The association of cardiac ventricular hypertrophy with congenital hyperinsulinism.
OBJECTIVE
Ventricular hypertrophy (VH) has been observed in children with congenital hyperinsulinism (CHI), a condition of hypoglycaemia characterised by dysregulated insulin secretion, but the prevalence is not known.


METHODS
Cardiac assessment was performed in children (n=49) with CHI at diagnosis and follow-up. Two dimensional and Doppler echocardiography studies were used to assess cardiac structures, while M-mode study was used to measure left ventricular (LV) dimensions, subsequently converted to Z scores. Where possible, LV hypertrophy was confirmed by LV mass index (g/m(2.7)) >95th centile.


RESULTS
Cardiac structural lesions were found in 14 (28%) children. At initial echocardiography, VH was present in 31 (65%) children with median (range) LV posterior wall dimension in diastole Z scores of +1.6 (-2.4 to +5.8) and interventricular septal wall dimension in end diastole Z scores of +1.9 (-1.7 to +17.2). At follow-up echocardiography, performed after an interval of 178 (45-390) days, VH persisted in 16 (33%) children. In regression analysis, the presence of VH (odds ratio (95% confidence intervals) 1.1 (1.0-1.2), P=0.04) at initial echocardiography was correlated with maximum glucose requirement at diagnosis, indicating that severity of CHI at presentation may play a role in the pathogenesis of VH.


CONCLUSIONS
A significant proportion of children with CHI have cardiac structural lesions. A majority also have VH, which may be associated with the severity of CHI at diagnosis. VH may persist in some children, which requires careful long-term cardiac review.
Department of Paediatric Endocrinology, Royal Manchester Children's Hospital, UK. indi.banerjee@cmft.nhs.uk
Indraneel
I
Banerjee
Bindu
B
Avatapalle
Anjali
A
Petkar
Mars
M
Skae
Raja
R
Padidela
Sarah
S
Ehtisham
Leena
L
Patel
Lindsey
L
Rigby
Sarah
S
Flanagan
Sian
S
Ellard
Caroline
C
Jones
Giovanna
G
Ciotti
Nilima
N
Malaiya
Peter E
PE
Clayton
D002306:Cardiac Volume; D006332:Cardiomegaly / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology*; D002648:Child; D044903:Congenital Hyperinsulinism / Q000150:complications* / Q000503:physiopathology*; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000209:etiology / Q000503:physiopathology; D017380:Hypertrophy, Right Ventricular / Q000209:etiology / Q000503:physiopathology; D008297:Male; D016017:Odds Ratio; D012720:Severity of Illness Index; D013997:Time Factors