Sudden cardiac death poses a significant risk in patients with surgically repaired tetralogy of Fallot. Despite extensive research, risk stratification practices vary. This study surveyed the Pediatric and Adult Congenital Electrophysiology Society to identify these differences. Results showed diverse practices in indications, methods, and interpretation of electrophysiology studies, highlighting a need for standardised algorithms to improve patient outcomes.

Hyperthermia is defined as an elevated body temperature above the normal range due to a failure of heat regulatory mechanisms. In addition to its effects on other organ systems, hyperthermia is associated with profound cardiovascular effects. We report the sentinel case of a 6-year-old girl with structurally and electrically normal heart, who presented with life-threatening hyperpyrexia-induced ventricular tachycardia, which was refractory to cardioversion and anti-arrhythmics but responded promptly to cooling. We emphasise the lifesaving role of immediate and aggressive cooling in such patients.

Accelerated idioventricular rhythm is a rare but benign form of ventricular tachycardia which might be challenging to differentiate from other more worrisome forms. We present the case of a healthy newborn diagnosed with an accelerated idioventricular rhythm which is spontaneously terminated without the need for medical therapy.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

“Torsades de pointes”, a life-threatening rhythm disorder, is a polymorphic ventricular tachycardia that usually develops in association with a prolonged QT interval. Fluconazole, an anti-fungal drug, may also induce QT prolongation, in some cases subsequent torsades de pointes. Herein, we report a 16-year-old female presenting “torsades de pointes” after administration of fluconazole and rapidly improved upon cessation of the drug.

Although rare, children with active coronavirus disease 2019 are at risk of developing malignant arrhythmia. Herein, we present the first paediatric case of refractory ventricular tachycardia from acute fulminant myocarditis secondary to acute COVID-19 infection. This 5-year-old boy required venoarterial extracorporeal membrane oxygenation support, but made a complete recovery without significant morbidity.

We report a case with Ebstein’s anomaly and pulmonary atresia with sustained monomorphic ventricular tachycardia in a patient without a ventriculotomy history. In the low voltage area between the atrialised right ventricle and hypoplastic right ventricle, there was a ventricular tachycardia substrate and slow conduction. The tachycardia circuit was eliminated by a point catheter ablation at the area with diastolic fractionated potentials.

We report two females with coronary artery occlusion caused by presumed Kawasaki disease that delivered children without any special treatment. After a 58-year-old female had ventricular tachycardia, a giant coronary artery aneurysm with calcification at the bifurcation of the left coronary artery and segmental stenosis of the right coronary artery were pointed out by CT angiography. She had an episode of sepsis when 3 years old. Further, she remembered chest pain during sleep after that episode. She had delivered twice without any complication during her 20s. Her diagnosis was undiagnosed coronary artery lesions caused by presumed Kawasaki disease and a previous myocardial infarction, and she underwent radiofrequency catheter ablation and implantable cardioverter defibrillator implantation. The other 48-year-old female was accidentally discovered to have coronary artery calcification on CT, while experiencing pneumonia. Her CT angiograms revealed a right coronary artery occlusion and coronary artery calcification at segments 1, 6, and 11. She had a history of “scarlet fever” before 12 months. Premature ventricular contractions were detected, while delivering her first child when 31 years old. However, she was not diagnosed as ischaemic heart disease and delivered twice by a vaginal delivery without any complication. Current guidelines recommend systemic anti-coagulation and anti-platelet therapy for all patients with giant aneurysms resulting from Kawasaki disease in childhood. The two women reported here were fortunate not to have had complications during pregnancy and delivery despite their severe coronary artery aneurysms, which were unrecognised clinically until later in life. They were lucky cases.

Conventional ambulatory heart rhythm monitoring is limited in its ability to provide rapid diagnosis of arrhythmias in athletes participating in water or high-intensity sports. This case report is of a 17-year-old female competitive swimmer who underwent loop recorder implantation with Confirm Rx™ ICM 3500 (Abbott, Minneapolis, MN) to monitor for arrhythmias during swimming. The purpose of this case report is to describe the utility of implantable loop recorders in arrhythmia diagnosis and symptom evaluation in water sport athletes.

