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Medical Author:

George Schiffman, MD, FCCP

George Schiffman, MD, FCCP

Dr. Schiffman received his B.S. degree with High Honors in biology from Hobart College in 1976. He then moved to Chicago where he studied biochemistry at the University of Illinois, Chicago Circle. He attended Rush Medical College where he received his M.D. degree in 1982 and was elected to the Alpha Omega Alpha Medical Honor Society. He completed his Internal Medicine internship and residency at the University of California, Irvine.

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Medical Author:

Siamak T. Nabili, MD, MPH

Siamak T. Nabili, MD, MPH

Dr. Nabili received his undergraduate degree from the University of California, San Diego (UCSD), majoring in chemistry and biochemistry. He then completed his graduate degree at the University of California, Los Angeles (UCLA). His graduate training included a specialized fellowship in public health where his research focused on environmental health and health-care delivery and management.

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Medical Editor:

William C. Shiel Jr., MD, FACP, FACR

William C. Shiel Jr., MD, FACP, FACR

Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

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In this Article

• What are pulmonary arteries?
• What is pulmonary hypertension?
• What are primary and secondary pulmonary hypertension?
• What causes pulmonary hypertension?
• What causes primary pulmonary hypertension?
• How common is pulmonary hypertension?
• What are the signs and symptoms of pulmonary hypertension?
• How is pulmonary hypertension diagnosed?
• What is the treatment for pulmonary hypertension?
• What is the life expectancy for pulmonary hypertension?
• Patient Comments: Pulmonary Hypertension - Share Your Experience
• Patient Comments: Pulmonary Hypertension - Treatments
• Patient Comments: Pulmonary Hypertension - Symptoms
• Find a local Cardiologist in your town
• Pulmonary Hypertension Index

The first step in diagnosis of pulmonary hypertension is to clinically suspect it. This may be done as part of an evaluation of another disease that can lead to pulmonary hypertension (such as scleroderma or chronic obstructive pulmonary disease), or based on patients and signs and symptoms as described above.

Many tests, such as echocardiogram, may be performed that may give clues to the possibility of pulmonary hypertension. But the gold standard (the best test available) is right heart catheterization. This test entails inserting a catheter through the groin into the femoral vein, a large vein in the lower body (or under the collar bone or in the upper arm into the subclavian vein, a large vein in the upper body) and advancing it to the right side of the heart. The catheter is connected to a device that can monitor and measure blood pressure in the right side of the heart and pulmonary arteries.

During right heart catheterization, response to certain medication to treat pulmonary hypertension can be assessed. This is done by administering medications for pulmonary hypertension while the patient still has the catheter placed in heart. Then pulmonary blood pressure is monitored and the response to treatment is compared to no treatment. This can give the physicians a clue as to if an individual is a candidate for a certain therapy and also what dosage of the medicine may be appropriate.

Pulmonary hypertension is defined as the mean pulmonary artery blood pressure greater than 25 millimeter of mercury (mmHg) measured by right heart catheterization. The pressures can be much higher than 25 mmHg in some people. Therefore, the pulmonary hypertension can be labeled as mild, moderate, or severe based on the pressures. Mean arterial pressure is two-thirds of the difference between systolic and diastolic blood pressure (systolic is the upper number and diastolic is the lower number in measuring blood pressure). Normally, the pulmonary blood pressure is much lower pressure system than the systemic blood pressure (which is usually measured with a blood pressure cuff).

What tests other than right heart catheterization may be used in diagnosing pulmonary hypertension?

Other tests available for diagnosing pulmonary hypertension include electrocardiogram (ECG), chest X-ray, and echocardiogram. An ECG may show some abnormalities that may be suggestive of right heart failure. Chest x-ray may also show enlargement of the chambers of the right heart. And echocardiogram (ultrasound of the heart) shows ultrasound images of the heart and can detect evidence of right heart failure and pressures in the pulmonary artery can be estimated. These tests, in the right clinical setting, are very useful in diagnosing pulmonary hypertension.

Other tests may be useful in evaluating the conditions leading to secondary pulmonary hypertension. For example, a ventilation-perfusion scan (V/Q scan) can suggest blood clots in the pulmonary arteries or sometimes a CAT scan of the chest can be used. The chest CAT scan can detect pulmonary arterial clots, but also can show abnormalities of the lung tissue and surrounding structures that can contribute to pulmonary hypertension. A pulmonary function testing can be useful in diagnosing chronic obstructive pulmonary disease (COPD). This test can be used to detect many aspects of lung function including airflow and evidence of obstruction, lung volumes, and the capacity for the lung to extract oxygen from the air.