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To reflect new developments in imaging, indications, and techniques in catheterisation of patients with CHD, the training recommendations of the Association for European Paediatric and Congenital Cardiology (AEPC) for the training in interventional catheterisation for CHD have been reviewed and updated after more than 7 years. They include detailed information about knowledge, skills, and approach to clinical practice expected from trainees at basic, intermediate, and advanced level.

A 14-year-old girl with trisomy 13 presented with signs of respiratory failure. This was caused by the rupture of a sinus of Valsalva aneurysm into the right atrium. A Konar Multifunctional Occluder was used for closure. This is the first report of this device for this indication.

Aorto-left ventricular tunnel is an extremely rare CHD clinically impressing as aortic regurgitation. This is usually corrected surgically, sometimes by percutaneous catheter intervention. Recurrent aorto-left ventricular tunnel has been reported occasionally. Reports of percutaneous closure of such recurrent aorto-left ventricular tunnels are rare. We hereby describe successful closure of a recurrent aorto-left ventricular tunnel with an Amplatzer Vascular Plug II.

NPC-QIC Registry data showed that angiotensin-converting enzyme inhibitors were described in almost 38% for patients with single ventricle physiology after stage-I Norwood palliation. However, mortality and ventricular dysfunction or atrioventricular valve insufficiency seems to be not improved by oral application of angiotensin-converting enzyme inhibitors. The final conclusion was that despite limited evidence of benefit for patients with hypoplastic left heart syndrome, prescription of angiotensin-converting enzyme inhibitors during interstage is still common. Taking into account of the predominant cardiovascular regulation in newborns and young infants by circulating catecholamines, no real improvement is to be expected from angiotensin-converting enzyme inhibitor monotherapy. The goals of drug therapy after stage-I Norwood palliation in hypoplastic left heart syndrome are prevention of systemic right ventricle failure, balancing pulmonary and systemic blood flow, and reduction of oxygen consumption with regard to limitations of oxygen supply by the single ventricle, furthermore, avoiding harmful effects of endogenous catecholamine production in the long term on somatic and cognitive development. In this light of knowledge, we want to recommend the use of a long-acting and highly specific ß1-adrenoreceptor blocker for almost all patients after stage-I Norwood palliation and a combination with angiotensin-converting enzyme inhibitors only by indication after exclusion of potential side effects.

Aims:Management strategies for pulmonary atresia with intact ventricular septum are variable and are based on right ventricular morphology and associated abnormalities. Catheter perforation of the pulmonary valve provides an alternative strategy to surgery in the neonatal period. We sought to assess the long-term outcome in terms of survival, re-intervention, and functional ventricular outcome in the setting of a 26-year single-centre experience of low threshold inclusion criteria for percutaneous valvotomy.Methods and results:Retrospective analysis of patients diagnosed with pulmonary atresia with intact ventricular septum from 1990 to 2016 at a tertiary referral centre, was performed. Of 71 patients, 48 were brought to the catheterisation laboratory for intervention. Catheter valvotomy was successful in 45 patients (94%). Twenty-three patients (51%) also underwent ductus arteriosus stenting. The length of intensive care and hospital stay was significantly shorter, and early re-interventions were significantly reduced in the catheterisation group. There were eight deaths (17%); all within 35 days of the procedure. Of the survivors, only one has required a Fontan circulation. Twenty-eight patients (74%) have undergone biventricular repair and nine patients (24%) have one-and-a-half ventricle circulation. Following successful valvotomy, 80% of patients required further catheter-based or surgical interventions.Conclusions:A low threshold for initial interventional management yielded a high rate of successful biventricular circulations. Although mortality was low in patients who survived the peri-procedural period, the rate of re-intervention remained high in all groups.

