Isolated left ventricular apical hypoplasia: Systematic review and analysis of the 37 cases reported so far

doi:10.12998/wjcc.v11.i23.5494

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World Journal of Clinical Cases

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World J Clin Cases. Aug 16, 2023; 11(23): 5494-5503
Published online Aug 16, 2023. doi: 10.12998/wjcc.v11.i23.5494

Isolated left ventricular apical hypoplasia: Systematic review and analysis of the 37 cases reported so far

Pier Paolo Bassareo, Sophie Duignan, Adam James, Esme Dunne, Colin J McMahon, Kevin P Walsh

Pier Paolo Bassareo, Kevin P Walsh, University College of Dublin, School of Medicine and Department of Cardiology at Mater Misericordiae University Hospital, Dublin D07 R2WY, Ireland

Sophie Duignan, Adam James, Esme Dunne, Department of Cardiology, Children's Health Crumlin, Dublin D12 N512, Ireland

Colin J McMahon, University College of Dublin, School of Medicine and Department of Cardiology, Children's Health Ireland, Dublin D12 N512, Ireland

Author contributions: Bassareo PP contributed to write the paper; Duignan S, James A, Dunne E, McMahon CJ collected the data and revised the manuscript critically; Walsh KP contributed to supervised the work and gave the final approval to publish the paper.

Conflict-of-interest statement: The authors declare that they have no conflict of interest.

PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.

Corresponding author: Pier Paolo Bassareo, DPhil, FACC, FESC, MD, MSc, Associate Professor, Consultant Physician-Scientist, Department of Cardiology at Mater Misericordiae University Hospital, University College of Dublin, Eccles Street, Dublin 7, Dublin D07 R2WY, Ireland. piercard@inwind.it

Received: May 10, 2023
Peer-review started: May 10, 2023
First decision: June 13, 2023
Revised: June 22, 2023
Accepted: July 17, 2023
Article in press: July 17, 2023
Published online: August 16, 2023
Processing time: 97 Days and 18.8 Hours

Abstract

BACKGROUND

Isolated left ventricular apical hypoplasia (ILVAH), also known as truncated left ventricle (LV), is a very unusual cardiomyopathy. It is characterised by a truncated, spherical, and non-apex forming LV. The true apex is occupied by the right ventricle. Due to the rarity of the disease, just a few case reports and limited case series have been published in the field.

AIM

To analysing the so far 37 reported ILVAH cases worldwide.

METHODS

The electronic databases PubMed and Scopus were investigated from their establishment up to December 13, 2022.

RESULTS

The majority of cases reported occurred in males (52.7%). Mean age at diagnosis was 26.1 ± 19.6 years. More than a third of the patients were asymptomatic (35.1%). The most usual clinical presentation was breathlessness (40.5%). The most commonly detected electrocardiogram changes were T wave abnormalities (29.7%) and right axis deviation with poor R wave progression (24.3%). Atrial fibrillation/flutter was detected in 24.3%. Echocardiography was performed in 97.3% of cases and cardiac MRI in 91.9% of cases. Ejection fraction was reduced in more than a half of patients (56.7%). An associated congenital heart disease was found in 16.2%. Heart failure therapy was administered in 35.1% of patients. The outcome was favorable in the vast majority of patients, with just one death.

CONCLUSION

ILVAH is a multifaceted entity with a so far unpredictable course, ranging from benign until the elderly to sudden death during adolescence.

Keywords: Isolated left ventricular apical hypoplasia; Truncated left ventricle; Electrocardiography; Echocardiography; Cardiac magnetic resonance imaging; Heart failure

Core Tip: The manuscript is focused on an interesting topic, e.g. a rare form of cardiomyopathy which is called isolated left ventricular hypoplasia or truncated left ventricle. The so far 37 reported cases worldwide have been reviewed and analysed to help clinicians in the difficult management of this extremely rare congenital heart disease. Nice images from our personal archive have been added.