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Paediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS) is a novel condition that was first reported in April, 2020. We aimed to develop a national consensus management pathway for the UK to provide guidance for clinicians caring for children with PIMS-TS. A three-phase online Delphi process and virtual consensus meeting sought consensus over the investigation, management, and research priorities from multidisciplinary clinicians caring for children with PIMS-TS. We used 140 consensus statements to derive a consensus management pathway that describes the initial investigation of children with suspected PIMS-TS, including blood markers to help determine the severity of disease, an echocardiogram, and a viral and septic screen to exclude other infectious causes of illness. The importance of a multidisciplinary team in decision making for children with PIMS-TS is highlighted throughout the guidance, along with the recommended treatment options, including supportive care, intravenous immunoglobulin, methylprednisolone, and biological therapies. These include IL-1 antagonists (eg, anakinra), IL-6 receptor blockers (eg, tocilizumab), and anti-TNF agents (eg, infliximab) for children with Kawasaki disease-like phenotype and non-specific presentations. Use of a rapid online Delphi process has made it possible to generate a national consensus pathway in a timely and cost-efficient manner in the middle of a global pandemic. The consensus statements represent the views of UK clinicians and are applicable to children in the UK suspected of having PIMS-TS. Future evidence will inform updates to this guidance, which in the interim provides a solid framework to support clinicians caring for children with PIMS-TS. This process has directly informed new PIMS-TS specific treatment groups as part of the adaptive UK RECOVERY trial protocol, which is the first formal randomised controlled trial of therapies for PIMS-TS globally.

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

BackgroundThere are several options available for aortic valve replacement (AVR), with few comparative reports in the literature. The optimal choice for AVR in each age group is not clear.ObjectivesThe study sought to report and compare outcomes after AVR in the young using data from a national database.MethodsAVR procedures were compared after advanced matching, both in pairs and in a 3-way manner, using a Bayesian dynamic survival model.ResultsA total of 1,501 patients who underwent AVR in the United Kingdom between 2000 and 2012 were included. Of these, 47.8% had a Ross procedure, 37.8% a mechanical AVR, 10.9% a bioprosthesis AVR, and 3.5% a homograft AVR, with Ross patients being significantly younger when compared to the other groups. Overall survival at 12 years was 94.6%. In children, the Ross procedure had a 12.7% higher event-free probability (death or any reintervention) at 10 years when compared to mechanical AVR (p = 0.05). We also compared all procedures except the homograft in a matched population of young adults, where the bioprosthesis had the lowest event-free probability of 78.8%, followed by comparable results in mechanical AVR and Ross, with 86.3% and 89.6%, respectively. Younger age was associated with mortality and pulmonary reintervention in the Ross group and with aortic reintervention in the mechanical AVR. Of all 3 options, only the patients undergoing the Ross procedure approached the survival of the general population.ConclusionsAVR in the young achieves good results, with the Ross being overall better suited for this age group, especially in children. Although freedom from aortic valve reintervention is superior after the Ross procedure, the need for homograft reinterventions is an issue to take into account. All methods have advantages and limitations, with reinterventions being an issue in the long term for all, more crucially in smaller children.

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.

Objective To describe the longer-term clinical experience and follow-up with perventricular device closure of ventricular septal defects (VSD) in children. Methods Between January 2005 and December 2013, muscular ventricular septal defect closure with the Amplatzer Muscular VSD Occluder was undertaken using a hybrid perventricular approach. Data including demographic, echocardiographic parameter, and clinical outcome were reviewed retrospectively. Results Median age at the time of procedure was 8.9 months (range 1.9–31.0 months) and median weight was 6.6 kg (range 4.5–12.9 kg). All had a moderate to large muscular VSD, three had more than one VSD, four had previous coarctation repair, and five had previous pulmonary artery (PA) banding. A single Amplatzer muscular occluder (range 8–18 mm) was deployed in each patient without cardiopulmonary bypass under echocardiographic guidance. Two of ten patients subsequently required a short period of cardiopulmonary bypass for reconstruction of PA after de-banding and closure of atrial septal defect in one. Occluder removal was necessary in one patient due to entrapment of the tricuspid valve and progressive tricuspid regurgitation. This patient underwent surgical repair with a good result and no device-related valve damage. In the remaining nine patients, no severe complications such as device embolization, arrhythmia, or significant valve regurgitation were noted in the post-operative period or follow-up. At a median time of 6.5 years (range 0.9–8.4 years) post device implantation, complete closure was achieved in five patients and four had small residual leaks, which were not hemodynamically significant. Conclusion Perventricular muscular VSD closure is effective in small children with suitable muscular defects and may avoid the morbidity associated with cardiopulmonary bypass and conventional surgical repair. There have been no late complications with this approach. © 2015 Wiley Periodicals, Inc.

