Outcomes of transcatheter pulmonary SAPIEN 3 valve implantation: an international registry.

BACKGROUND AND AIMS: Transcatheter pulmonary valve implantation (TPVI) is indicated to treat right-ventricular outflow tract (RVOT) dysfunction related to congenital heart disease (CHD). Outcomes of TPVI with the SAPIEN 3 valve that are insufficiently documented were investigated in the EUROPULMS3 registry of SAPIEN 3-TPVI. METHODS: Patient-related, procedural, and follow-up outcome data were retrospectively assessed in this observational cohort from 35 centres in 15 countries. RESULTS: Data for 840 consecutive patients treated in 2014-2021 at a median age of 29.2 (19.0-41.6) years were obtained. The most common diagnosis was conotruncal defect (70.5%), with a native or patched RVOT in 50.7% of all patients. Valve sizes were 20, 23, 26, and 29 mm in 0.4%, 25.5%, 32.1%, and 42.0% of patients, respectively. Valve implantation was successful in 98.5% [95% confidence interval (CI), 97.4%-99.2%] of patients. Median follow-up was 20.3 (7.1-38.4) months. Eight patients experienced infective endocarditis; 11 required pulmonary valve replacement, with a lower incidence for larger valves (P = .009), and four experienced pulmonary valve thrombosis, including one who died and three who recovered with anticoagulation. Cumulative incidences (95%CI) 1, 3, and 6 years after TPVI were as follows: infective endocarditis, 0.5% (0.0%-1.0%), 0.9% (0.2%-1.6%), and 3.8% (0.0%-8.4%); pulmonary valve replacement, 0.4% (0.0%-0.8%), 1.3% (0.2%-2.4%), and 8.0% (1.2%-14.8%); and pulmonary valve thrombosis, 0.4% (0.0%-0.9%), 0.7% (0.0%-1.3%), and 0.7% (0.0%-1.3%), respectively. CONCLUSIONS: Outcomes of SAPIEN 3 TPVI were favourable in patients with CHD, half of whom had native or patched RVOTs.

Post-infarction ventricular septal defect: percutaneous or surgical management in the UK national registry.

AIMS: Post-infarction ventricular septal defect (PIVSD) is a mechanical complication of acute myocardial infarction (AMI) with a poor prognosis. Surgical repair is the mainstay of treatment, although percutaneous closure is increasingly undertaken. METHODS AND RESUTS: Patients treated with surgical or percutaneous repair of PIVSD (2010-2021) were identified at 16 UK centres. Case note review was undertaken. The primary outcome was long-term mortality. Patient groups were allocated based upon initial management (percutaneous or surgical). Three-hundred sixty-two patients received 416 procedures (131 percutaneous, 231 surgery). 16.1% of percutaneous patients subsequently had surgery. 7.8% of surgical patients subsequently had percutaneous treatment. Times from AMI to treatment were similar [percutaneous 9 (6-14) vs. surgical 9 (4-22) days, P = 0.18]. Surgical patients were more likely to have cardiogenic shock (62.8% vs. 51.9%, P = 0.044). Percutaneous patients were substantially older [72 (64-77) vs. 67 (61-73) years, P < 0.001] and more likely to be discussed in a heart team setting. There was no difference in long-term mortality between patients (61.1% vs. 53.7%, P = 0.17). In-hospital mortality was lower in the surgical group (55.0% vs. 44.2%, P = 0.048) with no difference in mortality after hospital discharge (P = 0.65). Cardiogenic shock [adjusted hazard ratio (aHR) 1.97 (95% confidence interval 1.37-2.84), P < 0.001), percutaneous approach [aHR 1.44 (1.01-2.05), P = 0.042], and number of vessels with coronary artery disease [aHR 1.22 (1.01-1.47), P = 0.043] were independently associated with long-term mortality. CONCLUSION: Surgical and percutaneous repair are viable options for management of PIVSD. There was no difference in post-discharge long-term mortality between patients, although in-hospital mortality was lower for surgery.

Out-of-hours versus in-hours delivery of antenatally diagnosed transposition of the great arteries: outcomes from a United Kingdom Tertiary Centre.

