Pulmonary Hypertension

  • Medical Author:
    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.

  • Medical Editor: William C. Shiel Jr., MD, FACP, FACR
    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.

How is pulmonary hypertension diagnosed?

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.

Echocardiogram, Doppler, heart catheterization

  • Many tests, such as echocardiogram, may be performed that may give clues to the possibility of pulmonary hypertension. It is important that a Doppler study be performed with the echocardiogram, which enables the doctor to approximate the pulmonary artery pressures. These values are calculated based on the sound quality of the wave approaching and leaving the echocardiogram machine sensor/probe. This is based on the principle that explains why the sound of an approaching and then passing train varies.
  • 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 or neck into 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, oxygen levels are measured during various positions of the catheter in the pulmonary circulation. This can help determine if a congenital or acquired hole has formed in the heart contributing to the elevated pulmonary pressures. Certain medications or activities can be administered or performed during the procedure to help with the assessment. Nitric oxide can be inhaled and in certain forms of PAH a dramatic improvement in pressures can occur suggesting specific treatment options such as the use of calcium channel blocker medication. Some patients have an exaggerated pulmonary pressure response to exercise and this too can be measured by utilizing arm exercise during the procedure. Titration of medications for pulmonary hypertension while the he catheter is in place can help monitor the response to treatment and assist in optimizing therapy. This can assist in providing individualized therapy for patients with this complex disease.

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 calculated by multiplying the diagnostic pressure two times, and adding the systolic pressure to that number, and then divide by three (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 may be used in diagnosing pulmonary hypertension?

Other tests available for diagnosing pulmonary hypertension include electrocardiogram (ECG, EKG), 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 with the use of Doppler (as described previously) can estimate pressures in the pulmonary artery. These tests, in the right clinical setting, are very useful in diagnosing and managing 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 CT scan of the chest can be used. The chest CT scan can detect pulmonary arterial clots, but also can show abnormalities of the lung tissue and surrounding structures that can contribute to pulmonary hypertension.

Pulmonary function testing can be useful in diagnosing chronic obstructive pulmonary disease (COPD), and other lung diseases, and monitor disease progression. 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.

Sleep studies can confirm the diagnosis of sleep apnea and its severity and management options such as CPAP therapy.

Medically Reviewed by a Doctor on 3/30/2016

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