Objectives This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing

Objectives To describe the long-term clinical experience and follow-up with the Amplatzer Ductal Occluder II (ADO II) in children. Methods All patients undergoing attempted transcatheter closure of patent arterial duct (PDA) with the ADO II were included. Data collected included demographic, clinical, and echocardiographic parameters. Results From March 2008 until March 2013, 62 patients with a median age of 1.2 years (range 0.43–11.1 years) and median weight of 9 kg (range 4.7–31.4 kg) underwent the procedure. The median measurement for minimal ductal diameter was 2.7 mm (range 1.3–5 mm). An ADO II was implanted in 60 patients (96.8%). Two patients had significant residual shunting following deployment of the ADO II and underwent closure with the Amplatzer ductal occluder I (ADO I) during the same procedure. In six patients, the initial ADO II was unsatisfactory, and after recapture a different size ADO II was deployed. Device embolization of the ADO II to the pulmonary artery occurred in 6.7% of patients. Of these, one underwent surgical closure and three were closed with an ADO I. Complete occlusion on echocardiography was noted prior to discharge in 87.5% of the deployed occluders and 100% at first follow-up. Five year follow-up (n = 25) revealed a 100% occlusion rate. There were three cases of persistent mild left pulmonary artery stenosis at long-term follow-up. Conclusions The ADO II is effective for occlusion of PDA with variable anatomy from either arterial or venous approaches with a low profile delivery system. Stable occluder position is highly dependent on accurate device sizing, good quality imaging to visualize device configuration after deployment and operator experience. © 2014 Wiley Periodicals, Inc.

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. © 2009 Wiley-Liss, Inc.

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.

OBJECTIVES The purpose of the study was to determine the accuracy of the prenatal echocardiographic diagnosis and prognosis of fetuses with conotruncal anomalies. BACKGROUND The accuracy of prenatal echocardiographic diagnoses of cardiac lesions has been reported, but no previous reports specifically address fetal conotruncal anomalies. METHODS Medical records of 61 fetuses, in which a fetal diagnosis of a conotruncal anomaly was made, were reviewed. Disease entities included were tetralogy of Fallot (TOF), double outlet right ventricle (DORV), transposition of the great arteries (TGA), and truncus arteriosus (TA). RESULTS Fetal diagnosis was established at a median of 24.5 weeks' gestation. Termination of pregnancy was chosen in 31% (19/61) of cases. Postnatal assessment of the diagnosis was not obtained in 12 cases. Excluding two sets of conjoined twins, accurate prenatal diagnosis including definition of the great artery orientation was achieved in 36 of 47 cases (77%). Seven of 17 fetuses with DORV anatomy, of which 6 were thought to have a subpulmonary ventricular septal defect (VSD), had incorrect prenatal assessment of the great artery relationships. One fetus thought to show features of TA had aortic atresia with VSD and normal-sized left ventricle. Of the 42 pregnancies that continued, 15 had major extracardiac malformations and/or chromosomal abnormalities of which one died in utero with trisomy-13 and TA. A further nine died within the neonatal period. Among the 27 fetuses without a documented chromosomal or major extracardiac anomaly, 13 (48%) died. Overall, the survival rate beyond 28 days of life was 52% (22/42). In contrast, 75% (6/8) of fetuses with TOF, excluding the absent pulmonary valve syndrome, survived. CONCLUSIONS Conotruncal anomalies can be diagnosed by prenatal echocardiography with a high degree of accuracy. Defining the exact spatial relationship of the great arteries is problematic in some fetuses. The overall prognosis for fetuses with a conotruncal anomaly is poor, with the exception of uncomplicated TOF.

Objectives To describe early clinical experience with the amplatzer ductal occluder II additional sizes (ADO II AS) for percutaneous arterial duct occlusion in infants and small children. Methods Pre-, intra- and postprocedural data analysis of all patients undergoing arterial duct occlusion with the ADO II AS from three tertiary referral centers. Results 17 patients (10 female) with a median age of 6 months (range 1.0–48.1 months) and a median weight of 5.7 kg (range 1.7–17.4 kg) underwent attempted transcatheter ductal closure with the ADO II AS. Retrograde arterial approach was used in eight patients with transvenous femoral approach used in nine. The mean minimal ductal diameter was 2.2 ± 0.7 mm with mean ductal length of 6.8 ± 1.7 mm. Device sizes used were 5/6 (n = 5), 3/4 (n = 4), 4/4 (n = 3), 4/6 (n = 3), and 5/4 (n = 2) with four French delivery sheaths used in all cases. The median fluoroscopy time was 5.7 ± 1.8 min. Two patients underwent delivery under exclusive echocardiography guidance. Complete ductal occlusion was achieved by the end of the procedure in 13 patients. Device embolization to the left pulmonary artery occurred in one patient with successful surgical removal and ligation of the arterial duct. Three patients required device resizing following deployment of the initial device. Complete ductal occlusion without aortic arch or left pulmonary artery stenosis has been identified in all 16 remaining patients on transthoracic echocardiographic follow-up at median of 4.2 months. Conclusions The new amplatzer ductal occluder II AS achieves excellent ductal closure rates through low profile delivery systems in small infants and children with variable ductal anatomy. © 2012 Wiley Periodicals, Inc.

