Prevalence of left ventricular hypertrabeculation/
noncompaction among children with sickle cell disease
M. Louise Morrison MD
Corrina McMahon MD
Riona Tully MBBCh
Noelle Enright MBBCh
Ricardo Pignatelli MD
Jeffrey A. Towbin MD
Colin J. McMahon MBBCh
Department of Pediatric Cardiology,
Our Lady’s Hospital for Sick Children,
Crumlin, Dublin, Ireland
Department of Hematology, Our Lady’s
Hospital for Sick Children, Crumlin
Texas Children’s Hospital, Houston, Texas,
St. Jude Children’s Research Hospital, and
LeBonheur Children’s Hospital, University of
Tennessee Health Science Center, Memphis,
Prof. Colin McMahon, Department of
Paediatric Cardiology, Our Lady’s Children’s
Hospital, Cooley Road, Crumlin, Dublin,
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 (6 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 6 0.69 cm, LVEDD z-score of
1.44 6 1.9, and mean LVSF was 37.3% 615.7%. 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.
LVHT/noncompaction, pediatric, sickle cell disease
Sickle cell disease (SCD) is an autosomal recessive condition affect-
ing millions of African/Afro-Caribbean individuals worldwide. In Ire-
land the incidence has dramatically increased over the past 15 years
due to the influx of migrants. There are now over 400 children with
SCD and around 100 adults compared with just 2 children affected
Survival among those with SCD has steadily increased however in
adult life patients accumulate end organ damage and failure.
Cardiovascular manifestations are a common feature and an important
cause of morbidity. They include pulmonary hypertension and progres-
sive left ventricular dilatation and dysfunction.
A high proportion of
African/Afro-Caribbean patients with heart failure fulfill criteria for left
ventricular hypertrabeculation (LVHT)/noncompaction.
LVHT, a possi-
ble precursor to LV noncompaction cardiomyopathy (LVNC), has also
been recorded among black athletes.
Previous reports of adults with
SCD have noted LVHT occurring in up to 28% of patients.
to investigate the prevalence of LVHT among a large cohort of children
(<18 years) with SCD.
2018 Wiley Periodicals, Inc. wileyonlinelibrary.com/journal/chd Congenital Heart Disease.2018;13:1–4.
Received: 29 November 2017
Accepted: 23 January 2018