Feasibility and outcomes of ajmaline provocation testing for Brugada syndrome in children in a specialist paediatric inherited cardiovascular diseases centre.

OBJECTIVES: Brugada syndrome (BrS) is an inherited arrhythmia syndrome that causes sudden cardiac death in the young. The class Ia antiarrhythmic ajmaline can be used to provoke the diagnostic ECG pattern. Its use has been established in adults, but little data exist on the ajmaline provocation test in children. This study aims to determine the safety and feasibility of ajmaline provocation testing in a large paediatric cohort in a specialist paediatric inherited cardiac diseases centre. METHODS: 98 consecutive ajmaline tests were performed in 95 children between September 2004 and July 2012 for family history of BrS (n=46 (48%)); family history of unexplained sudden cardiac death (n=39 (41%); symptoms with suspicious ECG abnormalities (n=9 (10%)). Three patients were retested with age, due to the possibility of age-related penetrance. ECG parameters were measured at baseline and during maximal ajmaline effect. RESULTS: The mean patient age was 12.55 years, 43% were female. Nineteen patients (20%) had a positive ajmaline test. There were no arrhythmias or adverse events during testing. Ajmaline provoked significant prolongation of the PR, QRS and QTc in all patients. Mean follow-up was 3.62 years with no adverse outcomes reported in any patients with BrS. There were no predictors of a positive ajmaline provocation test on multivariable analysis. One patient who tested negative at 12 years of age, subsequently tested positive at 15 years of age. CONCLUSIONS: Ajmaline testing appears safe and feasible in children when performed in an appropriate setting by an experienced team. Test positivity may change with age in individuals, suggesting that the test should be repeated in the late teenage years or early adulthood.

A triad: cardiac rhabdomyosarcoma, stroke and tamponade.

The case report describes a 9-year-old boy who presented with an acute cerebrovascular accident and was found to have cardiac tamponade caused by cardiac rhabdomyosarcoma. Symptoms of rhabdomyosarcoma can be indolent and nonspecific, even with metastatic disease. Echocardiography and cardiac magnetic resonance imaging are explored as adjuncts to diagnosis. The radiologic features that helped with the diagnosis of this rare condition are described.

Cluster of neurological manifestations of rotavirus infection in children.

Rotavirus infection in children in the resource-rich countries is usually benign but complications are reported rarely. Four children presented within 4 weeks with neurological symptoms and stool virology positive for rotavirus. All four made a quick recovery and were discharged home after a few days without medication. At 6 weeks all were well, with no further neurological episodes. This is the first reported cluster of this nature. Potential mechanisms for how rotavirus causes its rare neurological complications are briefly discussed, along with clinical implications for clinicians managing such patients.

In patients undergoing cardiac surgery, thoracic epidural analgesia combined with general anaesthesia results in faster recovery and fewer complications but does not affect length of hospital stay.

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether the use of thoracic epidural anaesthesia (TEA) in combination with general anaesthesia during adult cardiac surgery resulted in a faster recovery, fewer complications and shorter length of hospital stay. Altogether 313 papers were identified on Medline and 368 on Embase using the reported search of which 15 represented the best evidence on the topic. The author, journal, date and country of publication, patient group studied, study type, relevant outcomes, results and study weaknesses were tabulated. We conclude that the use of TEA in combination with general anaesthesia results in more rapid extubation and significantly better pain relief in patients undergoing cardiac surgery. In addition, common postoperative complications such as chest infection, dysrhythmia, confusional states, renal failure and psychological sequelae may be decreased although there is currently no evidence that its use decreases length of hospital stay. Finally, whilst there are case reports of neurological injury secondary to epidural haematoma in the literature these are rare and a number of large studies have been performed confirming the safety of the technique when basic precautions regarding technical aspects of the procedure and coagulation management are followed.