Publications by authors named "Thomas Semple"

38 Publications

Extracorporeal membrane oxygenation: Bridging therapy in paediatric pulmonary Langerhans cell histiocytosis.

J Paediatr Child Health 2021 Sep 12. Epub 2021 Sep 12.

Department of Paediatrics, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom.

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http://dx.doi.org/10.1111/jpc.15712DOI Listing
September 2021

Tracheomegaly following antenatal treatment for congenital diaphragmatic hernia.

Arch Dis Child 2021 Aug 20. Epub 2021 Aug 20.

Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK.

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http://dx.doi.org/10.1136/archdischild-2021-322232DOI Listing
August 2021

Follow the Lead: The Challenges of Cardiogenic Shock in Device-Related Infective Endocarditis.

JACC Case Rep 2021 Aug 4;3(9):1163-1169. Epub 2021 Aug 4.

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom.

We present the challenging case of a young man with congenital heart disease who survived severe device-related infective endocarditis and new pulmonary hypertension. He required prolonged mechanical circulatory support and had multiple significant complications. His case posed a management dilemma that was successfully resolved by effective multidisciplinary, tertiary center care. ().
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http://dx.doi.org/10.1016/j.jaccas.2021.05.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8353571PMC
August 2021

The authors reply.

Crit Care Med 2021 Aug 9. Epub 2021 Aug 9.

Royal Brompton Hospital, Chelsea, London, United Kingdom National Heart & Lung Institute, Imperial College London, London, United Kingdom Lister Hospital, Stevenage, Hertfordshire, United Kingdom Imperial College Healthcare NHS Trust, London, United Kingdom.

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http://dx.doi.org/10.1097/CCM.0000000000005259DOI Listing
August 2021

Don't Stop Beleafing: A Unique Case of Fungal Infective Endocarditis.

JACC Case Rep 2021 Apr 31;3(4):672-677. Epub 2021 Mar 31.

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Trust, National Heart and Lung Institute, Imperial College, London, United Kingdom.

We present the case of a 60-year-old man who was successfully treated for obstructive fungal infective endocarditis of the ascending aorta caused by . This extremely rare cause of fungal infective endocarditis required surgical and prolonged medical management, facilitated by effective multidisciplinary cooperation. ().
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http://dx.doi.org/10.1016/j.jaccas.2021.02.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8302800PMC
April 2021

A Short extension to multiple breath washout provides additional signal of distal airway disease in people with CF: A pilot study.

J Cyst Fibros 2021 Jul 15. Epub 2021 Jul 15.

Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom; National Heart and Lung Institute, Imperial College London, Manresa Rd, London, United Kingdom; European Cystic Fibrosis Society Lung Clearance Index Core Facility, London, United Kingdom.

Background: Adding a slow vital capacity (SVC) to multiple breath washout (MBW) allows quantification of otherwise overlooked signal from under/un-ventilated lung units (UVLU) and may provide a more comprehensive assessment of airway disease than conventional lung clearance index (LCI).

Methods: We conducted a pilot study on people undergoing MBW tests: 10 healthy controls (HC) and 43 cystic fibrosis (CF) subjects performed an SVC after the standard end of test. We term the new outcome LCI with Short extension (LCI). We assessed (i) CF/ HC differences, (ii) variability (iii) effect of pulmonary exacerbation (PEx)/treatment and (iv) relationship with CF computed tomography (CFCT) scores.

Results: HC/ CF group differences were larger with LCI than LCI (P<0.001). Within the CF group UVLU was highly variable and when abnormal it did not correlate with corresponding LCI. Signal showed little variability during clinical stability (n = 11 CF; 2 visits; median inter-test variability 2.6% LCI 2.5% LCI). PEx signal was significantly greater for LCI both for onset and resolution. Both MBW parameters correlated significantly with total lung CT scores and hyperinflation but only LCI correlated with mucus plugging.