Background: Catheter ablation (CA) is the first-choice treatment for tachyarrhythmia in children. Currently available CA techniques differ in mechanism of catheter navigation and energy sources. There are no large studies comparing long-term outcomes between available CA techniques in a pediatric population with atrioventricular reentry tachycardia (AVRT) or atrioventricular nodal reentry tachycardia (AVNRT) mechanisms. Objective: This study aimed to compare procedural and long-term outcomes of remote magnetic navigation-guided radiofrequency (RF) ablation (RMN), manual-guided RF ablation (MAN) and manual-guided cryoablation (CRYO). Methods: This single-center, retrospective study included all first consecutive CA procedures for AVRT or AVNRT performed in children without structural heart disease from 2008 to 2019. Three study groups were defined by the ablation technique used: RMN, MAN or CRYO. Primary outcome was long-term recurrence of tachyarrhythmia. Results: In total, we included 223 patients, aged 14 (IQR 12–16) years; weighting 56 (IQR 47–65) kilograms. In total, 108 procedures were performed using RMN, 76 using MAN and 39 using CRYO. RMN had significantly lower recurrence rates compared to MAN and CRYO at mean follow-up of 5.5 ± 2.9 years (AVRT: 4.3% versus 15.6% versus 54.5%, P < 0.001; AVNRT: 7.7% versus 8.3% versus 35.7%, P = 0.008; for RMN versus MAN versus CRYO respectively). In AVNRT ablation, RMN had significantly lower fluoroscopy doses compared to CRYO [30 (IQR 20–41) versus 45 (IQR 29–65) mGy, P = 0.040). Conclusion: In pediatric patients without structural heart disease who underwent their first AV(N)RT ablation, RMN has superior long-term outcomes compared to MAN and CRYO, in addition to favorable fluoroscopy doses.

Background: Disconnected unilateral pulmonary arteries are frequently misdiagnosed as “absent”. They typically arise from the base of the innominate artery and are fed by an aberrant arterial duct. If diagnosed early enough, they can be reconnected with catheter techniques even after closure of this aberrant duct. Consecutive surgical anatomical correction at a later stage is possible. Methods: Four cases illustrate the anatomical findings on computed tomography and angiography, all show an outpouching at the base of the brachiocephalic artery. Results: The therapeutic approach consisted of stenting of the aberrant ductus and consecutive surgery. In the oldest patient, 13 years, such an approach was impossible. Conclusion: If identified early in life, disconnected pulmonary arteries can be recruited with catheter techniques, and reconnected surgically at a later stage. It is not yet known if this approach prevents pulmonary damage, which is frequently seen in older untreated patients.

Isolated atrial defects usually lead to left-to-right shunt and right ventricular volume load. Descriptions of cyanosis with this congenital heart defect are rare.We describe a rare case of inferior caval vein flow directed through an atrial septal defect in the fossa ovalis leading to severe cyanosis, but without any additional intracardiac anatomical abnormalities. The baby with clinical features of Marfan’s syndrome had an eventration of the right-sided diaphragm. Surgical closure of the defect resolved the cyanosis, but the child died 10 weeks later of severe valvar dysfunction, related to Marfan’s syndrome.Mechanisms of cyanosis in patients with atrial septal defects are discussed. Echocardiographic bubble studies both from the lower and upper half of the body may help to clarify the mechanism of an otherwise unexplained cyanosis.

Funding Acknowledgements Type of funding sources: None. Introduction – Catheter ablation (CA) is a first-choice treatment for tachyarrhythmia in pediatric patients. The currently available CA techniques differ in manner of catheter steering technique and energy sources. There are no large studies comparing long-term outcomes between the available CA techniques in pediatric patients with atrioventricular reentry (also known as accessory pathway mediated) tachycardia (AVRT) or atrioventricular nodal reentry tachycardia (AVNRT) mechanisms. Objective – The aim of this study was to compare procedural parameters and outcomes of remote magnetic navigation-guided radiofrequency (RF) ablation (RMN), manual-guided RF ablation (MAN) and manual-guided cryoablation (CRYO). Methods – This single-center, retrospective study included all first consecutive CA procedures for AVRT or AVNRT mechanisms performed in pediatric patients with no structural heart disease from January 2008 until June 2019. Three study groups were defined by the ablation technique used: RMN, MAN or CRYO. Primary outcome was recurrence of tachyarrhythmia and/or pre-excitation on ECG. Baseline clinical parameters, procedure times and complication rates were also evaluated. Results – In total, we included 223 patients, aged 13.8 ± 2.8 years, with a mean weight of 55.6 ± 14 kilograms. In total, 108 procedures were performed using RMN, 76 using MAN and 39 using Cryo. RMN had the lowest recurrence rates at a mean follow-up of 5.5 ± 2.9 years (AVRT ablation: 4% vs. 16% vs. 55%, P

