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Insights from examination of hearts from adults dying suddenly to the understanding of congenital cardiac malformations
Author(s) -
Westaby Joseph D.,
Cooper Susanna T. E.,
Edwards Khari A.,
Anderson Robert H.,
Sheppard Mary N.
Publication year - 2020
Publication title -
clinical anatomy
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.667
H-Index - 71
eISSN - 1098-2353
pISSN - 0897-3806
DOI - 10.1002/ca.23531
Subject(s) - medicine , tetralogy of fallot , heart disease , sudden cardiac death , cardiology , great arteries , sudden death , double outlet right ventricle , channelopathy
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. 33:394–404, 2020. © 2019 Wiley Periodicals, Inc.