Publications by authors named "Conal Austin"

43 Publications

Degradation of the Endothelial Glycocalyx Contributes to Metabolic Acidosis in Children Following Cardiopulmonary Bypass Surgery.

Pediatr Crit Care Med 2021 May 4. Epub 2021 May 4.

Paediatric Intensive Care, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom. Department of Women and Children's Health, School of Life Course Sciences, King's College London, St Thomas' Hospital, London, United Kingdom. Thrombosis and Vascular Biology Research Group, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom. Department of Cardiology, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom. Institute of Women and Children's Health, King's College London, St Thomas' Hospital, London, United Kingdom. Department of Infectious Diseases, School of Immunology and Microbial Sciences, King's College London, Guy's Hospital, London, United Kingdom. Department of Intensive Care, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom.

Objectives: Cardiopulmonary bypass surgery is complicated by metabolic acidosis, microvascular dysfunction, and capillary leak. The glycocalyx-a layer of proteins and sugars lining the vascular endothelium-is degraded during cardiopulmonary bypass. We aimed to describe the kinetics of glycocalyx degradation during and following cardiopulmonary bypass. We hypothesized that cleavage of negatively charged fragments of the glycocalyx would directly induce metabolic acidosis through changes in the strong ion gap (defined using Stewart's physicochemical approach to acid-base chemistry). We also investigated whether glycocalyx degradation was associated with failure of endothelial function and cardiovascular dysfunction.

Design: Single-center prospective cohort study.

Setting: Twenty-two bed surgical/medical PICU.

Patients: Twenty-seven term infants and children requiring cardiopulmonary bypass surgery for the correction/palliation of congenital heart disease.

Interventions: None.

Measurements And Main Results: We recruited 27 patients, 5 days to 57 months old. We prospectively sampled plasma prior to, during, and following cardiopulmonary bypass at predefined time points. We measured plasma concentrations of interleukin-6 (inflammatory marker), heparan sulfate (negatively charged glycocalyx glycosaminoglycan), and syndecan-1 (neutrally charged glycocalyx protein). We defined the following outcome measures: metabolic acidosis (strong ion gap), renal dysfunction (fold change in creatinine), capillary leak (fluid bolus volume), cardiovascular dysfunction (Vasoactive Inotropic Score), and length of ventilation. In linear regression models, maximum measured heparan sulfate concentration (negatively charged) was associated with metabolic acidosis (p = 0.016), renal dysfunction (p = 0.009), and length of ventilation (p = 0.047). In contrast, maximum measured syndecan-1 concentration (neutrally charged) was not associated with these clinical endpoints (p > 0.30 for all).

Conclusions: Our data show that metabolic acidosis (increased strong ion gap) is associated with plasma concentration of heparan sulfate, a negatively charged glycosaminoglycan cleaved from the endothelial glycocalyx during cardiopulmonary bypass. In addition, cleavage of heparan sulfate was associated with renal dysfunction, capillary leak, and global markers of cardiovascular dysfunction. These data highlight the importance of designing translational therapies to protect the glycocalyx in cardiopulmonary bypass.
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http://dx.doi.org/10.1097/PCC.0000000000002746DOI Listing
May 2021

Multisite Veno-Venous Cannulation for Neonates and Nonambulatory Children.

Pediatr Crit Care Med 2021 May 4. Epub 2021 May 4.

Department of Paediatric Intensive Care, Evelina London Children's Hospital, London, United Kingdom. Department of Cardiothoracic Surgery, Evelina London Children's Hospital, London, United Kingdom. Department of Paediatric Neuroscience, Faculty of population health King's College London, Evelina London Children's Hospital, London, United Kingdom. Department of Perfusion Department, Evelina London Children's Hospital, London, United Kingdom.

Objectives: Neonates with respiratory failure are ideally supported with veno-venous rather than veno-arterial extracorporeal membrane oxygenation due to the reduced rate of neurologic complications. However, the proportion of neonates supported with veno-venous extracorporeal membrane oxygenation is declining. We report multisite veno-venous extracorporeal membrane oxygenation, accessing the neck, returning to the inferior vena cava via the common femoral vein in neonates and children less than 10 kg.

Design: Retrospective case series with 1 year minimum follow-up.

Patients: Patients less than 10 kg supported with veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein.

Setting: A 30-bed pediatric intensive care delivering extracorporeal membrane oxygenation to approximately 20 children annually.

Interventions: Veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein was delivered using two single lumen cannulae.

Measurements And Main Results: January 2015 to August 2019, 11 patients underwent veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein with median weight of 3.6 kg (interquartile range 2.8-6.1 kg), and median corrected gestational age of 13 days (interquartile range, 2-175 d). The smallest patient weighed 2.1 kg. Seven patients had comorbidities. Extracorporeal membrane oxygenation was technically successful in all patients with median flows of 126 mL/kg/min (interquartile range, 120-138 mL/kg/min) and median arterial oxygenation saturation of 94% (interquartile range, 91-98%) at 24 hours. Nine survived to home discharge, and two were palliated. Common femoral vein occlusion was observed in all patients on ultrasound post decannulation. There was no clinical or functional deficit in the cannulated limb at follow-up, a minimum of 1 year post extracorporeal membrane oxygenation.

Conclusions: Veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein was performed safely in patients under 10 kg with the smallest patient weighing 2.1 kg. Although occlusion of the common femoral vein was observed in patients post decannulation, subsequent follow-up demonstrated no clinical implications. We challenge current practice that veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein cannot be performed in nonambulatory patients and suggest that this strategy is preferred over veno-arterial extracorporeal membrane oxygenation in infants requiring extracorporeal membrane oxygenation for respiratory failure.
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http://dx.doi.org/10.1097/PCC.0000000000002753DOI Listing
May 2021

The consequences of incomplete covering of the critical part of the aortic root in Personalized External Aortic Root Support.

