The diagnosis and surgical management of pulmonary sequestration in adults: a case series from a single centre in the UK.

Pulmonary sequestration (PS) is a rare congenital malformation where extrapulmonary lung tissue receives systemic blood supply from an anomalous branch directly from the thoracic or abdominal aorta. Whilst non-malignant, it can often present with similar symptoms as lung cancer. We present a retrospective review of 8 consecutive adult patients undergoing surgical management for PS within a single centre in the UK. Of our cohort, 62.5% had never smoked. PS in the right lung was seen in 62.5% of cases. Anomalous branches of the pulmonary artery, pulmonary vein or coeliac axis supplied 37.5% of the PS seen in our cohort, and 12.5% did not have a radiologically identifiable blood supply. Techniques varied from thoracotomy (n = 4), video-assisted thoracoscopic surgery (VATS) (n = 3) to robotic resection (n = 1) with no intra-operative or post-operative complications reported within hospital. The mean length of stay was 2 days. The post-operative mortality rate was 12.5%; one patient had died following the robotic resection of the mass of pneumonia in the local district hospital 26 days post-operatively after being discharged. No other complications nor recurrence was recorded over the follow-up period. Where pulmonary masses receive blood supply from anomalous branches of the pulmonary vein and coeliac axis, diagnoses of PS should be considered. The clinical feasibility of discharge in 2 days with no symptom recurrence should undergo further investigation with a larger sample size.

Assessment of post-infarct ventricular septal defects through 3D printing and statistical shape analysis.

Background: Post-infarct ventricular septal defect (PIVSD) is a serious complication of myocardial infarction. We evaluated 3D-printing models in PIVSD clinical assessment and the feasibility of statistical shape modeling for morphological analysis of the defects. Methods: Models (n = 15) reconstructed from computed tomography data were evaluated by clinicians (n = 8). Statistical shape modeling was performed on 3D meshes to calculate the mean morphological configuration of the defects. Results: Clinicians' evaluation highlighted the models' utility in displaying defects for interventional/surgical planning, education/training and device development. However, models lack dynamic representation. Morphological analysis was feasible and revealed oval-shaped (n = 12) and complex channel-like (n = 3) defects. Conclusion: 3D-PIVSD models can complement imaging data for teaching and procedural planning. Statistical shape modeling is feasible in this scenario.

Following heart attacks, the heart muscle becomes scarred and weaker, making it prone to tearing under high pressures. These tears are known as 'post-infarct ventricular septal defects'. Their shape varies greatly as the heart beats. The approach to fixing these can range from plugging them with a device or patching them by open heart surgery. We created 15 3D-printed models of hearts with these kinds of defects and made digital reconstructions of the tears to see the different sizes/shapes that they can have. Doctors agreed that 3D-printed models could help in planning repairs and training other doctors. The digital reconstructions of the tears showed that many were round, but some had irregular shapes which would mean devices used to fix them may not fit.

Artificial intelligence can detect left ventricular dilatation on contrast-enhanced thoracic computer tomography relative to cardiac magnetic resonance imaging.

