J Cardiovasc Ultrasound.  2015 Sep;23(3):186-190. 10.4250/jcu.2015.23.3.186.

Arrhythmogenic Noncompaction Cardiomyopathy: Is There an Echocardiographic Phenotypic Overlap of Two Distinct Cardiomyopathies?

• Aras D
• Ozeke O
• Cay S
• Ozcan F
• Baser K
• Dogan U
• Unlu M
• Demirkan B
• Tufekcioglu O
• Topaloglu

• KMID: 2068730
• DOI: http://doi.org/10.4250/jcu.2015.23.3.186

Abstract

The clinical diagnosis of right ventricular (RV) cardiomyopathies is often challenging. It is difficult to differentiate the isolated left ventricular (LV) noncompaction cardiomyopathy (NC) from biventricular NC or from coexisting arrhythmogenic ventricular cardiomyopathy (AC). There are currently few established morphologic criteria for the diagnosis other than RV dilation and presence of excessive regional trabeculation. The gross and microscopic changes suggest pathological similarities between, or coexistence of, RV-NC and AC. Therefore, the term arrhythmogenic right ventricular cardiomyopathy is somewhat misleading as isolated LV or biventricular involvement may be present and thus a broader term such as AC should be preferred. We describe an unusual case of AC associated with a NC in a 27-year-old man who had a history of permanent pacemaker 7 years ago due to second-degree atrioventricular block.

Keyword

Arrhythmogenic cardiomyopathy; Noncompaction cardiomyopathy

MeSH Terms

Adult
Arrhythmogenic Right Ventricular Dysplasia
Atrioventricular Block
Cardiomyopathies*
Diagnosis
Echocardiography*
Humans

Figure

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  Fig. 1 The 12-lead electrocardiogram showing (A) the typical of right ventricular pacing pattern, (B) the monomorphic ventricular tachycardia with a LBBB/inferior axis pattern; (C) 2:1 atrioventricular block (stars in C) with the epsilon (arrows in C and D) and negative T waves. LBBB: left bundle branch block.

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  Fig. 2 The endocardial bipolar electroanatomical mapping showing the earliest ventricular breaktrought site (A) and the scar area at preferentially RVOT (A) and a less extent degree at LVOT (B and C). RV: right ventricle, LV: left ventricle, RVOT: right ventricular outflow tract, LVOT: left ventricular outflow tract.

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  Fig. 3 The transthoracic echocardiography showing a compacted epicardial layer and a noncompacted endocardial layer that consisted of a prominent trabecular meshwork and deep intertrabecular recesses filled with blood from the ventricular cavity in the apical and mid portions of the LV (A-D), and an enlargement of RVOT with exaggerated trabecular pattern within the RV (D and E). RV: right ventricle, LV: left ventricle, LA: left atrium, RA: right atrium, RVOT: right ventricular outflow tract, PA: pulmonary artery, Ao: aorta.

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