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Insights from examination of hearts from adults dying suddenly to the understanding of congenital cardiac malformations

Lookup NU author(s): Professor Bob Anderson

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Abstract

© 2019 Wiley Periodicals, Inc.Congenital heart disease is a rare but important finding in adults who experience sudden death. Examination of the congenitally malformed heart has historically been considered esoteric and best left to those with expertise. The Cardiac Risk in the Young cardiovascular pathology laboratory based at St George's University of London has now received over 6,000 cases. Of these, 21 congenitally malformed hearts were retained for research and educational purposes. Hearts were assessed using sequential segmental analysis, and causes of death were adjudicated based on thorough macroscopic examination and histology. Congenital malformations that were encountered included atrial septal defects, ventricular septal defects, tetralogy of Fallot, and transposition of the great arteries in both its regular and congenitally corrected variants. Findings also included hearts with mirror-imaged and isomeric atrial appendages. Direct causes of death included myocardial fibrosis, pulmonary hypertension, and hemorrhage. A small but notable proportion did not reveal a substrate for arrhythmia, raising the question of whether the terminal event was due to the congenital heart disease itself, or an underlying channelopathy. Here, we demonstrate the value of simple sequential segmental analysis in describing and categorizing the cases, with the concept of the “morphological method” serving to identify the distinguishing features of the cardiac components. Clin. Anat., 2019. © 2019 Wiley Periodicals, Inc.

Publication metadata

Author(s): Westaby JD, Cooper STE, Edwards KA, Anderson RH, Sheppard MN

Publication type: Article

Publication status: Published

Journal: Clinical Anatomy

Year: 2019

Volume: 33

Issue: 3

Pages: 394-404

Print publication date: 09/03/2020

Online publication date: 09/12/2019

Acceptance date: 25/11/2019

ISSN (print): 0897-3806

ISSN (electronic): 1098-2353

Publisher: John Wiley and Sons Inc.

URL: https://doi.org/10.1002/ca.23531

DOI: 10.1002/ca.23531

PubMed id: 31769098

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