Conclusions: UVLU captured within the LCI varies between individuals; the lack of relationship with LCI demonstrates that new, additional information is being captured. LCI repeatability during clinical stability combined with its larger signal around episodes of PEx may lend it superior sensitivity as an outcome measure. Further studies will build on this pilot data to fully establish its utility in monitoring disease status.
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http://dx.doi.org/10.1016/j.jcf.2021.06.013DOI Listing
July 2021

Evolution of reproductive structures for in-flight mating in thynnine wasps (Hymenoptera: Thynnidae: Thynninae).

J Evol Biol 2021 Sep 9;34(9):1406-1422. Epub 2021 Aug 9.

Ecology and Evolution, Research School of Biology, The Australian National University, Canberra, Australia.

Thynnine wasps have an unusual mating system that involves concurrent in-flight copulation and nuptial feeding of wingless females by alate males. Consequently, thynnine genitalia play a multifunctional role and have likely been subject to various different selective pressures for both reproductive success and food provisioning. Here, we present a new molecular phylogeny for the Australian Thynninae and use 3D-geometric morphometrics and comparative methods to investigate the morphological evolution of select genital structures across the group. We found significant morphological integration between all male and female structures analysed, which is likely influenced by sexual selection, but also reproductive isolation requirements and mechanical constraints. The morphology of the primary male and female coupling structures was correlated with female body size, and female genitalia exhibited strong negative size allometry. Those male and female coupling structures have evolved at similar evolutionary rates, whereas female structures appear to have evolved a higher degree of morphological novelty over time. We conclude that the unique reproductive strategies of thynnine wasps have resulted in complex evolutionary patterns in their genital morphology, which has likely played a central role in the extensive diversification of the subfamily across Australasia and South America. Our study reinforces the need to treat composite characters such as genitalia by their component parts, and to consider the roles of both male and female reproductive structures in evolutionary studies.
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http://dx.doi.org/10.1111/jeb.13902DOI Listing
September 2021

The curious incident of the cast in the airway.

Thorax 2021 May 7. Epub 2021 May 7.

Respiratory Department, Royal Brompton Hospital, London, UK.

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http://dx.doi.org/10.1136/thoraxjnl-2020-216426DOI Listing
May 2021

Fructose 1,6-bisphosphatase deficiency as a cause of childhood interstitial lung disease.

Pediatr Pulmonol 2021 Jul 17;56(7):2362-2365. Epub 2021 Mar 17.

Paediatric Respiratory Medicine, Nottingham Children's Hospital, Nottingham University Hospitals NHS Trust, Queen's Medical Centre, Nottingham, UK.

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http://dx.doi.org/10.1002/ppul.25362DOI Listing
July 2021

Multi-institution assessment of the use and risk of cardiovascular computed tomography in pediatric patients with congenital heart disease.

J Cardiovasc Comput Tomogr 2021 Sep-Oct;15(5):441-448. Epub 2021 Jan 27.

Minneapolis Heart Institute and Foundation, Minneapolis, MN, USA; Children's Minnesota, Minneapolis, MN, USA. Electronic address:

Background: Cardiac computed tomography (CT) is increasingly used in pediatric patients with congenital heart disease (CHD). Variability of practice and of comprehensive diagnostic risk across institutions is not known.

Methods: Four centers prospectively enrolled consecutive pediatric CHD patients <18 years of age undergoing cardiac CT from January 6, 2017 to 1/30/2020. Patient characteristics, cardiac CT data and comprehensive diagnostic risk were compared by age and institutions. Risk categories included sedation and anesthesia use, vascular access, contrast exposure, cardiovascular medication, adverse events (AEs), and estimated radiation dose.

Results: Cardiac CT was performed in 1045 pediatric patients at a median (interquartile range, IQR) age of 1.7 years (0.3, 11.0). The most common indications were arterial abnormalities, suspected coronary artery anomalies, functionally single ventricle heart disease, and tetralogy of Fallot/pulmonary atresia. Sedation was used in 8% and anesthesia in 11% of patients. Peripheral vascular access was utilized for 93%. Median contrast volume was 2 ​ml/kg. Beta blockers were administered in 11% of cases and nitroglycerin in 2% of cases. The median (IQR) total procedural dose length product (DLP) was 20 ​mGy∗cm (10, 50). Sedation, vascular access, contrast exposure, use of cardiovascular medications and radiation dose estimates varied significantly by institution and age (p ​< ​0.001). Seven minor adverse events (0.7%) and no major adverse events were reported.

