Floris E.A. Udink ten Cate

Dilated cardiomyopathy in children with ventricular preexcitation: the location of the accessory pathway is predictive of this association.

BACKGROUND Ventricular preexcitation may be associated with dilated cardiomyopathy, even in the absence of recurrent and incessant tachycardia. METHODS This report describes the clinical and electrophysiologic characteristics of 10 consecutive children (6 males), with median age of 8 years (range, 1-17 years), who presented with dilated cardiomyopathy and overt ventricular preexcitation on the 12-lead electrocardiogram. Incessant tachycardia as the cause of dilated cardiomyopathy could be excluded. Coronary angiography, right ventricular endomyocardial biopsy (4/10 patients), and metabolic and microbiologic screening were nondiagnostic. RESULTS The electrocardiograms suggested right-sided pathways in all patients. A right-sided accessory pathway was demonstrated in 8 patients during invasive electrophysiologic study (superoparaseptal, n = 5; septal, n = 2; fasciculoventricular, n = 1). All pathways were successfully ablated (radiofrequency ablation in 7, cryoablation in 1). Two patients had spontaneous loss of ventricular preexcitation during follow-up. Left ventricular (LV) function completely recovered after a loss of preexcitation in all patients. CONCLUSIONS Right-sided accessory pathways with overt ventricular preexcitation and LV dyssynchrony may cause dilated cardiomyopathy. An association between such pathways and dilated cardiomyopathy is suggested by the rapid normalization of ventricular function and reverse LV remodeling after a loss of ventricular preexcitation.

Pacemaker therapy in isolated congenital complete atrioventricular block.

BREUR, J.M.P.J., et al.: Pacemaker Therapy in Isolated Congenital Complete Atrioventricular Block. The aim of this study was to evaluate the effect of pacemaker (PM) therapy in patients with isolated congenital complete atrioventricular block (CCAVB). Patients with CCAVB eventually qualify for PM implantation, however, timing remains controversial. Retrospective evaluation of left ventricular end‐diastolic diameter (LVEDD), shortening fraction (SF), and cardiothoracic ratio (CTR) in 149 CCAVB patients, before, at, and after PM implantation was carried out. LVEDD shows an average increase of 0.48%/month in non‐PM patients, and an average decrease of 0.88%/month in PM patients. SF shows an average increase of 0.10%/month in non‐PM, and an average decrease of 0.32%/month in PM patients. CTR shows an average increase of 0.02%/month in non‐PM, and an average decrease of 0.19%/month in PM patients. The difference between the non‐PM and PM groups is significant (P = 0.05) for all variables. Symptomatic patients show no significant change in LVEDD after PM therapy (from 66.5% before to 68.5% after PM therapy). Asymptomatic patients do show a significant (P < 0.001) decrease in LVEDD after PM therapy (from 78.4% before to 73.3% after PM therapy). CTR does not differ significantly between symptomatic and asymptomatic patients before PM therapy (58% and 57%, respectively). CTR does differ significantly (P < 0.001) between symptomatic and asymptomatic patients after PM therapy (52% and 48%, respectively). Heart size and SF are increased in most patients with isolated CCAVB. PM implantation is associated with a decrease in heart size and normalization of SF in most patients. Indications for PM therapy in children may require reevaluation in asymptomatic patients with increased cardiac size and decreased cardiac function.

Speckle tracking echocardiography in childhood myocarditis: Spontaneous reversal of severe left ventricular rotational abnormalities during follow-up

Network Heart Failure, funded by the Federal Ministry of Education and Research, grant no. 01GI0205. The authors are indebted to Mike T. John and Wolfgang Micheelis for the kind permission to use the reference data of the OHIP-G questionnaire [12] for validation purposes. The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology (Shewan and Coats 2010;144:1–2). They declare no conflict of interest.

Controversies in arrhythmias and arrhythmic syndromes of active children and young adults

Important advances in the diagnosis and therapy of various arrhythmic disorders have been made in the last two decades. These, in turn, have necessitated a re-examination of current practice guidelines, with a view to deciding on optimal management of young patients with suspected or proven arrhythmia syndromes and in assessing the risk of adverse arrhythmic events during sport participation. There has also been a concomitant emphasis on identifying individuals at risk by nationwide screening programs using the ECG and excluding them from competitive sport. This review identifies some of these issues, looks at the data critically and offers some suggestions for current care and future research.

Apical Sparing of Longitudinal Strain, Left Ventricular Rotational Abnormalities, and Short-Axis Dysfunctionin Primary Hyperoxaluria Type 1

A 24-year-old woman with end-stage renal failure because of primary hyperoxaluria type 1 was evaluated in our hospital for systemic calcium oxalate deposition in the course of long-term (5 years) hemodialysis therapy. Diagnosis of primary hyperoxaluria type 1, a hereditary cause of calcium oxalate kidney stones or progressive nephrocalcinosis that frequently results in end-stage renal failure,1 was made by liver biopsy (reduced alanine:glyoxylate aminotransferase activity, 3.0 µmol/h per milligram protein; normal, 19.1–47.9) and by genetic testing (homozygosity for the c.302 T>C, AGXT mutation). Her plasma oxalate level on regular hemodialysis (3× per week over 4 hours) was increased (86 µmol/L predialysis; normal, <10 µmol/L). Transthoracic echocardiography revealed increased wall thickness of the left ventricle (LV; Figure 1). The LV was mildly dilated and showed a decreased systolic function. Flow across the mitral valve demonstrated a restrictive filling pattern. Moreover, the myocardium had a characteristic echo-dense granular sparkling appearance (Movie in the online-only Data Supplement).2 Both atria were enlarged. Moderate tricuspid regurgitation was present. A right ventricle systolic pressure of 62 …

Stenting the arterial duct in neonates and infants with congenital heart disease and duct-dependent pulmonary blood flow

The primary aim of this multi‐institutional study was to describe our 18‐year experience of ductal stenting (DS) in infants with a duct‐dependent pulmonary circulation. The secondary aim sought to identify a subgroup of patients who may benefit the most using this evolving technique.

Screening children with suspected myocarditis for global and regional myocardial dysfunction using two-dimensional speckle tracking echocardiography: Is it of use?

components in the QRS complex by thewavelet transform inpatientswith idiopathic dilated cardiomyopathy. Jpn Circ J 1999;63:25–32. [13] Peters S, Trummel M, Koehler B. QRS fragmentation in standard ECG as a diagnostic marker of arrhythmogenic right ventricular dysplasiacardiomyopathy. Heart Rhythm 2008;5:1417–21. [14] Morita H, Kusano KF, Miura D, et al. Fragmented QRS as a marker of conduction abnormality and a predictor of prognosis of Brugada syndrome. Circulation 2008;118: 1697–704. [15] Wang DD, Buerkel DM, Corbett JR, Gurm HS. Fragmented QRS complex has poor sensitivity in detecting myocardial scar. Ann Noninvasive Electrocardiol 2010;15: 308–14. [16] Coats AJS, Shewan LG. Ethics in the authorship and publishingof scientific articles. Int J Cardiol 2011;153:239–40.

Stenting the arterial duct in neonates and infants with congenital heart disease and duct-dependent pulmonary blood flow: A multicenter experience of an evolving therapy over 18 years: Ductal Stenting in Congenital Heart Disease

The primary aim of this multi‐institutional study was to describe our 18‐year experience of ductal stenting (DS) in infants with a duct‐dependent pulmonary circulation. The secondary aim sought to identify a subgroup of patients who may benefit the most using this evolving technique.