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Objectives: Since rapid access chest pain clinics (RACPC) were established to streamline stable chest pain assessment, CT coronary angiography (CTCA) has become the recommended investigation for patients without known coronary artery disease (CAD), with well-defined indications. This single-centre retrospective study assessed the feasibility of General Practice (GP)-led CTCA prior to RACPC. Methods: RACPC pathway patients without pre-existing CAD electronic records were reviewed (September–October 2019). Feasibility assessments included appropriateness for RACPC, referral clinical data vs RACPC assessment for CTCA indication and safety, and a comparison of actual vs hypothetical pathways, timelines and hospital encounters. Results: 106/172 patients screened met inclusion criteria (mean age 61 ± 14, 51% female). 102 (96%) referrals were ‘appropriate’. No safety concerns were identified to preclude a GP-led CTCA strategy. The hypothetical pathway increased CTCA requests vs RACPC (84 vs 71), whilst improving adherence to guidelines and off-loading other services. 22% (23/106) had no CAD, representing cases where one hospital encounter may be sufficient. The hypothetical pathway would have reduced referral-to-diagnosis by at least a median of 27 days (interquartile range 14–33). Conclusion: A hypothetical GP-led CTCA pathway would have been feasible and safe in a real-world RACPC patient cohort without pre-existing CAD. This novel strategy would have increased referrals for CTCA, whilst streamlining patient pathways and improved NICE guidance adherence. Advances in knowledge: GP-led CTCA is a feasible and safe pathway for patients without pre-existing CAD referred to RACPC, reducing hospital encounters required and may accelerate time to diagnosis. This approach may have implications and opportunities for other healthcare pathways.

Background The world symposium on pulmonary hypertension (PH) has proposed that PH be defined as a mean pulmonary artery pressure (mPAP) > 20 mmHg as assessed by right heart catheterisation (RHC). Transthoracic echocardiography (TTE) is an established screening tool used for suspected PH. International guidelines recommend a multi-parameter assessment of the TTE PH probability although effectiveness has not been established using real world data. Study aims To determine accuracy of the European Society of Cardiology (ESC) and British Society of Echocardiography (BSE) TTE probability algorithm in detecting PH in patients attending a UK PH centre. To identify echocardiographic markers and revised algorithms to improve the detection of PH in those with low/intermediate BSE/ESC TTE PH probability. Methods TTE followed by RHC (within 4 months after) was undertaken in patients for suspected but previously unconfirmed PH. BSE/ESC PH TTE probabilities were calculated alongside additional markers of right ventricular (RV) longitudinal and radial function, and RV diastolic function. A refined IMPULSE algorithm was devised and evaluated in patients with low and/or intermediate ESC/BSE TTE PH probability. Results Of 310 patients assessed, 236 (76%) had RHC-confirmed PH (average mPAP 42.8 ± 11.7). Sensitivity and specificity for detecting PH using the BSE/ESC recommendations was 89% and 68%, respectively. 36% of those with low BSE/ESC TTE probability had RHC-confirmed PH and BSE/ESC PH probability parameters did not differ amongst those with and without PH in the low probability group. Conversely, RV free wall longitudinal strain (RVFWLS) was lower in patients with vs. without PH in low BSE/ESC probability group (− 20.6 ± 4.1% vs − 23.8 ± 3.9%) (P Conclusion Existing TTE PH probability guidelines lack sensitivity to detect patients with milder haemodynamic forms of PH. Combining additional TTE makers assessing RV radial, longitudinal and diastolic function enhance identification of milder forms of PH, particularly in those who have a low BSE/ESC TTE PH probability.

Since 1972, eighteen patients (10 females/8 males) have been detected by newborn bloodspot screening (NBS) with neonatal-onset Maple Syrup Urine Disease (MSUD) in Ireland. Patients were stratified into three clusters according to clinical outcome at the time of data collection, including developmental, clinical and IQ data. A fourth cluster comprised of two early childhood deaths; a third patient died as an adult. We present neuroimaging and EEG together with clinical and biochemical data. Incidence of MSUD (1972-2018) was 1 in 147,975. Overall good clinical outcomes were achieved with 15/18 patients alive and with essentially normal functioning (with only the lowest performing cluster lying beyond a single standard deviation on their full scale intelligence quotient, FSIQ). Molecular genetic analysis revealed genotypes hitherto not reported, including a possible digenic inheritance state for the BCKDHA and DBT genes in one family. Treatment has been based on early implementation of emergency treatment, diet, close monitoring and even dialysis in the setting of acute metabolic decompensation. A plasma leucine ≥400 μmol/L (outside therapeutic range) was more frequently observed in infancy or during adolescence, possibly due to infections, hormonal changes or noncompliance. Children require careful management during metabolic decompensations in early childhood as this represented a key risk period in our cohort. A high level of metabolic control can be achieved through diet with early implementation of a 'sick day' regime and, in some cases, dialysis as a rescue therapy. The Irish cohort, despite largely classical phenotypes, achieved good outcomes in the NBS era, underlining the importance of early diagnosis and skilled multidisciplinary team (MDT) management. This article is protected by copyright. All rights reserved.