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IntroductionIn general SARS-CoV-2-infection during pregnancy is not considered to be an increased risk for severe maternal outcomes, but has been associated with an increased risk for fetal distress. So far, there is no direct evidence of intrauterine vertical transmission and the mechanisms leading to the adverse outcomes are not well understoodResultsAn asymptomatic pregnant woman with preterm fetal distress during the COVID19 pandemic was included. We obtained multiple maternal, placental and neonatal swabs, which showed a median viral load in maternal blood, urine, oropharynx, fornix posterior over a period of 6 days was 5.0 log copies /mL. The maternal side of the placenta had a viral load of 4.42 log copies /mL, while the fetal side had 7.15 log copies /mL. Maternal breast milk, feces and all neonatal samples tested negative. Serology of immunoglobulins against SARS-CoV-2 was tested positive in maternal blood, but negative in umbilical cord and neonatal blood. Pathological examination of the placenta included immunohistochemical investigation against SARS-CoV-2 antigen expression in combination with SARS-CoV-2 RNA in situ hybridization and transmission electron microscopy. It showed the presence of SARS-CoV-2 particles with generalized inflammation characterized by histiocytic intervillositis with diffuse perivillous fibrin depositions with damage to the syncytiotrophoblasts.DiscussionPlacental infection by SARS-CoV-2 lead to fibrin depositions hampering fetal-maternal gas exchange most likely resulted in fetal distress necessitating a premature emergency caesarean section. Postpartum, the neonate showed a clinical presentation resembling a pediatric inflammatory multisystem syndrome including coronary artery ectasia, most likely associated with SARS-CoV-2 (PIMS-TS) for which admittance and care on the Neonatal Intensive Care unit (NICU) was required, despite being negative for SARS-CoV-2. This highlights the need for awareness of adverse fetal and neonatal outcomes during the current COVID-19 pandemic, especially considering that the majority of pregnant women appear asymptomatic.

A planned combined perventricular and “open heart” surgical closure of multiple ventricular septal defects had to be modified intraoperatively due to a technical fault disabling echocardiographic guidance. Through an atriotomy, device closure of a muscular defect and patch closure of a perimembranous ventricular septal defect were performed. In unusual situations, collaboration of the surgical and interventional team is crucial.

Veno-venous double lumen Extracorporeal membrane oxygenation (VVDL-ECMO) in neonates and infants for viral pneumonia is a well-established therapy. However even after initial correct positioning of the VVDL-ECMO cannula, there is a risk of unexpected malpositioning of this cannula during the ECMO run. We report a case of reposition of a 16-French Avalon Elite Bi-caval Dual Lumen cannula from its malposition in the right atrium to its correct position in the inferior venous cava. The procedure was achieved by interventional catheter techniques using a goose neck snare and was done without ECMO-flow interruption. Keywords: Infant, Dual lumen cannula, VVDL ECMO

Introduction Coarctation of the aorta in children under 3 months of age is usually treated surgically. However, there are clinical scenarios in which stenting of native or recurrent coarctation may become necessary in this age group. Case reports Four cases illustrate possible indications: left ventricular dysfunction increasing the operative risk, thrombus formation after coarctation surgery, patient size (i.e. in premature babies), and retrograde arch obstruction after hybrid palliation of hypoplastic left heart syndrome. In all babies, coarctation stenting was carried out successfully without complications. Conclusion Coarctation stenting can be carried out safely in small children. Usually, the stent has to be removed or redilated later. Results are encouraging.

textabstractBackground: Open cell stents are frequently used in interventional therapy of congenital heart disease. Overstenting of vessel branches may necessitate strut dilation. Methods and Results: The strut size achievable in Bard Valeo and Cook Formula stents, and the pressure necessary to fracture struts was assessed. In addition, a self expanding stent (Optimed SinusflexDS) was also tested. With the original balloon at nominal pressure, in Valeo stents side struts could be dilated to approximately 90% of the nominal stent diameter, in Formula stents to approximately 80%. With larger high pressure woven balloons, strut size increased to approximately 125% in Valeo stents, and to approximately 105% in the Formula. Strut fracture can connect two adjoining struts. Pressures were dependent on the balloon utilized. Sidestruts of the Sinusflex could lastingly overdilated with large balloons only. Conclusion: Dilation and overdilation of side struts in open cell stents can be achieved. Dependent on the clinical context, the original balloon used to place the premounted stent can be used to achieve strut dilation, but woven high pressure balloons maybe safer for patients. Should a larger diameter be required, these high pressure woven balloons can achieve bigger diameters and even strut fracture.

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 cm2 (47–461 mGy cm2), 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 cm2 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.

