Pulmonary artery banding: still a role for staged bi-ventricular repair of intracardiac shunts?

OBJECTIVES: Although pulmonary artery banding remains a useful palliation in bi-ventricular shunting lesions, single-stage repair holds several advantages. We investigate outcomes of the former approach in high-risk patients. METHODS: Retrospective cohort study including all pulmonary artery banding procedures over 9 years, excluding single ventricle physiology and left ventricular training. RESULTS: Banding was performed in 125 patients at a median age of 41 days (2-294) and weight of 3.4 kg (1.8-7.32). Staged repair was undertaken for significant co-morbidity in 81 (64.8%) and anatomical complexity in 44 (35.2%). The median hospital stay was 14 days (interquartile range 8-33.5) and 14 patients (11.2%) required anatomical repair before discharge. Nine patients died during the initial admission (hospital mortality 7.2 %) and five following discharge (inter-stage mortality 4.8%). Of 105 banded patients who survived, 19 (18.1%) needed inter-stage re-admission and 18 (14.4%) required unplanned re-intervention. Full repair was performed in 93 (74.4%) at a median age of 13 months (3.1-49.9) and weight of 8.5 kg (3.08-16.8). Prior banding, 54% were below the 0.4th weight centile, but only 28% remained so at repair. Post-repair, 5/93 (5.4%) developed heart block requiring permanent pacemaker, and 11/93 (11.8%) required unplanned re-intervention. The post-repair mortality (including repairs during the initial admission) was 6/93 (6.5%), with overall mortality of the staged approach 13.6% (17/125). CONCLUSIONS: In a cohort with a high incidence of co-morbidity, pulmonary artery banding is associated with a significant risk of re-intervention and mortality. Weight gain improves after banding, but heart block, re-intervention, and mortality remain frequent following repair.

Feasibility of non-invasive neuro-monitoring during extracorporeal membrane oxygenation in children.

INTRODUCTION: Detection of neurological complications during extracorporeal membrane oxygenation (ECMO) may be enhanced with non-invasive neuro-monitoring. We investigated the feasibility of non-invasive neuro-monitoring in a paediatric intensive care (PIC) setting. METHODS: In a single centre, prospective cohort study we assessed feasibility of recruitment, and neuro-monitoring via somatosensory evoked potentials (SSEP), electroencephalography (EEG) and near infrared spectroscopy (NIRS) during venoarterial (VA) ECMO in paediatric patients (0-15 years). Measures were obtained within 24h of cannulation, during an intermediate period, and finally at decannulation or echo stress testing. SSEP/EEG/NIRS measures were correlated with neuro-radiology findings, and clinical outcome assessed via the Pediatric cerebral performance category (PCPC) scale 30 days post ECMO cannulation. RESULTS: We recruited 14/20 (70%) eligible patients (median age: 9 months; IQR:4-54, 57% male) over an 18-month period, resulting in a total of 42 possible SSEP/EEG/NIRS measurements. Of these, 32/42 (76%) were completed. Missed recordings were due to lack of access/consent within 24 h of cannulation (5/42, 12%) or PIC death/discharge (5/42, 12%). In each patient, the majority of SSEP (8/14, 57%), EEG (8/14, 57%) and NIRS (11/14, 79%) test results were within normal limits. All patients with abnormal neuroradiology (4/10, 40%), and 6/7 (86%) with poor outcome (PCPC ≥4) developed indirect SSEP, EEG or NIRS measures of neurological complications prior to decannulation. No study-related adverse events or neuro-monitoring data interpreting issues were experienced. CONCLUSION: Non-invasive neuro-monitoring (SSEP/EEG/NIRS) during ECMO is feasible and may provide early indication of neurological complications in this high-risk population.

Anomalous coronary artery in Tetralogy of Fallot-Feasibility of right ventricular outflow tract stenting as initial palliation.

