Andrew J P Tometzki | The University of Sheffield (original) (raw)

Papers by Andrew J P Tometzki

Archives of Disease in Childhood-fetal and Neonatal Edition, Sep 1, 1995

Aim-To report the collaborative experience of extracorporeal membrane oxygenation (ECMO) in the t... more Aim-To report the collaborative experience of extracorporeal membrane oxygenation (ECMO) in the treatment of respiratory syncytial virus (RSV) bronchiolitis between April 1989 and January 1995. Methods-The medical records of patients with confirmed RSV bronchiolitis referred to three centres (Leicester, Glasgow, and Great Ormond Street) were reviewed. Results-Twenty four infants were identified. Seventeen had been born prematurely (gestational range 23-40 weeks, median 30 weeks). Thirteen infants had been mechanically ventilated after birth and seven of these had evidence of bronchopulmonary dysplasia (BPD). The age of onset of RSV infection varied from three to 64 weeks (mean 17*4 weeks, median 12 weeks). Ventilation before ECMO ranged from one to 16 days and oxygenation indices at the time of referral ranged from 21-73 (mean 39). Ribavirin was used in eight of the 24 patients. Sixteen patients received venoarterial and eight veno-venous ECMO. ECMO hours ranged from 32-647 (median 196 hours). One infant died (survival rate 96%). Cranial ultrasound abnormalities were detected in three patients. However, at follow up only one of the 23 survivors had evidence of developmental delay. Conclusion-A group of paediatric patients in whom ECMO can be of benefit has been identified. The use of ECMO should be considered when other means of support prove unsuccessfutl.

The Lancet Child & Adolescent Health, Feb 1, 2021

Elsevier has created a COVID-19 resource centre with free information in English and Mandarin on ... more Elsevier has created a COVID-19 resource centre with free information in English and Mandarin on the novel coronavirus COVID-19. The COVID-19 resource centre is hosted on Elsevier Connect, the company's public news and information website. Elsevier hereby grants permission to make all its COVID-19-related research that is available on the COVID-19 resource centre-including this research content-immediately available in PubMed Central and other publicly funded repositories, such as the WHO COVID database with rights for unrestricted research re-use and analyses in any form or by any means with acknowledgement of the original source. These permissions are granted for free by Elsevier for as long as the COVID-19 resource centre remains active.

Open heart, Jul 1, 2023

Background COVID-19 has caused significant worldwide morbidity and mortality. Congenital heart di... more Background COVID-19 has caused significant worldwide morbidity and mortality. Congenital heart disease (CHD) is likely to increase vulnerability and understanding the predictors of adverse outcomes is key to optimising care. Objective Ascertain the impact of COVID-19 on people with CHD and define risk factors for adverse outcomes. Methods Multicentre UK study undertaken 1 March 2020-30 June 2021 during the COVID-19 pandemic. Data were collected on CHD diagnoses, clinical presentation and outcomes. Multivariable logistic regression with multiple imputation was performed to explore predictors of death and hospitalisation. Results There were 405 reported cases (127 paediatric/278 adult). In children (age <16 years), there were 5 (3.9%) deaths. Adjusted ORs (AORs) for hospitalisation in children were significantly lower with each ascending year of age (OR 0.85, 95% CI 0.75 to 0.96 (p<0.01)). In adults, there were 24 (8.6%) deaths (19 with comorbidities) and 74 (26.6%) hospital admissions. AORs for death in adults were significantly increased with each year of age (OR 1.05, 95% CI 1.01 to 1.10 (p<0.01)) and with pulmonary arterial hypertension (PAH; OR 5.99, 95% CI 1.34 to 26.91 (p=0.02)). AORs for hospitalisation in adults were significantly higher with each additional year of age (OR 1.03, 95% CI 1.00 to 1.05 (p=0.04)), additional comorbidities (OR 3.23, 95% CI 1.31 to 7.97 (p=0.01)) and genetic disease (OR 2.87, 95% CI 1.04 to 7.94 (p=0.04)). Conclusions Children were at low risk of death and hospitalisation secondary to COVID-19 even with severe CHD, but hospital admission rates were higher in younger children, independent of comorbidity. In adults, higher likelihood of death was associated with increasing age and PAH, and of hospitalisation with age, comorbidities and genetic disease. An individualised approach, based on age and comorbidities, should be taken to COVID-19 management in patients with CHD. HOW THIS STUDY MIGHT AFFECT RESEARCH, PRACTICE OR POLICY ⇒ Identification of medical and non-medical factors associated with increased rates of death in children and adults with congenital heart disease could help inform future decision-making regarding shielding when rates of COVID-19 infection are high or there are new variants.

Catheterization and Cardiovascular Interventions, Oct 1, 2009

Objectives: To describe the early single-center clinical experience with the Amplatzer Ductal Occ... more Objectives: To describe the early single-center clinical experience with the Amplatzer Ductal Occluder II (ADO II). Methods: All patients undergoing attempted transcatheter closure of persistent arterial duct (PDA) with the ADO II were included. Data collected included demographic, clinical, and echocardiographic parameters. Results: From March until September 2008, 29 procedures were undertaken in 27 patients (21 female). Median age was 1.4 years (range 0.4-76 years) with median weight 9.4 kg (range 4.7-108 kg). A transarterial approach was used in 2 patients. The median minimum ductal diameter was 2.7 mm (range 1.7-5). ADO II was released in 25 patients (92.5%). Two patients had significant residual shunting following deployment of ADO II and underwent closure with Amplatzer ductal occluder (ADO I). Postprocedural echocardiography identified one occluder had changed position with development of a significant leak and one occluder had embolized to the left pulmonary artery. Both occluders were retrieved successfully at a second catheter procedure. Complete occlusion was noted predischarge in 22 of the remaining 23 occluders (96%). One patient had mild flow acceleration in the left pulmonary artery which has resolved. Conclusions: The ADO II is highly effective at providing rapid occlusion of morphologically varied PDAs. Occluder design allows closure with arterial or venous approach and delivery with 4 or 5 F delivery catheters. Stable occluder position is dependent on correct positioning of both aortic and pulmonary discs. A larger range of sizes and configurations of this occluder may be required to successfully occlude all ductal sizes and morphologies.