Conclusion: Cardiac CT for CHD is safe in pediatric patients when appropriate CT technology and expertise are available. Scans can be acquired at relatively low radiation exposure with few minor adverse events.
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http://dx.doi.org/10.1016/j.jcct.2021.01.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8313631PMC
January 2021

Prevalence of Thrombotic Complications in ICU-Treated Patients With Coronavirus Disease 2019 Detected With Systematic CT Scanning.

Crit Care Med 2021 05;49(5):804-815

Royal Brompton Hospital, London, United Kingdom.

Objectives: Severe coronavirus disease 2019 is associated with an extensive pneumonitis and frequent coagulopathy. We sought the true prevalence of thrombotic complications in critically ill patients with severe coronavirus disease 2019 on the ICU, with or without extracorporeal membrane oxygenation.

Design: We undertook a single-center, retrospective analysis of 72 critically ill patients with coronavirus disease 2019-associated acute respiratory distress syndrome admitted to ICU. CT angiography of the thorax, abdomen, and pelvis were performed at admission as per routine institution protocols, with further imaging as clinically indicated. The prevalence of thrombotic complications and the relationship with coagulation parameters, other biomarkers, and survival were evaluated.

Setting: Coronavirus disease 2019 ICUs at a specialist cardiorespiratory center.

Patients: Seventy-two consecutive patients with coronavirus disease 2019 admitted to ICU during the study period (March 19, 2020, to June 23, 2020).

Interventions: None.

Measurements And Main Results: All but one patient received thromboprophylaxis or therapeutic anticoagulation. Among 72 patients (male:female = 74%; mean age: 52 ± 10; 35 on extracorporeal membrane oxygenation), there were 54 thrombotic complications in 42 patients (58%), comprising 34 pulmonary arterial (47%), 15 peripheral venous (21%), and five (7%) systemic arterial thromboses/end-organ embolic complications. In those with pulmonary arterial thromboses, 93% were identified incidentally on first screening CT with only 7% suspected clinically. Biomarkers of coagulation (e.g., d-dimer, fibrinogen level, and activated partial thromboplastin time) or inflammation (WBC count, C-reactive protein) did not discriminate between patients with or without thrombotic complications. Fifty-one patients (76%) survived to discharge; 17 (24%) patients died. Mortality was significantly greater in patients with detectable thrombus (33% vs 10%; p = 0.022).

Conclusions: There is a high prevalence of thrombotic complications, mainly pulmonary, among coronavirus disease 2019 patients admitted to ICU, despite anticoagulation. Detection of thrombus was usually incidental, not predicted by coagulation or inflammatory biomarkers, and associated with increased risk of death. Systematic CT imaging at admission should be considered in all coronavirus disease 2019 patients requiring ICU.
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http://dx.doi.org/10.1097/CCM.0000000000004890DOI Listing
May 2021

Pulmonary manifestations of systemic vasculitis in childhood.

Breathe (Sheff) 2020 Dec;16(4):200211

Dept of Paediatric Respiratory Medicine, King's College Hospital NHS Foundation Trust, London, UK.

The systemic vasculitides are a heterogenous group of rare conditions with an incompletely understood aetiology. Any of the systemic vasculitides may cause respiratory disease, but some conditions are more likely to affect the pulmonary system, often through pulmonary infarction and diffuse alveolar haemorrhage. These conditions are often difficult to diagnose due to their rarity and significant clinical overlap with common respiratory conditions. Prompt diagnosis and management can significantly reduce morbidity and mortality.
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http://dx.doi.org/10.1183/20734735.0211-2020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7792777PMC
December 2020

Interstitial lung disease in infancy.

Early Hum Dev 2020 11 15;150:105186. Epub 2020 Sep 15.

