Standard measures of right ventricular function assessment in adult patients with acute sickle cell crises

Abstract

Chronic sickle cell (SC) disease is known to cause pulmonary hypertension (PH) which eventually affects right as well as left ventricular function. However, the acute effects of SC crisis on right ventricular (RV) function in adults have not been well described. Our echocardiography database was queried for patients followed in the outpatient hematology clinic, who were admitted with SC crises, and had an inpatient echocardiogram. Comparisons of RV fractional area change (RVFAC) and tricuspid annular peak systolic excursion (TAPSE) were then made to a group of healthy patients and a group with mild PH. TAPSE was normal in patients with SC disease (3.05 ± 0.56). However, the SC patients had significantly larger RV's compared to controls and mild PH patients (31.33 ± 7.23 cm 2 vs. 19.07 ± 4.49 cm 2 and 21.51 ± 6.07 cm 2, respectively, P < 0.0001). RVFAC was reduced in SC patients, and was comparable to patients with mild PH (46 ± 9% vs. 49 ± 14%, P = 0.4). We found no correlation between hemoglobin level (8.1 ± 2.5 g) and RVFAC, TAPSE, or PH severity. We found that adult patients admitted with SC crises have frequent dilatation of the RV with an abnormal reduction in RVFAC, despite normal TAPSE values. Therefore, caution should be exercised in using TAPSE to estimate RV function in this patient population.