Emergency arterial switch: Rescue therapy for life-threatening hypoxemia in infants with transposition of great arteries with intact intraventricular septum.

OBJECTIVE: A small percentage of infants with d-loop transposition of the great arteries with intact intraventricular septum have life-threatening refractory hypoxemia often due to coexistent persistent pulmonary hypertension of the newborn. In this case series we describe the outcomes of a "rescue" emergency arterial switch operation (ASO). METHODS: We undertook a retrospective medical record analysis of infants with d-loop transposition of the great arteries with intact intraventricular septum who underwent an ASO in New Zealand from January 1, 1996, to April 30, 2017. Data were compared for those who received an emergency ASO and those with a nonemergency ASO for descriptive purposes. An emergency ASO was defined as one that was undertaken for life-threatening refractory hypoxemia when the only alternative stabilization strategy was preoperative extracorporeal life support. Primary outcome measures were 30-day postoperative mortality and abnormal neurodevelopmental outcome in the survivors. Secondary outcomes were low cardiac output, arrhythmia, renal dysfunction, postoperative seizures, and length of stay. Other known risk factors for morbidity and mortality were also assessed. RESULTS: Two hundred seventy-two infants underwent an ASO with 25 (9%) who received an emergency ASO. No infants received preoperative extracorporeal life support. The emergency group had greater 30-day postoperative mortality (8.0% vs 0.4%; P = .01) with no difference in abnormal neurodevelopmental outcome among the survivors (17.4% vs 13.8%; P = .35). The emergency group had more therapies for low cardiac output syndrome, more postoperative seizures, and a longer length of stay. CONCLUSIONS: An emergency ASO is a definitive rescue therapy that can be undertaken with acceptable mortality and neurodevelopmental outcome with consideration of the preoperative clinical state.

Revisiting QT prolongation in acute rheumatic fever - Relevance for hydroxychloroquine treatment.

In-vitro evidence suggests hydroxychloroquine could be a potential immunomodulator for the inflammatory carditis of acute rheumatic fever (ARF). Hydroxychloroquine used as an anti-inflammatory agent has a low side effect profile but its use in the Covid-19 pandemic raised concerns about QTc interval prolongation and cardiac arrhythmias. The prolongation of QTc in ARF appears benign but has not been widely studied. We aim to report QTc intervals in a contemporary ARF population and consider implications for hydroxychloroquine use in ARF. The study cohort was 197 children <15 years of age with a clinical diagnosis of ARF. The QTc mean (SD) was 445 msec (28), range 370-545 msec. Eighteen percent of the cohort had a QTc > 99th percentile for normal by age and 8 patients (4%) had a QTc over 500 msec. There was no difference of QTc by age or gender. Inter-observer repeatability for QTc (n = 33) was 35 msec. The QTc is often prolonged in the early phase of ARF, meaning that QT prolonging medications should be used with caution in this setting. Serial ECG monitoring of the QT interval is recommended if hydroxycholoroquine is used in ARF.

Family acceptability of school-based echocardiographic screening for rheumatic heart disease in a high-risk population in New Zealand.

AIM: Echocardiographic screening for rheumatic heart disease has been piloted in high-risk areas in New Zealand and internationally, and fulfils most of the criteria for a targeted screening programme. The question of acceptability of rheumatic heart disease screening has not been assessed, and the aim of our study was to assess parental acceptability of a school-based echocardiographic screening programme in a high-risk population in New Zealand. METHODS: A post-screening questionnaire was developed to survey parents of children who underwent echocardiographic screening. The families of 34 children with abnormal scan results and a sample of 80 children with normal scan results were surveyed by phone within 4 months of screening. RESULTS: Positive results were seen in all survey questions in both normal and abnormal scan groups. All families were supportive of an ongoing screening programme. Of children with abnormal results, 62% of their parents reported that they would treat their child differently; however, all responses were positive health-promoting outcomes. CONCLUSION: The study showed strong positive support for school-based echocardiographic screening by a community with high acute rheumatic fever incidence. The study did not detect any short-term negative effects in those with abnormal results. The survey result shows family and community support for the establishment of echocardiographic screening programmes in high acute rheumatic fever areas provided there is adequate infrastructural support.