Introduction CT coronary angiography (CTCA) with fractional flow reserve (FFRCT) is a key diagnostic tool in the guidance led evaluation of chronic coronary syndrome (CCS). A small percentage of those patients presenting with CCS will have a pattern of coronary artery disease (CAD) where they would be better served with surgical revascularisation. CTCA alone is increasingly used to rule out important CAD pre-valvular surgery in the absence of an invasive coronary angiogram (ICA). Thus, this study tested to see if CTCA with FFRCT was sufficient to predict surgical CAD relative to subsequent ICA findings. Methods This retrospective single-centre study analysed all patients with CCS who underwent a CTCA with FFRCT, where findings led to a subsequent ICA from August 2018 to January 2021. Those patients who had significant left main stem (LMS) and/or flow limiting disease in three major epicardial blood vessels were included (3VD). Flow limiting disease was defined as an FFRCT of ≤0.8 (2 cm distal to the stenosis) in the left anterior descending (LAD), circumflex (LCx), principle obtuse marginal (OM) or right coronary artery (RCA). This was then compared to the ICA where significance was defined as a stenosis >50% for the LMS and >70% for the other epicardial vessels and/or iFR of ≤0.89 or FFR ≤0.8. Results A total of 565 patients had a CTCA with FFRCT, of which 164 had a subsequent ICA with sufficient data for analysis and 35 of these patients met inclusion criteria (LMS disease only 7/35, 3VD 25/35 and both LMS and 3VD 3/35 on CTCA with FFRCT). Relative to ICA the overall sensitivity, specificity, positive predictive value, negative predictive value and accuracy of CTCA and FFRCT for predicting surgical CAD was 83% (95% CI 61-95), 92% (95% CI 86-96), 61% (95% CI 47-74), 97% (95% CI 93-99) and 90% (95% CI 85-94) respectively. Conclusion CTCA with FFRCT was insufficient for a direct decision on surgical revascularisation in this cohort, particularly given the different risk profiles of ICA, PCI and bypass surgery. Importantly, however, the performance of CTCA with FFRCT for detection of surgical CAD would enable enhanced pre-procedural planning. This includes providing an opportunity to counsel patients in more detail on potential findings and their preference if a surgical pattern of disease is confirmed, consider pre-ICA MDT discussion, and ensure likely complex, high-risk cases are placed on an appropriate list. Conflict of Interest None

Phaeochromocytomas are rare neuroendocrine tumours, which can significantly increase the risk of cardiovascular morbidity and mortality. They are also recognised as ‘the great mimic’ and can present in many ways. A 42-year-old male patient presented with a non-ST elevation acute coronary syndrome and was medically treated pending an invasive coronary angiogram. During this procedure, he suffered a profound, symptomatic hypertensive surge documented with invasive pressure monitoring. This raised concern for potential secondary causes of hypertension, particularly given his age. He was subsequently diagnosed with a phaeochromocytoma, and after surgical resection of the tumour, his blood pressure control improved and he remains on single therapy only. As clinicians, it is important to remain alert for previously undiagnosed comorbidities contributing to common pathology, including rare, but life-threatening conditions as we present in this case.