Imperial College, UK; Royal Brompton and Harefield NHS Foundation Trust, UK.

There is a wide differential diagnosis of early onset respiratory distress especially in term babies, and interstitial lung disease (chILD) is a rare but important consideration in this context. chILD manifesting immediately after birth is usually related to mutations in surfactant protein genes, or conditions related to the Congenital Acinar Dysplasia -Alveolar capillary dysplasia - Congenital Alveolar Dysplasia (CAD-ACD) spectrum. There is currently no specific treatment for these conditions, and management is supportive. Prognosis is very poor in most of these babies if onset is early, with relentless respiratory deterioration unless transplanted. Ideally, the diagnosis is made on genetic analysis, but this may be time-consuming and complex in CAD-ACD spectrum, so lung biopsy may be needed to avoid prolonged and futile treatment being instituted. Milder forms with prolonged survival have been reported. Early onset, less severe chILD is usually related to neuroendocrine cell hyperplasia of infancy (NEHI), pulmonary interstitial glycogenosis (PIG) and less severe disorders of surfactant proteins. PIG and NEHI are not specific entities, but are pulmonary dysmaturity syndromes, and there may be a number of underlying genetic and other cause. If the child is stable and thriving, many will not be subject to lung biopsy, and slow improvement and weaning of supplemental oxygen can be anticipated. Where possible, a precise genetic diagnosis should be made in early onset cHILD allow for genetic counselling. chILD survivors and their families have complex respiratory and other needs, and co-ordinated, multi-disciplinary support in the community is essential.
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http://dx.doi.org/10.1016/j.earlhumdev.2020.105186DOI Listing
November 2020

Imaging of cerebral complications of extracorporeal membrane oxygenation in infants with congenital heart disease - ultrasound with multimodality correlation.

Pediatr Radiol 2020 06 15;50(7):997-1009. Epub 2020 Feb 15.

Radiology Department, The Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK.

Cranial ultrasound on neonatal intensive care units is generally performed by intensive care physicians, but radiologists often provide this crucial bedside test to children on specialist paediatric cardiac intensive care units. On a paediatric cardiac intensive care unit, complex congenital cardiac conditions are commonly encountered in both pre- and postoperative scenarios, often with the use of extracorporeal membrane oxygenation (ECMO), which both increases the risks of a number of neurologic complications and results in significant changes in vascular physiology. The aim of this pictorial essay is to discuss cranial ultrasound technique, demonstrate the changes in Doppler flow profiles resulting from veno-arterial extracorporeal membrane oxygenation and congenital cardiac conditions, and illustrate commonly encountered intracranial complications of extracorporeal membrane oxygenation support in congenital cardiac care.
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http://dx.doi.org/10.1007/s00247-019-04603-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7286855PMC
June 2020

Umbilical artery catheter, aortic dissection, carotid cannulation, and pseudoaneurysm in a neonate: A tale of propagating pathology.

Ann Pediatr Cardiol 2020 Jan-Mar;13(1):87-90. Epub 2019 Jul 19.

Department of Pediatric Intensive Care, Royal Brompton Hospital, London, United Kingdom.

Arterial dissections are uncommon in neonates. Complications include thrombosis, bleeding, dissection, aneurysm and pseudoaneurysm. We report an unusual case of multisite pathology (dissection and pseudoaneurysm) following common vascular interventions. A term neonate with antenatal diagnosis of congenital heart block secondary to maternal lupus deteriorated clinically at 5 days of life. He was found to have an abdominal aortic thrombus secondary to abdominal aortic dissection, following umbilical arterial catheter placement. Attempted percutaneous treatment was complicated by dissection of the left common carotid artery and formation of a large pseudoaneurysm. Neonatal lupus is associated with weakened vessel wall which may be vulnerable to injury from line placement and endovascular interventions. Various options are available to manage arterial dissection, thrombus, and pseudoaneurysm, but consequences of these options need to be carefully weighed to minimize further complications.
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http://dx.doi.org/10.4103/apc.APC_67_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6979014PMC
July 2019

A comprehensive and user-friendly framework for 3D-data visualisation in invertebrates and other organisms.

