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Ventricular repolarization abnormalities: the electrocardiographic track of cardiac tumoural involvement in an infant with tuberous sclerosis complex. A case report.

ABSTRACT: Background:Primary cardiac tumours are rare in children. Against this backdrop, Doppler echocardiogram is the main diagnostic procedure, while electrocardiogram (ECG) usually plays a secondary role, by detecting tumoural consequences as cardiac arrhythmias and chambers overload. We describe a case where an electrocardiographic sign was the cornerstone to diagnosis and surveillance of an infant with a cardiac rhabdomyoma. Case summary:A female infant was referred for cardiac evaluation to elucidate an electrocardiographic abnormality, detected during investigation of seizures. She had recently been diagnosed with epilepsy and was under three different anticonvulsants for appropriate control. Cardiovascular symptoms were absent. Skin inspection revealed hypochromic macules. Respiratory and cardiovascular examinations were normal, as well as laboratorial tests and chest radiography. Electrocardiogram (ECG) showed dome-shaped ST-segment elevation in V2 and V3. Transthoracic echocardiogram unveiled a single hyper-echogenic node (0.4 cm2) in the interventricular septum. Cardiac chambers had normal size and function and Doppler analysis was also normal. No specific medication was used to treat the tumour. During follow-up, she remained free of cardiac symptoms. Eighteen months after her first visit to the cardiologist, routine clinical assessment, ECG, and transthoracic Doppler echocardiogram normal results stated the spontaneous and complete involution of the tumoural lesion. Discussion:Convex ST-segment elevation, generally related to myocardial injury, is unusual in paediatric patients. Once it occurs in asymptomatic individuals within this age bracket, exclusion of cardiac tumours is mandatory. However, data regarding the accuracy of such electrocardiographic marker in this clinical setting are still to be defined.

SUBMITTER: Facin M

PROVIDER: S-EPMC7180701 | biostudies-literature | 2020 Apr

REPOSITORIES: biostudies-literature

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Ventricular repolarization abnormalities: the electrocardiographic track of cardiac tumoural involvement in an infant with tuberous sclerosis complex. A case report.

Facin Mirella M Pastore Carlos Alberto CA Samesima Nelson N Pereira Filho Horacio Gomes HG

European heart journal. Case reports 20200224 2

<h4>Background</h4>Primary cardiac tumours are rare in children. Against this backdrop, <i>Doppler</i> echocardiogram is the main diagnostic procedure, while electrocardiogram (ECG) usually plays a secondary role, by detecting tumoural consequences as cardiac arrhythmias and chambers overload. We describe a case where an electrocardiographic sign was the cornerstone to diagnosis and surveillance of an infant with a cardiac rhabdomyoma.<h4>Case summary</h4>A female infant was referred for cardiac ...[more]

PMID: 32352074

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Project description:Background Dispersion and gradients in repolarization have been associated with life-threatening arrhythmias, but are difficult to quantify precisely from surface electrocardiography. The objective of this study was to evaluate electrocardiographic imaging (ECGI) to noninvasively detect repolarization-based abnormalities. Methods and Results Ex vivo data were obtained from Langendorff-perfused pig hearts (n=8) and a human donor heart. Unipolar electrograms were recorded simultaneously during sinus rhythm from an epicardial sock and the torso-shaped tank within which the heart was suspended. Regional repolarization heterogeneities were introduced through perfusion of dofetilide and pinacidil into separate perfusion beds. In vivo data included torso and epicardial potentials recorded simultaneously in anesthetized, closed-chest pigs (n=5), during sinus rhythm, and ventricular pacing. For both data sets, ECGI accurately reconstructed T-wave electrogram morphologies when compared with those recorded by the sock (ex vivo: correlation coefficient, 0.85 [0.52-0.96], in vivo: correlation coefficient, 0.86 [0.52-0.96]) and repolarization time maps (ex-vivo: correlation coefficient, 0.73 [0.63-0.83], in vivo: correlation coefficient, 0.76 [0.67-0.82]). ECGI-reconstructed repolarization time distributions were strongly correlated to those measured by the sock (both data sets, R2 ≥0.92). Although the position of the gradient was slightly shifted by 8.3 (0-13.9) mm, the mean, max, and SD between ECGI and recorded gradient values were highly correlated (R2=0.87, 0.75, and 0.86 respectively). There was no significant difference in ECGI accuracy between ex vivo and in vivo data. Conclusions ECGI reliably and accurately maps potentially critical repolarization abnormalities. This noninvasive approach allows imaging and quantifying individual parameters of abnormal repolarization-based substrates in patients with arrhythmogenesis, to improve diagnosis and risk stratification.

Project description:Background Early repolarization pattern (ERP) is considered a common training-related and benign ECG finding in young adult athletes. Few data exist on ERP in the pediatric athletes population. Therefore, we aimed to evaluate the ERP prevalence, characteristics, and prognosis in pediatric athletes aged ≤16 years. Methods and Results Eight-hundred eighty-six consecutive pediatric athletes engaged in 17 different sports (mean age, 11.7±2.5 years; 7-16 years) were enrolled and prospectively evaluated with medical history, physical examination, resting and exercise ECGs, and transthoracic echocardiography during their preparticipation screening. Known cardiovascular diseases associated with sudden cardiac death was considered exclusion criteria. Athletes were followed up yearly for 4 years. The prevalence of ERP was 117 (13.2%), equally distributed in both sexes (P=0.072), irrespectively of body mass index and classification of sports. The most common ERP localizations were inferolateral and inferior leads (53.8% and 27.3%, respectively). Notching J-point morphology was the most prevalent (70%), and rapidly ascending ST elevation (96%) was the most common ST-segment morphology. Athletes with ERP were older (P<0.001) had lower rest and recovery heart rates (P<0.001), increased precordial and limb R-wave voltages (P<0.001), increased R/S Sokolow index (P<0.001), and longer PR interval (P=0.006) in comparison with the athletes without ERP. Neither major cardiovascular nor arrhythmic events, nor sudden cardiac death were recorded over a median follow-up of 4.2 years. One hundred seventeen (80.3%) athletes with ERP exhibited a persistent ERP. ERP localization and J-point morphology changed during follow-up in 11 (11.7%) and 17 (18%) of athletes, respectively. Conclusions ERP is common in pediatric athletes. It was mostly located in the inferolateral leads and associated with concave ascending ST segment with other training-related ECG changes. The lack of either sudden cardiac death or cardiomyopathies linked to sudden cardiac death over follow-up suggests that in pediatric athletes, ERP may be considered a benign training-related ECG phenomenon with a potential dynamic pattern.

Dataset Information

Ventricular repolarization abnormalities: the electrocardiographic track of cardiac tumoural involvement in an infant with tuberous sclerosis complex. A case report.

Publications

Ventricular repolarization abnormalities: the electrocardiographic track of cardiac tumoural involvement in an infant with tuberous sclerosis complex. A case report.

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