Ventricular fibrillation due to a likely pathogenic SOS1 variant: An unrecognized etiology of infantile sudden death?

Creator(s)

Christopher Follansbee, Children's Mercy HospitalFollow
Lindsey Malloy-Walton, Children's Mercy HospitalFollow

Document Type

Article

Publication Date

8-11-2021

Identifier

DOI: 10.1016/j.hrcr.2021.06.010; PMCID: PMC8377259

Abstract

We present the case of a female infant presenting after a ventricular fibrillation arrest found to have ectopic atrial tachycardia (EAT). Evaluation revealed a likely pathogenic variant in SOS1 not previously reported in affected individuals. SOS1 variants are associated with Noonan syndrome, which belongs to a family of related genetic syndromes affecting the RAS/MAPK signaling pathway. To date, this is the first case reported of a ventricular fibrillation arrest in a patient with a RASopathy-related variant prior to development of the typically associated structural cardiac phenotype and may represent a previously unrecognized etiology of sudden death during infancy.

Journal Title

HeartRhythm Case Rep

Volume

7

Issue

8

First Page

510

Last Page

513

Keywords

Ectopic atrial tachycardia; Noonan syndrome; Pediatric; RASopathy; SOS1; Ventricular fibrillation

Comments

This article is available under the Creative Commons CC-BY-NC-ND license and permits non-commercial use of the work as published, without adaptation or alteration provided the work is fully attributed.

Publisher's Link: https://www.heartrhythmcasereports.com/article/S2214-0271(21)00134-2/fulltext

Recommended Citation

Follansbee CW, Malloy-Walton L. Ventricular fibrillation due to a likely pathogenic SOS1 variant: An unrecognized etiology of infantile sudden death?. HeartRhythm Case Rep. 2021;7(8):510-513. Published 2021 Aug 11. doi:10.1016/j.hrcr.2021.06.010