OBJECTIVE: To study the impact of out-of-hours delivery on outcome for neonates with antenatally diagnosed transposition of the great arteries. SETTING: Tertiary paediatric cardiology centre (Yorkshire, United Kingdom), with co-located tertiary neonatal unit. PATIENTS: Neonates with antenatally diagnosed simple transposition of the great arteries delivered out-of-hours (Monday to Friday 17:00-08:00 and weekends) versus in-hours between 2015 and 2020. OUTCOME: The primary outcome was survival to hospital discharge. Secondary outcomes included neurological morbidity, length of stay, and time to balloon atrial septostomy. RESULTS: Of 51 neonates, 38 (75%) were delivered out-of-hours. All neonates born in the tertiary centre survived to discharge. Time to balloon atrial septostomy was slightly longer for out-of-hours deliveries compared to in-hours (median 130 versus 93 mins, p = 0.33). Neurological morbidity occurred for nine (24%) patients in the out-of-hours group and one (8%) in-hours (OR 3.72, 95% CI: 0.42-32.71, p = 0.24). Length of stay was also similar (18.5 versus 17.3 days, p = 0.59). Antenatal diagnosis of a restrictive atrial septum was associated with a lower initial pH (7.03 versus 7.13; CI: 0.03-0.17, p = 0.01), longer length of stay (22.6 versus 17.3 days; CI: 0.37-10.17, p = 0.04), and increased neurological morbidity (44% versus 14%; OR 4.80, CI 1.00-23.15, p = 0.05). A further three neonates were delivered in surrounding hospitals, with a mortality of 67% (versus 0 in tertiary centre; OR 172, CI 5-5371, p = 0.003). CONCLUSION: Neonates with antenatally diagnosed transposition of the great arteries have similar outcomes when delivered out-of-hours versus in-hours. Antenatal diagnosis of restrictive atrial septum is a significant predictor of worse outcomes. In our region, delivery outside the tertiary cardiac centre had a significantly higher risk of mortality.

Performance of a single-ventricle home-monitoring programme: survival and predictors of adverse outcome.

INTRODUCTION AND BACKGROUND: Mortality between stages 1 and 2 single-ventricle palliation is significant. Home-monitoring programmes are suggested to reduce mortality. Outcomes and risk factors for adverse outcomes for European programmes have not been published. AIMS: To evaluate the performance of a home-monitoring programme at a medium-sized United Kingdom centre with regards survival and compare performance with other home-monitoring programmes in the literature. METHODS: All fetal and postnatal diagnosis of a single ventricle were investigated with in-depth analysis of those undergoing stage 1 palliation and entered the home-monitoring programme between 2016 and 2020. The primary outcome was survival. Secondary outcomes included multiple parameters as potential predictors of death or adverse outcome. RESULTS: Of 217 fetal single-ventricle diagnoses during the period 2016-2020, 50.2% progressed to live birth, 35.4% to stage 1 and 29.5% to stage 2. Seventy-four patients (including 10 with postnatal diagnosis) entered the home-monitoring programme with six deaths making home-monitoring programme mortality 8.1%. Risk factors for death were the hybrid procedure as the only primary procedure (OR 33.0, p < 0.01), impaired cardiac function (OR 10.3, p < 0.025), Asian ethnicity (OR 9.3, p < 0.025), lower mean birth-weight (2.69 kg versus 3.31 kg, p < 0.01), and lower mean weight centiles during interstage follow-up (mean centiles of 3.1 versus 10.8, p < 0.01). CONCLUSION: Survival in the home-monitoring programme is comparable with other home-monitoring programmes in the literature. Hybrid procedure, cardiac dysfunction, sub-optimal weight gain, and Asian ethnicity were significant risk factors for death. Home-monitoring programmes should continue to raise awareness of these factors and seek solutions to mitigate adverse events. Future work to generalise home-monitoring programme and single-ventricle fetus to stage 2 outcomes in the United Kingdom will require multi-centre collaboration.

Surgical versus transcatheter palliation for insufficient pulmonary blood supply in infants with cyanotic CHD.