Eur J Cardiothorac Surg 2021 May;59(5):1095

Clinical Operational Research Unit, University College London, London, UK.

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http://dx.doi.org/10.1093/ejcts/ezab118DOI Listing
May 2021

Out-of-hospital cardiac arrest caused by ALCAPA syndrome in adulthood.

Eur Heart J 2021 03;42(11):1118

Cardiovascular Department, School of Cardiovascular Medicine and Sciences, King's College London British Heart Foundation Centre of Excellence, James Black Centre, 125 Coldharbour Lane, London, SE5 9NU, UK.

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http://dx.doi.org/10.1093/eurheartj/ehaa827DOI Listing
March 2021

Hybrid trans-apical atrioventricular valve-in-valve implantation in Fontan circulation.

Catheter Cardiovasc Interv 2020 04 8;95(5):950-953. Epub 2020 Jan 8.

Department of Paediatric and Adult Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.

Trans-apical approach has been proved successful in failing surgical bio-prosthesis in both mitral and aortic position in adult patients. Recently, valve-in-valve treatments have been applied even in patients with complex congenital heart disease. Here, we report the case of a 32 years old lady with left atrial isomerism, complete AV septal defect, interrupted inferior vena cava with azygos continuation who underwent Kawashima procedure with atrial Fontan. Severe systemic atrioventricular valve regurgitation necessitated a 33 mm Perimount valve implantation and conversion to lateral tunnel Fontan. After only 4 years there was severe valve stenosis and the patient underwent successful trans-apical transcatheter implantation of a 29 mm Sapien valve.
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http://dx.doi.org/10.1002/ccd.28714DOI Listing
April 2020

Application of the Boston Technical Performance Score to intraoperative echocardiography.

Echo Res Pract 2019 Sep 9;6(3):63-70. Epub 2019 Jul 9.

Department of Congenital Heart Disease, Evelina London Children's Hospital, London, UK.

Background: The Technical Performance Score (TPS) developed by Boston Children's Hospital showed surgical outcomes correlate with adequacy of technical repair when implemented on pre-discharge echocardiograms. We applied this scoring system to intraoperative imaging in a tertiary UK congenital heart surgical centre.

Methods: After a period of training, intraoperative TPS (epicardial and/or transesophageal echocardiography) was instituted. TPS was used to inform intraoperative discussions and recorded on a custom-made database using the previously published scoring system. After a year, we reviewed the feasibility, results and relationship between the TPS and mortality, extubation time and length of stay.

Results: From 01 September 2015 to 04 July 2016, there were 272 TPS procedures in 251 operations with 208 TPS recorded. Seven patients had surgery with no documented TPS, three had operations with no current TPS score template available. Patients left the operating theatre with TPS optimal in 156 (75%), adequate 34 (16%) and inadequate 18 (9%). Of those with an optimal score on leaving theatre, ten had more than one period of cardiopulmonary bypass. All four deaths <30 days after surgery (1.9%) had optimal TPS. There was a statistically significant difference in extubation times in the RACHS category 4 patients (3 days vs 5 days,  < 0.05) and in PICU and total length of stay in the RACHS category three patients (2 and 8 days vs 12.5 and 21.5 days respectively) if leaving theatre with an inadequate result.

Conclusions: Application of intraoperative TPS is feasible and provides a way of objectively recording intraoperative imaging assessment of surgery. An 'inadequate' TPS did not predict mortality but correlated with a longer ventilation time and longer length of stay compared to those with optimal or adequate scores.
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http://dx.doi.org/10.1530/ERP-19-0032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6689121PMC
September 2019

Surgery for high-risk locally advanced (pT3c) renal tumours: oncological outcomes and prognostic significance of a modified International Metastatic Renal Cell Cancer Database Consortium (IMDC) score.

BJU Int 2019 09 24;124(3):462-468. Epub 2019 Jun 24.

Urology, Guy's & St Thomas' NHS Foundation Trust, London, UK.

Objectives: To evaluate contemporary oncological outcomes and long-term survival in patients undergoing surgery for urological tumours involving the peridiaphragmatic inferior vena cava up to the level of the right atrium. To apply prognostic factors developed for metastatic renal cancer to patients with very-high-risk but apparently localized tumours, and develop a scoring system.

Patients And Methods: A retrospective cohort study of 54 patients referred between December 2007 and April 2018 to a single surgical and oncological team was conducted. Electronic patient records were used to obtain peri-operative data and oncological follow-up. For operated patients lost to follow-up, survival data were obtained from primary care physicians. We used Kaplan-Meier curves to estimate overall survival (OS) and disease-free survival. For the subgroup undergoing curative surgery (n = 32) the prognostic value of a renal cancer score developed at Guy's Hospital using five of the six criteria in the International Metastatic Renal Cell Carcinoma Database Consortium prognostic model (one point for each of anaemia, neutrophilia, thrombophilia, hypercalcaemia and Karnofsky performance status <80), in order to be relevant for M0 disease, was assessed using the log-rank test.

Results: The median (interquartile range [IQR]) OS of the whole cohort was 29 (11-57) months. The median (IQR) survival of the curative subgroup (n = 32) was 32 (16-57) months, vs 11 (4-upper limit not reached) months for the cytoreductive subgroup (n = 13; P = 0.14). The median (IQR) follow-up time was 14 (1-65) months for patients alive at analysis. Disease-free survival in the curative subgroup was 10 (6-30) months. The median (IQR) OS by risk category for curative cases, as defined by the Guy's renal cancer score, was not reached in the favourable risk group (score = 0 points) because there were no patient deaths, 43 (30-61) months in the intermediate-risk group (score = 1 point), and 18 months (11-32) months in the poor-risk group (score ≥ 2 points; P = 0.005).

