Which neonates should have a pre-operative echocardiography? Findings from a national survey and a retrospective tertiary single-centre analysis in the United Kingdom.

PURPOSE: The detection of congenital heart disease (CHD) before neonatal surgery is crucial for anaesthetic and perioperative management. There are no established criteria for pre-operative echocardiography in neonates. We aimed to survey current practice in the United Kingdom and evaluate the reliability of antenatal screening and postnatal clinical assessment in detecting CHD before surgery. METHOD: A 9-point questionnaire was sent to all paediatric surgical centres in the United Kingdom to assess their practice. Subsequently, a single-centre retrospective review of all neonatal surgery over 5 years (2015-2020) was conducted in our tertiary paediatric/neonatal hospital. Data included pre-operative clinical assessment, performance of chest radiograph and echocardiography. Indications for echocardiography were categorised and assessed using sensitivity, specificity, positive predictive value and negative predictive value. RESULTS: All 26 paediatric surgical centres responded to our survey. 23/26 (88.5%) did not have established criteria or guidelines for pre-operative echocardiography. There was a large variation in which surgical conditions required a pre-operative scan and whether a normal clinical examination was considered sufficient to not require one. For the retrospective review, 454 patients were identified. There were 40 cases with CHDs (8.8%), 13 were classed as major or moderate. Indications for echocardiography were categorised into abnormal foetal cardiac screening, medical/surgical conditions associated with CHD and an abnormal cardiorespiratory examination. Sensitivity, specificity, positive predictive value and negative predictive value for major and moderate CHD were 46%, 99%, 67%, 98% for abnormal foetal screening, 46%, 97%, 35%, 98% for associated medical conditions, 62%, 66%, 6%, 98% for associated surgical conditions, and 100%, 66%, 9%, 100% for abnormal clinical examination. CONCLUSION: The use of pre-operative echocardiography in neonates is not standardised across the UK. The results from our cohort demonstrate that foetal echocardiography is not sufficient to capture all major and moderate CHDs, but the absence of abnormal clinical examination is highly reliable in ruling out them out. Specifying a list of medical/surgical of conditions associated with CHD warranting pre-operative echocardiography may improve yield, but this depends on the availability of resources and expertise.

Anesthetic considerations for scoliosis surgery in a patient with recessive severe/lethal form of osteogenesis imperfecta.

Osteogenesis imperfecta is a heterogeneous genetic disorder characterized by bone fragility, with disease ranging from mild fractures to death in utero. We describe a child with autosomal recessive osteogenesis imperfecta type VIII (severe or lethal phenotype), who successfully underwent posterior spinal fusion, was extubated on postoperative day 1 and discharged home 25 days later. Recently identified recessive forms of osteogenesis imperfecta are associated with severe/lethal phenotype. Special consideration is needed in scoliosis surgery, with challenges arising from prone positioning, neurophysiology monitoring, and blood loss.