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The Left Ventricular Myocardium in Hypoplastic Left Heart Syndrome

Lookup NU author(s): Dr Bill Chaudhry, Dr Ahlam Alqahtani, Dr Lorraine Eley, Dr Louise CoatsORCiD, Dr Srinivas Annavarapu, Professor Deborah HendersonORCiD



This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


© 2022 by the authors. Hypoplastic left heart syndrome (HLHS) is a collective term applied to severe congenital cardiac malformations, characterised by a combination of abnormalities mainly affecting the left ventricle, associated valves, and ascending aorta. Although in clinical practice HLHS is usually sub-categorised based on the patency of the mitral and aortic (left-sided) valves, it is also possible to comprehensively categorise HLHS into defined sub-groups based on the left ventricular morphology. Here, we discuss the published human-based studies of the ventricular myocardium in HLHS, evaluating whether the available evidence is in keeping with this ventricular morphology concept. Specifically, we highlight results from histological studies, indicating that the appearance of cardiomyocytes can be different based on the sub-group of HLHS. In addition, we discuss the histological appearances of endocardial fibroelastosis (EFE), which is a common feature of one specific sub-group of HLHS. Lastly, we suggest investigations that should ideally be undertaken using HLHS myocardial tissues at early stages of HLHS development to identify biological pathways and aid the understanding of HLHS aetiology.

Publication metadata

Author(s): Chaudhry B, Alqahtani A, Eley L, Coats L, Moldovan C, Annavarapu SR, Henderson DJ

Publication type: Review

Publication status: Published

Journal: Journal of Cardiovascular Development and Disease

Year: 2022

Volume: 9

Issue: 8

Online publication date: 19/08/2022

Acceptance date: 15/08/2022

ISSN (electronic): 2308-3425

Publisher: MDPI


DOI: 10.3390/jcdd9080279