This is the first evidence to help guide which children with CHD need a preflight HCT. We suggest all children with an actual or potential R-L shunt should be tested.

A case of a baby born preterm with an antenatal diagnosis of aortic coarctation for which prostin was electively started at birth. The baby was found to be profoundly anaemic with no clear obstetric cause. Features consistent with antenatal intracerebral haemorrhage were noted on cranial ultrasonography in the context of severe coagulopathy, prompting investigations which confirmed fetal-maternal haemorrhage. It transpired that, following aortic and mitral valve replacements, the mother was anticoagulated with warfarin at conception, having misunderstood her cardiologist's advice that: 'you cannot get pregnant whilst on warfarin'. Following conversion to low molecular weight heparin, she suffered a stroke, thus warfarin was restarted, with an international normalised ratio of 3-4.7 during pregnancy. Following transfer to the paediatric intensive care unit, fetal warfarin syndrome was diagnosed. The coagulopathy and anaemia were corrected and aortic coarctation was excluded. The baby returned to the neonatal intensive care unit for ongoing care and was discharged home in good condition around his due date. At the present time, there is no clinically overt neurological deficit.

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Total anomalous pulmonary venous drainage (TAPVD) is a rare form of congenital heart disease where all four pulmonary veins drain to the systemic venous circulation. A term infant was found to have low oxygen saturations on the neonatal check and he was admitted to the neonatal intensive care unit. An increasing oxygen requirement necessitated invasive ventilation. A blood gas taken from the umbilical venous catheter (UVC) showed a pO(2) of 28.1kPa - a finding that at the time was considered to be erroneous. An x-ray showed the UVC tip was located in the liver. The following day the baby was transferred to a cardiology centre where a diagnosis of unobstructed infracardiac TAPVD was made on echocardiography. In retrospect the unusually oxygenated venous gas was consistent with pulmonary venous return draining directly to the hepatic venous system. This could have provided a vital clue to diagnosis in a situation where an echocardiogram was not available.

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IntroductionCommercial airplanes fly with an equivalent cabin FiO2 of 0.15 leading to reduced oxygen saturation (SpO2) in passengers. Although guidelines exist, the evidence-base for recommending supplemental O2 when flying in children with complex congenital heart disease (CHD) is practically non-existent. We conducted hypoxic challenge tests (HCT) to determine which children need a pre-flight assessment.MethodsChildren<16 years with complex CHD were recruited; exclusions were SpO2 <75%; pulmonary hypertension; oxygen requirement; or concomitant respiratory disease. Children had a standard HCT in a sealed body plethysmograph with FiO2 of 0.15. We measured SpO2, pulse rate, transcutaneous CO2 (PtcCO2), corrected QT interval (QTc), and total Hb by co-oximetry (SpHb). Supplemental O2 was given (which meant a ‘failed’ test) if (1) children with baseline SpO295%–100% desaturated to 85%, (2) or baseline SpO285%–94% desaturated to 15% of their baseline; (3) or baseline SpO275%–84% desaturated to 70%.ResultsThere were 68 children, mean age 3.3 years (range 10 weeks to 14.5 years); 53% were boys. Grouping by normal (≥95%) vs abnormal baseline SpO2(75%–94%), both groups had a significant fall in SpO2 (p<0.0001). 3/38 (8%) children failed with normal baseline SpO2 vs 5/32 (16%) with abnormal baseline (non-significant difference). In terms of cardiac status, both groups had a significant fall in SpO2 (p<0.0001); however in those with no residual for potential R-L shunt 0/27 failed vs those with residual potential R-L shunt or who had not undergone repair or who had palliative surgery in whom 8/41 (20%) failed (p<0.02). PtcCO2 did not change significantly (i.e., no-one hyperventilated to compensate for hypoxia); pulse rate and QTc were not different between groups, and unaffected by the hypoxic state.ConclusionsThis is the first evidence to help inform which children with CHD need a pre-flight HCT. We suggest all children with residual potential R-L shunt or who have not undergone repair or who have only had palliative surgery should be tested (as 20% are expected to need supplemental O2), whereas those with no potential for R-L shunt need not be. Baseline SpO2 does not help predict who will need supplemental O2 when flying.

Idiopathic ventricular fibrillation (IVF) is diagnosed in out-of-hospital VF survivors after comprehensive investigations have excluded structural heart disease or inherited channelopathies. Current guidelines recommend clinical screening of first-degree relatives of IVF survivors, but this approach has not been validated in children. This study aimed to assess the yield of clinical cardiac screening in child first-degree relatives of IVF victims. MethodsA retrospective observational study was conducted of all consecutive paediatric first-degree relatives of IVF patients referred to our centre between December 2007 and April 2020.Patients underwent systematic evaluation, including medical and family history; 12-lead resting, signal-averaged and ambulatory electrocardiogram (ECG); echocardiogram; exercise testing; cardiac magnetic resonance imaging; and ajmaline provocation testing. Results60 child first-degree relatives of 32 IVF survivors were included [median follow-up time of 55 months (IQR 27.0 -87.0 months); 30 (50%) females]. 8 patients (13.3%) from 6 families (18.8%) received a cardiac diagnosis: long QT syndrome (n=4); Brugada syndrome (n=3); and dilated cardiomyopathy (n=1). There were no deaths during follow-up. ConclusionThis study demonstrates a high yield of clinical screening for inherited cardiac disease in child first-degree relatives of IVF survivors. These findings highlight the variable expression of inherited cardiac conditions and the importance of comprehensive clinical evaluation in paediatric relatives, even when extensive investigations in the proband have not identified a

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