Archives of Disease in Childhood-fetal and Neonatal Edition, Mar 1, 1996

A neonate with myocardial infarction who failed to respond to conventional treatment was supporte... more A neonate with myocardial infarction who failed to respond to conventional treatment was supported by extracorporeal membrane oxygenation (ECMO). Severe mitral valve regurgitation necessitated mitral valve replacement while receiving ECMO following which the infant was successfully weaned. ECMO should be considered for potentially treatable causes of catastrophic heart failure.

Catheterization and Cardiovascular Interventions, 2008

Objectives: We describe our complete experience with covered stent implantation for aortic coarct... more Objectives: We describe our complete experience with covered stent implantation for aortic coarctation including short-to medium-term outcomes. Background: Coarctation of the aorta is a heterogeneous disease process with multiple associated complications both with and without treatment. Covered stents have evolved to provide greater support to the aortic wall and a varied approach with choice of stent tailored to the anatomy of the patient is required. Methods: We retrospectively analyzed consecutive patient data from two congenital heart centers from March 2003 to October 2007. Results: We implanted 38 covered stents in 37 patients (20 female) of which three were self-expanding stents. Median age was 29.6 years (9-65) with median weight of 71.5 kg (35-95). The indications for stent placement were native coarctation (n 5 13); recurrent coarctation following surgical treatment (n 5 11); aneurysm associated with previous coarctation surgery (n 5 7); aorto-bronchial fistula leading to acute hemoptysis (n 5 2); stent fracture (n 5 1); associated arterial duct (n 5 2). One patient had a combination of acquired coarctation and false aneurysm. The median screening time was 10 min (3-22). The median systolic gradient of 26 mm Hg (10-60) was reduced to 4 mm Hg (0-20) postprocedure (P < 0.001). There were no deaths on median follow up of 11.5 months (1-56). One patient developed aortic rupture during the procedure and required emergency surgery. Conclusion: Covered stent implantation for treatment of aortic coarctation is safe and highly effective in selected patients. Selfexpanding stent grafts may be preferable to balloon expandable stents when there is aneurysm formation in the setting of aortopathy.

European Journal of Echocardiography, 2020

A 16 month old, 8.2 kg patient came forward for evaluation of complex cardiac anatomy with multim... more A 16 month old, 8.2 kg patient came forward for evaluation of complex cardiac anatomy with multimodality imaging assessment to ascertain suitability for biventricular repair. A large ventricular septal defect was diagnosed before birth but possible straddling of the tricuspid valve identified postnatally. The patient developed symptoms of congestive cardiac failure and was palliated with a pulmonary artery band. In view of the difficult nature of the defect they were reviewed with 3D-transthoracic echo, transoesophageal echo and cardiac MRI. Their transthoracic echo confirmed situs solitus with levocardia, atrioventricular and ventriculoarterial concordance. There was a well placed pulmonary artery band with peak velocity of 5 m/s. Biventricular systolic function appeared good. There was a large ventricular septal defect at the inlet extending to the muscular septum. There were 2 prominent muscle bundles arising from the ventricular apex and it was difficult to distinguish on echocardiography which of these formed the true ventricular septum (Figures A & B). Although the tricuspid valve opened normally, there were multiple chords extending to overlie the right ventricular aspect of the ventricular septal defect (Figure C), some of which appeared to cross the through defect (*) and attach to the more leftward of the apical trabeculations (Figure D white dotted line illustrates the true plane of ventricular septum which overlies the attachments. Red dotted line represents the plane followed by the leftward apical trabeculation). Cardiac MRI showed that the trabeculation positioned to the left was the true ventricular septum, as it seemed to be in line with the plane of the atrial septum at the crux of the heart (Figure E & F). At surgery her heart was found to be unseptatable due to multiple straddling chords from the tricuspid valve inserting into multiple papillary muscle heads with the left ventricle. Conclusions: The key issue in this case is which of the muscular structures positioned at the ventricular apex is considered to be the true ventricular septum as this determines whether on not there is straddle of the tricuspid valve. In addition the complex and multiple nature of the chordal attachments below the valve made accessing and closing the defect not feasible. Even in the present era with wide availability of advanced, multimodality imaging techniques demonstrating anatomy can still prove challenging in planning surgical repair, especially within the setting of complex congenital heart disease. Many aspects of such cases still only become apparent at the time of surgery and this remains a key issue when counselling parents.

Catheterization and Cardiovascular Interventions, Jan 31, 2014

Catheterization and Cardiovascular Interventions, Mar 1, 2009

Despite the wide variety of occlusion devices available for closing the patent ductus arteriosus;... more Despite the wide variety of occlusion devices available for closing the patent ductus arteriosus; some defects with unusual morphology still remain a challenge. The drive for increasingly user friendly and lower caliber delivery systems is also ongoing. The new amplatzer ductal occluder II may prove to be a useful additional tool in these circumstances. We describe our initial experiences with this new device in three patients with long tubular (Type C) ducts.