OBJECTIVES: To assess the diagnostic accuracy of an automated algorithm to detect left ventricular (LV) dilatation on non-ECG gated CT, using cardiac magnetic resonance (CMR) as reference standard. METHODS: Consecutive patients with contrast-enhanced CT thorax and CMR within 31 days (2016-2020) were analysed (n = 84). LV dilatation was defined against age-, sex- and body surface area-specific values for CMR. CTs underwent automated artificial intelligence(AI)-derived analysis that segmented ventricular chambers, presenting maximal LV diameter and volume. Area under the receiver operator curve (AUC-ROC) analysis identified CT thresholds with ≥90% sensitivity and highest specificity and ≥90% specificity with highest sensitivity. Youden's Index was used to identify thresholds with optimised sensitivity and specificity. RESULTS: Automated diameter analysis was feasible in 92% of cases (77/84; 45 men, age 61 ± 14 years, mean CT to CMR interval 10 ± 8 days). Relative to CMR as a reference standard, 45% had LV dilatation. In males, an automated LV diameter measurement of ≥55.5 mm was ≥90% specific for CMR-defined LV dilatation (positive predictive value (PPV) 85.7%, negative predictive value (NPV) 61.2%, accuracy 68.9%). In females, an LV diameter of ≥49.7 mm was ≥90% specific for CMR-defined LV dilatation (PPV 66.7%, NPV 73.1%, accuracy 71.9%). AI CT volumetry data did not significantly improve AUC performance. CONCLUSION: Fully automated AI-derived analysis LV dilatation on routine unselected non-gated contrast-enhanced CT thorax studies is feasible. We have defined thresholds for the detection of LV dilatation on CT relative to CMR, which could be used to routinely screen for dilated cardiomyopathy at the time of CT. ADVANCES IN KNOWLEDGE: We show, for the first time, that a fully-automated AI-derived analysis of maximal LV chamber axial diameter on non-ECG-gated thoracic CT is feasible in unselected real-world cases and that the derived measures can predict LV dilatation relative to cardiac magnetic resonance imaging, the non-invasive reference standard for determining cardiac chamber size. We have derived sex-specific cut-off values to screen for LV dilatation on routine contrast-enhanced thoracic CT. Future work should validate these thresholds and determine if technology can alter clinical outcomes in a cost-effective manner.

Management of rheumatic aortic valve disease using the Ozaki procedure with autologous pericardium: a case report.

BACKGROUND: Rheumatic valve disease (RVD) is the most common cause of cardiovascular death in low-middle income nations. Surgical aortic valve (AV) interventions for RVD, especially in children, have proven problematic with graft failure, relapse, and poor compliance with anticoagulation. A novel technique involving neocuspidization of the aortic annulus using autologous pericardium to construct new AV leaflets (the Ozaki procedure) has shown promising outcomes in children with congenital AV disease; however, there are no previous recorded cases using this technique in children with RVD. CASE SUMMARY: We present the case of a 15-year-old male presenting with exertional angina and dyspnoea with a background of previous rheumatic fever. Echocardiography had shown a regurgitant tricuspid AV, left ventricular dilatation with mitral valve leaflet tethering. The patient underwent the Ozaki procedure for his AV regurgitation and was discharged following an uneventful post-operative recovery. The patient had full resolution of symptoms following the procedure and remains well 3 years following his operation. DISCUSSION: This case highlights that good outcomes with the Ozaki procedure in RVD are possible 3-years post-operatively and should prompt future studies to evaluate the procedure as a surgical option for paediatric patients in this clinical context. Additionally, the Ozaki procedure may also provide a cost-effective surgical technique requiring minimal additional operative resources and reduced follow-up demand, which would be critical in low-resource clinical settings where RVD is prevalent.

Role of 3D printing technology in paediatric teaching and training: a systematic review.

