Anthony C. McCanta

Predictors of myocardial recovery in pediatric tachycardia-induced cardiomyopathy

BACKGROUND Tachycardia-induced cardiomyopathy (TIC) carries significant risk of morbidity and mortality, although full recovery is possible. Little is known about the myocardial recovery pattern. OBJECTIVE The purpose of this study was to determine the time course and predictors of myocardial recovery in pediatric TIC. METHODS An international multicenter study of pediatric TIC was conducted. Children ≤18 years with incessant tachyarrhythmia, cardiac dysfunction (left ventricular ejection fraction [LVEF] <50%), and left ventricular (LV) dilation (left ventricular end-diastolic dimension [LVEDD] z-score ≥2) were included. Children with congenital heart disease or suspected primary cardiomyopathy were excluded. Primary end-points were time to LV systolic functional recovery (LVEF ≥55%) and normal LV size (LVEDD z-score <2). RESULTS Eighty-one children from 17 centers met inclusion criteria: median age 4.0 years (range 0.0-17.5 years) and baseline LVEF 28% (interquartile range 19-39). The most common arrhythmias were ectopic atrial tachycardia (59%), permanent junctional reciprocating tachycardia (23%), and ventricular tachycardia (7%). Thirteen required extracorporeal membrane oxygenation (n = 11) or ventricular assist device (n = 2) support. Median time to recovery was 51 days for LVEF and 71 days for LVEDD. Two (4%) underwent heart transplantation, and 1 died (1%). Multivariate predictors of LV systolic functional recovery were age (hazard ratio [HR] 0.61, P = .040), standardized tachycardia rate (HR 1.16, P = .015), mechanical circulatory support (HR 2.61, P = .044), and LVEF (HR 1.33 per 10% increase, p=0.005). For normalization of LV size, only baseline LVEDD (HR 0.86, P = .008) was predictive. CONCLUSION Pediatric TIC resolves in a predictable fashion. Factors associated with faster recovery include younger age, higher presenting heart rate, use of mechanical circulatory support, and higher LVEF, whereas only smaller baseline LV size predicts reverse remodeling. This knowledge may be useful for clinical evaluation and follow-up of affected children.

The spatial QRS-T angle outperforms the Italian and Seattle ECG-based criteria for detection of hypertrophic cardiomyopathy in pediatric patients

INTRODUCTION The spatial peaks QRS-T angle has been shown to differentiate adult patients with hypertrophic cardiomyopathy (HCM) from controls. We hypothesized that the spatial peaks QRS-T angle would, in isolation, be more accurate than the Italian 12-lead ECG Pre-participation Screening criteria or the Seattle criteria for detecting hypertrophic cardiomyopathy (HCM) in pediatric patients. METHODS A retrospective study of pediatric patients with HCM compared to age and gender-matched control patients was undertaken. Significance, odds ratios, sensitivity and specificity of HCM detection of the visually derived spatial peaks QRS-T angle were compared to those of traditional 12-lead ECG criteria using: 1) Italys National Pre-participation Screening Programme criteria; and 2) described criteria from Seattle. RESULTS ECG results from 130 pediatric HCM patients (14.2±4.4years) were compared to 470 control patients (normal echocardiograms, mean age 13.4±4.6years). Mean±standard deviation (SD) values for spatial peaks QRS-T angles were 120.4±40.7 and 21.3±13.7 degrees for HCM and controls, respectively (P<0.001). A spatial peaks QRS-T angle cutoff value of >54.9 degrees yielded greater sensitivity and specificity (93.1% and 98.7%, respectively) for detecting HCM over ECG criteria from Italy (68.5% and 48.1%, respectively) or Seattle (64.6% and 78.9%, respectively) with odds ratios at 1039.70 (95% CI 363.03 to 2977.67), 2.01 (95% CI 1.33 to 3.04) and 6.84 (4.49-10.44), respectively. CONCLUSION In our cohort, a visually derived spatial peaks QRS-T angle has increased sensitivity and specificity for detection of HCM in pediatric patients compared to currently utilized Italian or Seattle ECG criteria.

Constrictive pericarditis secondary to infection with Mycoplasma pneumoniae.

Pericardial effusions can be insidious, variable in presentation, and may result from a wide variety of causes. We report here a rare case of pericardial effusion in a pediatric patient secondary to infection with Mycoplasma pneumoniae that progressed to cardiac tamponade and constrictive pericarditis. The differential diagnosis of pericardial effusion is reviewed as well as current treatments for pericardial effusions and constrictive pericarditis.