Heart, Sep 10, 2009

Background Stenting for aortic coarctation is known to be effective in the medium term. Aneurysm ... more Background Stenting for aortic coarctation is known to be effective in the medium term. Aneurysm formation following stent implantation is a recognised complication. However, data regarding aortic wall injury and stent integrity following stent placement are sparse. Objectives We report comprehensive clinical, echocardiographic and prospective CT follow-up data following stenting for aortic coarctation from a single centre. Methods Full data analysis of all patients undergoing balloon expandable stent implantation and follow-up procedures in a single tertiary congenital cardiac unit. Results Between October 2002 and April 2008, we performed 102 coarctation stent procedures on 88 patients. Median age was 20.6 years (range 8.5e65) and median weight 65 kg (range 34e101). 94 stents (26 covered) were implanted. 12 procedures were re-dilatations. Stenting resulted in a reduction of the gradient across the site of coarctation, from a median of 20 mm Hg to 4 mm Hg. There were no procedure-related deaths. Four patients had immediate complications (one requiring emergency surgery). During median follow-up of 34.5 months (range 4.2e72.8), two patients had late complications requiring additional stent procedures. Follow-up CT data are available in 84 patients with MRI in one patient (96.5%). Only one patient developed a procedure-related aortic aneurysm. All stent fractures (n¼7) occurred with a single stent design. Conclusions Stenting for aortic coarctation and recoarctation is effective with low immediate complication rates. CT is useful in the longer term for assessment of stent integrity and post-procedural aneurysm formation. Overall incidence of post-procedural aneurysm is rare and stent fractures were not seen with newer generation stents.

Catheterization and Cardiovascular Interventions, 2006

We describe the use of a new vascular occlusion device to embolize vessels whose flow is detrimen... more We describe the use of a new vascular occlusion device to embolize vessels whose flow is detrimental to cardiac function in a variety of clinical situations in both adults and children with congenital cardiac disease. Our series includes four cases in which we have successfully occluded large venous vessels, to produce a beneficial effect on oxygen saturations and cardiac function. There were no adverse events because of the devices in our series, which provides evidence that the Amplatzer vascular plug is a safe and effective method of embolizing large venous vessels. © 2006 Wiley‐Liss, Inc.

Catheterization and Cardiovascular Interventions, 2006

Objectives: To assess the feasibility and results of trans‐axillary approach for balloon aortic v... more Objectives: To assess the feasibility and results of trans‐axillary approach for balloon aortic valvoplasty (BAV) in early infancy. Background: Severe aortic valve stenosis (SAVS) is rare but serious condition in infancy, which may be promptly treated either by surgical aortic valvotomy or BAV. BAV is usually performed via the femoral artery route, which is associated with significant vascular complications and long procedure times. Methods: BAV via the trans‐axillary approach was performed on twenty‐seven sequential infants with SAVS presenting to a single tertiary referral center over an 11‐year period. Maximum inflated balloon size was less than or equal to the aortic valve diameter. Results: Twenty‐seven infants aged 1–77 days underwent BAV. Weight at time of procedure was 2.0–4.42 kgs. The median procedure and screening times were 82 and 7.9 minutes, respectively. Mean instantaneous Doppler gradient across the aortic valve reduced from 68 ± 33 to 37 ± 14 mmHg ( p &lt; 0.0001). Three infants developed at least moderate aortic regurgitation. Right arm pulse volume was decreased in 12 infants; 5 received an intravenous heparin infusion. Longer‐term follow‐up demonstrated reduced or absent peripheral pulse in 5 infants. Transection of the axillary artery occurred in one infant requiring emergency microvascular repair. There was one post‐procedural and one late death due to non‐cardiac causes. Conclusions: In early infancy balloon aortic valvoplasty via the axillary artery approach for severe aortic stenosis is an acceptable and safe alternative to the femoral arterial approach and results in short procedure and screening times. Longer‐term vascular follow‐up is required. © 2006 Wiley‐Liss, Inc.

Archives of Disease in Childhood, Apr 22, 2012

Objective To assess the safety and efficacy of warfarin loading, in our paediatric congenital hea... more Objective To assess the safety and efficacy of warfarin loading, in our paediatric congenital heart disease patients, using the current protocols. By retrospective review of the data collected, implement change in line with the National Patient Safety Agency (NPSA) Alert 18 safety indicators,1 by formulating a departmental and regional guideline. Method A data collection sheet was designed and reviewed by the paediatric cardiac pharmacist, a cardiac nurse specialist and a paediatric cardiology registrar. The data collection was conducted by a medical student during the period of 28th June 2010 and 7th July 2010. All paediatric congenital heart disease inpatients during the study period were included in this preaudit data collection exercise. A retrospective study of patient medical notes and the paediatric congenital heart database (Heartsuite) was conducted between 1st July 2009 and 31st May 2010. The data collection reviewed; documentation in the patient's medical notes regarding commencement of warfarin, the loading dose given, the loading regimen over days 2–7 and the international normalised ratio (INR) target at discharge. Results There were eight new patients commenced on warfarin during the study period; seven postsurgery and one post pulmonary embolism. Documentation in the medical notes was variable; no documented reason why warfarin started or that verbal or written information given to patient/parents, the majority of loading doses used were documented and all of the INR results post surgery was detailed. As regards loading and target ranges; less than half of patients complied with the day 2–7 loading regimen protocol during the study period but at discharge seven out of the eight patients were in target range. The post discharge INR results were 46% within range, only one patient had an INR of greater than five and none had an INR greater than 8. Conclusion This data collection has highlighted that documentation and prescribing could be improved by the production of a comprehensive guideline that highlights the NPSA recommendations in Alert 18 that can be used within our department and regionally. This data collection has also shown that less than half of patients adhered to the current loading regimen that was used during the collection period, so our loading regimen used will need to be reviewed. Despite this there were no reports of adverse events such as major bleeding and only one patient had an INR greater than 5. Once a guideline has been written and approved, all of the standards outlined in this data collection will need to be re-audited.

