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Objective The ‘hybrid procedure’, consisting of surgical banding of the pulmonary arteries with intraoperative stenting of the arterial duct, was developed as primary palliation in hypoplastic left heart syndrome (HLHS), avoiding the risks of cardiopulmonary bypass. In many centres, it is reserved for low birth weight, premature or unstable neonates; however, its role in such high risk cases of HLHS has yet to be defined. Methods The preoperative condition of all patients with HLHS who underwent either the hybrid or the Norwood procedure for HLHS between 2005–2011 was analysed retrospectively, using a modified comprehensive Aristotle score. We then compared operative, interstage and 1 year mortalities between the groups after Aristotle adjustment via Cox proportional hazards analyses. Results Of 138 patients with HLHS, 27 had hybrid and 111 Norwood procedures. The hybrid group had significantly higher Aristotle scores (mean 4.1 vs 1.8; p<0.001); however, there was no significant difference in mortality at any stage. At 1 year, the overall unadjusted survival among Norwood and hybrid patients was 58.6% and 51.9%, respectively, yielding an Aristotle adjusted hazard ratio for mortality among hybrid patients of 1.09 (95% CI 0.56 to 2.11, p=0.80). Conclusions Applying a hybrid approach to high risk patients with HLHS produces a comparable early and interstage mortality risk to lower risk patients undergoing the Norwood procedure. Prospective studies are needed to establish whether the hybrid procedure is a viable alternative to the Norwood procedure in all HLHS patients in terms of both mortality and long term morbidity.

Background The Technical Performance Score (TPS) developed by Boston Children’s Hospital showed surgical outcomes correlate with adequacy of technical repair when implemented on pre-discharge echocardiograms. We applied this scoring system to intraoperative imaging in a tertiary UK congenital heart surgical centre. Methods After a period of training, intraoperative TPS (epicardial and/or transesophageal echocardiography) was instituted. TPS was used to inform intraoperative discussions and recorded on a custom-made database using the previously published scoring system. After a year, we reviewed the feasibility, results and relationship between the TPS and mortality, extubation time and length of stay. Results From 01 September 2015 to 04 July 2016, there were 272 TPS procedures in 251 operations with 208 TPS recorded. Seven patients had surgery with no documented TPS, three had operations with no current TPS score template available. Patients left the operating theatre with TPS optimal in 156 (75%), adequate 34 (16%) and inadequate 18 (9%). Of those with an optimal score on leaving theatre, ten had more than one period of cardiopulmonary bypass. All four deaths <30 days after surgery (1.9%) had optimal TPS. There was a statistically significant difference in extubation times in the RACHS category 4 patients (3 days vs 5 days, P < 0.05) and in PICU and total length of stay in the RACHS category three patients (2 and 8 days vs 12.5 and 21.5 days respectively) if leaving theatre with an inadequate result. Conclusions Application of intraoperative TPS is feasible and provides a way of objectively recording intraoperative imaging assessment of surgery. An ‘inadequate’ TPS did not predict mortality but correlated with a longer ventilation time and longer length of stay compared to those with optimal or adequate scores.

Background and objectivesIn personalised external aortic root support (PEARS), a custom-made, macroporous mesh is used to stabilise a dilated aortic root and prevent dissection, primarily in patients with genetically driven aortopathies. Data are needed on the safety and postoperative incidence of aortic events.MethodsWe present a multicentre cohort study evaluating the first 200 consecutive patients (median age 33 years) undergoing surgery with an intention to perform PEARS for aortic root dilatation in 23 centres between 2004 and 2019. Perioperative outcomes were collected prospectively while clinical follow-up was retrieved retrospectively. Median follow-up was 21.2 months.ResultsThe main indication was Marfan syndrome (73.5%) and the most frequent concomitant procedure was mitral valve repair (10%). An intervention for myocardial ischaemia or coronary injury was needed in 11 patients, 1 case resulting in perioperative death. No ascending aortic dissections were observed in 596 documented postoperative patient years. Late reoperation was performed in 3 patients for operator failure to achieve complete mesh coverage. Among patients with at least mild aortic regurgitation (AR) preoperatively, 68% had no or trivial AR at follow-up.ConclusionsThis study represents the clinical history of the first 200 patients to undergo PEARS. To date, aortic dissection has not been observed in the restrained part of the aorta, yet long-term follow-up is needed to confirm the potential of PEARS to prevent dissection. While operative mortality is low, the reported coronary complications reflect the learning curve of aortic root surgery in patients with connective tissue disease. PEARS may stabilise or reduce aortic regurgitation.