Vectorcardiographic predictors of ventricular arrhythmia inducibility in patients with tetralogy of Fallot

INTRODUCTION Vectorcardiography (VCG) may have predictive value in Tetralogy of Fallot (TOF) patients undergoing ventricular arrhythmia inducibility (VAI) electrophysiology studies (EPs). MATERIALS AND METHODS Blinded, retrospective analyses of 37 adult TOF patients undergoing EPs prior to pulmonary valve replacements were performed (21 female, median age 37years). VAI was evaluated from EPs and resting 12-lead electrocardiograms, respectively using QRS and heart rate adjusted Q-T intervals, spatial QRS-T angles (peaks), T-wave and QRS-wave (QRSwave vc) component vector root mean squares. Differences were assessed (Student t-tests, Mann Whitney U-tests, Analysis of Variance). Relative risks were calculated. RESULTS 16 patients had VAI (6 monomorphic, 10 polymorphic). Only the QRSwave vc showed significant differences between those with and without VAI, 10.5±2.4 dmV vs. 13.9±4.5dmV, respectively (p=0.002), area under the ROC curve of 0.78 and relative risk of 2.52. CONCLUSION VCG evidence of depolarization differences was significant between TOF patients with and without inducible VA.

Incidental Dual Atrioventricular Nodal Physiology in Children and Adolescents: Clinical Follow‐Up and Implications

Background:  Dual atrioventricular (AV) nodal physiology is a substrate for the development of AV nodal reentrant tachycardia (AVNRT). However, the risk of developing AVNRT in patients with dual AV nodal physiology is not known. The purpose of this study is to identify the risk of developing AVNRT in children and adolescents with incidental findings of dual AV nodal physiology after accessory pathway ablation.

Cardiomyopathy in a child with neutropenia and motor delay.

A 17-month boy with history of neutropenia and gross motor regression was found to have cardiomyopathy upon admission. He was diagnosed with Barth syndrome: dilated cardiomyopathy, neutropenia, skeletal myopathy, decreased stature, and 3-methylglutaconic aciduria, confirmed by tafazzin gene deletion. This diagnosis should be considered in boys with unexplained neutropenia.

Tachyarrhythmia Following Norwood Operation A Single-Center Experience

Background: The purpose of this study was to characterize tachyarrhythmias in children following the Norwood procedure. Methods: This is a single-center retrospective study including all children who underwent stage I Norwood procedure (n = 98; January 2003-September 2011). The primary outcome measure is the development of tachyarrhythmia during hospitalization after the Norwood procedure. Secondary aims include quantification of mortality in patients with tachyarrhythmias and evaluation of potential risk factors for the development of tachyarrhythmia. Results: Tachyarrhythmia occurred in 33 (34%) of 98 patients. The median time to onset of tachyarrhythmia was ten days (0-47 days). Tachyarrhythmia conferred no increase in overall mortality (P = .45), including operative mortality (P = .37) or interstage mortality (P = 1.00). There was no significant difference in the incidence of arrhythmia based on demographic, anatomic, or surgical variables, including shunt type (P = .23) except that patients with tachyarrhythmias were slightly larger (median weight 3.2 kg) at the time of stage I than those without tachyarrhythmia (median weight 2.93 kg; P = .02]. The odds of arrhythmia in males were 8.7 times higher than that in females (95% confidence interval 2.9-31.3; P < .0001). Conclusions: Postoperative tachyarrhythmia is common, occurring in 34% of patients after the Norwood operation. Onset of tachyarrhythmia occurred later after the Norwood operation than reported previously, and male gender is a risk factor. Further studies to elucidate the etiology and the timing of tachyarrhythmias after the Norwood procedure are necessary.

The fate of nontargeted endocardial leads during the extraction of one or more targeted leads in pediatrics and congenital heart disease.

Extraction of targeted endocardial leads can lead to inadvertent damage or dislodgement of nontargeted leads, though the frequency of this has not been reported in pediatrics and congenital heart disease (CHD).

Lower spatial QRS-T angle rules out sustained ventricular arrhythmias in children with hypertrophic cardiomyopathy.

Introduction The spatial peaks QRS-T angle accurately distinguishes children with hypertrophic cardiomyopathy from their healthy counterparts. The spatial peaks QRS-T angle is also useful in risk stratification for ventricular arrhythmias. We hypothesised that the spatial peaks QRS-T angle would be useful for the prediction of ventricular arrhythmias in hypertrophic cardiomyopathy patients under 23 years of age. METHODS Corrected QT interval and spatial peaks QRS-T angles were retrospectively assessed in 133 paediatric hypertrophic cardiomyopathy patients (12.4±6.6 years) with versus without ventricular arrhythmias of 30 seconds or longer. Significance, positive/negative predictive values, and odds ratios were calculated based on receiver operating characteristic curve cut-off values. RESULTS In total, 10 patients with ventricular arrhythmias were identified. Although the corrected QT interval did not differentiate those with versus without ventricular arrhythmias, the spatial peaks QRS-T angle did (151.4±19.0 versus 116.8±42.6 degrees, respectively, p<0.001). At an optimal cut-off value (124.1 degrees), the positive and negative predictive values of the spatial peaks QRS-T angle were 15.4 and 100.0%, respectively, with an odds ratio of 25.9 (95% CI 1.5-452.2). CONCLUSION In children with hypertrophic cardiomyopathy, the spatial peaks QRS-T angle is associated with ventricular arrhythmia burden with high negative predictive value and odds ratio.

Noninvasive predictors of perioperative atrial arrhythmias in patients with tetralogy of Fallot undergoing pulmonary valve replacement

Patients with tetralogy of Fallot (TOF) have increased risk of atrial arrhythmias.