J Morphol 2019 02;280(2):223-231

Department of Terrestrial Zoology, Western Australian Museum, Perth, Western Australia, Australia.

Methods for 3D-imaging of biological samples are experiencing unprecedented development, with tools such as X-ray micro-computed tomography (μCT) becoming more accessible to biologists. These techniques are inherently suited to small subjects and can simultaneously image both external and internal morphology, thus offering considerable benefits for invertebrate research. However, methods for visualising 3D-data are trailing behind the development of tools for generating such data. Our aim in this article is to make the processing, visualisation and presentation of 3D-data easier, thereby encouraging more researchers to utilise 3D-imaging. Here, we present a comprehensive workflow for manipulating and visualising 3D-data, including basic and advanced options for producing images, videos and interactive 3D-PDFs, from both volume and surface-mesh renderings. We discuss the importance of visualisation for quantitative analysis of invertebrate morphology from 3D-data, and provide example figures illustrating the different options for generating 3D-figures for publication. As more biology journals adopt 3D-PDFs as a standard option, research on microscopic invertebrates and other organisms can be presented in high-resolution 3D-figures, enhancing the way we communicate science.
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http://dx.doi.org/10.1002/jmor.20938DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6590182PMC
February 2019

Right ventricular dysfunction 10 years following a Bentall procedure.

J Card Surg 2018 09 22;33(9):563-564. Epub 2018 Aug 22.

Cardiology and Aortic Centre, Royal Brompton Hospital and National Heart and Lung Institute of Imperial College London, Sydney Street, London, UK.

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http://dx.doi.org/10.1111/jocs.13788DOI Listing
September 2018

Massive paediatric cardiac tumour: good medium-term outcome.

Arch Dis Child 2019 11 22;104(11):1098. Epub 2018 Aug 22.

Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK.

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http://dx.doi.org/10.1136/archdischild-2018-315201DOI Listing
November 2019

Accuracy of computed tomography in detection of great vessel stenosis or hypoplasia before superior bidirectional cavopulmonary connection: Comparison with cardiac catheterization and surgical findings.

Arch Cardiovasc Dis 2019 Jan 21;112(1):12-21. Epub 2018 Jul 21.

Department of paediatric cardiology, Royal Brompton Hospital, Sydney street, London SW3 6NP, UK.

Background: Cardiac catheterization is the gold-standard modality for investigation of cardiovascular morphology before bidirectional cavopulmonary connection, but requires general anaesthesia and is associated with procedural risk.

Aims: To assess the diagnostic accuracy and safety of computed tomography in diagnosing great vessel stenosis/hypoplasia compared with cardiac catheterization and surgical findings.

Methods: Twenty-seven patients (10 after Norwood stage I) underwent computed tomography before surgery between January 2010 and June 2016; 16 of these patients also underwent cardiac catheterization. Proximal and distal pulmonary artery, aortic isthmus and descending aorta measurements, radiation dose and complications were compared via Bland-Altman analyses and correlation coefficients.

Results: The accuracy of computed tomography in detecting stenosis/hypoplasia of either pulmonary artery was 96.1% compared with surgical findings. For absolute vessel measurements and Z-scores, there was high correlation between computed tomography and angiography at catheterization (r=0.98 for both) and a low mean bias (0.71mm and 0.48; respectively). The magnitude of intertechnique differences observed for individual patients was low (95% of the values ranged between -0.9 and 2.3mm and between -0.7 and 1.7, respectively). Four patients (25%) experienced minor complications from cardiac catheterization, whereas there were no complications from computed tomography. Patients tended to receive a higher radiation dose with cardiac catheterization than with computed tomography, even after exclusion of interventional catheterization procedures (median 2.5 mSv [interquartile range 1.3 to 3.4 mSv] versus median 1.3 mSv [interquartile range 0.9 to 2.6 mSv], respectively; P=0.13). All computed tomography scans were performed without sedation.

