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Cardiac function in sickle cell anemia.

B S Denenberg, G Criner, R Jones

    The American Journal of Cardiology
    |June 1, 1983
    PubMed
    Summary
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    Sickle cell anemia (SCA) patients show depressed left ventricular (LV) muscle function, masked by increased preload and decreased afterload. These compensatory mechanisms maintain normal ejection fraction and cardiac output despite underlying LV dysfunction.

    Area of Science:

    • Cardiology
    • Hematology
    • Physiology

    Background:

    • Congestive heart failure is suspected in sickle cell anemia (SCA), but ejection indexes of left ventricular (LV) pump performance are often normal.
    • Increased preload and decreased afterload in SCA can mask true LV dysfunction by artificially inflating ejection phase indexes.

    Purpose of the Study:

    • To compare the preload and afterload independent end-systolic stress-volume index in patients with SCA and healthy volunteers.
    • To investigate underlying left ventricular (LV) contractile performance in SCA, independent of preload and afterload variations.

    Main Methods:

    • Echocardiographic LV dimensions and end-systolic pressure were measured in 11 SCA patients and 11 controls.
    • Measurements were taken at rest and during interventions including leg raise, hand-grip, and amyl nitrite inhalation.

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  • Systemic vascular resistance (afterload) and end-diastolic volume index (preload) were calculated.

    • Main Results:

    • SCA patients had significantly decreased afterload and increased preload compared to controls.
    • Despite similar ejection fractions, SCA patients exhibited a decreased end-systolic stress-volume index ratio and slope.
    • Cardiac index was elevated in SCA patients.

    Conclusions:

    • Left ventricular (LV) muscle contractile performance is depressed in patients with sickle cell anemia (SCA).
    • Compensatory increases in preload and decreases in afterload maintain normal ejection fraction and cardiac output in SCA.
    • The end-systolic stress-volume index is a valuable tool for detecting subclinical LV dysfunction in SCA.