Conclusion: A median survival of 29 months appears to justify this type of surgery. A prognostic model, the Guy's renal cancer score, using five readily available clinical measures, appears promising in patients with very-high-risk locally advanced tumours.
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http://dx.doi.org/10.1111/bju.14755DOI Listing
September 2019

Catheter, MRI and CT Imaging in Newborns with Pulmonary Atresia with Ventricular Septal Defect and Aortopulmonary Collaterals: Quantifying the Risks of Radiation Dose and Anaesthetic Time.

Pediatr Cardiol 2018 Oct 9;39(7):1308-1314. Epub 2018 May 9.

Department of Congenital Heart Disease, Evelina Children's Hospital, London, UK.

A comprehensive understanding of the native pulmonary blood supply is crucial in newborns with pulmonary atresia with ventricular septal defect and aortopulmonary collaterals (PA/VSD/MAPCA). We sought to describe the accuracy in terms of identifying native pulmonary arteries, radiation dose and anaesthetic time associated with multi-modality imaging in these patients, prior to their first therapeutic intervention. Furthermore, we wanted to evaluate the cumulative radiations dose and anaesthetic time over the study period. Patients with PA/VSD/MAPCA diagnosed at < 100 days between 2004 and 2014 were identified. Cumulative radiation dose and anaesthetic times were calculated, with imaging results compared with intraoperative findings. We then calculated the cumulative risks to date for all surviving children. Of 19 eligible patients, 2 had echocardiography only prior to first intervention. The remaining 17 patients underwent 13 MRIs, 4 CT scans and 13 cardiac catheterization procedures. The mean radiation dose was 169 mGy cm (47-461 mGy cm), and mean anaesthetic time was 111 min (33-185 min). 3 children had MRI only with no radiation exposure, and one child had CT only with no anaesthetic. Early cross-sectional imaging allowed for delayed catheterisation, but without significantly reducing radiation burden or anaesthetic time. The maximum cumulative radiation dose was 8022 mGy cm in a 6-year-old patient and 1263 min of anaesthetic at 5 years. There is the potential to generate very high radiation doses and anaesthetic times from diagnostic imaging alone in these patients. As survival continues to improve in many congenital heart defects, the important risks of serial diagnostic imaging must be considered when planning long-term management.
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http://dx.doi.org/10.1007/s00246-018-1895-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6153876PMC
October 2018

Ventricular function and vascular dimensions after Norwood and hybrid palliation of hypoplastic left heart syndrome.

Heart 2018 02 28;104(3):244-252. Epub 2017 Jul 28.

Department of Pediatrics, UT Southwestern Medical Center, Children's Medical Center, Dallas, Texas, USA.

Objective: Norwood and hybrid procedure are two options available for initial palliation of patients with hypoplastic left heart syndrome (HLHS). Our study aimed to assess potential differences in right ventricular (RV) function and pulmonary artery dimensions using cardiac magnetic resonance (CMR) in survivors with HLHS.

Methods: 42 Norwood (mean age 2.4±0.8) and 44 hybrid (mean age 2.0±1.0 years) patients were evaluated by CMR after stage II palliation prior to planned Fontan completion. Initial stage I Norwood procedure was performed using a modified Blalock-Taussig shunt, while the hybrid procedure consisted of bilateral pulmonary artery banding and arterial duct stenting. Need for reinterventions and subsequent outcomes were also assessed.

Results: Norwood patients had larger RV end-diastolic dimensions (91±23 vs 80±31 mL/m, p=0.004) and lower heart rate (90±15 vs 102±13, p<0.001) than hybrid patients. Both Norwood and hybrid patients showed preserved global RV pump function (59±9 vs 59%±10%, p=0.91), while RV strain, strain rate and intraventricular synchrony were superior in the Norwood group. Pulmonary artery size was reduced (lower lobe index 135±74 vs 161±62 mm/m, p=0.02), and reintervention rate was significantly higher in the hybrid group whereas subsequent outcome did not differ significantly (p=0.24).

Conclusions: Norwood and hybrid strategy were associated with equivalent and preserved global RV pump function while development of the pulmonary arteries and reintervention rate were superior using the Norwood approach. Impaired RV myocardial deformation as a potential marker of early RV dysfunction in the hybrid group may have a negative long-term impact in this population.
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http://dx.doi.org/10.1136/heartjnl-2017-311532DOI Listing
February 2018

Plan, scan, model, print, manufacture, and implant personalized external aortic root support (PEARS).

J Thorac Cardiovasc Surg 2017 07;154(1):78

Cardiac Surgery, Royal Brompton Hospital, London, United Kingdom.

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http://dx.doi.org/10.1016/j.jtcvs.2017.03.037DOI Listing
July 2017

Is the medical treatment for arterial hypertension after primary aortic coarctation repair related to age at surgery? A retrospective cohort study.

Cardiol Young 2017 Nov 9;27(9):1701-1707. Epub 2017 Jun 9.

1Department of Cardiology and Cardiac Surgery,Evelina London Children's Hospital,London,United Kingdom.

Background: Hypertension following primary coarctation repair affects up to a third of subjects. A number of studies suggest that future hypertension risk is reduced if primary repair is performed at a younger age.

Objectives: The objective of this study was to evaluate the risk of future medical treatment for hypertension depending on age of primary coarctation repair.

Methods: This study was carried out at a tertiary paediatric cardiology referral centre. Retrospective database evaluation of children aged 28 days and ⩽12 months), and children (>12 months). Main outcome measure is the need for long-term anti-hypertensive medication. The risk for re-coarctation was also evaluated.

