Childhood/adolescent Sydenham's chorea in the UK and Ireland: a BPSU/CAPSS surveillance study.

OBJECTIVE: To conduct the first prospective surveillance study of Sydenham's chorea (SC) in the UK and Ireland, and to describe the current paediatric and child psychiatric service-related incidence, presentation and management of SC in children and young people aged 0-16 years. DESIGN: Surveillance study of first presentations of SC reported by paediatricians via the British Paediatric Surveillance Unit (BPSU) and all presentations of SC reported by child and adolescent psychiatrists through the Child and Adolescent Psychiatry Surveillance System (CAPSS). RESULTS: Over 24 months from November 2018, 72 reports were made via BPSU, of which 43 met the surveillance case definition of being eligible cases of suspected or confirmed SC. This translates to an estimated paediatric service-related incidence rate of new SC cases of 0.16 per 100 000 children aged 0-16 per year in the UK. No reports were made via CAPSS over the 18-month reporting period, although over 75% of BPSU cases presented with emotional and/or behavioural symptoms. Almost all cases were prescribed courses of antibiotics of varying duration, and around a quarter of cases (22%) received immunomodulatory treatment. CONCLUSIONS: SC remains a rare condition in the UK and Ireland but has not disappeared. Our findings emphasise the impact that the condition can have on children's functioning and confirm that paediatricians and child psychiatrists should remain vigilant to its presenting features, which commonly include emotional and behavioural symptoms. There is a further need for development of consensus around identification, diagnosis and management across child health settings.

Outcome after transcatheter occlusion of patent ductus arteriosus in infants less than 6 kg: A national study from United Kingdom and Ireland.

OBJECTIVES: This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing <6 kg. BACKGROUND: The technique of transcatheter PDA closure has evolved in the past two decades and is increasingly used in smaller patients but data on safety and efficacy are limited. METHODS: Patients weighing < 6 kg in whom transcatheter PDA occlusion was attempted in 13 tertiary paediatric cardiology units in the United Kingdom and Ireland were retrospectively analyzed to review the outcome and complications. RESULTS: A total of 408 patients underwent attempted transcatheter PDA closure between January 2004 and December 2014. The mean weight at catheterization was 4.9 ± 1.0 kg and mean age was 5.7 ± 3.0 months. Successful device implantation was achieved in 374 (92%) patients without major complication and of these, complete occlusion was achieved in 356 (95%) patients at last available follow-up. Device embolization occurred in 20 cases (5%). The incidence of device related obstruction to the left pulmonary artery or aorta and access related peripheral vascular injury were low. There were no deaths related to the procedure. CONCLUSIONS: Transcatheter closure of PDA can be accomplished in selected infants weighing <6 kg despite the manufacturer's recommended weight limit of 6 kg for most ductal occluders. The embolization rate is higher than previously reported in larger patients. Retrievability of the occluder and duct morphology needs careful consideration before deciding whether surgical ligation or transcatheter therapy is the better treatment option.

Two-Strip Technique to Repair Common Atrioventricular Valve Regurgitation in Single-Ventricle Palliation.

A common atrioventricular valve (CAVV) presents a challenge in single-ventricle palliation (SVP) because of its propensity to leak, with CAVV regurgitation being an important risk factor for repeated operation and mortality. We propose a new technique of CAVV repair in the context of SVP. The method uses the techniques of complete repair of antrioventricular septal defect while maintaining free communication at atrial and ventricular levels necessary for single-ventricle physiology.

Synergistic interaction between right ventricular mechanical dyssynchrony and pulmonary regurgitation determines early outcome following tetralogy of Fallot repair.

OBJECTIVE: The ability of the right ventricle to tolerate acute pulmonary regurgitation (PR) following tetralogy of Fallot (TOF) repair is variable and the mechanisms that underlie this are not completely understood. We hypothesise that dyssynchronous wall mechanics affects the RV tolerance to postoperative PR with adverse effect on early surgical outcome. METHODS: Twenty-four TOFs (mean age 19.5+/-15.5 months) undergoing elective repair were prospectively recruited. Ventricular wall mechanics was studied by tissue Doppler echocardiography following induction (preop) and postoperative day one (POD1) and compared with a control group (10 VSD/AVSD). Segmental dyssynchrony, defined as out-of-phase peak myocardial contraction, was determined at the base, mid, apical segments of the septum, RV and LV free walls and scored by the total number of affected segments. PR was graded from absent to severe and RV dimension was quantified by end-diastolic area index (RVEDAI). Cardiac index (CI) was measured by pulse contour cardiac output analysis. Outcome measures were CI, mixed venous oxygen saturation (SvO2), lactate, and duration of ventilation and critical care stay. RESULTS: Preoperatively, biventricular free-wall motion was synchronous in both groups. Following surgery, TOF developed RV-septal dyssynchrony (>2 segments in 11 (46%) vs none in control, p=0.01), while the LV free wall remained normal in both groups. RV-septal dyssynchrony correlated with the ventilation time (rho=0.69, p=0.003), critical care stay (rho=0.58, p=0.02) in the presence of PR (n=16), but not with other outcome measures. The relationships between dyssynchrony and early outcome were not seen when PR was absent. In the presence of PR, median RVEDAI was greater with higher dyssynchrony score (>3 segments; p=0.009). The degree of PR did not affect critical care/ventilation time or RVEDAI. The presence of transannular patch (p=0.007) or at least moderate PR (p=0.01) was associated with a more severe dyssynchrony. CONCLUSIONS: Dyssynchronous RV-septal wall mechanics occurs early after Fallot repair. The magnitude of dyssynchrony appears to interact synergistically with pulmonary regurgitation to influence RV dimension and early outcome.

Congenital heart defects in Central Australia.

OBJECTIVE: To determine the incidence of congenital heart defects (CHD) in Aboriginal and non-Aboriginal infants in Central Australia and to compare this with the incidence elsewhere in Australia. DESIGN AND SETTING: Data on cases were obtained from patient records of the Alice Springs Hospital, Central Australia, the sole referral centre for paediatric and initial cardiac diagnostic services for the region. PARTICIPANTS: Patients with CHD proven by echocardiography reported between 1 January 1993 and 30 June 2000. MAIN OUTCOME MEASURES: Incidence of CHD using all live births in Central Australia as the denominator. RESULTS: 108 patients with CHD were detected among 6156 live births (incidence, 17.5 per 1000; 95% CI, 14.9-21.7 per 1000); 57 of 2991 were Aboriginal (19.0 per 1000; 95% CI, 14.4-24.6 per 1000) and 51 of 3165 were non-Aboriginal (16.1 per 1000; 95% CI, 12.0-21.1 per 1000). The difference between the two groups was not statistically significant (relative risk, 1.18; 95% CI, 0.81-1.72). CHD incidence in Central Australia was significantly higher than that reported for other parts of Australia (4.3 per 1000 live births in New South Wales and the Australian Capital Territory, 1981-1984; 7.65 and 12 per 1000 total births in Western Australia, 1980-1989, and South Australia, 1993-2000, respectively). CONCLUSIONS: The high rates of CHD in Central Australia may partly reflect the high utilisation of echocardiography for assessing minor lesions. However, the incidence of both major and minor types of CHD was significantly higher than previously reported from other regions of Australia. The role of socioenvironmental factors in this high incidence should be explored.