Conclusions: Computed tomography may replace cardiac catheterization in identification of great vessel stenosis/hypoplasia before bidirectional cavopulmonary connection when no intervention before surgery is required. Computed tomography carries lower morbidity, can be performed without sedation and may be associated with less radiation.
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http://dx.doi.org/10.1016/j.acvd.2018.04.006DOI Listing
January 2019

Sleep disordered breathing and ventilatory support in children with Down syndrome.

Pediatr Pulmonol 2018 10 10;53(10):1414-1421. Epub 2018 Jul 10.

Department of Pediatric Respiratory Medicine, Royal Brompton Hospital, London, United Kingdom.

Study Objectives: Obstructive sleep apnoea (OSAS) in children with Down syndrome (DS) is now well recognized, but other forms of sleep disordered breathing (SDB) in this population are less well described. Anecdotally, respiratory support for SDB treatment in this population is not easily tolerated. We aimed to characterize the types of SDB in children with DS referred to a tertiary respiratory center and to assess the effectiveness and adherence to respiratory support.

Methods: Retrospective study of DS patients <18 years old under follow-up at a tertiary respiratory center. Anthropometrics, comorbidities, sleep study results, and details of respiratory support were collected. Satisfactory adherence to oxygen (O ), Continuous Positive Airway Pressure (CPAP), or bilevel noninvasive ventilation (NIV) was defined as use >4 h/night for >50% nights.

Results: Sixty patients were included, median age 1.5 (0.7-5.3) years; 49 (82%) had congenital heart disease, 16 (27%) pulmonary hypertension, 28 (47%) gastroesophageal reflux, 38 (63%) swallowing impairment; 16/17 who underwent CT scanning had evidence of aspiration. Forty-two had SDB: 27 (61%) OSAS (10 mild, 5 moderate, 12 severe), 11 (25%) central apnoeas, 19 (32%) nocturnal hypoventilation. Twenty-six had baseline saturations <95%. Lower SpO correlated with pulmonary hypertension (r  = 0.1, P = 0.04). Thirty-nine (65%) patients started respiratory support (14 O , 18 CPAP, 7 NIV) and 22 (56%) have regularly used it. After a 1.9 years follow up 11/24 had satisfactory adherence to CPAP/NIV (average use 8 h/night).

Conclusions: Our results confirm the high prevalence of OSAS in children with DS. A significant number also have low baseline saturations, central apnoeas, and nocturnal hypoventilation. Contrary to popular belief, more than half of children with DS had satisfactory adherence to respiratory support.
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http://dx.doi.org/10.1002/ppul.24122DOI Listing
October 2018

Development of a congenital cardiovascular computed tomography imaging registry: Rationale and implementation.

J Cardiovasc Comput Tomogr 2018 May - Jun;12(3):263-266. Epub 2018 Mar 16.

Minneapolis Heart Institute Foundation at Abbott Northwestern Hospital, Minneapolis, MN, USA.

Background: The cumulative exposure and risk of anesthesia, vascular access, contrast agents and radiation is emerging as a significant lifelong burden in patients with congenital heart disease (CHD). Latest generation computerized tomographic (CT) scanners are increasingly used for high resolution cardiovascular imaging and have both hardware and post processing radiation dose reduction strategies that can be implemented. Currently, these dose reduction strategies are not uniformly applied and there is a large variability in radiation dose used for the performance of CT in CHD.

Methods: We propose the development and implementation of a prospective, multi-center and multi-specialty consortium to measure the variability of use, risk and image quality of CT scans in patients of all ages with CHD. The primary goals of this collaboration are 1) define variability of use, diagnostic quality, and risk of cardiac CT 2) establish best practice guidelines designed to optimize diagnostic image quality with appropriate use of radiation and anesthesia exposure 3) provide institution specific feedback compared with the group norm across participating centers 4) improve the level of evidence for the use of CT in CHD through the collection of prospective and multi-institutional data.