Results: A total of 87 patients were analysed: 60 neonates, 17 infants, 10 children. Among them, 6.7% neonates, 29.4% infants, and 40% children required long-term anti-hypertensive medications. Group differences were statistically significant (p=0.004). After adjustment for type of repair, the risk of long-term anti-hypertensive therapy was 4.5 (95% confidence interval 1.2-16.9, p=0.025) and 10.5 times (95% confidence interval 2.6-42.3, p=0.001) higher if primary repair was carried out in infancy and childhood, respectively, compared with neonates. Among all, 13 patients developed re-coarctation: 21.7% in the neonatal group, 5.9% in the infant group, and 20% in the child group. We could not demonstrate a significant difference between these proportions or calculate a reliable risk for developing re-coarctation.

Conclusions: Risk of medical treatment for hypertension was lowest when primary repair was carried out during the neonatal period, rising 10-fold if first operated on as a child. Knowing the likelihood of hypertension development depending on age of primary repair is useful for long-term surveillance and counselling.
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http://dx.doi.org/10.1017/S1047951117001019DOI Listing
November 2017

Raised preoperative international normalised ratio (INR) identifies patients at high risk of perioperative death after simultaneous renal and cardiac surgery for tumours involving the peri-diaphragmatic inferior vena cava and right atrium.

BJU Int 2017 03 29;119(3):424-429. Epub 2016 Aug 29.

Department of Cardiothoracic Surgery, Guys and St Thomas' NHS Foundation Trust, London, UK.

Objective: To identify preoperative factors that predict 30-day mortality in patients undergoing simultaneous cardiac and renal surgery for urological tumours involving the peri-diaphragmatic vena cava and right atrium- The ability to predict mortality and therefore avoid surgery in those patients likely to die would be valuable.

Patients And Methods: We retrospectively reviewed perioperative outcomes in patients managed between December 2007 and January 2016 by a single team. The relationships of outcome measurements were analysed using Fisher's exact and Mann-Whitney U-tests.

Results: Of the 46 patients identified, 41 (89%) underwent surgery (20 males and 21 females). The median (range) age was 65 (17-95) years. Histology confirmed 37 renal cell cancers, one adrenal cancer, two primitive neuroectodermal tumours, and one leiomyosarcoma. The overall 30-day mortality rate was 7% (three of 41 patients). The international normalised ratio (INR), age, and estimated glomerular filtration rate (eGFR) correlated significantly with 30-day mortality. The mortality rate was high in patients with an INR ≥1.5 and <1.5 (with three of the five patients dying) compared to those with an INR <1.5 (0/36 patients died; 30 day mortality 0%). The INR correlated with serious complications (≥Clavien-Dindo Grade III), which occurred in all five patients with an INR ≥1.5 and <1.5 vs 12/36 (33%) with an INR <1.5 (P < 0.002). The median (range) eGFR in those that died was 36 (26-37) mL/min/1.73 m compared to 52 (24-154) mL/min/1.73 m in those that survived (P = 0.018).

Conclusions: In patients undergoing combined cardiac and renal tumour surgery raised preoperative INR is associated with a high risk of 30-day mortality when the patient is elderly (>70 years) and of significant post-operative complications in younger patients (<70 years). Surgery in patients with a normal INR is challenging but much safer.
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http://dx.doi.org/10.1111/bju.13587DOI Listing
March 2017

Personalized external aortic root support: a review of the current status.

Eur J Cardiothorac Surg 2016 Sep 31;50(3):400-4. Epub 2016 Mar 31.

NIHR Biomedical Research Unit, Royal Brompton Hospital, London, UK.

Personalized external aortic root support (PEARS) is an emerging technology. It is a pre-emptive operation to halt aortic root expansion and maintain aortic valve function in Marfan syndrome and is also applicable to aortic root aneurysms of other aetiologies. To fully evaluate PEARS, awareness of all those who advise these patients is necessary to ensure that patients are fully informed of the alternative operations, to carefully build experience, to ensure safety and quality and to monitor outcomes. Herein, we present a summary of published methods and outcomes and the arrangements in place for fuller evaluation.
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http://dx.doi.org/10.1093/ejcts/ezw078DOI Listing
September 2016

Hybrid Approach for Recanalization and Stenting of Acquired Pulmonary Vein Occlusion.

Pediatr Cardiol 2016 Jun 26;37(5):983-5. Epub 2016 Mar 26.

Department of Paediatric Cardiology, Evelina London Children's Hospital, St Thomas' Hospital, Lambeth Palace Road, London, SE1 7EH, UK.

A 5-year-old child with a Fontan circulation presented with acquired left pulmonary vein occlusion related to a previous surgical repair. We managed this lesion using a hybrid technique to perforate, dilate and stent the obstructed vessel. This approach should be considered when percutaneous access to the pulmonary veins is challenging (such as in a Fontan circulation). It also avoids the need for a high-risk redo sternotomy and cardiopulmonary bypass procedure.
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http://dx.doi.org/10.1007/s00246-016-1376-9DOI Listing
June 2016

Use of an Edwards Sapien S3 valve to replace a dysfunctional mechanical mitral valve in an 11-year old boy: another small step for surgical and interventional collaboration.

Eur J Cardiothorac Surg 2016 Sep 18;50(3):577-9. Epub 2016 Mar 18.

