What testing is recommended for a patient with elevated right ventricular systolic pressure?

From the Guidelines

For a patient with elevated right ventricular systolic pressure (RVSP), a comprehensive cardiac evaluation is recommended, starting with a transthoracic Doppler echocardiogram to confirm the elevation and assess right ventricular function, size, and wall thickness, as this is the most sensitive and specific non-invasive test for detecting moderate pulmonary hypertension 1.

Initial Evaluation

The initial evaluation should include:

• Transthoracic Doppler echocardiography to detect PH, with a sensitivity of 79% to 100% and specificity of 68% to 98% in detecting moderate PH 1
• Electrocardiogram (ECG) to detect right ventricular hypertrophy or strain patterns
• Chest X-ray to evaluate for cardiomegaly or pulmonary abnormalities
• Pulmonary function tests to rule out underlying lung disease

Further Testing

Further testing should include:

• Right heart catheterization, which is the gold standard for precise measurement of pulmonary pressures and calculation of pulmonary vascular resistance, and is necessary for confirmation of PH in patients with intermediate or high echocardiographic probability of PH before treatment initiation 1
• CT pulmonary angiography to exclude pulmonary embolism
• Ventilation-perfusion (V/Q) scan to detect chronic thromboembolic disease
• Laboratory tests including BNP/NT-proBNP, complete blood count, comprehensive metabolic panel, thyroid function tests, and autoimmune markers to help identify underlying causes
• Sleep studies may be warranted to evaluate for sleep-disordered breathing

Special Considerations

Patients at high risk for development of PAH, such as those with a known genetic mutation associated with PAH or a first-degree relative with idiopathic PAH, may benefit from screening using transthoracic Doppler echocardiography 1. The comprehensive approach is necessary because elevated RVSP indicates pulmonary hypertension, which can result from various cardiac, pulmonary, or systemic conditions, and proper diagnosis is essential for appropriate management.

From the FDA Drug Label

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From the Research

Testing for Elevated Right Ventricular Systolic Pressure

If a patient has elevated right ventricular systolic pressure (RVSP), several tests can be recommended to assess the presence and severity of pulmonary hypertension. The following are some of the key testing options:

• Echocardiography: This is a non-invasive test that uses sound waves to produce images of the heart and its blood vessels. It can be used to estimate RVSP and assess the presence of tricuspid regurgitation 2.
• Doppler ultrasound: This test uses sound waves to measure the velocity of blood flow through the tricuspid valve and estimate the systolic pressure gradient between the right ventricle and right atrium 2.
• Stress echocardiography: This test uses ultrasound imaging to visualize the heart during physical activity or pharmacological stress. It can be used to assess the safety of contrast agent use in patients with elevated RVSP 3.
• Right heart catheterization: This is an invasive test that involves inserting a catheter into the right side of the heart to directly measure pulmonary artery pressure and RVSP.

Factors to Consider When Interpreting Test Results

When interpreting test results, several factors should be considered, including:

• The severity of RVSP elevation: Patients with severely elevated RVSP (≥ 60 mm Hg) are at higher risk of adverse outcomes, such as hospitalization and mortality 4.
• The presence of tricuspid regurgitation: Tricuspid regurgitation can be used to estimate RVSP non-invasively using Doppler ultrasound 2.
• The impact of RVSP on mortality: Elevated RVSP has been shown to be a predictive factor of mortality in patients with scleroderma 5 and heart failure 4.
• The safety of contrast agent use: Contrast agents can be safely used during stress echocardiography in patients with elevated RVSP, without increasing the risk of adverse outcomes 3.

Additional Considerations

Additional considerations when testing for elevated RVSP include:

• The importance of serial measurements: Serial measurements of RVSP can help identify patients at increased risk of adverse outcomes 4.
• The role of calcium channel-blocking therapy: Calcium channel-blocking therapy may be effective in reducing pulmonary arterial pressure and regressing right ventricular hypertrophy in patients with primary pulmonary hypertension 6.