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). PROCEDURES: 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. 2005 Wiley-Liss, Inc.
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). PROCEDURES: 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-SCDpatients with respect to hemoglobin (Hb) levels (P = .003) and TR velocity (P = .03). T-SCDpatients showed elevated LVMPI compared to NT-SCDpatients with severe and mild disease (P = 0.002). Pair-wise comparisons demonstrated that T-SCDpatients had LVMPI that was significantly higher than NT-SCD (mild) patients (P = 0.01). CONCLUSIONS: Our study demonstrates that patientsSCD 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. 2005 Wiley-Liss, Inc.
Authors: Jane S Hankins; Mary Beth McCarville; Claudia M Hillenbrand; Ralf B Loeffler; Russell E Ware; Ruitian Song; Matthew P Smeltzer; Vijaya Joshi Journal: Pediatr Blood Cancer Date: 2010-09 Impact factor: 3.167
Authors: Michael V Di Maria; Hao H Hsu; Ghassan Al-Naami; Jeanine Gruenwald; K Scott Kirby; Fenella J Kirkham; Sharon E Cox; Adel K Younoszai Journal: J Am Soc Echocardiogr Date: 2014-12-30 Impact factor: 5.251