Introduction Computed Tomography Coronary Angiography (CTCA) is NICE recommended as the diagnostic investigation of choice for patients presenting with stable angina. The technology is well recognised for providing coronary anatomy and descriptive analysis of coronary disease. Fractional Flow Reserve derived from CTCA (FFRCT) is an additional, FDA approved non-invasive technique for defining the probability of flow-limiting coronary artery stenosis that correlates with invasive FFR measurements. Previous studies undertaken at this District General Hospital highlighted the value of this tool in streamlining invasive strategies. This follow-up study sought to assess the next step in the patient pathway, comparing those identified as intermediate to high risk based for flow limiting disease on FFRCT with the findings and management strategy employed at subsequent invasive coronary angiography. Methods A retrospective analysis of all CTCA’s (SOMATON Definition edge, Siemens) reports between April 2018 and January 2019 with FFRCT (Heartflow Inc.) undertaken were reviewed. Any imaging that reported an intermediate to high risk of flow limiting coronary disease based on FFRCT were included, (values of Results A total of 108 studies were sent for Heartflow analysis, of which 27 had intermediate or high likelihood of flow limiting coronary disease reported and have had subsequent invasive angiography. This consisted of 60% male, with a mean of age 67 (range 42-83 years). Invasive pressure wire assessment via iFR (instantaneous wave free ratio) and/or FFR was carried out in 9 (33%) patients at angiography. In total, 43 vessels with FFRCT intermediate or high likelihood vessels were assessed invasively. Table 1 below outlines the FFRCT findings versus invasive angiography management. FFRCTInvasive Coronary angiogram iFR/FFR -veiFR/FFR +veDirect Re-vascularisation (PCI)Direct Re-vascularisation (CABG)Not Invasively assessed Intermediate73115 High0112103 Table 1. This table compares FFRCT findings with invasive angiography strategy / findings. Of the 3 vessels with ‘high probability of flow-limiting disease’ that were not invasively assessed, all were branch vessels (2 diagonals and 1 obtuse marginal). Conclusions This study represents a real world NHS experience of activity undertaken in the catheter lab when functional information of coronary flow is known in advance of an invasive procedure. In some cases (13/16 [81%] of patients with a high probability of flow limiting disease) a direct decision on re-vascularisation was taken by the operator without further invasive pressure wire assessment, which may have reduced procedure duration. Further experience with FFRCT may increase operator confidence and thus increase the frequency of proceeding directly to re-vascularisation where indicated, thus reducing both procedure and fluoroscopic screening times. A further assessment of the role of FFRCT employed for stent planning pre-procedure is intended. Conflict of Interest None

AIM To quantify the real-world clinical and cost impact of computed tomography (CT) coronary angiography (CTCA)-derived fractional flow reserve (FFRCT) in the National Health Service (NHS). MATERIALS AND METHODS Consecutive clinical CTCA examinations from September to December 2018 with ≥1 stenosis of ≥25% underwent FFRCT analysis. The Heart Team reviewed clinical data and CTCA findings, blinded to FFRCT values, and documented hypothetical consensus management. FFRCT results were then unblinded and hypothetical consensus management re-recorded. Diagnostic waiting times for management pathways were estimated. A per-patient cost analysis for diagnostic certainty regarding coronary artery disease (CAD) management was performed using 2014–2020 NHS tariffs for pre- and post-FFRCT pathways. RESULTS Two hundred and fifty-one CTCAs were performed during the study period. Fifty-seven percent (145/251) had no CAD or stenosis CONCLUSIONS In the real-world NHS setting, FFRCT-guided management has the potential to rationalise patient management, accelerate diagnostic pathways, and depending on the stenosis severity modelled, may be cost-effective.

Brill’s Companion to Anarchism and Philosophy offers a broad thematic overview of the relationship between anarchism and philosophy.

Background The European Society of Cardiology (ESC) guidelines for management of pulmonary hypertension (PAH) advocate comprehensive assessment of patients to determine prognosis and to guide treatment decisions, using a set of risk assessment criteria based on expert advice. These criteria are coded Red (high), Amber (medium) and Green (low). It is unclear whether these criteria are associated with short term survival. Aim To determine whether red/amber/green risk status according to ESC guidelines is associated with 12 month mortality. Methods This was a “snapshot” observational study using routinely collected clinical data for patients eligible for targeted drug treatment at a regional centre, under shared care with a national centre. All data available at the latest visit within the study period were collated, including demographics, echocardiogram and right heart catheterisation data. Data are reported as mean/median/count/%. Characteristics of deceased and surviving patients were compared using Mann-Witney U-test. Association with 12 month mortality was assessed using Receiver Operator Characteristics (ROC) curve analysis. Results Routinely collected clinic data were available for 104 patients, echocardiograms for 88 and right heart catheter data for 68. 25% were male, mean age 68.2 years. 45.2% had connective tissue disease-associated PAH, 32.7% inoperable chronic thromboembolic PH, 18.3% Idiopathic PAH. 101 were on treatment, of which 35.6% were on monotherapy, 51.0% on dual oral therapy, 9.6% on intravenous treatments. Baseline data are shown in the table. 25% had one red criterion, 14.4% had two and 8.6% had three or more. 19 patients died in the 12 month follow-up period, 6 of whom had no red criteria. Deceased patients were older (p=0.015) and had shorter walking distance (p=0.003). Risk criteria were worse for symptom progression, WHO functional class, walking distance and for the overall number of red criteria. ROC-curve analysis showed that symptom progression (c-statistic 0.695, p=0.048), walking distance (0.748, p=0.012) and the overall number of red flags (0.710, p=0.033) were the only elements associated with 12 month mortality. Conclusions The ESC risk assessment criteria are associated with 12 month mortality in this cohort when all criteria are collated. Further work in a large cohort is needed to confirm the clinical utility of these criteria.