A modern catheter laboratory for the treatment of children with CHD should be in close proximity to the paediatric ICU, operating theatres, and imaging facilities. Space requirements and equipment for an up-to-date catheter laboratory are discussed. The document was endorsed by the council of the Association of European Paediatric and Congenital Cardiologists.

Objectives: To evaluate the procedural success and outcome of inter-atrial stenting. Background: Inter-atrial stenting has been shown to be an effective way to maintain inter-atrial blood flow, however it is considered a high risk procedure, usually performed urgently in patients with significant hemodynamic compromise. Methods: Between September 2004 and August 2016, inter-atrial stenting was attempted in 29 children. Procedural, clinical, and follow-up data were collected retrospectively. Results: The procedures were completed successfully in 27 patients. Twenty-five procedures were undertaken percutaneously, with the remaining four being performed as hybrid procedures. The patients were considered as high risk for adverse events (82% scored as CRISP 4 and 5) with four deaths during the first 24 hr (14%). Procedural complications occurred in eight patients (28%) with related death in three patients (10%). One further patient died after an uncomplicated technically successful stent implantation performed as a salvage procedure. Procedural complications (71% vs. 14%) and mortality (43% vs. 5%) were higher in those, who weighed 3 kg or less (P < 0.05). Patency of the stents was maintained until planned staged surgery in 22 patients at a mean of 302 days. Three patients underwent further balloon dilation for flow restriction at 58–201 days. In two un-operated patients the stents remained patent at follow-up. One patient with severe pulmonary hypertension died with a patent stent. Conclusions: Inter-atrial stenting produces reliable patency with a very good success rate. Morbidity and mortality were related to low weight at the time of the procedure.

A newborn with hypoplastic left heart underwent a Norwood operation. Obstruction of the Blalock–Thomas–Taussig shunt was treated with a stent. During resuscitation, this was compressed, which contributed to a fatal outcome.

Whilst stenosis of systemic and pulmonary arteries in Williams syndrome is frequently described, aneurysm formation is uncommon. We provide the first description of a Williams patient with development of an aneurysm of the arterial duct. This aneurysm developed concomitantly with supravalvar aortic, and peripheral pulmonary stenosis. The duct was closed interventionally to reduce the risk of rupture.

Pulmonary artery stenting may not be possible transcutaneously because of anatomic features. Although intraoperative stenting has been well described, we present a case in which stenting of the left pulmonary artery was performed transthoracically in a separate procedure. Unusual anatomic conditions may require a multi-disciplinary hybrid approach to achieve the desired results.

ObjectivesWe aimed to compare the procedural and mid-term performance of a specifically designed self-expanding stent with balloon-expandable stents in patients undergoing hybrid palliation for hypoplastic left heart syndrome and its variants.BackgroundThe lack of specifically designed stents has led to off-label use of coronary, biliary, or peripheral stents in the neonatal ductus arteriosus. Recently, a self-expanding stent, specifically designed for use in hypoplastic left heart syndrome, has become available.MethodsWe carried out a retrospective cohort comparison of 69 neonates who underwent hybrid ductal stenting with balloon-expandable and self-expanding stents from December, 2005 to July, 2014.ResultsIn total, 43 balloon-expandable stents were implanted in 41 neonates and more recently 47 self-expanding stents in 28 neonates. In the balloon-expandable stents group, stent-related complications occurred in nine patients (22%), compared with one patient in the self-expanding stent group (4%). During follow-up, percutaneous re-intervention related to the ductal stent was performed in five patients (17%) in the balloon-expandable stent group and seven patients (28%) in self-expanding stents group.ConclusionsHybrid ductal stenting with self-expanding stents produced favourable results when compared with the results obtained with balloon-expandable stents. Immediate additional interventions and follow-up re-interventions were similar in both groups with complications more common in those with balloon-expandable stents.

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.