Department of Congenital Cardiology, Guys and St Thomas' NHS Foundation Trust, London, UK

An 11-year old boy, with complex left ventricular morphology in the setting of repaired double outlet right ventricle developed progressive mitral regurgitation leading to a repair which failed, necessitating replacement of the valve with a 21 mm St. Jude mechanical prosthesis. He represented 3 weeks later in extremis with signs of severe mitral stenosis. The valve was replaced via a hybrid technique with a 26 mm Edwards Sapien 3 valve mounted on a MEMO 3D annuloplasty ring. One year later, the valve is functioning well with no regurgitation or evidence of an inflow gradient.
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http://dx.doi.org/10.1093/ejcts/ezw076DOI Listing
September 2016

Treatment of severe tracheobronchomalacia: Ten-year experience.

Int J Pediatr Otorhinolaryngol 2016 Apr 27;83:57-62. Epub 2016 Jan 27.

Department of Paediatric Otolaryngology, Evelina London Children's Hospital, Guy's and ST Thomas' NHS Foundation Trust, Westminster Bridge Road, London SE1 7EH, United Kingdom.

Introduction: Paediatric tracheobronchomalacia is a rare but potentially serious condition. Severe tracheobronchomalacia requires intervention or operation. This is an evaluation of a ten-year experience at an institution.

Methods: In this retrospective study all patients were included that required an intervention for severe tracheobronchomalacia from 2003 to 2012. Symptoms, aetiology, comorbidities, localisation of the malacia, age at diagnosis, therapeutic measures and associated complications were evaluated.

Results: Forty-four patients with severe tracheobronchomalacia underwent intervention/operation. The predominant aetiology was vascular compression in 48%. The majority of patients had complex comorbidities, most importantly cardiac pathology in 66%. The median age at diagnosis was 3 months. A total of 17 aortopexies, 21 tracheostomies and 25 stent placements were performed. The mean follow-up was 2.6 years. Severe complications occurred in 12 patients. The most common complications were stent obstruction/fracture and tracheostomy tube obstruction.

Conclusion: The management of severe tracheobronchomalacia is complex and the population of patients is very heterogeneous. Therefore the treatment has to be adapted for each patient individually. The decision strategies are discussed in this article. The surgical techniques for placement and safe removal of expandable bare metallic stents employed in our institution are presented. A multidisciplinary team of ENT surgeons, Intensivists, Cardiologists and Cardiac surgeons is of great importance.
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http://dx.doi.org/10.1016/j.ijporl.2016.01.022DOI Listing
April 2016

Personalized Aortic Root Support With Mesh Provides Optimal Valve Conservation.

Ann Thorac Surg 2015 Oct;100(4):1509-10

Clinical Operational Research Unit, UCL 4 Taviton St, London WC1H 0BT, United Kingdom. Electronic address:

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http://dx.doi.org/10.1016/j.athoracsur.2015.03.027DOI Listing
October 2015

Hybrid Procedure for Neonates With Hypoplastic Left Heart Syndrome at High-Risk for Norwood: Midterm Outcomes.

Ann Thorac Surg 2015 Dec 1;100(6):2286-90; discussion 2291-2. Epub 2015 Oct 1.

Department of Cardiology and Cardiac Surgery, Evelina London Children's Hospital, Guy's & St. Thomas' NHS Foundation Trust, London, United Kingdom.

Background: Hybrid procedure offers patients with severe congenital heart disease an alternative initial procedure to conventional surgical reconstruction. We report the midterm outcomes of a cohort of neonates who had a hybrid procedure for variants of hypoplastic left heart syndrome because they were at high risk for the Norwood procedure.

Methods: Between December 2005 and January 2013, 41 neonates underwent bilateral pulmonary artery banding followed by ductal stenting by means of a sternotomy at a median age of 6 days (range, 2 to 18 days) and weight of 2.6 kg (range, 1.5 to 3.7 kg). Thirty-five patients had hypoplastic left heart syndrome, and 6 patients had critical aortic stenosis with hypoplastic left ventricle. Primary indications for the hybrid procedure were low birth weight in 17 patients, hypoplastic left ventricle with the possibility of later biventricular repair in 6 patients, intact or near-intact atrial septum in 5 patients, and poor patient condition in 13 patients. Echocardiographic, angiographic, operative, and clinical data were reviewed. Outcomes were summarized with descriptive statistics and risk factors for mortality identified.

Results: All but 6 patients had an antenatal diagnosis, and 24 patients were from other congenital cardiac centers. Nine patients had perioperative balloon aortic valvuloplasty, 1 patient had fetal balloon aortic valvuloplasty, and 17 patients had intervention to their atrial septum (41.4%). There were 9 inpatient deaths (21.9%) and 4 interstage deaths (9.8%) after the hybrid procedure. Twenty-eight patients subsequently underwent either the Norwood procedure (11 patients), combined stage I and II (14 patients), or biventricular repair (3 patients). No patient had heart transplantation. Among the patients who had combined stage I and II as a second procedure after the hybrid procedure, there were 2 early deaths, 1 late death before the Fontan, and 1 late death after the Fontan completion after combined stage I and II. All patients who had subsequent Norwood procedure were midterm survivors. Three of the 4 patients who had biventricular repair were midterm survivors. Overall survival was 56.1% at a median follow-up of 32.0 months. By univariate analysis, patient factors, intact or near-intact atrial septum, and aortic atresia were associated with nonsurvival.

Conclusions: Hybrid procedure as an alternative to the Norwood procedure offers good midterm survival in patients deemed at high risk for neonatal reconstruction.
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http://dx.doi.org/10.1016/j.athoracsur.2015.06.098DOI Listing
December 2015

Second stage after initial hybrid palliation for hypoplastic left heart syndrome: Arterial or venous shunt?

J Thorac Cardiovasc Surg 2015 Aug 7;150(2):350-7. Epub 2015 May 7.

Department of Cardiovascular Imaging, Division of Imaging Sciences and Biomedical Engineering, King's College, London, United Kingdom; Evelina London Children Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom.

