Elsevier

Legal Medicine

Volume 17, Issue 5, September 2015, Pages 340-342
Legal Medicine

Case Report
Unexpected neonatal death caused by interrupted aortic arch, an uncommon congenital great vessel malformation: An autopsy case report

https://doi.org/10.1016/j.legalmed.2015.03.008Get rights and content

Highlights

Abstract

A 21-day-old Japanese male infant became inactive and then collapsed unexpectedly. On autopsy, there was no connection between the ascending and descending thoracic aorta. The site of interruption was the aortic isthmus. The heart showed hypertrophy and exhibited intracardiac malformations, including subaortic valve stenosis resulting from posterior deviation of the ventricular outlet septum and ventricular septal defect. The cause of death was diagnosed as prolonged physiological closure of the ductus arteriosus complicated by interrupted aortic arch and followed by assumed ductal shock.

Introduction

Autopsies on infants who die suddenly and unexpectedly can sometimes exhibit a lack of remarkable pathological findings; however, in a small number of cases, macroscopic abnormalities clarifying the cause of death may be present. We present an autopsy case in which interrupted aortic arch (IAA) was recorded along with intracardiac malformations and severe biventricular hypertrophy.

Section snippets

Clinical history

A 21-day-old Japanese male infant unexpectedly became inactive at home and stopped drinking milk; 3 h prior, he had looked normal and had ingested 70 ml of milk. The mother’s pregnancy and term delivery had been unremarkable, and the infant had a birth weight of 2655 g. He was taken to an emergency clinic and transferred to an intensive care unit because he was found to be in a serious hypoxic state. Despite mechanical ventilation and repeated adrenaline infusion, the infant died soon after

Discussion

In the present case, the isthmus was the site of aortic interruption; we diagnosed a type A IAA according to the Celoria and Patton classification [1], [2]. IAA is an uncommon congenital malformation, occurring in approximately 3 per million births [3]; it is defined as a complete lack of luminal continuity between the ascending and descending thoracic aorta. From the developmental point of view, this abnormality appears to result from regression or atrophy of the left dorsal aorta and left

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