How is cardiovascular involvement assessed in scleroderma?

Updated: Sep 09, 2019
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Elevated CXCL4 serum levels correlate with the severity of pulmonary fibrosis and progression of pulmonary hypertension. [104] Elevated serum levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) may correlate with early pulmonary hypertension. [105, 106, 107]

Hughes et al propose using cardiac troponins as a screening tool to detect asymptomatic cardiac involvement, with measurement of cardiac troponin T followed by confirmatory cardiac troponin I. Cardiac involvement is unlikely if levels of both troponins are normal and is probable if levels of both are high. Low-titer troponin T levels may reflect skeletal muscle involvement, but cardiac assessment is nevertheless warranted. [108]

Electrocardiograms (ECGs) should be performed routinely to identify arrhythmias and conduction defects. ECGs can identify early changes of right ventricular strain caused by pulmonary hypertension, and in advanced states, right atrial hypertrophy. Perform 24-hour ambulatory Holter monitoring to evaluate arrhythmias and serious conduction defects.

Transthoracic echocardiography is a noninvasive study for assessing pulmonary artery pressure. Conduct this test to evaluate the patient's pulmonary artery pressure at initial evaluation and during serial follow ups and to assess septal fibrosis or pericardial effusions.

Right-heart catheterization is the standard criterion and only definitive test for diagnosing pulmonary hypertension. It is usually performed after an elevated pulmonary artery pressure is found on echocardiographic screening. [109, 110]

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