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Left ventricular rotational mechanics in Tanzanian children with sickle cell disease
Michael V Di Maria1, Hao H Hsu2, Ghassan Al-Naami3
1University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, Colorado.
Insights
Children with sickle cell disease (SCD) show altered left ventricular (LV) rotation, indicating early cardiac changes. These findings highlight subclinical systolic and diastolic dysfunction in pediatric SCD patients.
Area of Science:
- Cardiology
- Pediatric Hematology
Background:
- Sickle cell disease (SCD) is a common inherited disorder.
- Adults with SCD experience cardiac dysfunction, but the onset in children is unclear.
- Left ventricular (LV) rotational mechanics have not been studied in pediatric SCD.
Purpose of the Study:
- To investigate differences in LV rotational mechanics between children with SCD and age-matched controls.
- To identify early signs of cardiac dysfunction in pediatric SCD.
Main Methods:
- Prospective acquisition of LV short-axis images in 213 children with SCD and 49 controls.
- Analysis of basal and apical rotation, net twist, torsion, and untwist rate using 2D speckle-tracking.
- Timing of events normalized to aortic valve closure.
Main Results:
- Significantly lower mean basal rotation in SCD patients (P = .012).
- Slower rotation rates at apex (P = .001) and base (P = .0004) in SCD patients.
- Significantly slower mean peak untwisting rate in SCD patients (P = .006).
Conclusions:
- Children with SCD exhibit altered LV rotational mechanics, including reduced basal rotation and untwist rate.
- These alterations suggest subclinical systolic and diastolic dysfunction in pediatric SCD.
- Rotational metrics may predict long-term outcomes in children with SCD.
Background:
Sickle cell disease (SCD) is a common inherited hemoglobinopathy. Adults with SCD manifest both systolic and diastolic cardiac dysfunction, though the age of onset of dysfunction has not been defined. Left ventricular (LV) rotational mechanics have not been studied in children with SCD. The aim of this study was to investigate whether cardiac rotational mechanics differed between children with SCD and age-matched controls.
Methods:
Basal and apical LV short-axis images were acquired prospectively in 213 patients with SCD (mean age, 14.1 ± 2.6 years) and 49 controls (mean age, 13.3 ± 2.8 years) from the Muhimbili Sickle Cohort in Dar es Salaam, Tanzania. The magnitude of basal and apical rotation, net twist angle, torsion, and untwist rate were obtained by two-dimensional speckle-tracking. The timing of events was normalized to aortic valve closure.
Results:
Mean basal rotation was significantly lower in patients with SCD compared with controls (P = .012), although no difference was observed in apical rotation (P = .37). No statistically significant differences in torsion or net twist angle were detected. Rotation rate at the apex (P = .001) and base (P = .0004) were significantly slower in subjects with SCD compared with controls. Mean peak untwisting rate was also significantly slower in patients with SCD (P = .006). No associations were found between hemoglobin concentration and apical rotation, basal rotation, net twist, and torsion.
Conclusion:
This study demonstrates alterations in LV rotational mechanics in children with SCD, including lower basal rotation, peak differential twist, and untwist rate. These abnormalities denote subclinical changes in LV systolic and diastolic performance in children with SCD. Future work may reveal an association between rotational metrics and long-term patient outcomes.