Objective: Hybrid palliation for hypoplastic left heart syndrome has been developed as an alternative to neonatal Norwood surgery. At the second stage, a source of pulmonary blood flow has to be established. Options include an arterial modified Blalock-Taussig or a venous superior cavopulmonary shunt.

Methods: We retrospectively reviewed patients who received second-stage palliation after the initial hybrid. Patients were stratified according to the source of pulmonary blood supply into the arterial shunt (n = 17 patients) or venous shunt (n = 26 patients).

Results: Age and weight at second stage were lower in the arterial group (85 [45-268] days vs 152.5 [61-496] days, P = .001 and 3.6 [2.7-9.4] kg vs 5.1 [2.97-9.4] kg, P = .001, respectively). All recorded surgical times were shorter in the arterial group. Mechanical ventilation and intensive care stay were shorter in the venous group (5.82 [2.01-14.9] days vs 2.42 [0.56-13.67] days, P = .005 and 8.5 [3.6-23.7] vs 5.75 [0.8-17.6] days, P = .036, respectively) There was no difference in mortality (2/17 vs 5/26; P = .685) or incidence of complications between the 2 groups. There was a tendency toward a higher need for intervention in the immediate postoperative period in the venous group, but this did not reach significance (6/17 vs 13/26, P = .342). The arterial group has shown better development of the branch pulmonary arteries with a higher lower lobe index (158.38 ± 39.43 mm(2)/m(2) vs 113.33 ± 43.96 mm(2)/m(2), respectively, P = .037).

Conclusions: Both arterial and venous shunts are viable options with mortality and morbidity results comparable to those in the literature. The arterial shunt pathway (2-stage Norwood I) may offer better pulmonary arterial growth than the venous shunt (comprehensive/combined Norwood I and II).
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http://dx.doi.org/10.1016/j.jtcvs.2015.04.039DOI Listing
August 2015

De novo obstruction after supravalvular aortic stenosis repair in Williams-Beuren syndrome.

Ann Thorac Surg 2015 May;99(5):1806-7

Department of Cardiology and Cardiac Surgery, Evelina Children's Hospital, London, United Kingdom. Electronic address:

Williams-Beuren syndrome is characterized by diffuse arteriopathy due to elastin gene deficiency. We present a patient with de novo supravalvular stenosis due to excessive intimal hyperplasia after a previous repair. This case report supports in vitro and animal studies that have linked elastin deficiency to increased cellular proliferation in the vessel wall with the subsequent development of obstructive lesions.
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http://dx.doi.org/10.1016/j.athoracsur.2014.06.108DOI Listing
May 2015

Technical and anatomical factors affecting the size of the branch pulmonary arteries following first-stage Norwood palliation for hypoplastic left heart syndrome.

Interact Cardiovasc Thorac Surg 2015 May 13;20(5):631-5. Epub 2015 Feb 13.

Department of Cardiology and Cardiac Surgery, Evelina London Children Hospital, Guy's & St Thomas' NHS Foundation Trust, London, UK Division of Imaging Sciences and Biomedical Engineering, King's College London, London, UK.

Objectives: Branch pulmonary artery (BPA) size is one of the factors that influence the efficacy of the Fontan circulation. Central pulmonary artery stenosis and small left pulmonary artery (LPA) are well-known problems following Norwood palliation for hypoplastic left heart syndrome (HLHS). We investigated anatomical and technical factors that may stand behind these problems.

Methods: A total of 47 consecutive patients were included in the study. All had complete magnetic resonance imaging (MRI) study pre-second-stage palliation. Measurements were taken using a first-pass 3D angiography technique after intravenous injection of an extravascular contrast agent. Factors investigated included the following: size and site of the pulmonary artery bifurcation stump in relation to the Damus-Kaye-Stansel (DKS) anastomosis, interaortic distance/ratio (neoaorta to descending aorta distance/antero-posterior dimension of the chest) (IAD/IAR), distance from the under surface of the arch and the size of native aorta and pulmonary artery. IAD/IAR were compared between two different arch reconstruction techniques.

Results: Stenosis occurred either centrally, at the origin of the BPA, or more distally, in the mid-LPA (posterior to DKS). There was a significant lower incidence of central BPA stenosis when the pulmonary artery stump was placed in the mid-position compared with right/left position (26 vs 67%; P = 0.011). A more bulky pulmonary artery stump was also found in those patients with central BPA stenosis (186 vs 137 mm(2)/m(2); P = 0.047). The mid-LPA consistently showed antero-posterior compression (mean cranio-caudal diameter 3.82 mm vs mean antero-posterior diameter 3.07 mm, P < 0.001). Indexed mid-LPA area was only correlated with IAD/IAR (r = 0.49 and 0.51, P < 0.001). No correlation was shown with the distance to the under surface of the arch (r = 0.14, P = 0.37), again confirming antero-posterior compression of the LPA rather than cranio-caudal. In multivariable analysis, the only predictor of indexed mid-LPA area was the IAR (P < 0.001). There was no significant difference in the IAD or IAR between the two arch reconstruction techniques [mean IAD 15.5 vs 13.5 mm (P = 0.14)]; [mean IAR 0.17 vs 0.19 (P = 0.21)].

Conclusions: Of all studied factors, IAR and the size and position of the pulmonary artery bifurcation plays the main role in LPA growth and central BPA stenosis.
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http://dx.doi.org/10.1093/icvts/ivv002DOI Listing
May 2015

Self-expanding stent implantation in arterial duct during hybrid palliation of hypoplastic left heart syndrome: midterm experience with a specially designed stent.

EuroIntervention 2015 Mar;10(11):1318-25

Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom.

