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Open Access 01-01-2020 | Cardiomyopathy | Review

Neonatal cardiac hypertrophy: the role of hyperinsulinism—a review of literature

Authors: Nina D. Paauw, Raymond Stegeman, Monique A. M. J. de Vroede, Jacqueline U. M. Termote, Matthias W. Freund, Johannes M. P. J. Breur

Published in: European Journal of Pediatrics | Issue 1/2020

Abstract

Hypertrophic cardiomyopathy (HCM) in neonates is a rare and heterogeneous disorder which is characterized by hypertrophy of heart with histological and functional disruption of the myocardial structure/composition. The prognosis of HCM depends on the underlying diagnosis. In this review, we emphasize the importance to consider hyperinsulinism in the differential diagnosis of HCM, as hyperinsulinism is widely associated with cardiac hypertrophy (CH) which cannot be distinguished from HCM on echocardiographic examination. We supply an overview of the incidence and treatment strategies of neonatal CH in a broad spectrum of hyperinsulinemic diseases. Reviewing the literature, we found that CH is reported in 13 to 44% of infants of diabetic mothers, in approximately 40% of infants with congenital hyperinsulinism, in 61% of infants with leprechaunism and in 48 to 61% of the patients with congenital generalized lipodystrophy. The correct diagnosis is of importance since there is a large variation in prognoses and there are various strategies to treat CH in hyperinsulinemic diseases.

Conclusion: The relationship between CH and hyperinsulism has implications for clinical practice as it might help to establish the correct diagnosis in neonates with cardiac hypertrophy which has both prognostic and therapeutic consequences. In addition, CH should be recognized as a potential comorbidity which might necessitate treatment in all neonates with known hyperinsulinism.

What is Known:

• Hyperinsulinism is currently not acknowledged as a cause of hypertrophic cardiomyopathy (HCM) in textbooks and recent Pediatric Cardiomyopathy Registry publications.

What is New:

• This article presents an overview of the literature of hyperinsulinism in neonates and infants showing that hyperinsulinism is associated with cardiac hypertrophy (CH) in a broad range of hyperinsulinemic diseases.

• As CH cannot be distinguished from HCM on echocardiographic examination, we emphasize the importance to consider hyperinsulinism in the differential diagnosis of HCM/CH as establishing the correct diagnosis has both prognostic and therapeutic consequences.

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Title: Neonatal cardiac hypertrophy: the role of hyperinsulinism—a review of literature
Authors: Nina D. Paauw
Raymond Stegeman
Monique A. M. J. de Vroede
Jacqueline U. M. Termote
Matthias W. Freund
Johannes M. P. J. Breur
Publication date: 01-01-2020
Publisher: Springer Berlin Heidelberg
Keywords: Cardiomyopathy
Insulins
Diazoxide
Insulins
Beckwith-Wiedemann Syndrome
Beckwith-Wiedemann Syndrome
Published in: European Journal of Pediatrics / Issue 1/2020
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-019-03521-6

At a glance: The STEP trials

Springer Medicine

Neonatal cardiac hypertrophy: the role of hyperinsulinism—a review of literature

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At a glance: The STEP trials

Springer Medicine

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Pigtail catheters versus traditional chest drains for pneumothorax treatment in two NICUs

Celiac disease and complement activation in response to Streptococcus pneumoniae

The perspectives of parents, general practitioners, and community pharmacists about treating an ill child in primary care: a comparative study

Lung ultrasound in preterm infants with respiratory distress: experience in a neonatal intensive care unit

Validation of the “Good2Go”: the first French-language transition readiness questionnaire

Treatment of opioid withdrawal in neonates with morphine, phenobarbital, or chlorpromazine: a randomized double-blind trial