Conclusions: Prospective multi-institutional data is needed to inform risk estimates of CT in CHD using current generation scanners and aggressive dose optimization techniques. This registry will provide a platform for future collaboration establishing a multi-modality risk assessment tool specific to patients with CHD.
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http://dx.doi.org/10.1016/j.jcct.2018.03.006DOI Listing
August 2018

False lumen intervention to promote remodelling and thrombosis-The FLIRT concept in aortic dissection.

Catheter Cardiovasc Interv 2018 10 30;92(4):732-740. Epub 2018 Mar 30.

Cardiology and Aortic Centre, Royal Brompton & Harefield NHS Foundation Trust; Imperial College London, London, SW3 6NP, United Kingdom.

Objective: Thoracic endovascular aortic repair (TEVAR) has changed the management of aortic dissection by induced remodelling. Beyond reconstructing the true lumen, we describe the concept of False Lumen Intervention to promote Remodelling and Thrombosis (FLIRT) in both type A and B aortic dissection.

Methods: Between 2011 and 2017, 10 patients with aortic dissection (5 type A; 5 type B) underwent FLIRT using a combination of patent foramen ovale (PFO) or atrial septal defect (ASD) occluders, coils and glue. Patients were followed by computed tomography (CT) angiogram prior to, and 6 months following, discharge to evaluate false lumen (FL) thrombosis and aortic remodelling. Outcomes analyzed comprised successful device delivery, completeness of FL thrombosis and aortic remodelling, procedure related complications and mortality.

Results: FLIRT induced aortic remodelling in all cases of proximal dissection, with aortic shrinkage from 63.8 ± 7.5 pre-FLIRT, to 50.2 ± 6.6 mm (P = 0.057) and an increase in true lumen area from 5.8 ± 3.6 to 11.4 ± 2.5 cm (P = 0.006). In distal dissection (after previous TEVAR with residual FL flow), FLIRT successfully induced FL thrombosis in 4 of 5 cases at first attempt (1 case required additional coiling of the gutter between left subclavian artery and stent-graft for complete thrombosis). While maximal aortic diameter remained unchanged (55.6 ± 9.1 pre-FLIRT and 54.4 ± 13.7 mm at follow-up), true lumen area increased from 7.8 ± 2.3 pre-procedure, to 10.6 ± 1.5 cm at follow-up (P = 0.016), consistent with remodelling.

Conclusion: Interventional FL management, using the FLIRT concept, is feasible in selected cases of aortic dissection, promotes FL thrombosis and induces successful remodelling.
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http://dx.doi.org/10.1002/ccd.27599DOI Listing
October 2018

Left circumflex coronary artery from the pulmonary artery in scimitar syndrome.

Pediatr Radiol 2018 05 14;48(5):632-637. Epub 2018 Mar 14.

Department of Radiology, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK.

Background: Scimitar syndrome is a rare combination of cardiopulmonary abnormalities found in 1-3 per 1000 live births. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is only found in 1 in 250-400 congenital heart disease patients.

Objective: We aimed to investigate the incidence of left circumflex ALCAPA within our referral center's cohort of scimitar syndrome patients.

Materials And Methods: A review of medical records, cardiac imaging and operative notes from all patients diagnosed with scimitar syndrome at our center between 1992 and 2016 was undertaken and all imaging reviewed.

Results: Fifty-four patients with scimitar syndrome and imaging were identified. Of these, 3 patients (1 male and 2 female) with ALCAPA were identified, representing an incidence of 5.5% (95% confidence interval [CI] 0-11.67%). In all three cases, the anomalous coronary arising from the pulmonary artery was the left circumflex coronary artery (LCx) and the point of origin was close to the pulmonary arterial bifurcation.

Conclusion: We hypothesize that the prevalence of LCx-ALCAPA, in the setting of scimitar syndrome, may be greater than previously thought. We suggest that any patient with scimitar syndrome, especially with evidence of ischaemia, should be investigated for ALCAPA. Given its noninvasive nature and simultaneous imaging of the lungs, we suggest that cardiovascular CT is the most appropriate first-line investigation for these patients.
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http://dx.doi.org/10.1007/s00247-017-4067-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5895679PMC
May 2018

'Porcelain aorta': a proposed definition and classification of ascending aortic calcification.

