested for genetic mutations.
Classification of pulmonary arterial hypertension
Once a diagnosis of pulmonary arterial hypertension has been made, the doctor can classify it using the guidelines of the New York Heart Association.
Grade I. Although pulmonary hypertension has been diagnosed, there are no symptoms.
Degree II. No symptoms at rest, but experience fatigue, shortness of breath, or chest pain with normal activities.
Degree III. Comfortable at rest but with symptoms when physically active.
Grade IV. Symptomatic even at rest.
Treatments and drugs
It often takes some time to find the best treatment for pulmonary hypertension. Treatments are often complex and require extensive follow-up care. Your doctor may also need to change your treatment if it is no longer working. When pulmonary hypertension is caused by another condition, your doctor will treat the underlying cause whenever possible.
Vasodilation. Vasodilators open narrowed blood vessels. One of the most commonly prescribed vasodilators for pulmonary hypertension is epoprostenol (Flolan). The limitation of epoprostenol is that its effects last only a few minutes. This medication is continuously pumped through a vein (IV) through a small pump. This means that you will learn to prepare your own medication mix, operate the pump and care for the IV catheters. Comprehensive follow-up care is required. Potential side effects of epoprostenol include jaw pain, nausea, diarrhea, leg cramps, as well as pain and infection at the IV infusion site.
Another form of the drug, iloprost (Ventavis). Iloprost can be inhaled every three hours through a nebulizer, making it more convenient and less painful to use. And because it's inhaled, it goes directly into the lungs. Side effects associated with iloprost include chest pain - often accompanied by headache and nausea and shortness of breath.
Endothelin receptor antagonist. These drugs reverse the effects of endothelin, a substance in the walls of blood vessels that causes the blood vessels to narrow. One of these drugs, bosentan (Tracleer), may improve symptoms. The drug is not intended for pregnant women. If bosentan is used, liver function should be monitored monthly, as it may cause liver damage.
Sildenafil. Revatio (sildenafil) is sometimes used to treat pulmonary hypertension. It works by opening up the blood vessels in the lungs to allow blood to flow more easily. Side effects include dizziness and vision problems.
Calcium channel blockers. These drugs help relax the wall muscles of the blood vessels. These include drugs such as amlodipine (Norvasc), diltiazem (Cardizem, Tiazac), and nifedipine (Adalat, Procardia). Although calcium channel blockers can be effective, only a small number of people with pulmonary hypertension respond to them.
Ambrisentan. Ambrisentan (Letairis) is a medicine that stops blood vessels from narrowing. This medicine can cause serious liver damage if not appropriate, and it should not be taken by pregnant women. Before taking this medicine, tell your doctor about anything about your liver.
Anticoagulants. Your doctor may prescribe the anticoagulant warfarin (Coumadin) to help prevent the formation of blood clots in the small pulmonary arteries. Because anticoagulants prevent normal blood clotting, it increases the risk of bleeding complications. Take warfarin exactly as prescribed, as warfarin can cause serious side effects if taken incorrectly. If you're taking warfarin, your doctor will ask for periodic blood tests to find out if the medications are working effectively. Many other drugs, herbal supplements, and foods can interact with warfarin, so make sure your doctor knows all of the medications you're taking.
Diuretic. This medicine helps to remove excess water from the body. This reduces the heart's workload. It can also be used to limit the accumulation of fluid in the lungs.
Oxygen. Your doctor may recommend sometimes breathing pure oxygen, a treatment called oxygen therapy, to help treat pulmonary hypertension, especially if you live at a high altitude or have sleep apnea. Some people with pulmonary hypertension eventually require continuous oxygen therapy.
Ball Blade Sepxostomy atrium. If medication does not control pulmonary hypertension, open-heart surgery may be an option. In atrial sepxostomy, the surgeon opens the right chamber of the heart to relieve pressure on the right side of the heart. Atrial sepsis can have serious complications, including an irregular heartbeat (arrhythmia).
Implant. In some cases, a lung transplant may be an option, especially for young people who have idiopathic pulmonary hypertension. The major risks of any type of transplant include organ rejection and serious infection, and lifelong immunosuppressive medication to help reduce the risk of rejection.
Lifestyle and remedies
Although medical treatment cannot cure pulmonary hypertension, it can relieve symptoms. Lifestyle changes can also help, so:
Rest much. Rest can reduce fatigue that can come from pulmonary hypertension.
Still working as possible. Even the most moderate forms of activity can be too tiring for some people with pulmonary hypertension. For others, moderate exercise such as walking may be beneficial, and using oxygen during exercise may be especially helpful. But first, talk to your doctor about specific exercise restrictions. In most cases, it is not recommended to lift more than 50 pounds (22.7 kg). Your doctor can help plan an appropriate exercise program.
No smoking. If you smoke, the single most important thing you can do for your heart and lung health is to quit. If you can't stop smoking on your own, ask your doctor about a treatment plan to help you quit. Also, avoid secondhand smoke if possible.
Avoid using birth control pills or get pregnant. If you are a woman of childbearing age, avoid pregnancy. Pregnancy can be life-threatening for both mother and baby. Also avoid using birth control pills, which can increase the risk of blood clots. Talk to your doctor about alternative forms of birth control.
Avoid traveling or living at high altitudes. High altitude can worsen symptoms of pulmonary hypertension. If you live at an altitude of 8,000 feet (2438 meters) or higher, your doctor may recommend moving to a lower altitude.
Avoid situations that can lower blood pressure. This includes sitting in a hot tub or sauna or taking a long bath or shower. These activities lower blood pressure and cause fainting and even death. Also avoid activities that cause prolonged stress, such as heavy lifting.
Find ways to relieve stress. These can range from yoga, meditation, and biofeedback to a warm bath, music, or a book. Try allowing at least 30 minutes a day for a relaxing activity. Many people with pulmonary hypertension find that simply reducing stress can greatly improve their quality of life.
Follow a nutritious diet and maintain a healthy weight. Your doctor will recommend limiting the amount of salt in your diet to minimize swelling of the body's tissues. Most experts agree that you should eat no more than 1,500 to 2,400 mg of salt per day. Remember that processed foods are often high in salt, so it's important to check labels carefully.