Aims: The lack of a specifically designed stent for the arterial duct has led to the off-label use of stents during hybrid palliation of hypoplastic left heart syndrome (HLHS). We evaluated the implantation and performance of a specially designed self-expanding stent in hybrid palliation of HLHS.

Methods And Results: We implanted 39 sinus-SuperFlex (SSF) stents in 24 patients at a median age of seven days (range 2-27 days) and median weight of 2.85 kg (range 1.3-3.8 kg). A single stent was implanted in nine patients and two overlapping stents in 15 patients. There was one intraoperative death, not related to ductal stenting and one additional in-hospital death. During median follow-up of 137 days (range 38-522 days), nine patients required 11 interstage interventions. Four patients have undergone a biventricular repair, 11 have undergone the next stage of univentricular palliation and seven are awaiting a second-stage palliation.

Conclusions: The SSF stent provides effective maintenance of ductal patency in patients undergoing hybrid palliation of HLHS and its variants. It conforms to the ductal anatomy but the lack of stents longer than 20 mm has required overlapping stents in longer ducts. This has not been of haemodynamic consequence.
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http://dx.doi.org/10.4244/EIJY15M01_08DOI Listing
March 2015

Juxtaposed right atrial appendage in complex Transposition of the Great Arteries.

Rev Port Cardiol 2015 Jan 10;34(1):79-80. Epub 2015 Jan 10.

Cardiovascular MRI Department, Kings College, London, UK; Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's & St. Thomas NHS Foundation Trust, London, UK.

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http://dx.doi.org/10.1016/j.repc.2014.08.015DOI Listing
January 2015

Stenting the atrial septum: a hybrid approach guided solely by echocardiography.

J Card Surg 2014 Jul 18;29(4):561-3. Epub 2014 Feb 18.

Department of Congenital Heart Disease, Evelina London Children's Hospital, London, United Kingdom.

Stenting the atrial septum to achieve decompression of the atrium is well described and commonly performed via a percutaneous catheter approach under combined fluoroscopic and echocardiographic guidance. We describe two successful cases of a hybrid approach to atrial stenting under echocardiographic guidance alone, as a viable alternative approach.
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http://dx.doi.org/10.1111/jocs.12310DOI Listing
July 2014

Analysis of preoperative condition and interstage mortality in Norwood and hybrid procedures for hypoplastic left heart syndrome using the Aristotle scoring system.

Heart 2014 May 10;100(10):775-80. Epub 2014 Jan 10.

Evelina London Children's Hospital, Guy's & St Thomas NHS Foundation Trust, , London, UK.

Objective: The 'hybrid procedure', consisting of surgical banding of the pulmonary arteries with intraoperative stenting of the arterial duct, was developed as primary palliation in hypoplastic left heart syndrome (HLHS), avoiding the risks of cardiopulmonary bypass. In many centres, it is reserved for low birth weight, premature or unstable neonates; however, its role in such high risk cases of HLHS has yet to be defined.

Methods: The preoperative condition of all patients with HLHS who underwent either the hybrid or the Norwood procedure for HLHS between 2005-2011 was analysed retrospectively, using a modified comprehensive Aristotle score. We then compared operative, interstage and 1 year mortalities between the groups after Aristotle adjustment via Cox proportional hazards analyses.

Results: Of 138 patients with HLHS, 27 had hybrid and 111 Norwood procedures. The hybrid group had significantly higher Aristotle scores (mean 4.1 vs 1.8; p<0.001); however, there was no significant difference in mortality at any stage. At 1 year, the overall unadjusted survival among Norwood and hybrid patients was 58.6% and 51.9%, respectively, yielding an Aristotle adjusted hazard ratio for mortality among hybrid patients of 1.09 (95% CI 0.56 to 2.11, p=0.80).

Conclusions: Applying a hybrid approach to high risk patients with HLHS produces a comparable early and interstage mortality risk to lower risk patients undergoing the Norwood procedure. Prospective studies are needed to establish whether the hybrid procedure is a viable alternative to the Norwood procedure in all HLHS patients in terms of both mortality and long term morbidity.
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http://dx.doi.org/10.1136/heartjnl-2013-304759DOI Listing
May 2014

Late bidirectional glenn anastomosis in a 54-year-old patient with unoperated functionally univentricular heart.

World J Pediatr Congenit Heart Surg 2013 Oct;4(4):444-6

Northwest Adult Congenital Heart Disease Unit, Manchester Heart Centre, Central Manchester Foundation Trust, Oxford Road, Manchester, United Kingdom.

We report the case of a 54-year-old patient with complex univentricular physiology who presented with worsening exercise intolerance, chronic cyanosis, and uncontrolled heart failure. Investigations included echocardiography, cardiac magnetic resonance imaging, and cardiac catheterization. After discussion in a multidisciplinary meeting, the patient underwent successful surgery that included a bidirectional Glenn anastomosis and repair of the atrioventricular (AV) junction. She recovered well and on follow-up had substantially less cyanosis and heart failure. We demonstrated that common AV valve repair and Glenn shunt can be used together successfully in appropriately selected older patients as palliation for complex congenital heart disease with functionally univentricular physiology.
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http://dx.doi.org/10.1177/2150135113485759DOI Listing
October 2013

Serial magnetic resonance imaging in hypoplastic left heart syndrome gives valuable insight into ventricular and vascular adaptation.

J Am Coll Cardiol 2013 Feb 26;61(5):561-70. Epub 2012 Dec 26.

Division of Imaging Sciences and Biomedical Engineering, Rayne Institute, King's College London, St. Thomas' Hospital, London, United Kingdom.

Objectives: This study sought to investigate changes in magnetic resonance imaging (MRI) ventricular volumes and vascular dimensions before hemi-Fontan (HF) and before total cavopulmonary connection (TCPC) in children with hypoplastic left heart syndrome (HLHS).