ObjectiveNorwood and hybrid procedure are two options available for initial palliation of patients with hypoplastic left heart syndrome (HLHS). Our study aimed to assess potential differences in right ventricular (RV) function and pulmonary artery dimensions using cardiac magnetic resonance (CMR) in survivors with HLHS.Methods42 Norwood (mean age 2.4±0.8) and 44 hybrid (mean age 2.0±1.0 years) patients were evaluated by CMR after stage II palliation prior to planned Fontan completion. Initial stage I Norwood procedure was performed using a modified Blalock-Taussig shunt, while the hybrid procedure consisted of bilateral pulmonary artery banding and arterial duct stenting. Need for reinterventions and subsequent outcomes were also assessed.ResultsNorwood patients had larger RV end-diastolic dimensions (91±23 vs 80±31 mL/m2, p=0.004) and lower heart rate (90±15 vs 102±13, p<0.001) than hybrid patients. Both Norwood and hybrid patients showed preserved global RV pump function (59±9 vs 59%±10%, p=0.91), while RV strain, strain rate and intraventricular synchrony were superior in the Norwood group. Pulmonary artery size was reduced (lower lobe index 135±74 vs 161±62 mm2/m2, p=0.02), and reintervention rate was significantly higher in the hybrid group whereas subsequent outcome did not differ significantly (p=0.24).ConclusionsNorwood and hybrid strategy were associated with equivalent and preserved global RV pump function while development of the pulmonary arteries and reintervention rate were superior using the Norwood approach. Impaired RV myocardial deformation as a potential marker of early RV dysfunction in the hybrid group may have a negative long-term impact in this population.

Conventional surgical approaches for aortic root aneurysms, including valved grafts and valve-sparing techniques, present inherent limitations such as the requirement for anticoagulation and the potential for late reoperation. Personalized External Aortic Root Support (PEARS), utilizing the ExoVasc® implant, represents a novel approach that aims to overcome these limitations. This report presents the initial clinical experience with the ExoVasc® PEARS implant in the Americas, encompassing 10 patients (six males, age range 30 - 52 years, mean age 37.8 years) diagnosed with aortic root aneurysms. Indications for PEARS included Marfan syndrome (eight patients, including one reoperation), bicuspid aortic valve (two patients, including one with anomalous coronary artery), and associated valvular dysfunction. Cardiopulmonary bypass was utilized in four cases. No major adverse postoperative events were observed. Postoperative recovery was generally uneventful, with minor complications, including pericarditis and atrial fibrillation, successfully managed with medical therapy. Aortic dimensions remained stable at 30-day and one-year follow-ups. This initial experience demonstrates the feasibility, safety, and efficacy of the PEARS technique for the treatment of aortic root aneurysms. Potential advantages over traditional approaches include the possibility for off-pump procedures, reduced risk of aortic valve dysfunction, shorter hospital stays, and elimination of the need for long-term anticoagulation therapy. Further investigation is warranted to evaluate the long-term durability and clinical outcomes of this innovative approach.

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

IntroductionSystemic hypertension manifesting after surgical coarctation repair is a known long term sequela and affects about two thirds of subjects. Previous studies suggest the risk of developing hypertension is reduced if the coarctation repair is performed at a younger age with the optimal time for repair between the first year of life up to 9 years of age.ObjectiveUsing our experience of patients operated on since the opening of the new Evelina London Children’s Hospital in 2005, we sought to further clarify the optimal age at which coarctation repair is most effective in reducing the risk of systemic hypertension. Risk of developing re-coarctation related to the age of primary surgical repair was also examined.MethodsRetrospective analysis of patients with coarctation undergoing primary surgical repair from October 2005 to October 2014, identified from the institutional database (Heartsuite, Systeria, Glasgow, UK). Included patients were all <16 years age. Patients with complex congenital heart disease and no follow up data were excluded. Owing to inconsistent documentation of blood pressure at follow up (the documentation of blood pressure was available on only 195 (65%) out of 299 follow up episodes) a surrogate marker of hypertension, regular antihypertensive medication, was used. The two primary outcome events at follow up were: 1) age when commenced on regular anti-hypertensive medication and 2) the presence of re-coarctation of the aorta.ResultsEighty seven patients were included: 58 neonates (<4 weeks), 19 infants (4 weeks to 1 year), 10 children (over 1 year). Median time of follow up was 700 days. The proportion of those requiring long term anti-hypertensives rose the older the child was at the time of first coarctation surgery, being 6.9% in the neonatal group, 26.3% as an infant and 40% if over the age of 1 year. These differences were statistically significant (Chi2 9.83, P = 0.0073). The relative risk of requiring long term anti-hypertension medication if repaired during the neonatal period versus during infancy was 0.26 (95% CI 0.078 to 0.88, P = 00.03), 0.65 (95% CI 0.23 to 1.92, P = 00.44) for repair during infancy versus over 1 year of age, and 0.17 (95% CI 0.051 to 0.58, P = 00.0045) for repair during the neonatal period versus being over 1 year of age. There was no significant difference in the proportion of those developing re-coarctation depending on the age they were first operated, though the prevalence was lowest in the infant age group, 5.3%, versus 22.4% in the neonatal group and 20% in those over 1 year of age.ConclusionWe have demonstrated that the risk of being on long term anti-hypertensive medication if operated on during neonatal period was 74% less compared to being operated on during infancy and 83% less if operated in the neonatal period compared to surgery after the first year of life. Therefore primary surgical repair of coarctation of the aorta to reduce the future risk of hypertension is optimal in the neonatal period. We were unable to demonstrate a significant difference in the prevalence of re-coarctation depending on age of first coarctation repair.