A 14-year-old girl suddenly developed ventricular tachycardia and severe chest pain during hospitalisation for trauma surgery. CT revealed a needle in the pericardium. Careful interview elicited that she had inserted the needle by herself, and Munchausen syndrome was diagnosed. This is the first report of ventricular tachycardia caused by a foreign body in a patient with Munchausen syndrome.

Zn plays an important role in maintaining the anti-oxidant status within the heart and helps to counter the acute redox stress that occurs during myocardial ischaemia and reperfusion. Individuals with low Zn levels are at greater risk of developing an acute myocardial infarction; however, the impact of this on the extent of myocardial injury is unknown. The present study aimed to compare the effects of dietary Zn depletion with in vitro removal of Zn (N,N,N′,N′-tetrakis(2-pyridinylmethyl)-1,2-ethanediamine (TPEN)) on the outcome of acute myocardial infarction and vascular function. Male Sprague–Dawley rats were fed either a Zn-adequate (35 mg Zn/kg diet) or Zn-deficient (<1 mg Zn/kg diet) diet for 2 weeks before heart isolation. Perfused hearts were subjected to a 30 min ischaemia/2 h reperfusion (I/R) protocol, during which time ventricular arrhythmias were recorded and after which infarct size was measured, along with markers of anti-oxidant status. In separate experiments, hearts were challenged with the Zn chelator TPEN (10 µm) before ischaemia onset. Both dietary and TPEN-induced Zn depletion significantly extended infarct size; dietary Zn depletion was associated with reduced total cardiac glutathione (GSH) levels, while TPEN decreased cardiac superoxide dismutase 1 levels. TPEN, but not dietary Zn depletion, also suppressed ventricular arrhythmias and depressed vascular responses to nitric oxide. These findings demonstrate that both modes of Zn depletion worsen the outcome from I/R but through different mechanisms. Dietary Zn deficiency, resulting in reduced cardiac GSH, is the most appropriate model for determining the role of endogenous Zn in I/R injury.

Acute rheumatic fever is the most commonly acquired heart disease in developing countries. The most common cardiac presentation is valvular disease. Although some rhythm disturbances may occur during the acute stages of the disease, ventricular tachycardia is extremely rare. Here, a case of acute rheumatic fever with severe endocarditis involving four valves and ventricular tachycardia is presented.

We report a case of primary hyperparathyroidism with hypercalcaemia in a 16-year-old boy who had spontaneous attack of ventricular tachycardia. To our knowledge, presentation of ventricular tachycardia caused by primary hyperparathyroidism in such a young patient has not been reported in medical literature so far.

Marine n-3 PUFA may improve autonomic dysfunction by an increase in heart rate variability (HRV) and may reduce the risk of malignant ventricular arrhythmias. Only a few smaller studies have examined such effects in patients on chronic dialysis, who often have autonomic dysfunction and a high risk of sudden cardiac death, which accounts for almost 30 % of all deaths. This cross-sectional study investigated the association between the plasma phospholipid content of n-3 PUFA and 24-h HRV or ventricular arrhythmias in patients on chronic dialysis. A 48-h Holter monitoring was performed on 169 patients on in-centre dialysis (83 %), home haemodialysis (10 %) or peritoneal dialysis (7 %) obtaining data on arrhythmias (n 152) and 24-h HRV (n 135). The mean overall HRV (standard deviation of normal intervals (SDNN)) was low and 71 % had a reduced overall HRV (SDNN<100 ms) indicating autonomic dysfunction. No significant associations between plasma phospholipid content of total marine n-3 PUFA, EPA (22 : 5n-3) or DHA (22 : 6n-3) and time-domain or frequency-domain HRV were detected in crude or adjusted linear regression analysis. However, a higher plasma phospholipid content of DHA was associated with a significantly lower proportion of patients with ventricular tachycardia (higher DHA-tertile: 9 % v. lower DHA-tertile: 28 %, P=0·02). In conclusion, the content of marine n-3 PUFA in plasma phospholipids was not associated with 24-h HRV, but a higher plasma phospholipid content of DHA was associated with a lower occurrence of ventricular tachycardia suggesting an antiarrhythmic effect of marine n-3 PUFA in patients on chronic dialysis.