Catheterization and Cardiovascular Interventions, 2007

We report on two patients who developed significant aortic regurgitation during transcatheter clo... more We report on two patients who developed significant aortic regurgitation during transcatheter closure of a perimembranous ventricular septal defect, both requiring further surgical intervention. Both patients were noted prior to the procedure to have limited subaortic margins to the defect. The potential mechanisms for development of aortic regurgitation in this setting are discussed.

Clinical and Experimental Dermatology, 2001

ABSTRACT Kawasaki disease is one of the commonest vasculitides seen in children. It presents with... more ABSTRACT Kawasaki disease is one of the commonest vasculitides seen in children. It presents with prolonged fever and a polymorphic exanthem. It is a major cause of acquired heart disease in western society. Its exact cause is not known, but exposure to a superantigen has been suggested as a possible aetiological factor. Diagnosis of Kawasaki disease still relies on clinical criteria (Table 1) and investigations are done mainly to exclude other diseases and to detect early or established cardiac complications. Coronary complications can be reduced significantly by the use of intravenous immunoglobulin therapy combined with oral aspirin. The serious consequences of Kawasaki disease require a heightened awareness of this condition when dealing with childhood exanthems.

Cardiology in The Young, Oct 1, 2004

Cardiology in The Young, Oct 23, 2012

The presence of an atrial septal defect in pulmonary hypertension has benefits and detractions. E... more The presence of an atrial septal defect in pulmonary hypertension has benefits and detractions. Even in idiopathic pulmonary arterial hypertension, a significant left-to-right shunt at atrial level may increase the pulmonary arterial pressure and exacerbate the disease. However, it is well recognised that the presence of an atrial communication may be protective in subgroups with severe disease, allowing maintenance of cardiac output during times of increased pulmonary resistance. In the present paper, we present the case of a young boy with significant idiopathic pulmonary arterial hypertension and an atrial septal defect. We report our technique of septal occlusion using a device to decrease left-to-right shunting with concomitant stent insertion in that device to maintain the potential for right-to-left shunting during times of high pulmonary arterial pressure.

Pediatric Cardiology, Aug 5, 2017

To report our experience of coarctation stent therapy in small children weighing less than 30 kg,... more To report our experience of coarctation stent therapy in small children weighing less than 30 kg, with the low profile dilatable Valeo stent and review the literature on coarctation stent therapy in this patient population. Coarctation stent implantation was undertaken in 14 consecutive children using the Bard Valeo Stent. Demographic, angiographic, echocardiographic and clinical data were reviewed retrospectively. The median age at the time of procedure was 5.1 (2.6-7.5) years and median weight was 20.8 (14.7-27) kg. There was improvement in median coarctation diameter from 4 (1.3-5.2) to 9.5 (5.8-12.7) mm, p \ 0.001; and a reduction in the median peak pressure gradient across the coarctation from 35 (20-49) to 9 (0-15) mmHg, p \ 0.001. Median stent recoil was 7.9 (0-20)%. There was one case of access related complication that resolved without sequelae. Follow-up was a median of 15 (3.0-57.2) months. CT angiogram performed at a median time of 3.3 (2.6-10.2) months post procedure showed no aortic wall injury and preserved stent integrity in all cases. Two children underwent re-intervention for stent dilation and further stent implantation due to in-stent stenosis and somatic growth after 3 years. Six of fourteen children remained on a single antihypertensive agent post-intervention at last follow-up. Implantation of the dilatable Valeo stent is a feasible treatment strategy in native or recurrent coarctation in small children, accepting that additional stent implantation may be necessary with somatic growth. Further study is required to determine longer-term stent efficacy and clinical outcome.

Circulation Research, Aug 3, 2018

Objective: Though a significant number of low birth weight and premature neonates are born with a... more Objective: Though a significant number of low birth weight and premature neonates are born with aortic coarctation, waiting for the child to grow before intervening rarely permits significant useful weight gain and puts the child at risk of interim complications. Previous studies of early operation on these patients have shown a high hospital mortality up to 25% (25 out of 100) and 30% (30 out of 100) recurrence in one year. We reviewed our data to ascertain whether modern approaches allow better outcomes for these children. Methods: All patients weighing less than 2 kg with isolated coarctation between August 2008 and November 2015 were studied by retrospective chart review to ensure greater than 24 months follow-up. Eleven patients underwent extended end-to side surgical repair. In-hospital and medium term follow-up data were collected. Data are expressed as median (range). Results: Weight at time of surgery was 1.8kg (1.6-1.9). There were no deaths, in-hospital or during follow-up. One patient developed postoperative chylothorax. In-hospital stay was 18 days (4-47). At follow-up of 68 months (28-115) echocardiographic velocity across the repair was 1.6m/sec (1.4-4.5). One patient with William’s syndrome had evidence of recoarctation and required balloon coarctoplasty followed by stenting. This patient had grossly abnormal vessels at the time of initial surgery with aortic wall thickness greater than 3mm. Conclusions: Neonates below 2kg can undergo coarctation repair safely with low incidence of recurrence. Waiting for growth in this cohort of patients cannot therefore be justified.