This study addresses the outcome of right ventricle outflow tract (RVOT) stenting in Tetralogy of Fallot (ToF) with anomalous coronaries crossing the RVOT. RVOT stenting in ToF patients has emerged as an alternative to Blalock Taussig shunting. This is a single center study of patients who underwent RVOT stenting for symptomatic ToF at Birmingham Children's Hospital between 2005 and 2020. A total of 122 patients underwent RVOT stenting as initial palliation over a 15-year period, 10 patients had anomalous coronaries crossing the RVOT (study group) and 112 not (comparative group). Median age of the study group was 72.5 days (interquartile range [IQR]: 28-103) with a weight of 4.7 kg (IQR: 3.5-4.9). No significant differences were found between the two groups regarding the patients' weights and ages, procedure and screening times, or hospital stay. Four had valve sparing stenting. Oxygen saturations increased from a median of 75.5% (IQR: 70-82) to 94.5% (IQR: 90-95), p < 0.002. Postprocedure median hospital stay was 3 days (IQR: 2-6). Six patients underwent interstage catheterization reintervention and one needed early surgical palliation due to stent suboptimal position. Complete repair could be delayed for a median of 11.1 months (IQR: 5.6-19.2). At surgical repair, the patients had a median age of 12.3 months (IQR: 7.5-25.6) and weight of 7.7 kg (IQR: 6.8-10.8). There were no deaths. RVOT stenting in ToF with anomalous coronaries is safe and effective. Dilatable stents should be used when two-stage delayed conduit repair is the default approach.

Validation of the 'paediatric extracorporeal membrane oxygenation prediction' model in a UK extracorporeal membrane oxygenation centre.

INTRODUCTION: There is a need for a universal risk-adjustment model that may be used regardless of the indication and nature of neonatal or paediatric extracorporeal membrane oxygenation support. The 'paediatric extracorporeal membrane oxygenation prediction' model appeared to be a promising candidate but required external validation. METHODS: We performed a validation study using institutional database of extracorporeal membrane oxygenation patients (2008-2019). We used the published paediatric extracorporeal membrane oxygenation prediction score calculator to derive estimated mortality based on the model in this cohort of patients in our institutional database. We used standardized mortality ratio, area under the receiver operating characteristic curve and Hosmer-Lemeshow goodness-of-fit test in 10 deciles to assess model performance. RESULTS: We analysed 154 extracorporeal membrane oxygenation episodes in 150 patients. About 53% of the patients were full term (age ⩽30 days and gestation at birth ⩾37 weeks) neonates. The commonest category of extracorporeal membrane oxygenation support was cardiac (42%). The overall in-paediatric intensive care unit mortality was 37% (57/154) and the in-hospital mortality was 42% (64/154). Distribution of estimated mortality risk was similar to the derivation study. The calculated standardized mortality ratio was 0.81 based on the paediatric extracorporeal membrane oxygenation prediction model of risk-adjustment. The area under the receiver operating characteristic curve was 0.55 (0.45-0.64) and Hosmer-Lemeshow-test p value <0.001 was unable to support goodness-of-fit. CONCLUSION: This small single-centre study with a small number of events was unable to validate the paediatric extracorporeal membrane oxygenation prediction-model of risk-adjustment. Although this remains the most promising of all the available models, further validation in larger data sets and/or refinement may be required before widespread use.

Cardioplegia practice in paediatric cardiac surgery: a UK & Ireland survey.

INTRODUCTION: Many techniques are available for cardioplegic arrest in children, but there is a lack of late phase clinical trials to guide practice. We surveyed paediatric cardiac surgeons and perfusionists to establish current practice and willingness to change within a clinical trial. METHODS: An online survey was sent to all consultant paediatric cardiac surgeons and chief perfusionists in paediatric centres in the UK and Ireland. Information was sought on cardioplegia type, composition, temperature, topical cooling, dosing for induction and maintenance, interval between doses, whether practice changed with patient age or complexity and whether respondents would be willing and able to use different cardioplegia solutions within a randomised trial. RESULTS: Responses were obtained from 32 (78.0%) surgeons and 12 (100%) perfusionists. Twenty-seven (84.4%) surgeons use blood cardioplegia in infants, with St. Thomas' Harefield preparation the most popular (19, 59.4%), used routinely in eight (66.7%) centres. Twenty-two (68.8%) administer at 4-6°C, 18 (56.3%) use topical cooling, 18 (56.3%) give 30 ml/kg induction and 15 ml/kg maintenance, with 23 (71.9%) re-dosing every 20-25 minutes. Thirty (93.8%) surgeons were open to randomising patients in a trial, with del Nido (29, 90.6%) the most popular. CONCLUSIONS: This survey demonstrates heterogeneity in cardioplegia practice. Whilst most surgeons use blood cardioplegia, there is variation in type, temperature, topical cooling, dosing and intervals. Combined with a lack of evidence from late phase trials, our findings support the presence of clinical equipoise. Surgeons are willing to change practice, suggesting that a pragmatic, multi-centre, randomised, controlled trial of cardioplegia in children is feasible.

