Electrocardiographic Parameters Associated with Adverse Outcomes in Children with Cardiomyopathies

Cardiomyopathies have a low prevalence in children and thus may lead to malignant ventricular arrhythmias or the progression of heart failure, resulting in death. In adults, the QRS-T angle derived from ECG has been associated with adverse outcomes in patients with hypertrophic and dilated cardiomyopathies. We aimed to assess the electrocardiographic parameters, including QRS-T angle, associated with adverse cardiac events in children with cardiomyopathies. Forty-two children with cardiomyopathies were included in this study: 19 with dilated cardiomyopathy, 17 with hypertrophic cardiomyopathy, and 6 with left ventricular non-compaction. Additionally, 19 control subjects were recruited. In terms of ECG parameters, the QRS-T angle was significantly greater among patients with adverse outcomes compared to patients without the end points of the study (133° vs. 65°, p < 0.001). On Kaplan–Meier survival curves, QRS-T angle > 120°, increased serum concentrations of NT-proBNP and troponin I levels as well as greater NYHA or Ross scale were associated with the greatest risk of unfavorable outcome. The QRS-T angle appears to be a valuable component of 12-lead ECG interpretation, and might be helpful in outlining patients with the greatest cardiovascular risk. Additionally, serum biomarkers such as NT-proBNP (p = 0.003) and troponin (p < 0.001) are useful in outlining patients with the worst survival.

[1]  M. Emdin,et al.  Magnetic Resonance for Differential Diagnosis of Left Ventricular Hypertrophy: Diagnostic and Prognostic Implications , 2022, Journal of clinical medicine.

[2]  A. Hierlemann,et al.  Deciphering the pathogenic role of a variant with uncertain significance for short QT and Brugada syndromes using gene‐edited human‐induced pluripotent stem cell‐derived cardiomyocytes and preclinical drug screening , 2021, Clinical and translational medicine.

[3]  J. Haas,et al.  Identification of SCN5a p.C335R Variant in a Large Family with Dilated Cardiomyopathy and Conduction Disease , 2021, International journal of molecular sciences.

[4]  A. Saguner,et al.  Preclinical short QT syndrome models: studying the phenotype and drug-screening. , 2021, Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology.

[5]  R. D. de Boer,et al.  Usefulness of High-Sensitivity Cardiac Troponin T to Predict Long-Term Outcome in Patients with Hypertrophic Cardiomyopathy. , 2021, The American journal of cardiology.

[6]  R. Weintraub,et al.  The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy , 2021, European journal of preventive cardiology.

[7]  I. Akin,et al.  Different genotypes of Brugada syndrome may present different clinical phenotypes: electrophysiology from bench to bedside. , 2021, European heart journal.

[8]  A. Bogers,et al.  Predicting outcome in children with dilated cardiomyopathy: the use of repeated measurements of risk factors for outcome , 2021, ESC heart failure.

[9]  K. Nassenstein,et al.  QRS-T angle in patients with Hypertrophic Cardiomyopathy - A comparison with Cardiac Magnetic Resonance Imaging , 2021, International journal of medical sciences.

[10]  C. Pappone,et al.  The omics of channelopathies and cardiomyopathies: what we know and how they are useful , 2020, European heart journal supplements : journal of the European Society of Cardiology.

[11]  S. Aslan,et al.  Impaired repolarization parameters may predict fatal ventricular arrhythmias in patients with hypertrophic cardiomyopathy (from the CILICIA Registry). , 2020, Journal of electrocardiology.

[12]  P. Platonov,et al.  Vectorcardiography Findings Are Associated with Recurrent Ventricular Arrhythmias and Mortality in Patients with Heart Failure Treated with Implantable Cardioverter-Defibrillator Device , 2020, Cardiology.

[13]  C. Lenz,et al.  Intronic CRISPR Repair in a Preclinical Model of Noonan Syndrome–Associated Cardiomyopathy , 2020, Circulation.

[14]  R. Płoski,et al.  Can Circulating Cardiac Biomarkers Be Helpful in the Assessment of LMNA Mutation Carriers? , 2020, Journal of clinical medicine.

