Prolonged QT and Torsades de Pointes in a child with late-onset post-operative complete heart block.

A young child presented with syncope attacks. Late-onset post-operative complete atrioventricular block and Torsades de Pointes were diagnosed. She was treated with surgical epicardial pacemaker implantation. This report is the description of Torsades de Pointes due to late-onset post-operative complete atrioventricular block followed by R on T phenomenon in a child.

Cardiac resynchronization therapy in paediatric patients with congenital heart disease: single centre with 10 years of experience.

OBJECTIVES: In recent years, cardiac resynchronization therapy (CRT) has also started to be performed in the paediatric and CHD population. This study aimed to evaluate the efficacy of CRT in children with CHD. PATIENTS AND METHODS: Patients with CHD who underwent CRT treatment in our paediatric cardiology clinic between January, 2010 and January, 2020 were included in the study. Demographic findings, 12-lead electrocardiograms, echocardiograms, clinical characteristics, management strategies, and outcomes were reviewed systematically. RESULTS: The study population consisted of 18 CHD patients who had been treated with CRT for 10 years in our institution. The median age was 11 years (2.2-18 years) and the median weight was 39 kg (10-81 kg). Systemic ventricle was left ventricle in 13 patients, right ventricle in 4 patients, and 1 patient had single-ventricle physiology. CRT implantation indications were as follows: dysfunction after permanent pacemaker in 11 patients, dysfunction after left bundle branch block in 4 patients, and systemic ventricular dysfunction in 3 patients. CRT implantation techniques were epicardial (n = 13), hybrid (n = 4), and transvenous (n = 1) methods. QRS duration significantly decreased after CRT implantation (160 versus 124 m/second, p < 0.05). Median systemic ventricle ejection fraction (EF) significantly increased after the procedure (30 versus 50%, p < 0.05). Fourteen patients (78%) were responders, two patients (11%) were superresponders, and two patients (11%) were non-responders after the CRT treatment. One patient deceased during follow-up. Median follow-up duration was 40 months (6-117 months). CONCLUSION: When electromechanical dyssynchrony occurs in paediatric cases with CHD and developing heart failure, patients should be evaluated in terms of CRT to improve ventricular function. Alternative CRT therapy will be beneficial in these cases that do not improve clinically despite optimal medical treatment.

Is N-terminal pro-brain natriuretic peptide a reliable marker for body fluid status in children with chronic kidney disease?

INTRODUCTION: Brain natriuretic peptides, released in response to left ventricular stress, have a strong prognostic value in dialysis patients. However, their role in detecting abnormalities of fluid status is under debate; the relationship between volume status and brain natriuretic peptides (BNPs) differs among various studies. The aim of our study was to evaluate the clinical utility of N-terminal proBNP in the assessment of fluid status and cardiovascular risk in this setting. MATERIAL AND METHODS: The study included 65 children: 10 pre-dialysis, 13 hemodialysis, 12 peritoneal dialysis patients and 30 healthy controls. Volume status was determined by multifrequency bioimpedance and NT-pro-BNP, as well as echocardiography to estimate the left ventricle structure and function. RESULTS: The median log NT-proBNP values of hemodialysis and peritoneal dialysis patients were 3.66 (2.05-4.90) and 3.57 (2.51-4.13) pg/ml, respectively, and significantly higher compared with the control group (p < 0.001, p < 0.001). On simple correlation, NT-proBNP was correlated with markers of volume overload and cardiac dysfunction. On multivariate regression analysis, only left ventricle mass index (ß = 0.402, p = 0.003) and left atrium diameter (ß = 0.263, p = 0.018) were independently associated with NT-proBNP (adjusted R 2 of the model: 0.707, p < 0.001). CONCLUSIONS: Our research suggested that NT-proBNP, which was correlated with LV systolic and diastolic dysfunction and fluid overload as assessed by bioimpedance, can be used to evaluate cardiovascular states in a chronic kidney disease (CKD) population. From the early stages of CKD, periodic monitoring of NT-proBNP levels may be essential for early detection of patients with high risk of cardiovascular events, and for taking preventive intervention as soon as possible.

Accelerated idioventricular rhythm resulting in torsades de pointes and cardiac arrest in a child: successfully cryoablated in left'coronary cusp.

Known as a benign arrhythmia and normally requiring no specific treatment, accelerated idioventricular rhythm can rarely degenerate to a life-threatening arrhythmia. Here, we present a child with left coronary cusp-originating accelerated idioventricular rhythm, degenerating into torsades de pointes and resulting in cardiac arrest, which was ablated with a cryocatheter. An 11-year-old boy, followed due to asymptomatic accelerated idioventricular rhythm before, was referred to our department because he had experienced an aborted cardiac arrest during sleep. He had been resuscitated for 5 minutes. Twenty-four-hour Holter-ECG revealed incessant accelerated idioventricular rhythm, consisting up to 90% of the whole record and two torsades de pointes attacks, triggered by accelerated idioventricular rhythm-induced "R on T" phenomenon, and resulting in syncope and cardiac arrest. Transthoracic echocardiography revealed no structural cardiac defect but mild left ventricular systolic dysfunction with an ejection fraction of 45% and shortening fraction 23%. An electrophysiologic study was conducted, and accelerated idioventricular rhythm focus was mapped to left aortic coronary cusp. A cryocatheter with an 8-mm tip was preferred for successful ablation of the accelerated idioventricular rhythm focus, due to close neighbourhood to coronary ostium. The patient was discharged in 3 days without any premature ventricular contractions or accelerated idioventricular rhythm and with normalised cardiac functions. After 9 months on follow-up, he was still asymptomatic, without any premature ventricular contractions or accelerated idioventricular rhythm and with normal cardiac functions. Although the clinical course of accelerated idioventricular rhythm is known as benign, accelerated idioventricular rhythm can rarely degenerate to a life-threatening arrhythmia. In such cases, electrophysiologic study and catheter ablation are a good option in such cases with accelerated idioventricular rhythm for an ultimate cure.