Retrospective Cohort Study of Additional Procedures and Transplant-Free Survival for Patients With Functionally Single Ventricle Disease Undergoing Staged Palliation in England and Wales.

BACKGROUND: Reinterventions may influence the outcomes of children with functionally single-ventricle (f-SV) congenital heart disease. METHODS AND RESULTS: We undertook a retrospective cohort study of children starting treatment for f-SV between 2000 and 2018 in England, using the national procedure registry. Patients were categorized based on whether they survived free of transplant beyond 1 year of age. Among patients who had transplant-free survival beyond 1 year of age, we explored the relationship between reinterventions in infancy and the outcomes of survival and Fontan completion, adjusting for complexity. Of 3307 patients with f-SV, 909 (27.5%), had no follow-up beyond 1 year of age, among whom 323 (35.3%) had ≥1 reinterventions in infancy. A total of 2398 (72.5%) patients with f-SV had transplant-free survival beyond 1 year of age, among whom 756 (31.5%) had ≥1 reinterventions in infancy. The 5-year transplant-free survival and cumulative incidence of Fontan, among those who survived infancy, were 93.4% (95% CI, 92.4%-94.4%) and 79.3% (95% CI, 77.4%-81.2%), respectively. Both survival and Fontan completion were similar for those with a single reintervention and those who had no reinterventions. Patients who had >1 additional surgery (adjusted hazard ratio, 3.93 [95% CI, 1.87-8.27] P<0.001) had higher adjusted risk of mortality. Patients who had >1 additional interventional catheter (adjusted subdistribution hazard ratio, 0.71 [95% CI, 0.52-0.96] P=0.03) had a lower likelihood of achieving Fontan. CONCLUSIONS: Among children with f-SV, the occurrence of >1 reintervention in the first year of life, especially surgical reinterventions, was associated with poorer prognosis later in childhood.

Bilateral remote ischemic conditioning in children: A two-center, double-blind, randomized controlled trial in young children undergoing cardiac surgery.

Objective: The study objective was to determine whether adequately delivered bilateral remote ischemic preconditioning is cardioprotective in young children undergoing surgery for 2 common congenital heart defects with or without cyanosis. Methods: We performed a prospective, double-blind, randomized controlled trial at 2 centers in the United Kingdom. Children aged 3 to 36 months undergoing tetralogy of Fallot repair or ventricular septal defect closure were randomized 1:1 to receive bilateral preconditioning or sham intervention. Participants were followed up until hospital discharge or 30 days. The primary outcome was area under the curve for high-sensitivity troponin-T in the first 24 hours after surgery, analyzed by intention-to-treat. Right atrial biopsies were obtained in selected participants. Results: Between October 2016 and December 2020, 120 eligible children were randomized to receive bilateral preconditioning (n = 60) or sham intervention (n = 60). The primary outcome, area under the curve for high-sensitivity troponin-T, was higher in the preconditioning group (mean: 70.0 ± 50.9 µg/L/h, n = 56) than in controls (mean: 55.6 ± 30.1 µg/L/h, n = 58) (mean difference, 13.2 µg/L/h; 95% CI, 0.5-25.8; P = .04). Subgroup analyses did not show a differential treatment effect by oxygen saturations (pinteraction = .25), but there was evidence of a differential effect by underlying defect (pinteraction = .04). Secondary outcomes and myocardial metabolism, quantified in atrial biopsies, were not different between randomized groups. Conclusions: Bilateral remote ischemic preconditioning does not attenuate myocardial injury in children undergoing surgical repair for congenital heart defects, and there was evidence of potential harm in unstented tetralogy of Fallot. The routine use of remote ischemic preconditioning cannot be recommended for myocardial protection during pediatric cardiac surgery.