[15]  M. Türe,et al.  The relationship between electrocardiographic data and mortality in children diagnosed with dilated cardiomyopathy , 2020, European Journal of Pediatrics.

[16]  S. Colan,et al.  Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association. , 2019, Circulation.

[17]  Daniel P. Morin,et al.  Sudden cardiac death in nonischemic cardiomyopathy. , 2019, Progress in cardiovascular diseases.

[18]  Jason Imundo,et al.  Pediatric noncompaction patients with high spatial QRS‐T angles are at increased risk for ventricular tachycardia , 2018, Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc.

[19]  J. Utikal,et al.  Ion Channel Dysfunctions in Dilated Cardiomyopathy in Limb-Girdle Muscular Dystrophy , 2018, Circulation. Genomic and precision medicine.

[20]  Michael J Ackerman,et al.  ECG-derived spatial QRS-T angle is strongly associated with hypertrophic cardiomyopathy. , 2017, Journal of electrocardiology.

[21]  Biao Xu,et al.  Prognostic Significance of Frontal QRS-T Angle in Patients with Idiopathic Dilated Cardiomyopathy , 2016, Chinese medical journal.

[22]  J. Alejos,et al.  Lower spatial QRS-T angle rules out sustained ventricular arrhythmias in children with hypertrophic cardiomyopathy , 2016, Cardiology in the Young.

[23]  C. Almond,et al.  Electrocardiographic repolarization abnormalities and increased risk of life-threatening arrhythmias in children with dilated cardiomyopathy. , 2016, Heart rhythm.

[24]  L. Mestroni,et al.  In Hypertrophic Cardiomyopathy, the Spatial Peaks QRS‐T Angle Identifies Those With Sustained Ventricular Arrhythmias , 2016, Clinical cardiology.

[25]  J. Carlin,et al.  Sudden death in childhood cardiomyopathy: results from a long-term national population-based study. , 2015, Journal of the American College of Cardiology.

[26]  Andrew C Oehler,et al.  QRS‐T Angle: A Review , 2014, Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc.

[27]  F. Rutten,et al.  2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). , 2014, European heart journal.

[28]  W. Hua,et al.  The prognostic use of serum concentrations of cardiac troponin-I, CK-MB and myoglobin in patients with idiopathic dilated cardiomyopathy. , 2014, Heart & lung : the journal of critical care.

[29]  T. Sugiura,et al.  Significance of high-sensitivity cardiac troponin T in hypertrophic cardiomyopathy. , 2013, Journal of the American College of Cardiology.

[30]  A. Dawnay,et al.  Relation between serum N-terminal pro-brain natriuretic peptide and prognosis in patients with hypertrophic cardiomyopathy. , 2013, European heart journal.

[31]  M. Horie,et al.  Prognostic role of high-sensitivity cardiac troponin T in patients with nonischemic dilated cardiomyopathy. , 2011, Circulation journal : official journal of the Japanese Circulation Society.

[32]  S. Colan,et al.  Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. , 2010, Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography.

[33]  A. Kadish,et al.  Prognostic Value and Temporal Behavior of the Planar QRS-T Angle in Patients With Nonischemic Cardiomyopathy , 2008, Circulation.

[34]  P. Elliott,et al.  B-type natriuretic peptide predicts disease severity in children with hypertrophic cardiomyopathy , 2007, Heart.

[35]  P. Kaufmann,et al.  Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy , 2001, Heart.

[36]  A. Sittig,et al.  Reconstruction of the Frank vectorcardiogram from standard electrocardiographic leads: diagnostic comparison of different methods. , 1990, European heart journal.

[37]  S. Daniels,et al.  Plasma norepinephrine levels in infants and children with congestive heart failure. , 1987, The American journal of cardiology.

[38]  Nandita Sharma,et al.  Visual transform applications for estimating the spatial QRS-T angle from the conventional 12-lead ECG: Kors is still most Frank. , 2014, Journal of electrocardiology.