Open Heart 2018;5(1):e000703. Epub 2018 Jan 26.

Department of Radiology, Royal Brompton Hospital, London, UK.

Introduction: 'Porcelain aorta' is listed in the second consensus document of the Valve Academic Research Consortium as a risk factor in aortic valve replacement. However, the extent of circumferential involvement is poorly defined with great variability in reported incidence. We present a simple, reproducible classification to describe the extent of aortic calcification and thus appropriately define 'porcelain aorta', aiding clinical decision-making and registry data collection.

Methods: 175 consecutive CT aortograms were reviewed. The aorta was divided into three sections, and each section divided into quadrants. These were individually scored using a 5-point scale (0-no calcification, 5-complete contiguous calcification).Results for each quadrant were summated for each segment to provide an indication of the distribution of calcification.

Results: Only one patient (0.6%) had a 'true' porcelain aorta, defined as contiguous calcification across all quadrants at any aortic level. Intraobserver and interobserver variation was excellent for the ascending aorta (K=0.85-0.88 and 0.81-0.96, respectively) while the interobserver variation in the transverse arch was good at 0.75.

Conclusions: Our data suggest the incidence of 'true' porcelain aorta may be significantly lower than reported in the literature. The predominance of calcification within the anterior wall of the proximal ascending aorta and the superior wall of the transverse arch may be clinically important. Application of this quick, simple and reproducible grading system, with no requirement for advanced software, may provide a tool to support accurate assessment of focal aortic calcification and its relationship to subsequent procedural risk.
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http://dx.doi.org/10.1136/openhrt-2017-000703DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5786940PMC
January 2018

A crown of thorns-right ventricular outflow tract obstruction caused by calcific pericardial ring.

Eur Heart J Cardiovasc Imaging 2018 01;19(1):83

Department of Cardiac MRI, Royal Brompton Hospital, National Heart and Lung Institute, Imperial College London, UK.

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http://dx.doi.org/10.1093/ehjci/jex228DOI Listing
January 2018

Interstitial Lung Disease in Children Made Easier…Well, Almost.

Radiographics 2017 Oct;37(6):1679-1703

From the Department of Imaging, Royal Brompton Hospital, Sydney Street, London, England SW3 6NP (T.R.S.); and Departments of Imaging (T.R.S., C.M.O.) and Histopathology (M.T.A.), Great Ormond Street Hospital, London, England.

Interstitial lung disease (ILD) in pediatric patients is different from that in adults, with a vast array of pathologic conditions unique to childhood, varied modes of presentation, and a different range of radiologic appearances. Although rare, childhood ILD (chILD) is associated with significant morbidity and mortality, most notably in conditions of disordered surfactant function, with respiratory failure in 100% of neonates with surfactant protein B dysfunction and 100% mortality without lung transplantation. The authors present a summary of lung development and anatomy, followed by an organized approach, using the structure and nomenclature of the 2013 update to the chILD Research Network classification system, to aid radiologic diagnosis of chILD. Index radiologic cases with contemporaneous histopathologic findings illustrate a summary of recent imaging studies covering the full spectrum of chILD. chILD is best grouped by age at presentation from infancy (diffuse developmental disorders, lung growth abnormalities, specific conditions of unknown origin, surfactant dysfunction mutations) to later childhood (disorders of the normal host, disorders related to systemic disease processes, disorders related to immunocompromise). Appreciation of the temporal division of chILD into infant and later childhood onset, along with a sound understanding of pulmonary organogenesis and surfactant homeostasis, will aid in providing useful insight into this important group of pediatric conditions. Application of secondary lobular anatomy to interpretation of thin-section computed tomographic images is pivotal to understanding patterns of ILD and will aid in selecting and narrowing a differential diagnosis. RSNA, 2017.
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http://dx.doi.org/10.1148/rg.2017170006DOI Listing
October 2017
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