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9 - The abnormal heart

Published online by Cambridge University Press:  05 September 2014

By
Glenn P. Taylor ,
Mary N. Sheppard  and
S. Yen Ho
Edited by
Marta C. Cohen  and
Irene Scheimberg
Glenn P. Taylor
Affiliation:
The Hospital for Sick Children, Toronto
Mary N. Sheppard
Affiliation:
Royal Brompton and Harefield NHS Foundation Trust, London, UK
S. Yen Ho
Affiliation:
Royal Brompton Hospital, London, UK
Marta C. Cohen
Affiliation:
Sheffield Children’s Hospital
Irene Scheimberg
Affiliation:
Barts and the London NHS Trust, London
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Summary

Introduction

The pathology of the heart in the pediatric age group can be divided into that of congenital lesions and postnatally acquired heart disease. Acquired disease in the setting of congenital heart disease is achieving increasing significance, with more surgery being performed on even very complex cases with long-term survival. Pathologists need not only be familiar with congenital heart lesions, but the complex surgical techniques which have evolved since the 1940s in dealing with these cardiac lesions.

While adult cardiac autopsy concentrates on coronary artery disease, in children congenital cardiac lesions predominate [1,2]. The incidence of congenital heart disease has been estimated at approximately 8 per 1000 live births [3]. Incidence in stillbirths is higher [4]. Many pathologists are totally ignorant and apprehensive concerning a congenitally malformed heart and the complex procedures evolved over the years in dealing with these lesions.

Although congenital heart disease is the most prevalent form of heart pathology in the fetus and child, other types of heart disease occur, with their own challenges for the autopsy prosector. These include cardiomyopathies with a very wide range of rare metabolic and genetic causes, myocarditis that can masquerade as sudden infant death syndrome (SIDS) or cause sudden death, and certain heart tumors that primarily affect children. Many of these conditions have genetic associations with potential consequences to current and future siblings, necessitating an accurate and clinically relevant diagnosis. Autopsy of the fetal or pediatric heart is further challenged by the macerated stillbirth, very small pre-term fetus, and disrupted terminated conceptus that often present to the pathology laboratory. Congenital heart disease in a fetus or infant may have been identified by ante mortem clinical investigations or be a complete surprise at autopsy.

Type
Chapter
Information
The Pediatric and Perinatal Autopsy Manual , pp. 139 - 172
Publisher: Cambridge University Press
Print publication year: 2000

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