OBJECTIVE: To report initial experience with a new occlusion device for native and residual patent ductus arteriosus. DESIGN: Descriptive study of consecutive non-randomised patients undergoing a new method of patent ductus arteriosus closure with detachable coils. SETTING: Tertiary centres for paediatric cardiology. PATIENTS: 71 consecutive patients, aged 1.2-22 years, with a patent ductus arteriosus (PDA) underwent elective transcatheter closure. 45 had native PDAs (group A) with a minimum diameter of 1.0 mm-5.0 mm (median 2.0 mm). A further 26 had undergone one or more previous occlusion attempts (group B). INTERVENTIONS: A total of 133 detachable (Cook) spring coils were successfully implanted in 70 patients. The procedure was performed transvenously in 51 patients, retrograde arterially in 13, and by both routes in a further 6 patients. One 5 mm coil migrated but was successfully retrieved. MAIN OUTCOME MEASURES: In group A colour flow Doppler echocardiography showed that complete occlusion was achieved in 40/45 (89%) at 24 hours, 41/45 (91%) at 1 month, and 44/45 (98%) by 6 months post procedure. Occlusion rates in residual PDAs were 22/25 (88%) occluded at 24 hours, 23/25 (92%) at 1 month, and 24/25 (96%) at 6 months follow up. CONCLUSIONS: Transcatheter occlusion using detachable (Cook) spring coils is a safe and effective alternative to presently available devices. The delivery system allows full retrieval of the coil until a satisfactory position is obtained.

Background The technique of transcatheter patent ductus arteriosus (PDA) closure has evolved in the past two decades and is increasingly used in smaller patients. We sought to report a multicentre experience of transcatheter PDA occlusion in infants weighing Methods A multicentre, retrospective analysis of children weighing Results A total of 361 patients underwent attempted transcatheter PDA closure. The mean weight at catheterization was 4.8 ± 1.0 kg and mean age was 5.7 ± 2.7 months. Successful device closure was achieved in 321(89%) patients without major complication. Device placement was attempted but failed in in 13 cases. Device malposition or embolization post release occurred in the remaining 27 cases. Of these, 11 patients had surgical retrieval of the embolized device and PDA ligation; 6 patients were referred for surgery at a later date after transcatheter retrieval of device and 10 patients had ductal closure with a different device after transcatheter retrieval of the initial device. In the 331 patients who had transcatheter PDA occlusion, 34 had coils and 297 had occluders, including Amplatzer Duct Occluder (ADO) (n = 197); ADO II (n = 51); ADO II Additional Sizes (n = 42); Amplatzer Vascular Plug (n = 6); Occlutech Duct Occluder (n = 1). Complete occlusion was achieved in a 312(94%) patients at either catheterization or last available follow-up. Three of 19 patients had mild residual flow which persisted at less than a year post procedure; but follow up data was not complete in the rest. There were 17 cases of mild left pulmonary artery (LPA) stenosis related to device impingement with velocity >2m/s; 11 of which resolved with time, 5 had persistent mild LPA stenosis at last review with Doppler velocity of 2.5m/s or less; and 1 underwent LPA stenting. Mild aortic obstruction was recognised in 10 patients during catheterisation or on post procedure echocardiogram, 7 of which resolved; one had persistent increased velocity at the arch with no diastolic tail at last review and 2 had incomplete follow up data. Other complications include loss of pulse requiring TPA (n = 5) temporary reduction in pulse requiring heparin infusion (n = 16); bleeding requiring transfusion (n = 2); anaesthesia or airway related (n = 1); small pericardial effusion (n = 1); haemopericardium requiring percutaneous drainage (n = 1); tricuspid valve injury (n = 1); and cerebrovascular event (n = 1). There were 13 deaths in the cohort which were unrelated to the procedure. The causes of death were mainly related to underlying chronic lung disease and pulmonary hypertension. Conclusions Transcatheteter closure of PDA can be safely accomplished in selected infants who are less than 6 kg despite the manufacturer’s recommended weight limit of 6 kg for most ductal occluders. The embolization rate (7%) in this group appeared to be higher than published series in infants >6kg, therefore thorough delineation of ductal anatomy and attention to device selection are vital to the success of the procedure.

