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Left ventricular non-compaction in patients with single ventricle heart disease

Published online by Cambridge University Press:  09 January 2020

Preeti Choudhary Open the ORCID record for Preeti Choudhary [Opens in a new window] ,
Wendy Strugnell ,
Rajesh Puranik ,
Christian Hamilton-Craig ,
Shelby Kutty Open the ORCID record for Shelby Kutty [Opens in a new window]  and
David S Celermajer
Preeti Choudhary
Affiliation:
Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia Faculty of Medicine and Heath, University of Sydney, Sydney, Australia
Wendy Strugnell
Affiliation:
Richard Slaughter Centre of Excellence in Cardiovascular Magnetic Resonance Imaging, The Prince Charles Hospital, Brisbane, Australia
Rajesh Puranik
Affiliation:
Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia Faculty of Medicine and Heath, University of Sydney, Sydney, Australia
Christian Hamilton-Craig
Affiliation:
Richard Slaughter Centre of Excellence in Cardiovascular Magnetic Resonance Imaging, The Prince Charles Hospital, Brisbane, Australia University of Queensland, Brisbane, Australia Griffith University School of Medicine, Griffith, Australia
Shelby Kutty
Affiliation:
Department of Cardiology, University of Nebraska Medical Centre, Omaha, NE, USA
David S Celermajer*
Affiliation:
Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia Faculty of Medicine and Heath, University of Sydney, Sydney, Australia
*
Author for correspondence: Professor David S Celermajer, MBBS (Hons 1) DSc FRACP PhD, Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, NSW 2050, Australia. Tel: +612 9515 7110; Fax: +612 9550 6262; E-mail: David.Celermajer@health.nsw.gov.au
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Abstract

Objective:

Left ventricular non-compaction is an architectural abnormality of the myocardium, associated with heart failure, systemic thromboembolism, and arrhythmia. We sought to assess the prevalence of left ventricular non-compaction in patients with single ventricle heart disease and its effects on ventricular function.

Methods:

Cardiac MRI of 93 patients with single ventricle heart disease (mean age 24 ± 8 years; 55% male) from three tertiary congenital centres was retrospectively reviewed; 65 of these had left ventricular morphology and are the subject of this report. The presence of left ventricular non-compaction was defined as having a non-compacted:compacted (NC:C) myocardial thickness ratio >2.3:1. The distribution of left ventricular non-compaction, ventricular volumes, and function was correlated with clinical data.

Results:

The prevalence of left ventricular non-compaction was 37% (24 of 65 patients) with a mean of 4 ± 2 affected segments. The distribution was apical in 100%, mid-ventricular in 29%, and basal in 17% of patients. Patients with left ventricular non-compaction had significantly higher end-diastolic (128 ± 44 versus 104 ± 46 mL/m2, p = 0.047) and end-systolic left ventricular volumes (74 ± 35 versus 56 ± 35 mL/m2, p = 0.039) with lower left ventricular ejection fraction (44 ± 11 versus 50 ± 9%, p = 0.039) compared to those with normal compaction. The number of segments involved did not correlate with ventricular function (p = 0.71).

Conclusions:

Left ventricular non-compaction is frequently observed in patients with left ventricle-type univentricular hearts, with predominantly apical and mid-ventricular involvement. The presence of non-compaction is associated with increased indexed end-diastolic volumes and impaired systolic function.

Keywords

Fontanadult congenital heart diseasecardiac MRIheart failurenon-compactionuniventricular physiology
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 1 , January 2020 , pp. 12 - 18
Copyright
© Cambridge University Press 2020

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