The Role of Speckle Tracking Echocardiography in the Evaluation of Common Inherited Cardiomyopathies in Children and Adolescents: A Systematic Review.

Dan M. Dorobantu,A. Graham Stuart,Curtis A. Wadey,Nurul H. Amir,Guido E. Pieles,Craig A. Williams

doi:10.3390/diagnostics11040635

Abstract

Speckle tracking echocardiography (STE) has gained importance in the evaluation of adult inherited cardiomyopathies, but its utility in children is not well characterized. We conducted a systematic review to evaluate the role of STE in pediatric inherited cardiomyopathies. PubMed, EMBASE, Web of Science, Scopus, CENTRAL and CINAHL databases were searched up to May 2020, for terms related to inherited cardiomyopathies and STE. Included were dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), left ventricular non-compaction (LVNC) and arrhythmogenic cardiomyopathy (ACM). A total of 14 cohorts were identified, of which six were in DCM, four in HCM, three in LVNC and one in ACM. The most commonly reported STE measurements were left ventricular longitudinal strain (Sl), circumferential strain (Sc), radial strain (Sr) and rotation/torsion/twist. Sl, Sc and were abnormal in all DCM and LVNC cohorts, but not in all HCM. Apical rotation and twist/torsion were increased in HCM, and decreased in LVNC. Abnormal STE parameters were reported even in cohorts with normal non-STE systolic/diastolic measurements. STE in childhood cardiomyopathies can detect early changes which may not be associated with changes in cardiac function detectable by non-STE methods. Longitudinal and circumferential strain should be introduced in the cardiomyopathy echocardiography protocol, reflecting current practice in adults.

Highlights

The publication of recent registry-based reports has led to an increasing interest in the pediatric inherited cardiomyopathies, including hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), left ventricle non-compaction cardiomyopathy (LVNC)
Rare [1,2,3], they carry a significant mortality and morbidity burden: DCM is the main indication for pediatric heart transplant [1]; 7% of older children or adolescents with HCM died or received a heart transplant [2]; isolated LVNC, diagnosed in late childhood or adolescence, has 6% mortality at 5 years [3]; pediatric arrhythmogenic cardiomyopathy (ACM) has been associated with a high prevalence of life threatening arrhythmia at first presentation, compared to adults [4]
Mean age ranged from 4.5–15 years, and males accounted from 46–86% of samples

Summary

Introduction

Diagnosis and follow-up of cardiomyopathy patients can be complicated by heterogeneity in clinical presentation and etiology, as well as overlap with acquired causes, such as myocarditis, or normal variants, such as athletes. Two dimensional speckle tracking echocardiography (STE) is used routinely in the management of adult cardiomyopathies and has been associated with clinical outcomes in HCM [5], DCM [6], LVNC [7] and ACM [8], aiding screening and early diagnosis [9,10,11,12,13]. Despite overwhelming data from the adult cardiomyopathy practice, STE has only recently started to gain importance on the pediatric side

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