Archives of Disease in Childhood-fetal and Neonatal Edition, Sep 1, 1995

Aim-To report the collaborative experience of extracorporeal membrane oxygenation (ECMO) in the t... more Aim-To report the collaborative experience of extracorporeal membrane oxygenation (ECMO) in the treatment of respiratory syncytial virus (RSV) bronchiolitis between April 1989 and January 1995. Methods-The medical records of patients with confirmed RSV bronchiolitis referred to three centres (Leicester, Glasgow, and Great Ormond Street) were reviewed. Results-Twenty four infants were identified. Seventeen had been born prematurely (gestational range 23-40 weeks, median 30 weeks). Thirteen infants had been mechanically ventilated after birth and seven of these had evidence of bronchopulmonary dysplasia (BPD). The age of onset of RSV infection varied from three to 64 weeks (mean 17*4 weeks, median 12 weeks). Ventilation before ECMO ranged from one to 16 days and oxygenation indices at the time of referral ranged from 21-73 (mean 39). Ribavirin was used in eight of the 24 patients. Sixteen patients received venoarterial and eight veno-venous ECMO. ECMO hours ranged from 32-647 (median 196 hours). One infant died (survival rate 96%). Cranial ultrasound abnormalities were detected in three patients. However, at follow up only one of the 23 survivors had evidence of developmental delay. Conclusion-A group of paediatric patients in whom ECMO can be of benefit has been identified. The use of ECMO should be considered when other means of support prove unsuccessfutl.

The Lancet Child & Adolescent Health, Feb 1, 2021

Elsevier has created a COVID-19 resource centre with free information in English and Mandarin on ... more Elsevier has created a COVID-19 resource centre with free information in English and Mandarin on the novel coronavirus COVID-19. The COVID-19 resource centre is hosted on Elsevier Connect, the company's public news and information website. Elsevier hereby grants permission to make all its COVID-19-related research that is available on the COVID-19 resource centre-including this research content-immediately available in PubMed Central and other publicly funded repositories, such as the WHO COVID database with rights for unrestricted research re-use and analyses in any form or by any means with acknowledgement of the original source. These permissions are granted for free by Elsevier for as long as the COVID-19 resource centre remains active.

Open heart, Jul 1, 2023

Background COVID-19 has caused significant worldwide morbidity and mortality. Congenital heart di... more Background COVID-19 has caused significant worldwide morbidity and mortality. Congenital heart disease (CHD) is likely to increase vulnerability and understanding the predictors of adverse outcomes is key to optimising care. Objective Ascertain the impact of COVID-19 on people with CHD and define risk factors for adverse outcomes. Methods Multicentre UK study undertaken 1 March 2020-30 June 2021 during the COVID-19 pandemic. Data were collected on CHD diagnoses, clinical presentation and outcomes. Multivariable logistic regression with multiple imputation was performed to explore predictors of death and hospitalisation. Results There were 405 reported cases (127 paediatric/278 adult). In children (age <16 years), there were 5 (3.9%) deaths. Adjusted ORs (AORs) for hospitalisation in children were significantly lower with each ascending year of age (OR 0.85, 95% CI 0.75 to 0.96 (p<0.01)). In adults, there were 24 (8.6%) deaths (19 with comorbidities) and 74 (26.6%) hospital admissions. AORs for death in adults were significantly increased with each year of age (OR 1.05, 95% CI 1.01 to 1.10 (p<0.01)) and with pulmonary arterial hypertension (PAH; OR 5.99, 95% CI 1.34 to 26.91 (p=0.02)). AORs for hospitalisation in adults were significantly higher with each additional year of age (OR 1.03, 95% CI 1.00 to 1.05 (p=0.04)), additional comorbidities (OR 3.23, 95% CI 1.31 to 7.97 (p=0.01)) and genetic disease (OR 2.87, 95% CI 1.04 to 7.94 (p=0.04)). Conclusions Children were at low risk of death and hospitalisation secondary to COVID-19 even with severe CHD, but hospital admission rates were higher in younger children, independent of comorbidity. In adults, higher likelihood of death was associated with increasing age and PAH, and of hospitalisation with age, comorbidities and genetic disease. An individualised approach, based on age and comorbidities, should be taken to COVID-19 management in patients with CHD. HOW THIS STUDY MIGHT AFFECT RESEARCH, PRACTICE OR POLICY ⇒ Identification of medical and non-medical factors associated with increased rates of death in children and adults with congenital heart disease could help inform future decision-making regarding shielding when rates of COVID-19 infection are high or there are new variants.

Catheterization and Cardiovascular Interventions, Oct 1, 2009

Objectives: To describe the early single-center clinical experience with the Amplatzer Ductal Occ... more Objectives: To describe the early single-center clinical experience with the Amplatzer Ductal Occluder II (ADO II). Methods: All patients undergoing attempted transcatheter closure of persistent arterial duct (PDA) with the ADO II were included. Data collected included demographic, clinical, and echocardiographic parameters. Results: From March until September 2008, 29 procedures were undertaken in 27 patients (21 female). Median age was 1.4 years (range 0.4-76 years) with median weight 9.4 kg (range 4.7-108 kg). A transarterial approach was used in 2 patients. The median minimum ductal diameter was 2.7 mm (range 1.7-5). ADO II was released in 25 patients (92.5%). Two patients had significant residual shunting following deployment of ADO II and underwent closure with Amplatzer ductal occluder (ADO I). Postprocedural echocardiography identified one occluder had changed position with development of a significant leak and one occluder had embolized to the left pulmonary artery. Both occluders were retrieved successfully at a second catheter procedure. Complete occlusion was noted predischarge in 22 of the remaining 23 occluders (96%). One patient had mild flow acceleration in the left pulmonary artery which has resolved. Conclusions: The ADO II is highly effective at providing rapid occlusion of morphologically varied PDAs. Occluder design allows closure with arterial or venous approach and delivery with 4 or 5 F delivery catheters. Stable occluder position is dependent on correct positioning of both aortic and pulmonary discs. A larger range of sizes and configurations of this occluder may be required to successfully occlude all ductal sizes and morphologies.