Outcomes of the arterial switch for transposition during infancy using a standardized approach over 30 years.

OBJECTIVES: The aim of this study was to describe the early and late outcomes of the arterial switch for transposition. METHODS: A single-centre retrospective cohort study was conducted to assess the early and late outcomes of arterial switch performed during infancy using a standardized institutional approach between 1988 and 2018, compared by morphological groups. RESULTS: A total of 749 consecutive patients undergoing arterial switch during infancy were included, 464 (61.9%) with intact septum, 163 (21.8%) with isolated ventricular septal defect and 122 (16.3%) with complex transposition with associated lesions, including 67 (8.9%) with Taussig-Bing anomaly. There were 34 early deaths [4.5%, 95% confidence interval (CI) 3.1-6.1] with only 10 (2.6%) early deaths since 2000. Complex morphology (odds ratio 11.44, 95% CI 4.76-27.43) and intramural coronary artery (odds ratio 5.17, 95% CI 1.61-15.91) were identified as the most important risk factors for 90-day mortality. Overall survival was 92.7% (95% CI 90.8-94.6) at 5 years and 91.9% (95% CI 89.9-94.1) at 20 years; in hospital survivors, there were 15 (2.1%) late deaths during a median follow-up of 13.7 years. Cumulative incidence of surgical or catheter reintervention was 16.0% (95% CI 14.5-17.5) at 5 years and 22.7% (95% CI 21.0-24.0) at 20 years; early and late reinterventions were more common in the complex group, with no difference between the other groups. CONCLUSIONS: Using a standardized approach, the arterial switch can be performed with low early mortality, moderate rates of reintervention and excellent long-term survival. Concomitant lesions were the most important risk factor for early death and were associated with increased risk of late reintervention.

Perioperative electroencephalography in cardiac surgery with hypothermic circulatory arrest: a narrative review.

OBJECTIVES: Cardiac surgery with hypothermic circulatory arrest (HCA) is associated with neurological morbidity of variable severity and electroencephalography (EEG) is a sensitive proxy measure of brain injury. We conducted a narrative review of the literature to evaluate the role of perioperative EEG monitoring in cardiac surgery involving HCA. METHODS: Medline, Embase, Central and LILACS databases were searched to identify studies utilizing perioperative EEG during surgery with HCA in all age groups, published since 1985 in any language. We aimed to compare EEG use with no use but due to the lack of comparative studies, we performed a narrative review of its utility. Two or more reviewers independently screened studies for eligibility and extracted data. RESULTS: Fourty single-centre studies with a total of 3287 patients undergoing surgery were identified. Most were observational cohort studies (34, 85%) with only 1 directly comparing EEG use with no use. EEG continuity (18, 45%), seizures (15, 38%) and electrocerebral inactivity prior to circulatory arrest (15, 38%) were used to detect, monitor, prevent and prognose neurological injury. Neurological dysfunction was reported in almost all studies and occurred in 0-21% of patients. However, the heterogeneity of reported clinical and EEG outcome measures prevented meta-analysis. CONCLUSIONS: EEG is used to detect cortical ischaemia and seizures and predict neurological abnormalities and may guide intraoperative cerebral protection. However, there is a lack of comparative data demonstrating the benefit of perioperative EEG monitoring. Use of a standardized methodology for performing EEG and reporting outcome metrics would facilitate the conduct of high-quality clinical trials.

Research priorities in children and adults with congenital heart disease: a James Lind Alliance Priority Setting Partnership.

