Quantitative assessment of ventricular function in sickle cell disease: effect of long-term erythrocytapheresis.
BACKGROUND
Previous studies on cardiac function in patients with sickle cell disease (SCD) demonstrated abnormalities of systolic and diastolic function including elevated left ventricular myocardial performance index (LVMPI) on chronic transfusion protocols. LVMPI has been validated as a useful and easy non-invasive measure of overall cardiac function. Up to now, there are no reported studies on cardiac function in patients with SCD maintained on long-term erythrocytapheresis (LTE).


METHODS
We recorded LVMPI in 22 patients with SCD aged 3-20 years and we compared the results between non-transfused patients (NT-SCD) and patients on LTE (T-SCD).


RESULTS
Males with SCD had higher mean LVMPI than females (P = 0.04). There were significant differences among T-SCD, severe NT-SCD, and mild NT-SCD patients with respect to hemoglobin (Hb) levels (P = .003) and TR velocity (P = .03). T-SCD patients showed elevated LVMPI compared to NT-SCD patients with severe and mild disease (P = 0.002). Pair-wise comparisons demonstrated that T-SCD patients had LVMPI that was significantly higher than NT-SCD (mild) patients (P = 0.01).


CONCLUSIONS
Our study demonstrates that patients SCD on LTE have cardiac dysfunction based on elevated LVMPI. This may be a reflection of the global severity of disease. Our findings merit further investigation with serial monitoring of LVMPI on a larger number of patients with SCD.
Raj
Ashok B
AB
Department of Pediatrics, Division of Pediatric Hematology/Oncology, University of Louisville, Louisville, Kentucky 40202, USA. a0raj001@louisville.edu
Condurache
Tania
T
Bertolone
Salvatore
S
Williams
David
D
Lorenz
Douglas
D
Sobczyk
Walter
W
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000000981:diagnostic imaging / Q000628:therapy*; D002312:Cardiomyopathy, Hypertrophic / Q000209:etiology; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D016238:Cytapheresis; D015150:Echocardiography, Doppler / Q000379:methods; D004562:Electrocardiography; D017707:Erythrocyte Transfusion / Q000009:adverse effects; D005260:Female; D006337:Heart Murmurs; D006801:Humans; D008297:Male; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging / Q000209:etiology*