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

Westaby, JD; Cooper, STE; Edwards, KA; Anderson, RH; Sheppard, MN (2020) Insights from examination of hearts from adults dying suddenly to the understanding of congenital cardiac malformations. Clin Anat, 33 (3). pp. 394-404. ISSN 1098-2353 https://doi.org/10.1002/ca.23531
SGUL Authors: Westaby, Joseph David

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Abstract

Congenital heart disease is a rare but important finding in adults that 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 6000 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 haemorrhage. 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 categorising the cases, with the concept of the "morphological method" serving to identify the distinguishing features of the cardiac components. This article is protected by copyright. All rights reserved.

Item Type: Article
Additional Information: This is the peer reviewed version of the following article: Westaby, J.D., Cooper, S.T.E., Edwards, K.A., Anderson, R.H. and Sheppard, M.N. (2020), Insights from examination of hearts from adults dying suddenly to the understanding of congenital cardiac malformations. Clin. Anat., 33: 394-404., which has been published in final form at https://doi.org/10.1002/ca.23531. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions.
Keywords: Morphological method, cardiac morphology, sequential segmental analysis, 1116 Medical Physiology, 1103 Clinical Sciences, Anatomy & Morphology
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Journal or Publication Title: Clin Anat
ISSN: 1098-2353
Language: eng
Dates:
DateEvent
9 March 2020Published
9 December 2019Published Online
19 November 2019Accepted
Publisher License: Publisher's own licence
PubMed ID: 31769098
Go to PubMed abstract
URI: http://openaccess.sgul.ac.uk/id/eprint/111438
Publisher's version: https://doi.org/10.1002/ca.23531

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