A noninvasive comparison of left ventricular performance in sickle cell anemia and chronic aortic regurgitation

Abnormalities of the cardiovascular system are common in patients with sickle cell anemia (SS). Noninvasive testing to document left ventricular dysfunction has yielded conflicting results. Left ventricular performance was evaluated in 27 patients with SS by M-mode and 2-D echocardiography, and systolic time intervals. Comparisons were made to 25 normal controls, and to 22 patients with chronic aortic regurgitation. Left ventricular diastolic diameter (LVDD) and cardiac index (CI) were significantly greater in the patients with SS than in controls (LVDD 5.3 +/- 0.4 vs. 4.7 +/- 0.5 cm; CI 4.2 +/- 1.3 vs. 3.1 +/- 0.8 liters/min/m2; both p less than 0.001). Left ventricular ejection fraction (EF) was slightly, but significantly less (62.9 +/- 7.3 vs. 67.0 +/- 5.4; p less than 0.05). In comparison to the patients with chronic aortic regurgitation, the LVDD in the patients with SS was slightly, but significantly lower (LVDD 5.3 +/- 0.4 vs. 5.9 +/- 0.6 cm; p less than 0.05). There was no significant difference between the patients in EF or CI (EF 62.9 +/- 7.3 vs. 63.3 +/- 4.4; CI 4.2 +/- 1.3 vs. 5.0 +/- 1.0 liters/min/m2; NS). Left ventricular EF was below 55 in three patients who also had hypertension at the time of examination. We conclude that patients with SS have resting left ventricular performance consistent with a high output state. Significant left ventricular dysfunction related to sickle cell disease alone was not demonstrated in this population, although the addition of hypertension appears to deleteriously affect resting left ventricular performance.