Infants with complex cyanotic CHD can become symptomatic from insufficient pulmonary blood supply following either ductal closure or due to outflow tract obstruction. Blalock-Taussig shunt mortality remains significant and recent studies have highlighted the advantages of using transcatheter alternatives. We present here our experience in changing our primary choice of palliation from the Blalock-Taussig shunt to transcatheter palliation with either a ductal stent or, if antegrade flow is present, a right ventricular outflow tract stent.This is a retrospective, single-unit cohort study. Eighty-seven infants underwent palliation for insufficient pulmonary blood flow at under 3 months of age between 2012 and 2019. On an intention-to-treat basis, 29 underwent insertion of a Blalock-Taussig shunt, 36 duct stents, and 22 right ventricular outflow tract stents at median ages of 15, 9, and 32 days, respectively, and median weights of 3.3, 3.1, and 3.1 kg, respectively. No primary Blalock-Taussig shunts have been performed in our institution since 2017.At 30-days there had been one death in each group (univariable p = 0.93) and deaths prior to repair totalled three in the shunt group, four in the ductal stent group, and two in the right ventricular outflow tract stent group (univariable p = 0.93). Reintervention on the pulmonary circuit prior to next stage of surgery was more frequent in those undergoing transcatheter intervention, reaching statistical significance by logrank (p = 0.012).In conclusion, within this work we provide further evidence of the safety and efficacy of transition from a primary surgical to primary transcatheter palliation pathway in infants with insufficient pulmonary blood supply.

Whole Exome Sequencing Reveals the Major Genetic Contributors to Nonsyndromic Tetralogy of Fallot.

RATIONALE: Familial recurrence studies provide strong evidence for a genetic component to the predisposition to sporadic, nonsyndromic Tetralogy of Fallot (TOF), the most common cyanotic congenital heart disease phenotype. Rare genetic variants have been identified as important contributors to the risk of congenital heart disease, but relatively small numbers of TOF cases have been studied to date. OBJECTIVE: We used whole exome sequencing to assess the prevalence of unique, deleterious variants in the largest cohort of nonsyndromic TOF patients reported to date. METHODS AND RESULTS: Eight hundred twenty-nine TOF patients underwent whole exome sequencing. The presence of unique, deleterious variants was determined; defined by their absence in the Genome Aggregation Database and a scaled combined annotation-dependent depletion score of ≥20. The clustering of variants in 2 genes, NOTCH1 and FLT4, surpassed thresholds for genome-wide significance (assigned as P<5×10-8) after correction for multiple comparisons. NOTCH1 was most frequently found to harbor unique, deleterious variants. Thirty-one changes were observed in 37 probands (4.5%; 95% CI, 3.2%-6.1%) and included 7 loss-of-function variants 22 missense variants and 2 in-frame indels. Sanger sequencing of the unaffected parents of 7 cases identified 5 de novo variants. Three NOTCH1 variants (p.G200R, p.C607Y, and p.N1875S) were subjected to functional evaluation, and 2 showed a reduction in Jagged1-induced NOTCH signaling. FLT4 variants were found in 2.4% (95% CI, 1.6%-3.8%) of TOF patients, with 21 patients harboring 22 unique, deleterious variants. The variants identified were distinct to those that cause the congenital lymphoedema syndrome Milroy disease. In addition to NOTCH1, FLT4 and the well-established TOF gene, TBX1, we identified potential association with variants in several other candidates, including RYR1, ZFPM1, CAMTA2, DLX6, and PCM1. CONCLUSIONS: The NOTCH1 locus is the most frequent site of genetic variants predisposing to nonsyndromic TOF, followed by FLT4. Together, variants in these genes are found in almost 7% of TOF patients.

Duct Stenting Versus Modified Blalock-Taussig Shunt in Neonates With Duct-Dependent Pulmonary Blood Flow: Associations With Clinical Outcomes in a Multicenter National Study.

