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Insights of Clinical and Medical Images

Ebstein's anomaly associated with left ventricular non-compression can be detected via cardiac magnetic resonance imaging.
k.Sabine

Department of Clinical Genetics, Academic Medical Center, Amsterdam, The Netherlands.

Correspondence to Author: k.Sabine
Abstract:

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Hematopoietic immature microorganism transplantation (SCT) is a possibly healing treatment for patients with hematologic malignancies. There has been huge advancement in the beyond a very long while in allogeneic SCT with improved results through upgrades in steady care, extension of undifferentiated cell contributor choices (HLA-matched irrelevant benefactors (MUD), haploidentical related contributors, and rope blood units

ABBREVIATIONS:

Cardio-Magnetic Resonance Imaging, or CMR SSFP stands for steady-state free precession, while NC stands for noncompacted.

Introduction:

Ebstein's anomaly and isolated left ventricular non-compaction cardiomyopathy are both uncommon diseases. We discuss the case of a female patient, age 47, whose cardiac magnetic resonance imaging (CMR) revealed both Ebstein's abnormality and non-compaction cardiomyopathy of the left ventricle.

CASES PRESENTED:

Due to abnormal myocardial texture and tricuspid valve regurgitation, a 47-year-old woman was admitted to our clinic for cardiac magnetic resonance imaging. The patient first came in for a standard heart check-up. The patient denied having dyspnea, syncope, or angina pectoris.

The left ventricle was shown to have normal function with a left ventricular ejection fraction of 62% by the CMR volumetric assessment using left-ventricular short axis SSFP sequences. The antero-septal, anterior, lateral, and inferior regions of the midventricular region all had non-compacted myocardium. Due to abnormal myocardial texture and tricuspid valve regurgitation, a 47-year-old woman was admitted to our clinic for cardiac magnetic resonance imaging. The patient first came in for a standard heart check-up. The patient denied having dyspnea, syncope, or angina pectoris. The left ventricle was shown to have normal function with a left ventricular ejection fraction of 62% by the CMR volumetric assessment using left-ventricular short axis SSFP sequences. A ratio of more than 2.3:1 between non-compacted (NC) and compacted (C) myocardium was identified in the antero-septal, anterior, lateral, and inferior segments midventricular to apical.

A 2.5 cm apical displacement of the septal tricuspid leaflet was visible in SSFP sequences. Additionally, tricuspid regurgitation due to insufficient coaptation of the tricuspid leaflets could be seen in the SSFP 4-chamber image .

A normal systolic function was determined by volumetric analysis of the right ventricle's short axis cine stack.

DISCUSSION:

Ebstein's anomaly and isolated left ventricular non-compaction cardiomyopathy are both uncommon diseases. In the absence of other heart conditions, isolated left ventricular non-compaction is an uncommon cardiomyopathy characterised by large left ventricular trabeculae and deep intertrabecular recesses. A congenital heart condition known as Ebstein's abnormality is defined by the apical displacement of the tricuspid valve leaflets and the resultant "atrialization" of the right ventricle. Ebstein's abnormality is present in 0.17 to 0.72/10.000 live births [1, 2].

Noncompacted myocardium can also be an uncommon complication of Ebstein's abnormality [3, 4].

We present a case of combined left ventricular non-compaction cardiomyopathy and Ebstein's abnormality identified by CMR. A solid criteria for the diagnosis of noncompaction cardiomyopathy is the ratio of non-compacted to compacted myocardium. With a sensitivity and specificity of 86% and >2.3 in diastole, respectively, non-compaction cardiomyopathy is detected [5]. CMR offers volumetric evaluation of the right ventricle and estimation of right ventricular function with regard to Ebstein's abnormality. Additionally, CMR can be used to assess the level of tricuspid regurgitation and visualise the posterior tricuspid valve [6].

Modern imaging methods like CMR help to better diagnose uncommon cardiac disorders including Ebstein's abnormality and left ventricular non-compaction.

REFERENCES:

1. Lupo PJ, Langlois PH, Mitchell LE. Epidemiology of Ebstein anomaly:prevalence and patterns in Texas, 1999-2005. Am J Med Genet A. 2011; 155:1007-1014.

2. Pradat P, Francannet C, Harris JA, Robert E. The epidemiology of cardiovascular defects, part I: a study based on data from three large registries of congenital malformations. Pediatr Cardiol. 2003; 24: 195-221.

3. Attenhofer Jost CH, Connolly HM, O’Leary PW, Warnes CA, Tajik AJ, Seward JB. Left heart lesions in patients with Ebstein anomaly. Mayo Clin Proc. 2005; 80: 361-368.

4. Stahli BE, Gebhard C, Biaggi P, Klaassen S, Valsangiacomo Buechel E, Attenhofer Jost CH, Jenni R, Tanner FC, Greutmann M. Left ventricular non-compaction: prevalence in congenital heart disease. Int J Cardiol. 2013; 167: 2477-2481.

5. Petersen SE, Selvanayagam JB, Wiesmann F, Robson MD, Francis JM, Anderson RH, et al. Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol. 2005; 46: 101-105.

6. Attenhofer Jost CH, Edmister WD, Julsrud PR, Dearani JA, Savas Tepe M, Warnes CA, et al. Prospective comparison of echocardiography

versus cardiac magnetic resonance imaging in patients with Ebstein’s anomaly. Int J Cardiovasc Imaging. 2012; 28:1147-1159.

Citation:

k.Sabine. Ebstein's anomaly associated with left ventricular non-compression can be detected via cardiac magnetic resonance imaging. Insights of Clinical and Medical Images 2022.