Background: The systemic right ventricle (RV) in HLHS is subject to significant changes in volume loading throughout the surgical stages of palliation, particularly after the HF.

Methods: Fifty-eight patients had paired pre-HF and pre-TCPC MRI for assessment of changes of RV volumes, neoaortic flow, and vascular dimensions.

Results: Comparison of pre-HF and pre-TCPC MRI results showed a decrease of indexed RV end-diastolic volume and end-systolic volume (98 ml/m(2) to 87 ml/m(2) and 50 ml/m(2) to 36 ml/m(2), respectively) with stroke volume remaining constant (49 ml/m(2) vs. 51 ml/m(2)), leading to an increased RV ejection fraction (51% vs. 59%). These findings persisted after excluding the 3 patients who underwent tricuspid valve repair as part of their HF procedure. Indexed RV end-diastolic volume plotted against neoaortic stroke volume demonstrated a Frank-Starling-like curve that shifted upward after HF. The indexed distal left and right cross-sectional pulmonary artery areas were reduced after HF.

Conclusions: In HLHS, serial MRI shows the adaptation of the systemic RV after HF with volume reduction in the context of a preserved stroke volume and an increased ejection fraction. The staged palliation in HLHS may be a risk factor particularly for reduced left pulmonary artery growth in itself as no factors investigated in this study were found to significantly impact on this.
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http://dx.doi.org/10.1016/j.jacc.2012.11.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3573231PMC
February 2013

Three-dimensional echocardiography in the management of parachute mitral valve.

Eur Heart J Cardiovasc Imaging 2012 May 29;13(5):446. Epub 2011 Dec 29.

Department of Congenital Heart Disease, Evelina Children' s Hospital, Guy' s and St Thomas Foundation Trust, London, UK.

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http://dx.doi.org/10.1093/ejechocard/jer299DOI Listing
May 2012

Outcomes following the Kawashima procedure for single-ventricle palliation in left atrial isomerism.

Eur J Cardiothorac Surg 2012 Mar 18;41(3):574-9. Epub 2011 Oct 18.

Department of Congenital Heart Disease, Evelina Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.

Objectives: Patients with left atrial isomerism and interrupted inferior vena cava palliated with a superior cavopulmonary connection or Kawashima procedure (KP) have a high incidence of developing pulmonary arteriovenous malformations. The necessity for hepatic vein redirection (HVR) and its timing remains a controversy. We aimed to assess the clinical outcome of patients with left atrial isomerism following a KP. The main end points were death, requirement for HVR and the impact of HVR on oxygen saturation.

Methods: Retrospective review of 21 patients with a diagnosis of left atrial isomerism, interruption of the inferior vena cava and single-ventricle physiology managed with a KP at a single centre between January 1990 and March 2010.

Results: Twenty-one patients had a KP, with 12 subsequently undergoing HVR. There was relatively a constant monthly decrement in the proportion of patients who were free from death or HVR up until 60 months following the KP, with a dramatic increase in the hazard after this time. The Cox proportional hazards regression model demonstrated a reduced early risk for HVR or death in patients who underwent pulmonary artery banding versus arterial shunt as the primary procedure (hazard ratio: 0.10; P = 0.01), and an increased risk with bilateral superior vena cavas (SVCs) (hazard ratio: 3.4; P = 0.04) and age at KP (hazard ratio: 1.02 per month increase in age at KP; P = 0.02). HVR mortality was relatively high with 3 of 12 patients dying in the early postoperative period with profound cyanosis. The timing of HVR after the KP did not influence the postoperative rate of increase in oxygen saturation.

Conclusion: These findings confirm that the majority of patients who undergo a KP will require HVR. Patients who are older at the time of the KP or having an initial arterial shunt or bilateral SVCs are at higher risk of HVR or death. The relatively high mortality at HVR was characterized by severe postoperative cyanosis.
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http://dx.doi.org/10.1093/ejcts/ezr003DOI Listing
March 2012

Growth of left heart structures following the hybrid procedure for borderline hypoplastic left heart.

Eur J Echocardiogr 2010 Dec 4;11(10):870-4. Epub 2010 Aug 4.

Department of Congenital Heart Disease, Evelina Children's Hospital, Guy's and St Thomas Hospital Foundation Trust, 6th Floor, Westminster Bridge Road, London SE1 7EH, UK.

Aims: The 'hybrid' procedure is an alternative to the Norwood operation in classical hypoplastic left heart syndrome to support the systemic circulation until subsequent palliation. This approach has been extended to infants with the borderline development of left heart structures. We investigated whether or not a hybrid procedure for 'borderline' cases of underdevelopment of the left heart would lead to any improvement in the growth of those structures relative to body size or would impact on eventual repair.

Methods And Results: Serial echocardiograms were reviewed in cases in whom left heart development was judged borderline for adequacy to support the systemic circulation. z-scores of left heart structures and aortic discriminant scores were plotted sequentially following the hybrid procedure and random-coefficient linear-mixed models were applied to quantify growth rates. Seven infants met the inclusion criteria. At birth, the median (range) of aortic discriminant scores was -2.67 (-3.26 to -1.22), suggesting that a biventricular repair would not be feasible. Following a hybrid procedure, aortic discriminant scores increased with time and three infants were managed with a biventricular repair. The rate of change was significantly higher in infants who achieved a biventricular repair compared with those who did not (P = 0.01).

Conclusion: The hybrid procedure allows time for growth of left heart structures in selected infants, and serial echocardiography may assist in identifying those children who may ultimately achieve a biventricular circulation.
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http://dx.doi.org/10.1093/ejechocard/jeq085DOI Listing
December 2010