BACKGROUND: Infants born with cardiac abnormalities causing dependence on the arterial duct for pulmonary blood flow are often palliated with a shunt usually between the subclavian artery and either pulmonary artery. A so-called modified Blalock-Taussig shunt allows progress through early life to an age and weight at which repair or further more stable palliation can be safely achieved. Modified Blalock-Taussig shunts continue to present concern for postprocedural instability and early mortality such that other alternatives continue to be explored. Duct stenting (DS) is emerging as one such alternative with potential for greater early stability and improved survival. METHODS: The purpose of this study was to compare postprocedural outcomes and survival to next-stage palliative or reparative surgery between patients undergoing a modified Blalock-Taussig shunt or a DS in infants with duct-dependent pulmonary blood flow. All patients undergoing cardiac surgery and congenital interventions in the United Kingdom are prospectively recruited to an externally validated national outcome audit. From this audit, participating UK centers identified infants <30 days of age undergoing either a Blalock-Taussig shunt or a DS for cardiac conditions with duct-dependent pulmonary blood flow between January 2012 and December 31, 2015. One hundred seventy-one patients underwent a modified Blalock-Taussig shunt, and in 83 patients, DS was attempted. Primary and secondary outcomes of survival and need for extracorporeal support were analyzed with multivariable logistic regression. Longer-term mortality before repair and reintervention were analyzed with Cox proportional hazards regression. All multivariable analyses accommodated a propensity score to balance patient characteristics between the groups. RESULTS: There was an early (to discharge) survival advantage for infants before next-stage surgery in the DS group (odds ratio, 4.24; 95% confidence interval, 1.37-13.14; P=0.012). There was also a difference in the need for postprocedural extracorporeal support in favor of the DS group (odds ratio, 0.22; 95% confidence interval, 0.05-1.05; P=0.058). Longer-term survival outcomes showed a reduced risk of death before repair in the DS group (hazard ratio, 0.25; 95% confidence interval, 0.07-0.85; P=0.026) but a slightly increased risk of reintervention (hazard ratio, 1.50; 95% confidence interval, 0.85-2.64; P=0.165). CONCLUSIONS: DS is emerging as a preferred alternative to a surgical shunt for neonatal palliation with evidence for greater postprocedural stability and improved patient survival to destination surgical treatment.

Permanent pacing post-Fontan is not associated with reduced long-term survival.

BACKGROUND: The Fontan procedure is the final stage of surgical palliation for a single-ventricle circulation. Significant complications are common including rhythm disturbance necessitating implantation of a permanent pacemaker. This has been widely considered a negative prognostic indicator. METHODS: This single-centre, retrospective case control study involved all patients who underwent the Fontan procedure at the Leeds Congenital Heart Unit between 1990 and 2015 and have had regular follow-up in Yorkshire and Humber, United Kingdom. 167 Fontan patients were identified of which 2 were excluded for having a pre-procedure pacemaker. Of the remainder, 23 patients required a pacemaker. Outcomes were survival, early and late complications, need for further intervention and oxygen saturation in long-term follow-up. RESULTS: There was no difference in survival (30-day survival pacemaker 92.6%, sinus rhythm 90.5%, p = 0.66, 1-year pacemaker 11.1%, sinus rhythm 10.1%, p = 1). The pacemaker group was more likely to have cerebral or renal complications in the first-year post-procedure (acute kidney injury: sinus rhythm 0.8%, pacemaker 19.1%, p = 0.002). No difference was observed in longer term complications including protein losing enteropathy (sinus rhythm 3.5%, pacemaker 0% p = 1). There was no difference in saturations between the two groups at follow-up. Paced patients were more likely to have required further intervention, with a higher incidence of cardiopulmonary bypass procedures (sinus rhythm 6.3%, pacemaker 35%, p < 0.001). CONCLUSIONS: Despite an increase in early complications and the need for further interventions, pacemaker requirement does not appear to affect long-term survival following the Fontan procedure.

Fetal single ventricle journey to first postnatal procedure: a multicentre UK cohort study.

