Kawasaki Disease in the Australian Population: An Australian Tertiary Hospital Experience.

AIM: To describe the contemporary experience of Kawasaki Disease (KD) in a tertiary paediatric hospital. METHODS: This was a retrospective analysis of admissions with suspected/confirmed KD to John Hunter Children's Hospital from 1 January 2015 to 31 December 2016, with follow-up. Patients were stratified into classical, incomplete, unlikely and uncertain KD cohorts based on the 2004 American Heart Association (AHA) guidelines. RESULTS: Forty (40) patients had 45 admissions with suspected KD. Twenty-four (24) patients (60%) had complete and incomplete KD. Twenty-nine per cent (29%) (7/24) were not diagnosed at presentation. Thirty per cent (30%) (12/40) were incompletely assessed according to the AHA pathway. Seventy-one per cent (71%) of KD patients (17/24) were treated with aspirin and intravenous immunoglobulin. The incidence was 22-26 per 100,000 in children less than 5 years during the study period, higher compared to prior data. CONCLUSION: The diagnosis of KD remains challenging, with some children not diagnosed at the time of initial presentation. The increase in KD incidence is hypothesis generating and requires further investigation.

Fascicular tachycardia in infancy and the use of verapamil: a case series and literature review.

OBJECTIVE: Guidelines state that verapamil is contraindicated in infants. This is based on reports of cardiovascular collapse and even death after rapid intravenous administration of verapamil in infants with supraventricular tachycardia (SVT). We wish to challenge this contraindication for the specific indication of verapamil sensitive ventricular tachycardia (VSVT) in infants. DESIGN: Retrospective case series and critical literature review. SETTING: Hospitals within New Zealand. PATIENTS: We present a series of three infants/young children with VSVT or 'fascicular VT'. RESULTS: Three children aged between 8 days and 2 years presented with tachycardia 200-220 beats per minute with right bundle brunch block and superior axis. Adenosine failed to cardiovert and specialist review diagnosed VSVT. There were no features of cardiovascular shock. Verapamil was given as a slow infusion over 10-30 min (rather than as a push) and each successfully cardioverted without incident. Critical review of the literature reveals that cardiovascular collapses were associated with a rapid intravenous push in cardiovascularly compromised infants and/or infants given other long-acting antiarrhythmics prior to verapamil. CONCLUSIONS: Verapamil is specifically indicated for the treatment of fascicular VT, and for this indication should be used in infancy, as well as in older children, as first-line treatment or after failure of adenosine raises suspicion of the diagnosis. We outline how to distinguish this tachycardia from SVT and propose a strategy for the safe intravenous slow infusion of verapamil in children, noting that extreme caution is necessary with pre-existing ventricular dysfunction.

Two Ventricles Are Not Better Than One in the Fontan Circulation: Equivalent Late Outcomes.

BACKGROUND: A subset of patients who underwent Fontan operations has two adequate-sized ventricles, but an anatomic biventricular circulation cannot be achieved because of complex morphology or for technical reasons. This study sought to determine whether these patients with two-ventricle Fontan circulation had superior outcomes compared with those with a single ventricle. METHODS: A binational Fontan Registry of patients (n = 1,377) was analyzed to identify those patients with two adequate ventricles. This cohort was compared with patients with single-ventricle Fontan circulation. The primary end point was a composite end point called "Fontan failure" encompassing death, heart transplantation, Fontan takedown or conversion, protein-losing enteropathy, plastic bronchitis, or New York Heart Association functional class III or IV. RESULTS: A total of 79 Fontan patients with two adequate ventricles (2V) were compared with 1,291 single ventricle (1V) Fontan patients. Median follow-up for the entire cohort was 11.5 years (interquartile range, 5.1 to 18.8 years). There was no difference in unadjusted 15-year freedom from Fontan failure (2V: 81% [95% confidence interval (CI), 69% to 94%] vs 1V: 86% [95% CI, 83% to 88%], p = 0.4). Propensity-score matching for potential confounding factors yielded 75 two-ventricle Fontan patients matched with 604 single-ventricle Fontan patients, in which 15-year freedom from Fontan failure was also not different (2V: 79% [95% CI, 67% to 94%] vs 1V: 87% [95% CI, 84% to 91%], p = 0.3). CONCLUSIONS: The two-ventricle Fontan circulation does not have better outcomes compared with the single-ventricle Fontan circulation. Late outcomes may depend more on other characteristics of the Fontan circulation. This finding is relevant when the Fontan procedure is being considered as an alternative to anatomic repair in patients with complex two-ventricle morphologies.

Transcatheter Closure of Atrial Septal Defects using the GORE(®) Septal Occluder in Children Less Than 10 kg of Body Weight.

The GORE(®) Septal Occluder (GSO) is a well-evaluated device for interventional ASD closure with closure rates comparable to the Amplatzer(®) Septal Occluder (ASO), but there are no published reports of its use in small children weighing less than 10 kg. This may be due to the necessity of a large-sized introducing sheath of at least 10 Fr and therefore the assumed risk of complications in vascular access. The GSO is an alternative option for interventional ASD closure in children weighing less than 10 kg. Fourteen infants and children with a median body weight 8900 g (range 6350-9650 g) underwent successful ASD closure using the GSO. The closure was performed under fluoroscopic and transthoracic echocardiographic guidance. Postprocedure, the vessels passed by the occluder and delivery catheter were examined by duplex sonography. The median ASD diameter was 11 mm (5-17 mm), and the median GSO size was 22.5 mm (15-30 mm), whereas the median ASO left disc size that would have been recommended was 25 mm (17-31 mm). All ASDs were successfully closed. During a median follow-up of 1.57 years (range 0.5-4.2), no complications like erosion, embolization, arrhythmias, or vascular injuries occurred. Although using a 10-Fr introducer sheath, no vascular complications were detected. Our data suggest that the small usable size as well as the soft and flexible design of the device allows successful use of the GSO in young children.