Haemodynamic assessment in the catheterization laboratory is the process by which various aspects of a patient’s haemodynamics are measured invasively, with parameters such as the pressures in various cardiac chambers, blood flow and oxygen saturations being recorded. These data, along with information from other non-invasive tests, are used to assess the cardiac status of the patient and can be vital in planning treatment strategies. This article looks at the utility of such assessments, and discusses the clinical importance of this aspect of work in the catheter laboratory.

We report two cases of non-cardiogenic pulmonary edema as a complication of basiliximab induction therapy in young pediatric renal transplant patients identified following a retrospective review of all pediatric renal transplant cases performed in the National Paediatric Transplant Centre, Childrens University Hospital, Temple Street, Dublin, Ireland. Twenty-eight renal transplantations, of which five were living-related (LRD) and 23 were from deceased donors (DD), were performed in 28 children between 2003 and 2006. In six cases, transplantations were pre-emptive. Immunosuppression was induced pre-operatively using a combination of basiliximab, tacrolimus and methylprednisolone in all patients. Basiliximab induction was initiated 2 h prior to surgery in all cases and, in 26 patients, basiliximab was re-administered on post-operative day 4. Two patients, one LRD and one DD, aged 6 and 11 years, respectively, developed acute non-cardiogenic pulmonary edema within 36 h of surgery. Renal dysplasia was identified as the primary etiological factor for renal failure in both cases. Both children required assisted ventilation for between 4 and 6 days. While both grafts had primary function, the DD transplant patient subsequently developed acute tubular necrosis and was eventually lost within 3 weeks due to thrombotic microangiopathy and severe acute antibody-mediated rejection despite adequate immunosuppression. Non-cardiogenic pulmonary edema is a potentially devastating post-operative complication of basiliximab induction therapy in young pediatric patients following renal transplantation. Early recognition and appropriate supportive therapy is vital for patient and, where possible, graft survival.

Ludwig von Mises tried, in Bureaucracy (2007 [1944]), to make the bureaucratic or entrepreneurial character of an organization a simple matter of its organizational goals rather than its function. He argued that the corporate hierarchy as such was not a bureaucracy. He defined bureaucracy as rules-based management, with processes set on Weberian lines, rather than profit-based management, because it produced no marketable product and its output had no market price. The large business enterprise, on the other hand, was—thanks to the miracle of double-entry bookkeeping—an extension of the entrepreneur’s will. The entrepreneur could track the profits and losses of each subdivision, and accordingly shift investment between subdivisions and discipline or replace managers. The motivation of all corporate employees would be profit seeking, their wills in harmony with those of the shareholders, because they belonged to the shareholders’ organization. By treating the firm as permeated by the entrepreneur’s will, Mises, like the neoclassicals, essentially treated it as a unitary actor in the marketplace and its internal workings as a black box. Although at one point he explicitly denied that the entrepreneur was omnipresent, in practice Mises viewed his entrepreneur as a brooding omnipresence whose influence guided the action of every employee from CEO to janitor.

A 13-year-old adolescent presented for correction of an 85-degree idiopathic thoracic scoliosis. She was known to have Prader-Willi syndrome. Previous general anaesthesia for non-spinal surgery had been uneventful. On two occasions following uneventful induction and total intravenous anaesthesia (TIVA) maintenance, she developed severe recalcitrant bradycardia with hypotension that was resistant to anticholinergics, inotropes and vasopressors upon prone positioning. Immediate resolution occurred upon a return to the supine position. On each occasion, she emerged from anaesthesia with no untoward sequelae. Cardiac investigations, including echocardiography, electrocardiography (ECG), troponin and creatine kinase-MB fraction levels were all within the normal range, and ventricular function was good. It was necessary to urgently proceed with the surgery as the scoliosis was progressive, with risks of cardiovascular and respiratory compromise. Additionally, she was scheduled to recommence growth hormone therapy postoperatively to treat her growth retardation. Ultimately, she received propofol for induction of anaesthesia and TIVA with propofol and remifentanil infusions for the maintenance of anaesthesia. Post-induction, a transvenous pacing wire was placed under ECG guidance. A transoesphageal probe was inserted and cardiac function monitored throughout the procedure. Upon prone positioning, she again developed a bradycardia which responded to pacing and surgery was carried out uneventfully. Clinical examination and extensive investigations had failed to demonstrate any specific underlying cause for her repeated positional arrhythmia. Therefore, we deduced that the bradycardia was due to a hypervagal response provoked by prone positioning and because of the severity of the scoliosis. We are unaware of reports of such a complication in the literature.Keywords: anaesthesia, cardiovascular, scoliosis, bradycardia, pacing

An 87-year-old woman from a nursing home with a history of ventricular rate responsive demand (VVIR) Microny II SR (St. Jude Medical AB, Jarfalla, Sweden) pacemaker insertion for syncope with complete heart block in 2003 was admitted following an episode of loss of consciousness. This occurred as she was being helped to the toilet by two carers each supporting her under one arm. …