OBJECTIVES: UK single ventricle (SV) palliation outcomes after first postnatal procedure (FPP) are well documented. However, survival determinants from fetal diagnosis to FPP are lacking. To better inform parental-fetal counselling, we examined factors favouring survival at two large UK centres. DESIGN: Retrospective multicentre cohort study. SETTING: Two UK congenital cardiac centres: Leeds and Birmingham. PATIENTS: SV fetal diagnoses from 2015 to 2021. MAIN OUTCOME MEASURES: Survival from fetal diagnosis with intention to treat (ITT) to birth and then FPP. Maternal, fetal and neonatal risk factors were assessed. RESULTS: There were 666 fetal SV diagnoses with 414 (62%) ITT. Of ITT, 381 (92%) were live births and 337 (81%) underwent FPP. Survival (ITT) to FPP was notably reduced for severe Ebstein's 14/22 (63.6%), unbalanced atrioventricular septal defect 32/45 (71%), indeterminate SV 3/4 (75%), mitral atresia 8/10 (80%) and hypoplastic left heart syndrome 127/156 (81.4%). Biventricular pathway was undertaken in five (1%). After multivariable adjustment, prenatal risk factors for mortality were increasing maternal age (OR 1.05, 95% CI 1.0 to 1.1), non-white ethnicity (OR 2.6, 95% CI 1.4 to 4.8), extracardiac anomaly (OR 6.34, 95% CI 1.8 to 22.7) and hydrops (OR 7.39, 95% CI 1.2 to 45.1). Postnatally, prematurity was significantly associated with mortality (OR 6.3, 95% CI 2.3 to 16.8). CONCLUSIONS: Around 20% of ITT fetuses diagnosed with SV will not reach FPP. Risk varies according to the cardiac lesion and is significantly influenced by the presence of an extracardiac anomaly, fetal hydrops, ethnicity, increasing maternal age and gestation at birth. These data highlight the need for fetal preprocedure data to be used in conjunction with procedural outcomes for fetal counselling.

Early clinical experience with a modified Amplatzer ductal occluder for transcatheter arterial duct occlusion in infants and small children.

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.

Early clinical experience with a modified amplatzer ductal occluder for transcatheter arterial duct occlusion in infants and small children.

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. © 147.

Post-myocardial infarction ventricular septal defect closure with Gore Cardioform atrial septal defect occluder to improve tissue interactions: case report.

Background: Gore Cardioform Atrial Septal Defect Occluder (GCA) is composed of a platinum-filled nitinol wire frame covered with expanded polytetrafluoroethylene (ePTFE). This makes the device highly occlusive and resistant to residual shunts through the device, as well as conforming well to the surrounding anatomy. In defects with poor rims to hold a device for closure or where one of the 'rims' is the free wall of the left ventricle, such as in a post-infarct apical ventricular septal defect (VSD), successful closure with standard nitinol mesh devices can be unachievable. Case summary: We present the occlusion of a post-myocardial infarction VSD with a GCA device in a critically ill patient at risk for closure failure and intravascular haemolysis with conventional nitinol mesh devices. The device conformed well to the anatomy even in the absence of an apical interventricular septum. Discussion: GCA can be used for intracardiac high-velocity shunts in selected cases where conventional devices are unsuitable due to haemolysis or poor tissue and poor rims. With growing experience using GCA for the closure of atrial septal defects, interventionists should consider the potential advantages of ePTFE material and apply them to other lesions where these could be beneficial for patients.

Clinical applications of contactless photoplethysmography for vital signs monitoring in pediatrics: A systematic review and meta-analysis.

Background: Contactless photoplethysmography (PPG) potentially affords the ability to obtain vital signs in pediatric populations without disturbing the child. Most validity studies have been conducted in laboratory settings or with healthy adult volunteers. This review aims to evaluate the current literature on contactless vital signs monitoring in pediatric populations and within a clinical setting. Methods: OVID, Webofscience, Cochrane library, and clinicaltrials.org were systematically searched by two authors for research studies which used contactless PPG to assess vital signs in children and within a clinical setting. Results: Fifteen studies were included with a total of 170 individuals. Ten studies were included in a meta-analysis for neonatal heart rate (HR), which demonstrated a pooled mean bias of -0.25 (95% limits of agreement (LOA), -1.83 to 1.32). Four studies assessed respiratory rate (RR) in neonates, and meta-analysis demonstrated a pooled mean bias of 0.65 (95% LOA, -3.08 to 4.37). All studies were small, and there were variations in the methods used and risk of bias. Conclusion: Contactless PPG is a promising tool for vital signs monitoring in children and accurately measures neonatal HR and RR. Further research is needed to assess children of different age groups, the effects of skin type variation, and the addition of other vital signs.

Successful treatment of refractory chylothorax with MEK inhibitor trametinib in a child with Noonan syndrome: case report.