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 8.5–65) and median weight 65 kg (range 34–101). 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 4.2–72.8), 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 (n=7) occurred with a single stent design. Conclusions Stenting for aortic coarctation and re-coarctation 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.

Objectives To describe the evolution of transcatheter closure of perimembranous ventricular septal defects (PMVSD) using either the Amplatzer membranous or muscular occluders in a single centre. Methods Retrospective analysis of all patients referred for transcatheter PMVSD closure from December 2003 to December 2007. All patients met unit criteria for surgical closure. Results There were 27 procedures on 25 patients (11 male) with a preprocedure diagnosis of a PMVSD. Median age was 9.6 years (1.8-32.8). Median weight was 28 kg (10.2-86). Defect size on TOE ranged from 5 to 12 mm. Median Qp:Qs was 1.6:1. A muscular occluder was used in six patients. Median procedure time was 93.5 min (51-214). Implantation was ultimately successful in 23 patients (92%). Acute aortic incompetence resulting in occluder removal occurred in two cases, one requiring surgical removal. Another patient had an aborted attempt but had subsequent successful closure in another unit. Median follow-up is 19.5 months (1-42). Five patients (22%) have trivial/mild residual leak across the occluder at their latest assessment, the majority of which had an aneurysmal perimembranous septum (n = 4). Two patients (8%) developed new trivial to mild aortic incompetence. To date, none of the patients in our group have developed complete heart block. Conclusions Transcatheter closure of PMVSD is evolving and should be considered an acceptable alternative to surgery in selected subgroups. Avoidance of oversized occluders and use of muscular occluders in those with aneurysmal defects may help to avoid heart block and aortic regurgitation. Muscular occluders may however interfere with tricuspid valve function.

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.

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 = 13); recurrent coarctation following surgical treatment (n = 11); aneurysm associated with previous coarctation surgery (n = 7); aorto-bronchial fistula leading to acute hemoptysis (n = 2); stent fracture (n = 1); associated arterial duct (n = 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. Self-expanding stent grafts may be preferable to balloon expandable stents when there is aneurysm formation in the setting of aortopathy. © 2008 Wiley-Liss, Inc.

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. © 2007 Wiley-Liss, Inc.

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 < 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. (c) 2006 Wiley-Liss, Inc.

A 6-week-old infant presenting in heart failure was suspected on echocardiography of having anomalous origin of the whole coronary circulation from the pulmonary artery arising from a single ostium. This was confirmed at operation, and the child underwent successful correction. The management of cardiopulmonary bypass and myocardial protection in this abnormality is discussed.

Fetuses can survive with complete airway obstruction caused by placental gas exchange until birth when full ventilatory function is required. The authors present a case in which prenatal scans suggested that adequate ventilation would not be achievable because of the presence of an intrathoracic tumor. An EXIT procedure (exutero intrapartum treatment) was therefore performed, which permitted sufficient lung expansion for adequate ventilation.

Objectives—Description and evaluation of current experience with the use of balloon expandable stents for the relief of systemic venous pathway stenosis late after Mustard's operation. Design—Retrospective observational study of technical procedures, angiographic, and haemodynamic findings. Patients—Twenty long term survivors of Mustard's operation for transposition of the great arteries (TGA) with angiographic evidence of systemic venous pathway narrowing. Intervention—Systemic venous pathway stenoses were stented using balloon expandable Palmaz stents. Results—Twenty seven stents were deployed across 24 stenoses. Seventeen stents were placed in the inferior baffle (16 patients), with an increase in mean (range) minimum diameter from 9.6 (4.5-15.9) to 16.5 (11.9-22.2) mm (p = 0.007), and a reduction in mean pressure gradient from 3.1 (0-8) to 0.67 (0-3) mm Hg (p = 0.002). Eight stents were placed in the superior pathways of eight patients, with diameters widened from 9.1 (3.5-14.1) to 15.2 (8.7-19.2) mm (p = 0.018), and gradients reduced from 6.4 (2-11) to 0.9 (0-2) mm Hg (p = 0.02). Two badly deployed stents were safely withdrawn from their intracardiac positions and redeployed in the iliac vein. Transvenous pacemaker insertion was facilitated by prior stent insertion. Conclusions—The use of balloon expandable stents for late systemic pathway narrowing after Mustard's operation is safe and effective. The beneficial effects of stenting are likely to be more durable than those of balloon angioplasty alone, but longer term follow up is required. Keywords: stents; Mustard procedure; systemic venous baffle stenosis; adult congenital heart disease