What is the role of ECG in the management of sickle cell disease (SCD)?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Echocardiography is used to identify patients with pulmonary hypertension based on tricuspid regurgitant jet velocity. Patients with sickle cell disease may have an array of abnormalities of systolic and diastolic function. Adults should be tested for evidence of pulmonary hypertension with Doppler echocardiography. Cardiac echocardiography should be performed for patients with dyspnea.

Left ventricular diastolic dysfunction (LVDD) is commonly reported in SCD patients and is linked to premature death. Echocardiography is the most widely used method to evaluate LV diastolic function, but the majority of patients with SCD-associated cardiomyopathy have high-output left heart failure, and the evaluation of diastolic function is more challenging in this setting. [53]

The diagnosis of high-output heart failure, especially when it is at an early stage, could be missed by echocardiography and/or right heart catheterization performed with the patient at rest. However, early-stage high-output heart failure can be identified on invasive low-level exercise testing that shows exercise-induced elevation of LV filling pressure despite a normal resting value. Consequently, Hammoudi et al recommend considering low-level invasive exercise testing in SCD patients with inconclusive measurements at rest when there is clinical suspicion of heart failure (specifically, symptoms with exercise and no history of congestion, or use of diuretics). [53]

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