Abstract

BackgroundNoncompaction cardiomyopathy (NCCM) is characterized by a prominent trabecular meshwork and deep intertrabecular recesses. Although systolic dysfunction is common, limited information is available on differences in wall motion of the normal compacted and noncompacted segments. The purpose of this study was to assess radial wall motion and longitudinal wall velocity in patients with NCCM, according to the extent and severity of noncompaction.MethodsThe study comprised 29 patients in sinus rhythm (age 41 ± 15 years, 15 men), who fulfilled stringent diagnostic criteria for NCCM and compared to 29 age and gender matched healthy controls. Segmental radial wall motion of all compacted and noncompacted segments was assessed with the standard visual wall motion score index and longitudinal systolic (Sm) wall velocity with tissue Doppler imaging of the mitral annulus. For each LV wall a normalized Sm value was calculated. The extent and severity of NC in each LV segment was assessed both in a qualitative and quantitative manner.ResultsHeart failure was the primary clinical presentation in half of the patients. NCCM patients had a wall motion score index of 1.68 ± 0.43 and a normalized Sm of 82 ± 20%. The total and maximal noncompaction scores were not related to the wall motion score index and the normalized Sm. NCCM patients with and without heart failure had similar total and maximal noncompaction scores.ConclusionsIn NCCM patient's radial wall motion and longitudinal LV wall velocity is impaired but not related to the extent or severity of noncompaction.

Highlights

  • Noncompaction cardiomyopathy (NCCM) is characterized by a prominent trabecular meshwork and deep intertrabecular recesses

  • Noncompaction of the left ventricle (LV) or noncompaction cardiomyopathy (NCCM), is a relatively new clinico-pathologic entity, first described by Engberding and Bender in 1984 [1]. It is characterized by a prominent trabecular meshwork and deep intertrabecular recesses communicating with the LV cavity and is thought to be caused by an arrest of normal embryogenesis of the myocardium [2,3]

  • The advantage of assessment of mitral annular velocities is that the region of interest from which the measurements are taken is not involved in the process of NC but the measurements reflect function of walls involved in the process of NC

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Summary

Introduction

Noncompaction cardiomyopathy (NCCM) is characterized by a prominent trabecular meshwork and deep intertrabecular recesses. The purpose of this study was to assess radial wall motion and longitudinal wall velocity in patients with NCCM, according to the extent and severity of noncompaction. Noncompaction of the left ventricle (LV) or noncompaction cardiomyopathy (NCCM), is a relatively new clinico-pathologic entity, first described by Engberding and Bender in 1984 [1] It is characterized by a prominent trabecular meshwork and deep intertrabecular recesses communicating with the LV cavity and is thought to be caused by an arrest of normal embryogenesis of the myocardium [2,3]. Still limited information is available on differences in wall motion of the normal compacted (C) and abnormal NC segments [4,5,6]. The purpose of this study was to assess radial wall motion and longitudinal wall velocity in patients with NCCM, according to the extent and severity of NC

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