OBJECTIVE: To bring together patients, parents, charities and clinicians in a Priority Setting Partnership to establish national clinical priorities for research in children and adults with congenital heart disease. METHODS: The established James Lind Alliance methodology was used to identify and prioritise research on the management of congenital heart disease, focusing on diagnosis, treatment and outcomes. An initial open survey was used to gather potential uncertainties which were filtered, categorised, converted into summary questions and checked against current evidence. In a second survey, respondents identified the unanswered questions most important to them. At two final workshops, patients, parents, charities and healthcare professionals agreed the top 10 lists of priorities for child/antenatal and adult congenital heart disease research. RESULTS: 524 respondents submitted 1373 individual questions, from which 313 out of scope or duplicate questions were removed. The remaining 1060 questions were distilled into summary questions and checked against existing literature, with only three questions deemed entirely answered and removed. 250 respondents completed the child/antenatal survey (56 uncertainties) and 252 completed the adult survey (47 uncertainties). The questions ranked the highest by clinicians and non-clinicians were taken forward to consensus workshops, where two sets of top 10 research priorities were agreed. CONCLUSIONS: Through an established and equitable process, we determined national clinical priorities for congenital heart disease research. These will be taken forward by specific working groups, a national patient and public involvement group, and through the establishment of a UK and Ireland network for collaborative, multicentre clinical trials in congenital heart disease.

Detection of feline haemoplasma species in experimental infections by in-situ hybridisation.

The aim of this study was to use fluorescence in-situ hybridisation (FISH) to search for the tissues and cell types important in survival and persistence of Mycoplasma haemofelis, "Candidatus Mycoplasma haemominutum" or "Candidatus Mycoplasma turicensis" in infected cats. A 16S rDNA probe for each species was applied to formalin-fixed, paraffin wax-embedded tissues sections collected from experimentally infected cats. Tissues (n = 12) were collected, at necropsy, from ten cats which had been infected with M. haemofelis, and one each with "Ca. M. haemominutum" and "Ca. M. turicensis". M. haemofelis specific hybridisation was present on red blood cells (RBCs) in all tissues from acutely infected cats, but not the majority of tissues from chronically infected cats. "Ca. M. haemominutum" specific hybridisation was present on scattered RBCs within the spleen and liver. Specific probe hybridisation was not detected in any of the "Ca. M. turicensis" infected tissues. Haemoplasmas were detected on the surface of RBCs only and not any other cell type. Additionally, FISH was limited by sensitivity and could not detect the lower numbers of organisms present in tissues of cats chronically infected with M. haemofelis. Occasional organisms were detected in cats acutely infected with "Ca. M. haemominutum" but not "Ca. M. turicensis".

The Senning procedure as part of the double-switch operations for congenitally corrected transposition of the great arteries.

Anatomic correction of congenitally corrected transposition of the great arteries (ccTGA) has brought about the renaissance of the atrial switch. The Senning procedure has become the most widely used variant because of the lower incidence of pathway obstruction, baffle leak, and significant late arrhythmias. It is for this reason the Senning is discussed in detail here. The technical steps of the Senning are both ingenious and unique amongst cardiac surgical procedures. They must be made as safe and reproducible as possible because the procedure is no longer commonly performed and trainee surgeons may have only very limited exposure to these types of operation. In addition to its infrequency, there are additional technical issues regarding the atrial switch in the setting of ccTGA, particularly in relation to associated malposition of the heart and the conduction system. Outcomes for the Senning procedure in ccTGA have been very good, with early complications being extremely rare. Obstruction to the superior vena cava pathway has been recorded in less than 3% of cases and can usually be managed by interventional catheterization. Late problems with atrial arrhythmias have not been widely reported, but this may reflect the relatively short follow-up for these patient cohorts compared with older series in d-TGA.

Understanding parents' decision-making on participation in clinical trials in children's heart surgery: a qualitative study.

