We hereby present the case of a 47-year old female who was admitted to our outpatient clinic to perform cardiac magnetic resonance imaging (CMR) because of unusual texture of the myocardium and tricuspid valve regurgitation in echocardiography. Cardiac magnetic resonance imaging revealed not only noncompaction cardiomyopathy of the left ventricle but also Ebstein´s anomaly in this patient. Isolated non-compaction cardiomyopathy as well as Ebstein´s anomaly is a rare condition and the combination of both is seldom seen. However, both entities have an impact on the patient´s life. This case report in combination with high quality CMR pictures underlines that CMR is a modern imaging technique, which further improves diagnosis of rare cardiac conditions and thereby helps establishing the necessary therapy.

• Ebstein’s anomaly
• Left ventricular non-compaction
• Cardiac magnetic resonance imaging
• Cardiac imaging

Loeffler F, Zwadlo C, Westhoff-Bleck M, Pirr J, Bauersachs J (2017) Cardiac Magnetic Resonance Imaging to Diagnose Ebstein’s Anomaly Associated with Left Ventricular Non-Compaction. JSM Clin Med Imaging Cases Rev 2(1): 1010.

CMR: Cardiac Magnetic Resonance Imaging, NC: NonCompacted, C: Compacted, SSFP: Steady-State Free Precessio

Both isolated left ventricular non-compaction cardiomyopathy and Ebstein’s anomaly are rare conditions. We present a case of a 47-year old female in whom cardiac magnetic resonance imaging (CMR) revealed not only non-compaction cardiomyopathy of the left ventricle but also Ebstein´s anomaly.

A 47-year-old female was admitted to our clinic to perform cardiac magnetic resonance imaging because of unusual texture of the myocardium and tricuspid valve regurgitation. The initial presentation was for routine cardiac check-up. The patient negated any angina pectoris, dyspnea, dizziness, or syncopes.

The CMR volumetric assessment by left-ventricular short axis SSFP sequences showed a normal function of the left ventricle with a left ventricular ejection fraction of 62%. Non-compacted myocardium was found in antero-septal, anterior, lateral, and inferior segments midventricular to apical with a ratio of > 2.3:1 of non-compacted (NC) to compacted (C) myocardium in diastole (Video 1). 

Video 1 SSFP cine imaging of 4-chamber view: non-compacted myocardium is found in midventricular to apical parts of the left ventricle with a ratio of > 2.3:1 of non-compacted (NC) to compacted (C) myocardium in diastole. In the right ventricle, apical displacement of about 2.5cm of the septal tricuspid leaflet is visible with tricuspid regurgitation

SSFP sequences showed an apical displacement of the septal tricuspid leaflet about 2.5 cm. Furthermore, tricuspid regurgitation could be noted in the SSFP 4-chamber view resulting from failing coaptation of the tricuspid leaflets (Figure). The volumetric assessment of the short axis cine stack of the right ventricle rendered a normal systolic function.

Both isolated left ventricular non-compaction cardiomyopathy and Ebstein’s anomaly are rare conditions. Isolated left ventricular non-compaction is a rare cardiomyopathy characterized by extensive left ventricular trabeculae and deep intertrabecular recesses in the absence of other cardiac disorders. Ebstein’s anomaly denotes a congenital heart disease characterized by the apical displacement of the leaflets of the tricuspid valve and the subsequent “atrialization” of the right ventricle. Prevalence of Ebstein’s anomaly accounts to 0.17-0.72/10.000 live births [1, 2]. In rare cases, Ebstein’s anomaly can also be associated with noncompacted myocardium [3, 4].

We report a case combining both Ebstein’s anomaly and left ventricular non-compaction cardiomyopathy, which was diagnosed through CMR. The ratio of non-compacted to compacted myocardium poses a reliable criterion for the diagnosis of noncompaction cardiomyopathy. A NC/C ratio of >2.3 in diastole identifies non-compaction cardiomyopathy with a sensitivity of 86% and specificity of 86% [5]. Regarding Ebstein’s anomaly CMR enables volumetric assessment of the right ventricle and quantification of the right ventricular function. Additionally, CMR is helpful in visualizing the posterior tricuspid valve and to quantify the degree of tricuspid regurgitation [6].

CMR is a modern imaging technique, which further improves diagnosis of rare cardiac conditions such as Ebstein’s anomaly accompanied by left ventricular non-compaction.

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.