Pulmonary hypertension is a rare and very serious blood vessel disorder of the lung. Primary pulmonary hypertension (PPH) – which is pulmonary hypertension that isn't a side effect of (or secondary to) another condition such as emphysema or lupus – strikes only between one and two people per million.
Managing this illness and its complications, including right heart failure, requires expertise and access to sophisticated treatment such as oral and continuous intravenous medication, and lung transplantation. Treatments for pulmonary hypertension can be highly complex and require intensive ongoing follow-up, all of which are available through our Pulmonary Hypertension Program.
Our approach to pulmonary hypertension
UCSF has been at the forefront of evaluating and treating pulmonary hypertension (high blood pressure in the lungs) for more than 30 years. We care for patients with either primary pulmonary hypertension, a condition that occurs by itself, or secondary pulmonary hypertension, which is a side effect of another disease and is often misdiagnosed. Our hospital is one of only three medical centers on the West Coast designated a comprehensive center of care by the Pulmonary Hypertension Association.
Managing this rare illness and its complications requires the expertise and sophisticated care that UCSF can provide. Our patients have access to the full range of treatments, including oral, inhaled and catheter-based therapies, as well as experimental approaches being evaluated in clinic trials.
In some cases, the best option may be a lung transplant. UCSF performs 40 to 50 lung transplants each year and leads the nation in higher-than-expected rates of patient survival and transplant function.
Awards & recognition
Among the top hospitals in the nation
No. 8 in the nation for pulmonology & lung surgery
One of the nation’s best in cardiology & heart surgery
Accredited center of comprehensive care (Pulmonary Hypertension Association)
Signs & symptoms
Pulmonary hypertension is classified into five different types:
- Pulmonary arterial hypertension (PAH). This form affects blood vessels in the lungs that carry blood from the heart to the lungs where it picks up oxygen. This category is divided into two types:
- Primary pulmonary hypertension (PPH) is very rare and can occur for no known reason. In about 10 percent of cases, it's inherited. It most often occurs in young adults and is more than twice as common in women as men.
- PAH related to exposure to toxins including diet drugs such as fenphen; street drugs including cocaine and methamphetamine; HIV; collagen vascular diseases including scleroderma, lupus and rheumatoid arthritis; chronic liver disease; and congenital heart diseases.
- Pulmonary venous hypertension (PVH). This form is caused by diseases of the left side of the heart, such as heart failure or mitral valve disease. This can increase pulmonary artery blood pressure but usually doesn't become severe PAH.
- Respiratory system. Pulmonary hypertension can be associated with diseases of the respiratory system including interstitial lung disease, emphysema, asthmatic bronchitis, sleep apnea and chronic exposure to high altitude.
- Chronic blood clots. Blood clots in the lung blood vessels
- Blood vessel disorders. Pulmonary hypertension due to disorders directly affecting the blood vessels in the lungs such as parasites, or inflammation of the blood vessels.
There are no specific signs and symptoms in the early stages of the disease. The symptoms that may occur – such as shortness of breath, fatigue and chest pain – also are common to many other conditions. Other symptoms that may occur include dizziness, swollen ankles and legs, fainting and a bluish cast to lips and skin.
Over time, pulmonary arterial hypertension can damage your heart to the point of danger and result in complications that can interfere with your daily life. Complications from pulmonary hypertension include:
- Enlarged right ventricle
- Blood clots
Pulmonary hypertension in its early stages is difficult to recognize on routine physical exam. It may be two or three years after onset that symptoms become severe enough to be noticed.
Pulmonary hypertension is diagnosed primarily with an echocardiogram, which is an ultrasound examination of the heart. The echocardiogram measures the heart's size and shape by using sound waves to create an image of the heart and can estimate the pulmonary artery pressure.
- Electrocardiogram (ECG or EKG) to measure your heart's electrical function
- Right heart catheterization to measure blood pressure inside the heart and blood vessels of the lungs and measure blood flow. This test is performed in all patients to confirm the diagnosis and to help guide therapy.
- Six-minute walk test
- Assessment of how well you accomplish daily living tasks
If your doctor determines that you have pulmonary hypertension, the next task is to find out what is causing it. The tests to determine the cause include:
- Computerized tomography, called CT or CAT, scan to see if there is a blood clot or other disease in your lungs
- Pulmonary function test to rule out obstructive lung disease
- Sleep study to see if you have sleep apnea, a condition in which you may stop breathing during sleep
- Lab tests to rule out hepatitis, collagen disease, HIV or other conditions
If no discernible cause for the pulmonary hypertension is found, then the diagnosis of primary pulmonary hypertension is made. If a cause is elucidated, then the diagnosis is secondary pulmonary hypertension.
The New York Heart Association and the World Health Organization (WHO) use the following system to classify pulmonary arterial hypertension.
Pulmonary hypertension is divided into four classifications:
- Class 1. You've been diagnosed as having the disease but show no symptoms. Ordinary physical activity doesn't cause undue discomfort.
- Class 2. Your symptoms occur only with activity but not when you're at rest
- Class 3. There are no symptoms at rest but they occur with even slight exertion
- Class 4. You have symptoms even at rest
The treatment for secondary pulmonary hypertension usually begins with treating the underlying cause. For instance, if a blood clot, or pulmonary embolism, is causing the hypertension, the clot may need to be surgically removed.
Several treatments are used to manage primary pulmonary hypertension, although they don't cure the condition. These include:
- Calcium channel blocking drugs. These help the heart pump better by relaxing muscles in the walls of blood vessels.
- Endothelin receptor antagonists (ERAs). These medications reverse the effects of endothelin, a substance in blood vessels that causes constriction.
- Continuously infused epoprostenol. This drug dilates and reverses thickening of the blood vessels of the lungs, and helps prevent platelets from clotting the blood. It has to be administered continuously via an implanted catheter and a portable, battery operated pump. The drug lasts less than six minutes, which is why it must be infused continuously into the bloodstream.
- Anticoagulants. These drugs help keep blood from clotting, which makes it flow better.
- Diuretics. Prescribed to minimize water retention, a condition that makes the heart work harder.
- Digoxin. This drug can help the right side of the heart pump better.
- Lifestyle changes. Adequate rest, a healthy diet, exercise and stress reduction can help control the disease. People with pulmonary hypertension should avoid smoking, pregnancy, birth control pills and high altitudes.
- Supplemental oxygen. This is used for patients with low oxygen level in the blood.
- Lung or heart-lung transplantation. These procedures are employed for patients with severe pulmonary hypertension and extremely poor quality of life. It is the procedure of last resort.
UCSF Health medical specialists have reviewed this information. It is for educational purposes only and is not intended to replace the advice of your doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your provider.