Archives of Disease in Childhood-fetal and Neonatal Edition, Mar 1, 1996

A neonate with myocardial infarction who failed to respond to conventional treatment was supporte... more A neonate with myocardial infarction who failed to respond to conventional treatment was supported by extracorporeal membrane oxygenation (ECMO). Severe mitral valve regurgitation necessitated mitral valve replacement while receiving ECMO following which the infant was successfully weaned. ECMO should be considered for potentially treatable causes of catastrophic heart failure.

Catheterization and Cardiovascular Interventions, 2008

Objectives: We describe our complete experience with covered stent implantation for aortic coarct... more Objectives: We describe our complete experience with covered stent implantation for aortic coarctation including short-to medium-term outcomes. Background: Coarctation of the aorta is a heterogeneous disease process with multiple associated complications both with and without treatment. Covered stents have evolved to provide greater support to the aortic wall and a varied approach with choice of stent tailored to the anatomy of the patient is required. Methods: We retrospectively analyzed consecutive patient data from two congenital heart centers from March 2003 to October 2007. Results: We implanted 38 covered stents in 37 patients (20 female) of which three were self-expanding stents. Median age was 29.6 years (9-65) with median weight of 71.5 kg (35-95). The indications for stent placement were native coarctation (n 5 13); recurrent coarctation following surgical treatment (n 5 11); aneurysm associated with previous coarctation surgery (n 5 7); aorto-bronchial fistula leading to acute hemoptysis (n 5 2); stent fracture (n 5 1); associated arterial duct (n 5 2). One patient had a combination of acquired coarctation and false aneurysm. The median screening time was 10 min (3-22). The median systolic gradient of 26 mm Hg (10-60) was reduced to 4 mm Hg (0-20) postprocedure (P < 0.001). There were no deaths on median follow up of 11.5 months (1-56). One patient developed aortic rupture during the procedure and required emergency surgery. Conclusion: Covered stent implantation for treatment of aortic coarctation is safe and highly effective in selected patients. Selfexpanding stent grafts may be preferable to balloon expandable stents when there is aneurysm formation in the setting of aortopathy.

European Journal of Echocardiography, 2020

A 16 month old, 8.2 kg patient came forward for evaluation of complex cardiac anatomy with multim... more A 16 month old, 8.2 kg patient came forward for evaluation of complex cardiac anatomy with multimodality imaging assessment to ascertain suitability for biventricular repair. A large ventricular septal defect was diagnosed before birth but possible straddling of the tricuspid valve identified postnatally. The patient developed symptoms of congestive cardiac failure and was palliated with a pulmonary artery band. In view of the difficult nature of the defect they were reviewed with 3D-transthoracic echo, transoesophageal echo and cardiac MRI. Their transthoracic echo confirmed situs solitus with levocardia, atrioventricular and ventriculoarterial concordance. There was a well placed pulmonary artery band with peak velocity of 5 m/s. Biventricular systolic function appeared good. There was a large ventricular septal defect at the inlet extending to the muscular septum. There were 2 prominent muscle bundles arising from the ventricular apex and it was difficult to distinguish on echocardiography which of these formed the true ventricular septum (Figures A & B). Although the tricuspid valve opened normally, there were multiple chords extending to overlie the right ventricular aspect of the ventricular septal defect (Figure C), some of which appeared to cross the through defect (*) and attach to the more leftward of the apical trabeculations (Figure D white dotted line illustrates the true plane of ventricular septum which overlies the attachments. Red dotted line represents the plane followed by the leftward apical trabeculation). Cardiac MRI showed that the trabeculation positioned to the left was the true ventricular septum, as it seemed to be in line with the plane of the atrial septum at the crux of the heart (Figure E & F). At surgery her heart was found to be unseptatable due to multiple straddling chords from the tricuspid valve inserting into multiple papillary muscle heads with the left ventricle. Conclusions: The key issue in this case is which of the muscular structures positioned at the ventricular apex is considered to be the true ventricular septum as this determines whether on not there is straddle of the tricuspid valve. In addition the complex and multiple nature of the chordal attachments below the valve made accessing and closing the defect not feasible. Even in the present era with wide availability of advanced, multimodality imaging techniques demonstrating anatomy can still prove challenging in planning surgical repair, especially within the setting of complex congenital heart disease. Many aspects of such cases still only become apparent at the time of surgery and this remains a key issue when counselling parents.

Catheterization and Cardiovascular Interventions, Jan 31, 2014

Catheterization and Cardiovascular Interventions, Mar 1, 2009

Despite the wide variety of occlusion devices available for closing the patent ductus arteriosus;... more Despite the wide variety of occlusion devices available for closing the patent ductus arteriosus; some defects with unusual morphology still remain a challenge. The drive for increasingly user friendly and lower caliber delivery systems is also ongoing. The new amplatzer ductal occluder II may prove to be a useful additional tool in these circumstances. We describe our initial experiences with this new device in three patients with long tubular (Type C) ducts.