BACKGROUND: In the UK, undergraduate paediatric training is brief, resulting in trainees with a lower paediatric knowledge base compared with other aspects of medicine. With congenital conditions being successfully treated at childhood, adult clinicians encounter and will need to understand these complex pathologies. Patient-specific 3D printed (3DP) models have been used in clinical training, especially for rarer, complex conditions. We perform a systematic review to evaluate the evidence base in using 3DP models to train paediatricians, surgeons, medical students and nurses. METHODS: Online databases PubMed, Web of Science and Embase were searched between January 2010 and April 2020 using search terms relevant to "paediatrics", "education", "training" and "3D printing". Participants were medical students, postgraduate trainees or clinical staff. Comparative studies (patient-specific 3DP models vs traditional teaching methods) and non-comparative studies were included. Outcomes gauged objective and subjective measures: test scores, time taken to complete tasks, self-reported confidence and personal preferences on 3DP models. If reported, the cost of and time taken to produce the models were noted. RESULTS: From 587 results, 15 studies fit the criteria of the review protocol, with 5/15 being randomised controlled studies and 10/15 focussing on cardiovascular conditions. Participants using 3DP models demonstrated improved test scores and faster times to complete procedures and identify anatomical landmarks compared with traditional teaching methods (2D diagrams, lectures, videos and supervised clinical events). User feedback was positive, reporting greater user self-confidence in understanding concepts with users wishing for integrated use of 3DP in regular teaching. Four studies reported the costs and times of production, which varied depending on model complexity and printer. 3DP models were cheaper than 'off-the-shelf' models available on the market and had the benefit of using real-world pathologies. These mostly non-randomised and single-centred studies did not address bias or report long-term or clinically translatable outcomes. CONCLUSIONS: 3DP models were associated with greater user satisfaction and good short-term educational outcomes, with low-quality evidence. Multicentred, randomised studies with long-term follow-up and clinically assessed outcomes are needed to fully assess their benefits in this setting. PROSPERO REGISTRATION NUMBER: CRD42020179656.

Language in a Time of COVID-19: Literacy Bias Ethnic Minorities Face During COVID-19 from Online Information in the UK.

OBJECTIVES: To investigate the readability and presence of translated online information readily available to the British public during COVID-19. DESIGN: A cross-sectional study was performed. The terms "Coronavirus", "COVID-19", "Lockdown", "Social Distancing", "Handwashing", "Furlough Scheme" and "Sick pay" were inputted into the popular search engine, Google. Websites were categorised by their source (i.e. Government, Non-Governmental Organisation, NHS and Commercial) and theme (i.e. general COVID-19 information, population practise and employment rulings). Reliable calculators for assessing readability (Simple Measure of Gobbledygook, Gunning Fog Index, Flesch-Kincaid Grade Level, Coleman-Liau Index and Automated Readability Index) were used. MAIN OUTCOME MEASURES: The median scores with the interquartile range from each calculator of the pooled data were observed. The presence of accompanying translated material and graphic information was also counted and presented as counts and percentages. The number of readable websites (i.e. a score ≤ 8) for each source and theme category were also recorded. SETTING: UK Internet servers. RESULTS: The median scores of the pooled data (n = 148) had shown that the majority of websites were unreadable to the average British reader according to each calculator used (SMOG 1.3%; GF 6.8%; FK 35.8%; CL 2.6%; ARI 40%). Only 3.4% and 6.8% of the pooled websites had readily available translated material and accompanying graphic material, respectively. CONCLUSION: Readability of COVID-19 information is below national standards and that there is a lack of accompanying translated and graphics-based material online. This may lead to an amplified level of misunderstanding in BAME populations about the COVID-19 pandemic and the rulings put in place.

Congenital thrombotic thrombocytopenic purpura presenting in adulthood with recurrent cerebrovascular events.

Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare, life-threatening disease, characterised by episodes of microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and small vessel thrombosis. We describe a case of cTTP first diagnosed at age 70 years in a female presenting with an acute ischaemic stroke and thrombocytopenia, in whom A Disintegrin And Metalloproteinase with a Thrombospondin type 1 Motif, member 13 (ADAMTS13) levels were <10%, suggestive of thrombotic thrombocytopaenic purpura (TTP). The patient underwent plasma exchange and started rituximab for presumed immune TTP; however, anti-ADAMTS13 antibody titres were negative on two occasions. This, together with a history of pregnancies complicated by presumed disseminated intravascular coagulation, and two previous episodes of sepsis with MAHA, prompted investigation for cTTP, which was confirmed by genetic testing. Despite treatment with infusions of solvent/detergent-treated, virus-inactivated fresh frozen plasma, she has re-presented with further neurological deficit, associated with new infarcts on imaging. cTTP has a varied phenotype which, as demonstrated in this case, can include large vessel occlusion.