OBJECTIVES: Few children undergoing heart surgery are recruited to clinical trials and little is known about the views and attitudes of parents towards trials. This study explored parents' perspectives on decision-making about their child's participation in a clinical trial during their elective cardiac surgery. DESIGN: Qualitative interview study. SETTING: Single-centre substudy of a multicentre, double-blind, randomised controlled trial to investigate the effects of remote ischaemic preconditioning in children undergoing cardiac surgery. PARTICIPANTS: Parents of children approached to participate in the trial, both consenters and decliners. METHODS: Semistructured interviews were conducted face-to-face or by telephone following discharge, digitally audio-recorded, transcribed and thematically analysed. RESULTS: Of 46 patients approached for the trial, 24 consenting and 2 declining parents agreed to participate in an interview (21 mothers, 5 fathers). Parental decision-making about research was influenced by (1) potential risks or additional procedures; (2) personal benefit and altruism for the 'cardiac community'; (3) information, preparation, timing and approach; and (4) trust in the clinical team and collaboration with researchers. All of these were placed within the context of their understanding of the trial and knowledge of research. CONCLUSIONS: Parents of children undergoing cardiac surgery attach value to clinical research and are supportive of clinical trials when there is no or minimal perceived additional risk. These findings enhance our understanding of the factors that influence parents' decision-making and should be used to inform the design and conduct of future paediatric surgical trials. TRIAL REGISTRATION NUMBER: ISRCTN12923441; Pre-results.

Four right ventricle to pulmonary artery conduit types.

OBJECTIVE: The most durable valved right ventricle to pulmonary artery conduit for the repair of congenital heart defects in patients of different ages, sizes, and anatomic substrate remains uncertain. METHODS: We performed a retrospective analysis of 4 common right ventricle to pulmonary artery conduits used in a single institution over 30 years, using univariable and multivariable models of time-to-failure to analyze freedom from conduit dysfunction, reintervention, and replacement. RESULTS: Between 1988 and 2018, 959 right ventricle to pulmonary artery conduits were implanted: 333 aortic homografts, 227 pulmonary homografts, 227 composite porcine valve conduits, and 172 bovine jugular vein conduits. Patients weighed 1.6 to 98.3 kg (median 15.3 kg), and median duration of follow-up was 11.4 years, with 505 (52.2%) conduits developing dysfunction, 165 (17.2%) requiring catheter intervention, and 415 (43.2%) being replaced. Greater patient weight, conduit z-score, type and position, as well as catheter intervention were predictors of freedom from replacement. Multivariable analysis demonstrated inferior durability for smaller composite porcine valve conduits, with excellent durability for larger diameter conduits of the same type. Bovine jugular vein conduit longevity was inferior to that of homografts in all but the smallest patients. Freedom from dysfunction at 8 years was 60.7% for aortic homografts, 72% for pulmonary homografts, 51.2% for composite porcine valve conduits, and 41.3% for bovine jugular vein conduits. Judicious oversizing of the conduit improved conduit durability in all patients, but to the greatest extent in patients weighing 5 to 20 kg. CONCLUSIONS: Pulmonary and aortic homografts had greater durability than xenograft conduits, particularly in patients weighing 5 to 20 kg. Judicious oversizing was the most significant surgeon-modifiable factor affecting conduit longevity.

Hypoplastic left heart syndrome.

Hypoplastic left heart syndrome is a rare congenital heart defect in which the left side of the heart is underdeveloped. Surgical management of hypoplastic left heart syndrome has changed the prognosis of the condition that was previously regarded as fatal. We discuss surgical strategies based on staged procedures, with the right ventricle supporting both systemic and pulmonary circulation. We also discuss other management options, such as neonatal transplantation and the recent innovation of hybrid techniques. Surgical techniques and the understanding of the pathophysiology of this condition have been at the forefront of neonatal cardiac surgery and intensive care. The management of the syndrome remains a challenge because affected children grow into adolescence and adulthood posing various new problems and demands.

What tissue bankers should know about the use of allograft heart valves.

Heart valve allografts (usually referred to as 'homografts') have been used in cardiac surgery for over 45 years when they were amongst the first valves ever used. Today they remain an important part of valve replacement and reconstructive surgery, particularly in the field of congenital heart disease. There are currently seven tissue banks on the UK and Eire that procure, prepare and store these homografts for surgical implantation, currently providing around 700 grafts per year. This article reviews the history and applications of homografts and compares their performance and outcomes with current prosthetic alternatives. It also describes the processes of valve procurement and storage and describes their clinical applications, hopefully providing tissue bankers with the surgeon's insight into what is required. Homograft degeneration and the natural history of these tissues is discussed, together with future expectations and developments in homograft valve technology.

