Abstract

Sudden cardiac death is an uncommon but yet catastrophic event, which can occur in neonates and young children. Although extensive research has been carried out assessing the underlying causes, there still remains a degree of uncertainty around this area. Congenital heart disease (CHD) is one known cause of sudden cardiac death in children, the aetiology of which embraces virally induced mechanisms, genetic susceptibility, drug-induced, and maternal factors. Screening tools and investigations including electrocardiograms and echocardiograms alongside a concise history taking and physical examination can be used to identify the potential cardiovascular risk factors of sudden death. This review has comprehensively studied the causes and risk factors for sudden cardiac death in children with CHD and provides a collation and summary of the evidence available so far underpinning the complex link between the two. Moreover, current screening and prevention methods are discussed in detail in order to increase awareness and understanding of how we can improve patient outcomes.

Highlights

  • Congenital heart disease (CHD) is one of the most common birth defects in newborn babies.[1]

  • CHD and structural heart disease form one part of a spectrum of congenital cardiac disorders, which predispose an individual to sudden cardiac death, and so we found that further classification of disease types based on anatomy and physiology aids in the understanding of the specific mechanisms underlying premature death

  • This review found that those CHDs considered being complex in both pathophysiology and management were more likely to lead to sudden cardiac death

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Summary

Introduction

CHD is one of the most common birth defects in newborn babies.[1]. extremely rare, these malformations can lead to sudden cardiac death during early childhood. Common CHDs in patients with Noonan’s syndrome include pulmonary valve stenosis, atrial septal defects, and congenital hypertrophic cardiomyopathy and mitral valve defects.[13] Sudden cardiac death is a concern in patients with Noonan’s syndrome, due to congenital hypertrophic cardiomyopathy being a major risk factor; for example, Ramond et al in 2017 reported a fatal case of a patient with Noonan’s syndrome, who had a sudden cardiac death event whilst running.[14]. Another risk factor that is linked with foetal development of CHD is maternal diabetes mellitus.

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