AimsIn newborns with pulmonary atresia with ventricular septal defect (PA/VSD), the pulmonary arteries (PAs) may be either hypoplastic or absent, with or without highly variable aortopulmonary collaterals. Comprehensive imaging of the native pulmonary blood supply is important to inform initial management. We sought to describe the accuracy, cumulative radiation dose and anaesthetic time associated with multi-modality imaging in newly diagnosed patients with PA/VSD prior to their first intervention.MethodsThe records of all patients diagnosed with PA/VSD between 2004 and 2014 were reviewed retrospectively, excluding all patients over 100 days old at the time of diagnosis. We determined the accuracy and benefits (if any) of serial investigations up to the time of the first intervention, comparing the results to the anatomical findings at operation. We then calculated the cumulative radiation dose and anaesthetic time per patient.Results30 patients were identified, of which 13 were excluded (7 had no further imaging and 6 were >100 days old at diagnosis). In total, there were 13 catheters and 4 CT scans performed in the whole group before intervention. All were within 100 days of life. Aside from one patient with 2 MRI scans, no investigation was repeated. Echocardiography correctly identified PAs in 12/17 patients (71%), MRI in 9/13 patients (69%), CT in 1/4 patients (25%), and in all patients who underwent catheterisation (13/13, 100%). In the 4 patients who underwent cross-sectional imaging only (3 MRI, 1 CT), PAs were also correctly identified.The mean radiation dose during catheterisation was 119mGy*cm2 (47–231mGy*cm2), and for CT was 92mGy*cm2 (66–123mGy*cm2). 3 patients underwent both catheterisation and CT, with a mean cumulative dose of 297mGy*cm2 (191–461mGy*cm2). 3 underwent MRI only with no ionising radiation. The mean total anaesthetic time before intervention was 111 min (33–185 min). In the one child who had CT only, no anaesthetic was used.The mean age of those undergoing primary catheterisation was 3 days (1 – 6 days, n = 3). In those undergoing primary MRI or CT, the mean age at subsequent catheterisation was 35 days (8–86 days, n = 10, P = 00.09); however, there was no difference in the mean radiation dose or anaesthetic time of these procedures.Over the ten year period of our study, the maximum cumulative radiation dose in a single patient was 8022mGy/cm2 at the age of 6 years (via 5 catheters), and 1263 min of anaesthetic time at 5 years (via 5 catheters, 1 CT and 4 MRIs).ConclusionsEchocardiography may not identify native PAs in all neonates and infants with PA/VSD. Catheterisation is highly accurate, but also carries the highest risk. Cross-sectional imaging alone may be appropriate in some cases; in others, it may allow for delayed catheterisation, but may not reduce the radiation dose or anaesthetic time.The cumulative effects of repeated diagnostic and interventional procedures can lead to large radiation doses and anaesthetic times, both before and after primary intervention. These risks are an important consideration when planning management in this complex patient group.

A comprehensive understanding of the native pulmonary blood supply is crucial in newborns with pulmonary atresia with ventricular septal defect and aortopulmonary collaterals (PA/VSD/MAPCA). We sought to describe the accuracy in terms of identifying native pulmonary arteries, radiation dose and anaesthetic time associated with multi-modality imaging in these patients, prior to their first therapeutic intervention. Furthermore, we wanted to evaluate the cumulative radiations dose and anaesthetic time over the study period. Patients with PA/VSD/MAPCA diagnosed at < 100 days between 2004 and 2014 were identified. Cumulative radiation dose and anaesthetic times were calculated, with imaging results compared with intraoperative findings. We then calculated the cumulative risks to date for all surviving children. Of 19 eligible patients, 2 had echocardiography only prior to first intervention. The remaining 17 patients underwent 13 MRIs, 4 CT scans and 13 cardiac catheterization procedures. The mean radiation dose was 169 mGy cm 2 (47–461 mGy cm 2 ), and mean anaesthetic time was 111 min (33–185 min). 3 children had MRI only with no radiation exposure, and one child had CT only with no anaesthetic. Early cross-sectional imaging allowed for delayed catheterisation, but without significantly reducing radiation burden or anaesthetic time. The maximum cumulative radiation dose was 8022 mGy cm 2 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.