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Prevalence of left ventricular hypertrabeculation/noncompaction among children with sickle cell disease
M Louise Morrison1, Corrina McMahon2, Riona Tully1
1Department of Pediatric Cardiology, Our Lady's Hospital for Sick Children, Crumlin, Dublin, Ireland.
Insights
Left ventricular hypertrabeculation (LVHT) affects 11% of children with sickle cell disease (SCD) in Ireland. Left ventricular systolic function remains preserved in this pediatric SCD cohort.
Area of Science:
- Cardiology
- Pediatrics
- Hematology
Background:
- Sickle cell disease (SCD) incidence is rising in Ireland, with improved survival leading to increased focus on cardiovascular complications.
- Left ventricular hypertrabeculation (LVHT), also known as noncompaction, is a recognized cardiovascular manifestation in SCD.
- Understanding the prevalence of LVHT in pediatric SCD populations is crucial for long-term management.
Purpose of the Study:
- To determine the prevalence of left ventricular hypertrabeculation (LVHT) in a large cohort of children diagnosed with sickle cell disease (SCD).
- To assess left ventricular systolic function and identify potential risk factors associated with LVHT in pediatric SCD patients.
Main Methods:
- Retrospective review of echocardiography records for pediatric patients with SCD at Our Lady's Children's Hospital Crumlin (1998-2015).
- Data collected included demographics, hemoglobin phenotype, treatment, left ventricular systolic function (LVSF), and assessment for LVHT and pulmonary arterial hypertension.
- Analysis of 236 patient echocardiograms to identify features of LVHT and borderline LVHT.
Main Results:
- A total of 236 pediatric patients with SCD underwent echocardiography.
- Eleven percent (11%) of patients exhibited features of LVHT, with an additional 4.7% showing borderline features.
- No significant differences in age, left ventricular end-diastolic diameter (LVEDD), LVEDD z-score, or LVSF were observed between patients with and without LVHT.
Conclusions:
- The prevalence of LVHT/noncompaction in children with SCD is lower compared to adult populations.
- Left ventricular systolic function is generally well-preserved across the studied pediatric SCD cohort.
- Further research is needed to elucidate the mechanisms of LVHT development in SCD, and longitudinal monitoring is recommended for these patients.
Objectives:
Incidence of sickle cell disease (SCD) in Ireland has dramatically increased. Disease survival has also steadily improved however cardiovascular manifestations remain important causes of morbidity. These include reports of left ventricular hypertrabeculation (LVHT)/noncompaction. We sought to investigate the prevalence of LVHT among a large cohort of children with SCD.
Methods:
We retrospectively reviewed the records of all patients with a diagnosis of SCD who had undergone surveillance echocardiography at Our Lady's Children's Hospital Crumlin (OLCHC) from 1998 to 2015. Demographics, hemoglobin phenotype and treatment information was recorded. LV systolic function, evidence of LVHT, and possible pulmonary arterial hypertension was assessed.
Results:
Two hundred thirty-six patients had echocardiograms available for interpretation. One hundred twenty-one (51.3%) were female; mean age was 11.3 years (± 4.1 years). Twenty-six patients (11%) had features of LVHT on echocardiography. Eleven patients (4.7%) had borderline features of LVHT. Mean LVEDD across the whole cohort was 4.2 ± 0.69 cm, LVEDD z-score of 1.44 ± 1.9, and mean LVSF was 37.3% ±15.7%. There were no significant differences in terms of age, LVEDD, LVEDD z-score, or LVSF between patients with and those without LVHT.
Conclusions:
The prevalence of LVHT/noncompaction in children with SCD is lower than the adult population and LV systolic function is well preserved throughout our patient group. The mechanism behind the development of LVHT in this population remains speculative. Further work is required in this field. Sickle cell patients require longitudinal evaluation to ascertain changes in left ventricular function and the presence of LVHT/noncompaction.
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