Objectives: Pulmonary hypertension is one of the main causes of mortality and morbidity in patients with congenital diaphragmatic hernia. Currently, it is unknown whether pulmonary hypertension persists or recurs during the first year of life. Design: Prospective longitudinal follow-up study. Setting: Tertiary university hospital. Patients: Fifty-two congenital diaphragmatic hernia patients admitted between 2010 and 2014. Interventions: None. Measurements and Main Results: Pulmonary hypertension was measured using echocardiography and electrocardiography at 6 and 12 months old. Characteristics of patients with persistent pulmonary hypertension were compared with those of patients without persistent pulmonary hypertension. At follow-up, pulmonary hypertension persisted in four patients: at 6 months old, in three patients (patients A–C), and at 12 months old, in two patients (patients C and D). Patients with persistent pulmonary hypertension had a longer duration of mechanical ventilation (median 77 d [interquartile range, 49–181 d] vs median 8 d [interquartile range, 5–15 d]; p = 0.002) and hospital stay (median 331 d [interquartile range, 198–407 d) vs median 33 d (interquartile range, 16–59 d]; p = 0.003) than patients without persistent pulmonary hypertension. The proportion of patients with persistent pulmonary hypertension (n = 4) treated with inhaled nitric oxide (100% vs 31%; p = 0.01), sildenafil (100% vs 15%; p = 0.001), and bosentan (100% vs 6%; p < 0.001) during initial hospital stay was higher than that of patients without persistent pulmonary hypertension (n = 48). At 6 months, all patients with persistent pulmonary hypertension were tube-fed and treated with supplemental oxygen and sildenafil. Conclusions: Less than 10% of congenital diaphragmatic hernia patients had persistent pulmonary hypertension at ages 6 and/ or 12 months. Follow-up for pulmonary hypertension should be reserved for congenital diaphragmatic hernia patients with echocardiographic signs of persistent pulmonary hypertension at hospital discharge and/or those treated with medication for pulmonary hypertension at hospital discharge

Pulmonary valve stenosis is common in patients with Noonan’s syndrome. The response to balloon valvoplasty varies.We assessed the correlation between re-intervention rate, immediate response, and the progress of the valve gradient over time after intervention.MethodsThis is a retrospective study conducted from 1995 to 2014.ResultsOf 14 patients identified, seven had re-intervention 28±54 months (range 3–149, median 3.3) after valvoplasty. These patients did not have a significant decrease in gradient after intervention. Their gradient subsequently decreased during follow-up and then became static before increasing years after intervention. In contrast, the gradient of patients not requiring further intervention continually reduced over time. Demographics did not differ between these groups.ConclusionWe could not identify predisposing factors for long-term success of pulmonary valvoplasty in Noonan’s patients, but the trajectory of gradients differs significantly between patients needing re-intervention from those who remain free from re-intervention.

The Melody valve, designed for implantation into the pulmonary outflow tract, can also be used to treat the pathology of atrioventricular (AV) valves. Increasing gradients are seen as an indication for re-dilating the valve. Our case demonstrates the heart rate dependency of the gradient across a Melody implanted in the left AV valve position in an infant. Beta blockers were used to lower both heart rate and gradient.

Background Little is known about the necessity for long-term follow-up after interventional closure of persistent arterial duct (PDA). Potential side effects and complications include residual shunts, haemolysis, device embolization, and obstruction to flow in the adjoining vessels. Methods Single centre retrospective study of paediatric patients undergoing interventional PDA occlusion. Results 315 patients who underwent interventional occlusion of a PDA between November 2002 and September 2013 were included. Of these, eight needed re-intervention (three for device embolization, five for residual shunt). Seven had mild obstruction to flow in the adjoining vessels, but did not require any intervention. All sequelae were found latest at the first follow-up appointment after the procedure (usually within 3 months); whilst none developed during further follow-up. Conclusion Complications of interventional closure of PDA were apparent immediately after the procedure or by three months of follow-up. Long-term follow-up is not indicated in cases when no complications are seen early after the procedure. © 2015 Wiley Periodicals, Inc.

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.

Endocarditis of congenital coronary fistulas in the cardiac chambers is rare, especially in the paediatric age group. We describe the case of a 9-year-old boy with a fistula from the dilated right coronary artery to the junction of the superior caval vein to the right atrium, complicated by endocarditis. Treatment consisted of 6 weeks of antibiotics and interventional closure of the fistula 3 months later with an Amplatzer vascular plug.