Morphology of vascular ring arch anomalies influences prognosis and management.

OBJECTIVE: This study aimed to explore the anatomical features of aortic arch anomalies associated with vascular rings, hoping to identify those which may increase the risk of symptomatic presentation and surgical intervention. METHODS: This was a retrospective observational study at a single cardiac unit. Individuals diagnosed with an aortic arch anomaly, either isolated or non-isolated, between June 2014 and September 2018 were included. The morphology of the aortic arch was established via analysis of postnatal echocardiography, CT or MRI scans. CT and magnetic resonance studies were evaluated for the presence of a Kommerell diverticulum in those with aberrant vessels. Case notes were reviewed for relevant clinical data. RESULTS: Of those with aberrant subclavian arteries, 24/79 (30.4%) were shown to have a Kommerell diverticulum. Additional forms of congenital heart disease were present in 133/227 (58.6%) individuals. Surgical division of the vascular ring was performed in 30/227 (13.2%), most commonly in the setting of a double aortic arch (70.8%). In those with aberrant subclavian arteries, no children without a Kommerell diverticulum were referred for surgery. In those with a Kommerell diverticulum confirmed on imaging, 11/24 underwent surgery. CONCLUSION: Individuals with a double aortic arch, or an aberrant subclavian artery arising from a Kommerell diverticulum, have the highest requirement for surgical intervention, especially in isolated anomalies. These individuals should remain under monitoring. The subjective nature of symptoms remains problematic. Longitudinal research is required further to understand the natural history of vascular rings and how it links to morphology.

Strategies to Minimise Need for Prosthetic Aortic Valve Replacement in Congenital Aortic Stenosis-Value of the Ross Procedure.

To examine the role and outcomes of all interventions for aortic stenosis in children, with focus on freedom from reintervention and the aim to minimise prosthetic aortic valve replacement (pAVR) during childhood. Retrospective analysis of 194 consecutive children who underwent any aortic valve intervention for a biventricular repair strategy at a single institution between 1995 and 2017. Data were obtained from hospital records and follow-up was 100% complete. Over a 22-year period, 194 children underwent total 313 aortic valve procedures: Primary interventions were surgical valvotomy (SV)/surgical repair (SR) in 94 (48.5%), balloon valvuloplasty (BV) in 60 (30.9%), pAVR in 8 (4.1%) and Ross/Ross-Konno procedure in 32 (16.5%). Median age at first intervention was 1.1 years (interquartile range [IQR] 0.1-9.4) and varied with type of intervention: SV/SR were most common in neonates (33, 75%) and infants (35, 68%), whilst BV was most frequent in older children (42, 42%). Operative survival was 99% (2 early deaths, both in neonates with critical aortic stenosis and poor left ventricular function) and 15-year survival was 95%. A Ross procedure was performed in 79 (40.7%) patients over the 15-year study period, 1 of whom required late pAVR for autograft failure. Freedom from any reintervention after SV/SR and BV was 41% and 40% at 10 years, compared to 90% at 10 years with the Ross procedure (P < 0.001). Among neonatal SV/SR and BV, 98% required reintervention during childhood with no difference between groups. Valve morphology did not influence freedom from ultimate valve replacement. In patients who went on to have a Ross procedure, median time from initial intervention to Ross was 2.8 years (IQR 0.1-11.9) in neonates and 6.0 years (IQR 3.1-7.5) in all other age groups. Overall freedom from pAVR was 97% at 10 years and was similar in the SV/SR and BV groups. A strategy of simple valve repair and primary Ross procedure provides excellent survival and good freedom from pAVR. However, reintervention rates after simple interventions for congenital AS are high, especially in younger age groups. The Ross procedure offers the best freedom from reintervention of any technique and wider use of primary Ross in younger age groups should be considered.