The association of cardiac ventricular hypertrophy with congenital hyperinsulinism

Abstract

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. Patients and methods: Cardiac assessment was performed in children (nZ49) 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 ) O95th 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 C1.6 (K2.4 to C5.8) and interventricular septal wall dimension in end diastole Z scores of C1.9 (K1.7 to C17.2). 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), PZ0.04) 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.

Percutaneous left main coronary artery stent for acute myocardial ischemia after repaired ALCAPA

“…This patient had a previously unreported association of ALCAPA, peripheral pulmonary artery stenosis, and congenital hyperinsulinism. Infants with congenital hyperinsulinism have been reported to have an increased incidence of cardiac structural lesions and hypertrophic cardiomyopathy , however coronary or pulmonary artery abnormalities have not been previously reported.…”

Gene Biomarkers in Congenital Hyperinsulinism

“…24 Also, INS as a growth factor plays a role in the differentiation process of stem cells into brain and nerve cells 25, 26 The present study showed that INS is the most effective biomarker related to CHI, in line with the results of other studies. 27 In a study conducted on patients with congenital hyperinsulinism, they found that a large percentage of infants with this disease have ventricular hypertrophy, which may be related to the severity of CHI at the time of diagnosis, making the previous care of these patients even more severe. 28 The results showed that PRKACA is the second most effective biomarker related to CHI.…”

Integrating genetic and imaging investigations into the clinical management of congenital hyperinsulinism

“…Correction of hypoglycaemia can also be complicated by the development of heart failure from exuberant dextrose containing fluid overload while attempting to maintain a high carbohydrate intake. It is well recognized that children with CHI commonly have cardiac ventricular hypertrophy, which may predispose these children to pulmonary oedema . Furthermore, oral treatment with Diazoxide, a K ATP channel opening agent, may cause fluid retention in the whole body, and sometimes specifically in the pericardial cavity, leading to acute cardiovascular compromise.…”