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Systematic Review

A Systematic Review of Ebstein’s Anomaly with Left Ventricular Noncompaction

by
Suma K. Thareja
1,2,
Michele A. Frommelt
3,4,
Joy Lincoln
3,4,
John W. Lough
1,
Michael E. Mitchell
2,3,4 and
Aoy Tomita-Mitchell
2,3,4,*
1
Department of Cell Biology, Neurobiology, and Anatomy, Medical College of Wisconsin, Milwaukee, WI 53226, USA
2
Department of Surgery, Division of Congenital Heart Surgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA
3
Department of Pediatrics, Division of Pediatric Cardiology, Children’s Wisconsin, Milwaukee, WI 53226, USA
4
Herma Heart Institute, Children’s Wisconsin, Milwaukee, WI 53226, USA
*
Author to whom correspondence should be addressed.
J. Cardiovasc. Dev. Dis. 2022, 9(4), 115; https://doi.org/10.3390/jcdd9040115
Submission received: 12 March 2022 / Revised: 31 March 2022 / Accepted: 7 April 2022 / Published: 13 April 2022
(This article belongs to the Special Issue Congenital Heart Defects: Diagnosis, Management, and Treatment)
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Abstract

Traditional definitions of Ebstein’s anomaly (EA) and left ventricular noncompaction (LVNC), two rare congenital heart defects (CHDs), confine disease to either the right or left heart, respectively. Around 15–29% of patients with EA, which has a prevalence of 1 in 20,000 live births, commonly manifest with LVNC. While individual EA or LVNC literature is extensive, relatively little discussion is devoted to the joint appearance of EA and LVNC (EA/LVNC), which poses a higher risk of poor clinical outcomes. We queried PubMed, Medline, and Web of Science for all peer-reviewed publications from inception to February 2022 that discuss EA/LVNC and found 58 unique articles written in English. Here, we summarize and extrapolate commonalities in clinical and genetic understanding of EA/LVNC to date. We additionally postulate involvement of shared developmental pathways that may lead to this combined disease. Anatomical variation in EA/LVNC encompasses characteristics of both CHDs, including tricuspid valve displacement, right heart dilatation, and left ventricular trabeculation, and dictates clinical presentation in both age and severity. Disease treatment is non-specific, ranging from symptomatic management to invasive surgery. Apart from a few variant associations, mainly in sarcomeric genes MYH7 and TPM1, the genetic etiology and pathogenesis of EA/LVNC remain largely unknown.
Keywords: Ebstein’s anomaly; left ventricular noncompaction; congenital heart disease; cardiomyopathy; genetic etiology Ebstein’s anomaly; left ventricular noncompaction; congenital heart disease; cardiomyopathy; genetic etiology

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MDPI and ACS Style

Thareja, S.K.; Frommelt, M.A.; Lincoln, J.; Lough, J.W.; Mitchell, M.E.; Tomita-Mitchell, A. A Systematic Review of Ebstein’s Anomaly with Left Ventricular Noncompaction. J. Cardiovasc. Dev. Dis. 2022, 9, 115. https://doi.org/10.3390/jcdd9040115

AMA Style

Thareja SK, Frommelt MA, Lincoln J, Lough JW, Mitchell ME, Tomita-Mitchell A. A Systematic Review of Ebstein’s Anomaly with Left Ventricular Noncompaction. Journal of Cardiovascular Development and Disease. 2022; 9(4):115. https://doi.org/10.3390/jcdd9040115

Chicago/Turabian Style

Thareja, Suma K., Michele A. Frommelt, Joy Lincoln, John W. Lough, Michael E. Mitchell, and Aoy Tomita-Mitchell. 2022. "A Systematic Review of Ebstein’s Anomaly with Left Ventricular Noncompaction" Journal of Cardiovascular Development and Disease 9, no. 4: 115. https://doi.org/10.3390/jcdd9040115

APA Style

Thareja, S. K., Frommelt, M. A., Lincoln, J., Lough, J. W., Mitchell, M. E., & Tomita-Mitchell, A. (2022). A Systematic Review of Ebstein’s Anomaly with Left Ventricular Noncompaction. Journal of Cardiovascular Development and Disease, 9(4), 115. https://doi.org/10.3390/jcdd9040115

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