Heart, Sep 10, 2009

Background Stenting for aortic coarctation is known to be effective in the medium term. Aneurysm ... more Background Stenting for aortic coarctation is known to be effective in the medium term. Aneurysm formation following stent implantation is a recognised complication. However, data regarding aortic wall injury and stent integrity following stent placement are sparse. Objectives We report comprehensive clinical, echocardiographic and prospective CT follow-up data following stenting for aortic coarctation from a single centre. Methods Full data analysis of all patients undergoing balloon expandable stent implantation and follow-up procedures in a single tertiary congenital cardiac unit. Results Between October 2002 and April 2008, we performed 102 coarctation stent procedures on 88 patients. Median age was 20.6 years (range 8.5e65) and median weight 65 kg (range 34e101). 94 stents (26 covered) were implanted. 12 procedures were re-dilatations. Stenting resulted in a reduction of the gradient across the site of coarctation, from a median of 20 mm Hg to 4 mm Hg. There were no procedure-related deaths. Four patients had immediate complications (one requiring emergency surgery). During median follow-up of 34.5 months (range 4.2e72.8), two patients had late complications requiring additional stent procedures. Follow-up CT data are available in 84 patients with MRI in one patient (96.5%). Only one patient developed a procedure-related aortic aneurysm. All stent fractures (n¼7) occurred with a single stent design. Conclusions Stenting for aortic coarctation and recoarctation is effective with low immediate complication rates. CT is useful in the longer term for assessment of stent integrity and post-procedural aneurysm formation. Overall incidence of post-procedural aneurysm is rare and stent fractures were not seen with newer generation stents.

Catheterization and Cardiovascular Interventions, 2006

We describe the use of a new vascular occlusion device to embolize vessels whose flow is detrimen... more We describe the use of a new vascular occlusion device to embolize vessels whose flow is detrimental to cardiac function in a variety of clinical situations in both adults and children with congenital cardiac disease. Our series includes four cases in which we have successfully occluded large venous vessels, to produce a beneficial effect on oxygen saturations and cardiac function. There were no adverse events because of the devices in our series, which provides evidence that the Amplatzer vascular plug is a safe and effective method of embolizing large venous vessels. © 2006 Wiley‐Liss, Inc.

Catheterization and Cardiovascular Interventions, 2006

Objectives: To assess the feasibility and results of trans‐axillary approach for balloon aortic v... more Objectives: To assess the feasibility and results of trans‐axillary approach for balloon aortic valvoplasty (BAV) in early infancy. Background: Severe aortic valve stenosis (SAVS) is rare but serious condition in infancy, which may be promptly treated either by surgical aortic valvotomy or BAV. BAV is usually performed via the femoral artery route, which is associated with significant vascular complications and long procedure times. Methods: BAV via the trans‐axillary approach was performed on twenty‐seven sequential infants with SAVS presenting to a single tertiary referral center over an 11‐year period. Maximum inflated balloon size was less than or equal to the aortic valve diameter. Results: Twenty‐seven infants aged 1–77 days underwent BAV. Weight at time of procedure was 2.0–4.42 kgs. The median procedure and screening times were 82 and 7.9 minutes, respectively. Mean instantaneous Doppler gradient across the aortic valve reduced from 68 ± 33 to 37 ± 14 mmHg ( p &lt; 0.0001). Three infants developed at least moderate aortic regurgitation. Right arm pulse volume was decreased in 12 infants; 5 received an intravenous heparin infusion. Longer‐term follow‐up demonstrated reduced or absent peripheral pulse in 5 infants. Transection of the axillary artery occurred in one infant requiring emergency microvascular repair. There was one post‐procedural and one late death due to non‐cardiac causes. Conclusions: In early infancy balloon aortic valvoplasty via the axillary artery approach for severe aortic stenosis is an acceptable and safe alternative to the femoral arterial approach and results in short procedure and screening times. Longer‐term vascular follow‐up is required. © 2006 Wiley‐Liss, Inc.

Archives of Disease in Childhood, Apr 22, 2012

Objective To assess the safety and efficacy of warfarin loading, in our paediatric congenital hea... more Objective To assess the safety and efficacy of warfarin loading, in our paediatric congenital heart disease patients, using the current protocols. By retrospective review of the data collected, implement change in line with the National Patient Safety Agency (NPSA) Alert 18 safety indicators,1 by formulating a departmental and regional guideline. Method A data collection sheet was designed and reviewed by the paediatric cardiac pharmacist, a cardiac nurse specialist and a paediatric cardiology registrar. The data collection was conducted by a medical student during the period of 28th June 2010 and 7th July 2010. All paediatric congenital heart disease inpatients during the study period were included in this preaudit data collection exercise. A retrospective study of patient medical notes and the paediatric congenital heart database (Heartsuite) was conducted between 1st July 2009 and 31st May 2010. The data collection reviewed; documentation in the patient's medical notes regarding commencement of warfarin, the loading dose given, the loading regimen over days 2–7 and the international normalised ratio (INR) target at discharge. Results There were eight new patients commenced on warfarin during the study period; seven postsurgery and one post pulmonary embolism. Documentation in the medical notes was variable; no documented reason why warfarin started or that verbal or written information given to patient/parents, the majority of loading doses used were documented and all of the INR results post surgery was detailed. As regards loading and target ranges; less than half of patients complied with the day 2–7 loading regimen protocol during the study period but at discharge seven out of the eight patients were in target range. The post discharge INR results were 46% within range, only one patient had an INR of greater than five and none had an INR greater than 8. Conclusion This data collection has highlighted that documentation and prescribing could be improved by the production of a comprehensive guideline that highlights the NPSA recommendations in Alert 18 that can be used within our department and regionally. This data collection has also shown that less than half of patients adhered to the current loading regimen that was used during the collection period, so our loading regimen used will need to be reviewed. Despite this there were no reports of adverse events such as major bleeding and only one patient had an INR greater than 5. Once a guideline has been written and approved, all of the standards outlined in this data collection will need to be re-audited.