Aims Coronary artery fistulae represent one of the most challenging anatomical defects to define accurately. We aimed to investigate the additional benefit conferred by volume rendering of tomographic images and 3D printing for diagnosis and interventional planning. Methods and results Four cases of coronary fistulae were considered for transcatheter closure. Multidetector computed tomography (three cases) or cardiac magnetic resonance (one case) images were acquired and segmented using Mimics software. Each case was reviewed after incremental consideration of diagnostic resources: two cardiologists reported source and volume-rendered images; device closure was discussed by the interventional cardiology team. All diagnoses and planned management were reviewed after inspection of a 3D model. Using source images alone, both cardiologists correctly described the course and drainage in two out of four cases. Aided by volume rendering, this improved to three out of four cases. Inspection of the 3D printed model prompted the planned interventional approach and device sizing to be altered in two out of four cases. In one out of four cases, the intervention was abandoned after inspection of the 3D printed model. Conclusions Diagnosis and management of patients with coronary artery fistulae rely on detailed image analyses. 3D models add value when determining the feasibility of, and the approach to intervention in these cases.

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

BackgroundHypertension 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.ObjectivesThe objective of this study was to evaluate the risk of future medical treatment for hypertension depending on age of primary coarctation repair.MethodsThis 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.ResultsA 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.ConclusionsRisk 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.

The evolution of the treatment and palliation of congenital heart disease has been celebrated over the last 20 years as, the literature describes improving longevity in all but the most extreme conditions.

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.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.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; p0.001); 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).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.

Kawasaki disease (KD) is a generalized systemic vasculitis, although the coronary artery system is typically involved. Coronary artery lesions (CAL) develop in 25% of children during the acute stage of KD and may lead to infarction, sudden death, or chronic coronary artery insufficiency [(1)][1]. In

Background Transcatheter balloon aortic valvuloplasty has become the first-line treatment for critical or severe aortic stenosis in neonates in many centers. Aortic regurgitation following balloon aortic valvuloplasty remains a major concern. An optimal balloon size to aortic annulus ratio in order to minimize aortic regurgitation postprocedure, while relieving the obstruction, has not yet been identified. Methods and Results In this retrospective study, data from 29 neonates with critical or severe congenital aortic valve stenosis, who underwent balloon aortic valvuloplasty in the first 28 days of life, over an 11-year period, was evaluated. The balloon size used, ranged from 71% to 160% of the annulus size, with an average of 89%, based on the aortic annulus size as measured on angiography. The aortic regurgitation immediately following the procedure was trivial in 8 (27.6%), mild in 13 (44.8%), moderate in 7 (24.1%), and severe in 1 (3.4%) patient. The balloon to annulus ratio had no statistically significant effect on the degree of aortic regurgitation immediately after the procedure (P-value of 0.25), at first follow-up within 6 weeks of the procedure (P-value of 0.76) or at follow-up at 1 year (P-value of 0.52). Conclusion This study did not show any significant relationship between the balloon to annulus ratio during interventional dilatation and the degree of aortic regurgitation following the procedure. (J Interven Cardiol 2013;26:200-207)

In neonates and infants with a shunt-dependent pulmonary circulation, stenosis of the shunt needs to be treated aggressively to prevent a fatal outcome. We report the technique and outcome in seven consecutive neonates and infants with Blalock–Taussig shunt stenosis, who were treated with intraluminal stents. Stenting of classical or modified Blalock–Taussig shunts is technically feasible and can be used as a rescue procedure. Neointimal dissection is less likely compared with balloon angioplasty of the shunt alone.

The indication to intervene on congential heart disease can be dependent on clinical symptoms such as breathlessness, cyanosis and heart failure signs, but in many cases echocardiographical findings lead to further investigations and even interventional treatment (i.e., an atrial septal defect with right heart volume load is typically clinically asymptomatic for a long time, and transthoracic echocardiography (TTE) may be the only investigation carried out prior to treatment). The same appeals to purely valvar stenosis of the semilunatic valves; however, if there is doubt about outflow tract obstruction or sub-/supra-valvar stenosis, a transesophageal echocardigram (TOE) can delineate the anatomy in high detail. On the other hand, especially in children, this will have to be carried out under general anesthesia.

Objectives Despite improvements in the surgical management of neonates with the hypoplastic left heart syndrome and its variants, there is a subset of children who have a high predicted mortality. We have analyzed and report our results with the hybrid approach in the management of these high-risk patients. Methods Children identified by our selection criteria underwent the initial hybrid procedure performed in the catheterization laboratory. This consists of surgical banding of the branch pulmonary arteries and stenting of the arterial duct. This was followed 3 to 6 months later by the combined stage I and II Norwood operation or biventricular repair. Results Between December 2005 and May 2008, 21 neonates were selected for the hybrid procedure, of whom 16 had typical hypoplastic left heart syndrome. The remaining 5 neonates had severe aortic stenosis with borderline left ventricular dimensions or poor left ventricular function at presentation. Four neonates had a virtually intact interatrial septum and required urgent intervention immediately after birth. There were 6 deaths as inpatients or up to 30 days after the procedure and 1 interstage death before the combined stage I and II operation. Thus far, of 12 babies who have undergone the next operation, 9 have had the combined stage I and II operation and 3 have had a biventricular repair. Two of these 12 babies have died after the combined stage I and II operation. Conclusion A hybrid approach may be a valuable alternative to the management of high-risk neonates with hypoplastic left heart syndrome and its variants.

