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Unclassified Cardiomyopathies: Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) and Left Ventricular Non-Compaction Cardiomyopathy (LVNC)

  • Anita Sadeghpour
  • Azin Alizadehasl
Chapter

Abstract

Echocardiography has the main role in the diagnosis and management of arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy. An RV outflow tract dimension greater than 30 mm from the parasternal long-axis view has a the high sensitivity (89%) and specificity (86%) for the diagnosis of arrhythmogenic RV dysplasia. RV function is abnormal in 62%, and RV regional wall motion abnormalities is common—most frequently affecting the apex and the anterior wall. RV trabecular derangements, hyper-reflective moderator bands, and sacculations are present in about 54, 34, and 17%—respectively. In some patients, LV dilation and dysfunction also happen. The tricuspid regurgitation velocity is usually less than 2 m/s, suggesting an RV systolic pressure in the low-normal range. Contrast echocardiography is helpful in delineating RV morphology and contractility. Cardiac magnetic resonance imaging or computed tomography for fat in RV myocardium has been useful in some patients.

Isolated ventricular non-compaction occurs from the embryonic arrest of compaction, previously described as persistent intramyocardial sinusoids. It is characterized by segmental thickening in LV wall, comprising a compacted epicardial layer and a thickened endocardial layer with marked trabeculations and deep intertrabecular recesses. Complications associated with non-compaction cardiomyopathy include heart failure (53%), thromboembolic events (24%), ventricular tachycardia (41%), and sudden death.

Keywords

Echocardiography Arrhythmogenic right ventricular dysplasia/cardiomyopathy Isolated ventricular non-compaction Contrast echocardiography RV trabecular derangements 

Abbreviations

ARVD/C

Arrhythmogenic right ventricular dysplasia/cardiomyopathy

FAC

Fractional area change

ICD

Implantable cardioverter–defibrillator

LV

Left ventricular

LVNC

Left ventricular non-compaction

RV

Right ventricular

RVOT

Right ventricular outflow tract

TAPSE

Tricuspid annular plane systolic excursion

TDI

Tissue doppler imaging

TFC

Task force criteria

TTE

Transthoracic echocardiography

Supplementary material

Movie 24.1

ARVC; Para-sternal short axis view; dilated proximal part of RVOT (AVI 8970 kb)

Movie 24.2

ARVC; significant RV and RA enlargement; no confirmed shunt by contrast study (AVI 33078 kb)

Movie 24.3

(Related to Fig. 24.2): ARVC; severe RV enlargement with prominent moderator band (AVI 8509 kb)

Movie 24.4

LVNC; significant trabeculation (Spongy myocardium) (AVI 7481 kb)

Movie 24.5

LVNC; significant trabeculation with color flow in it (AVI 6810 kb)

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Anita Sadeghpour
    • 1
  • Azin Alizadehasl
    • 2
  1. 1.Professor of CardiologyEchocardiography Research Center, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical ScienceTehranIran
  2. 2.Associate Professor of Cardiology, Echocardiologist, Echocardiography and Cardiogenetic Research CentersCardio-Oncology Department, Rajaie Cardiovascular Medical and Research CenterTehranIran

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