Catheterization and Cardiovascular Interventions, 2007

We report on two patients who developed significant aortic regurgitation during transcatheter clo... more We report on two patients who developed significant aortic regurgitation during transcatheter closure of a perimembranous ventricular septal defect, both requiring further surgical intervention. Both patients were noted prior to the procedure to have limited subaortic margins to the defect. The potential mechanisms for development of aortic regurgitation in this setting are discussed.

Clinical and Experimental Dermatology, 2001

ABSTRACT Kawasaki disease is one of the commonest vasculitides seen in children. It presents with... more ABSTRACT Kawasaki disease is one of the commonest vasculitides seen in children. It presents with prolonged fever and a polymorphic exanthem. It is a major cause of acquired heart disease in western society. Its exact cause is not known, but exposure to a superantigen has been suggested as a possible aetiological factor. Diagnosis of Kawasaki disease still relies on clinical criteria (Table 1) and investigations are done mainly to exclude other diseases and to detect early or established cardiac complications. Coronary complications can be reduced significantly by the use of intravenous immunoglobulin therapy combined with oral aspirin. The serious consequences of Kawasaki disease require a heightened awareness of this condition when dealing with childhood exanthems.

Cardiology in The Young, Oct 1, 2004

Cardiology in The Young, Oct 23, 2012

The presence of an atrial septal defect in pulmonary hypertension has benefits and detractions. E... more The presence of an atrial septal defect in pulmonary hypertension has benefits and detractions. Even in idiopathic pulmonary arterial hypertension, a significant left-to-right shunt at atrial level may increase the pulmonary arterial pressure and exacerbate the disease. However, it is well recognised that the presence of an atrial communication may be protective in subgroups with severe disease, allowing maintenance of cardiac output during times of increased pulmonary resistance. In the present paper, we present the case of a young boy with significant idiopathic pulmonary arterial hypertension and an atrial septal defect. We report our technique of septal occlusion using a device to decrease left-to-right shunting with concomitant stent insertion in that device to maintain the potential for right-to-left shunting during times of high pulmonary arterial pressure.

Pediatric Cardiology, Aug 5, 2017

To report our experience of coarctation stent therapy in small children weighing less than 30 kg,... more To report our experience of coarctation stent therapy in small children weighing less than 30 kg, with the low profile dilatable Valeo stent and review the literature on coarctation stent therapy in this patient population. Coarctation stent implantation was undertaken in 14 consecutive children using the Bard Valeo Stent. Demographic, angiographic, echocardiographic and clinical data were reviewed retrospectively. The median age at the time of procedure was 5.1 (2.6-7.5) years and median weight was 20.8 (14.7-27) kg. There was improvement in median coarctation diameter from 4 (1.3-5.2) to 9.5 (5.8-12.7) mm, p \ 0.001; and a reduction in the median peak pressure gradient across the coarctation from 35 (20-49) to 9 (0-15) mmHg, p \ 0.001. Median stent recoil was 7.9 (0-20)%. There was one case of access related complication that resolved without sequelae. Follow-up was a median of 15 (3.0-57.2) months. CT angiogram performed at a median time of 3.3 (2.6-10.2) months post procedure showed no aortic wall injury and preserved stent integrity in all cases. Two children underwent re-intervention for stent dilation and further stent implantation due to in-stent stenosis and somatic growth after 3 years. Six of fourteen children remained on a single antihypertensive agent post-intervention at last follow-up. Implantation of the dilatable Valeo stent is a feasible treatment strategy in native or recurrent coarctation in small children, accepting that additional stent implantation may be necessary with somatic growth. Further study is required to determine longer-term stent efficacy and clinical outcome.

Circulation Research, Aug 3, 2018

Objective: Though a significant number of low birth weight and premature neonates are born with a... more Objective: Though a significant number of low birth weight and premature neonates are born with aortic coarctation, waiting for the child to grow before intervening rarely permits significant useful weight gain and puts the child at risk of interim complications. Previous studies of early operation on these patients have shown a high hospital mortality up to 25% (25 out of 100) and 30% (30 out of 100) recurrence in one year. We reviewed our data to ascertain whether modern approaches allow better outcomes for these children. Methods: All patients weighing less than 2 kg with isolated coarctation between August 2008 and November 2015 were studied by retrospective chart review to ensure greater than 24 months follow-up. Eleven patients underwent extended end-to side surgical repair. In-hospital and medium term follow-up data were collected. Data are expressed as median (range). Results: Weight at time of surgery was 1.8kg (1.6-1.9). There were no deaths, in-hospital or during follow-up. One patient developed postoperative chylothorax. In-hospital stay was 18 days (4-47). At follow-up of 68 months (28-115) echocardiographic velocity across the repair was 1.6m/sec (1.4-4.5). One patient with William’s syndrome had evidence of recoarctation and required balloon coarctoplasty followed by stenting. This patient had grossly abnormal vessels at the time of initial surgery with aortic wall thickness greater than 3mm. Conclusions: Neonates below 2kg can undergo coarctation repair safely with low incidence of recurrence. Waiting for growth in this cohort of patients cannot therefore be justified.