A prolongation of the corrected QT interval (QTc) in the routine ECG of otherwise healthy neonates has been suspected to be a risk factor for sudden infant death. None of the studies published so far differentiated between awake and sleeping neonates. This study was designed to find possible differences of the QTc when neonates were asleep or awake, and to provide guide values for neonatal QTc in 24-h ECGs. Hundred neonates (51 male, 49 female, age 2–11 days) underwent ambulatory ECG with defined lead positions. The QT interval was measured constantly in three different leads, and the corrected QT interval was calculated using Bazett’s formula. We compared the QTc obtained during sleep with the QTc obtained from wake periods. The heart rate was significantly higher when the neonates were awake, and when awake higher in girls than in boys. While there was no statistically significant difference between the QTc when the babies were awake or asleep in leads 1 and 3, this reached the level of mathematical significance in the most cranio-caudal lead 2. Changes of the corrected QT interval in neonates seems to be dependent on the lead position of ambulatory ECGs, and could be found only in one lead. Guide values for the sleeping and awake neonate are provided.

Evelina London Children’s Hospital, Department of Paediatric Cardiology, Westminster Bridge Road, London, SE1 7EH, UK Percutaneous intervention for congenital heart disease (CHD) has been established as a core treatment modality since Rashkind pioneered balloon septostomy for neonates with transposition of the great arteries [1]. Since then, cardiac catheterization has progressed from mainly being a diagnostic procedure to interventions in the majority of cases, especially as a large amount of diagnostic information can be obtained with imaging modalities, such as echocardiography, computed tomography and MRI. Catheter interventions for CHD (as well as for coronary disease) are traditionally performed under f luoroscopic guidance using radiation. Especially in pediatric practice, any use of radiation carries the potential risk of DNA damage and the development of malignancy years to decades after the procedure [2,3]. Hence, the ‘as low as reasonably achievable’ principle has been introduced, and efforts have been made to minimize the dose used for common interventions [4]. This can be facilitated by detailed visualization of complex anatomy with imaging modalities, such as MRI, prior to intervention. Such preprocedural planning allows preselection of the angulations needed for interventional angiography, hence decreasing procedure time and minimizing contrast and radiation load [5]. Echocardiography and angiography do not always correlate well, as shown for neonatal aortic valves [6]. Hence, in these cases, angiography as the gold standard to determine balloon size cannot be replaced by echocardiography. Image fusion is a technique in which preexisting MRI or computed tomography images are overlaid on the live fluoroscopic images and are used as a ‘road map’ to guide catheter interventions (i.e., in coarctation of the aorta) [7].

Primary repair of tetralogy of Fallot (TOF) has been favored in many centers for years now and results and advantages of this management seem to verify this procedure. The authors wanted to know, if the age at the time of surgery and the surgical techniques had an influence on the long-term results. Between 1992 and 2003, 124 patients underwent complete repair of TOF at the University Hospital Munster, Germany. Patients were subdivided into two groups based on their age ( 1 year of age). Patients in whom a transannular patch (TAP) was used were compared with those without (NTAP), or in whom a conduit was used. Overall mortality was 8%, with an average age of death of 9.53 years (range 0.06–19.77 years). The patients’ age at the time of surgery affected their survival as only two cases of death were reported among the group of children < 1 year of age (3.2%) whereas eight patients were older (12.9%; p = 0.0483). Six patients died within the first 30 days post surgery. Reoperation had to be performed in 21 cases, 13 (61.9%) of these patients were < 1 year of age at the time of surgery, eight were older (38.1%). A TAP, NTAP or conduit treatment did not show significant differences in long-term survival or freedom from reoperation. Early repair of TOF within the 1st year of life can be recommended, because mortality is lower than in patients treated at a higher age. There seems no significant difference in the reintervention rate between patients treated within the 1st year of life or later.