Background: Refractory chylous effusions due to lymphatic dysplasia related to Noonan syndrome cause significant morbidity and mortality due to protein and immunoglobulin losses. Very few cases have been published reporting successful treatment of patients with trametinib where all conventional treatments had failed. Case summary: We present a girl with Noonan syndrome and hypertrophic cardiomyopathy who presented with life-threatening refractory chylothorax where all conventional treatment options failed. She was successfully treated with mitogen-activated extracellular signal-regulated kinase inhibitor trametinib. Discussion: MEK inhibition with trametinib is emerging as a possible salvage treatment option for a subset of patients with Noonan syndrome and severe pulmonary lymphangiectasia. More experience is required to establish optimal treatment regimen and long-term outcomes.

Cardiac output monitoring in children: a review.

Cardiac output monitoring enables physiology-directed management of critically ill children and aids in the early detection of clinical deterioration. Multiple invasive techniques have been developed and have demonstrated ability to improve clinical outcomes. However, all require invasive arterial or venous catheters, with associated risks of infection, thrombosis and vascular injury. Non-invasive monitoring of cardiac output and fluid responsiveness in infants and children is an active area of interest and several proven techniques are available. Novel non-invasive cardiac output monitors offer a promising alternative to echocardiography and have proven their ability to influence clinical practice. Assessment of perfusion remains a challenge; however, technologies such as near-infrared spectroscopy and photoplethysmography may prove valuable clinical adjuncts in the future.

Educational attainment of children with congenital Heart disease in the United Kingdom.

BACKGROUND: Educational attainment in children with congenital heart disease (CHD) within the UK has not been reported, despite the possibility of school absences and disease-specific factors creating educational barriers. METHODS: Children were prospectively recruited to the Born in Bradford birth cohort between March 2007 and December 2010. Diagnoses of CHD were identified through linkage to the congenital anomaly register and independently verified by clinicians. Multivariable regression accounted for relevant confounders. Our primary outcome was the odds of 'below expected' attainment in Maths, Reading and Writing at ages 4-11 years. RESULTS: Educational records of 139 children with non-genetic CHD were compared to 11 188 age-matched children with no major congenital anomaly. Children with CHD had significantly higher odds of 'below expected' attainment in Maths at age 4-5 years (Odds Ratio 1.64, 95% CI 1.07-2.52), age 6-7 (OR 2.03, 95% CI 1.32-3.12), and age 10-11 (OR 2.28, 95% CI 1.01-5.14). Odds worsened with age, with similar results for Reading and Writing. The odds of receiving special educational needs support reduced with age for children with CHD relative to controls (age 4-5: OR 4.84 (2.06-11.40); age 6-7: OR 3.65 (2.41-5.53); age 10-11: OR 2.73 (1.84-4.06)). Attainment was similar for children with and without exposure to cardio-pulmonary bypass. Lower attainment was strongly associated with the number of pre-school hospital admissions. CONCLUSIONS: Children with CHD have lower educational attainment compared to their peers. Deficits are evident from school entry and increase throughout primary school.

Transcatheter closure of persistent ductus arteriosus in adults.

OBJECTIVE: To describe the technical aspects and outcome of duct occlusion in adults over a 12-year period. METHODS: A single center review of all transcatheter duct closures performed between 2000 and 2012. RESULTS: Of 518 transcatheter duct closures performed, 31 patients were over the age of 16 at the time of procedure (6%). In 10 of the 31 cases, it was not possible to cross the duct from the pulmonary artery. In 4 of those, the duct was small enough to be closed with coils delivered from the aorta (although 1 required a second procedure for a residual shunt). In the remaining 6 cases, it was necessary to cross the duct from the aorta and create an arterio-venous "circuit" using a snare to deliver an Amplatzer device from the femoral vein. In none of the 487 children who underwent transcatheter duct closure during the same time period was it necessary to deliver the device using an arterio-venous wire circuit. The increased complexity of the procedure in adults compared with children was further reflected by longer procedure times (median of 37 minutes in adults vs. 24 minutes in children [P < 0.01]) and longer fluoroscopy times (median of 8.4 minutes in adults vs. 4.3 minutes in children [P < 0.025]). There were no major complications. CONCLUSIONS: Closure of the arterial duct in adults is safe and effective but ductal anatomy may differ from that seen in childhood, making transcatheter closure technically much more demanding than in children.