BACKGROUND AND AIMS:Introduction of the three-vessel trachea view to the second trimester foetal anomaly screening programme increased detection of right (RAA) and double (DAA) aortic arch. Aim was to investigate incidence, associations, and medium-term outcome of RAA/DAA. METHODS:Retrospective, population-based, multicentre, observational study of foetuses diagnosed with RAA/DAA between 1 April 2015 and 31 March 2019. All cardiology units in England, Scotland and Wales participated. Foetuses with major congenital heart disease were excluded. RESULTS:Overall, 1046 foetuses were diagnosed with RAA (n = 898, 85.9%) or DAA (n = 141, 13.5%), uncategorized in seven (0.6%). Estimated incidence of RAA and DAA was 17.98 and 4.58 per 10 000 pregnancies screened/year (0.23%). Nine hundred forty cases (89.9%) were isolated, with prenatal extracardiac anomaly (ECA) and/or minor cardiac difference in the remainder. Genetic results were abnormal in 80 of 1046 (7.6%): 22q11.2 microdeletion (n = 28, 35%). Prenatal ECAs increased the risk of genetic anomaly (risk ratio 3.39, 95% confidence interval 1.89-5.73, P < .0001). Nine hundred eighty six (94.3%) were liveborn, while five died postnatally from unrelated problems. Mean follow-up is 4.1 years (standard deviation 1.4), and symptoms of tracheoesophageal compression were found in 278 of 986 (28.2%). Vascular ring relief was performed in 234 of 986 liveborn (23.7%) with no deaths. There was variation in management pathways. Surgery was performed in 74.6% with DAA, 24.4% with RAA-left arterial duct (LAD)-aberrant left subclavian artery (ALSA); median age at surgery was 5.29 months [interquartile range (IQR): 2.99-11.26 months] and 12.62 months (IQR: 8.68-17.32 months), respectively (P < .0001). CONCLUSION:Incidence of RAA/DAA is higher than previously reported. Genetic anomaly is identified in at least 7.6%, particularly in the presence of ECA. Liveborn with DAA have earlier and more surgical intervention compared to those with RAA-LAD-ALSA.

BACKGROUND:Population studies of congenital heart disease (CHD) often include only children receiving cardiac interventions, underestimating the burden of cases without intervention. We evaluated outcomes for all detected structural CHD cases in England from fetal life to the age of 1 year. METHOD:We linked the National Congenital Anomaly and Rare Disease Registration Service, the National Congenital Heart Disease Audit, and Office for National Statistics mortality records to construct an incident cohort with estimated delivery/birth dates 2018-2020. Outcomes were: termination of pregnancy, fetal loss (miscarriage/stillbirth), live birth with no cardiac intervention in infancy, and live birth with intervention(s) in infancy. Infant mortality at the age of 1 year was assessed. RESULTS:Among 11 265 CHD cases, 63.7% were antenatally detected (95% CI 62.8% to 64.6%), rising to 94.2% (92.0% to 96.0%) for hypoplastic left heart syndrome (HLHS). There were 1766 terminations (15.7%, 95% CI 14.7% to 16.7%), 295 fetal losses (2.6%, 95% CI 1.6% to 3.6%), 4538 live births with no infant cardiac intervention (40.3%, 95% CI 39.3% to 41.3%) and 4666 with intervention(s) (41.4%, 95% CI 40.4% to 42.4%). Termination was higher with greater CHD complexity (eg, HLHS 51.1% (95% CI 46.8% to 55.5%) versus isolated ventricular septal defect 6.0% (95% CI 4.3% to 7.7%), p<0.001), non-cardiac comorbidities (23.6% (95% CI 21.9% to 25.4%) vs 11.3% (95% CI 10.1% to 12.6%), p<0.001), and least versus most deprived areas (20.3% (95% CI 17.5% to 23.1%) vs 11.6% (95% CI 9.7% to 13.5%), p<0.001). Infant mortality was 13.3% (602/4538) in the no-intervention group and 5.2% (243/4666) in the intervention group; those deaths without intervention (n=602) were predominantly cases with critical CHD (n=154), preterm birth (n=301) and/or comorbidity (n=362). CONCLUSION:This national, linked cohort shows that un-intervened cases account for most infant deaths and that antenatal detection exceeds 90% for the most complex lesions. Registries and quality improvement should include all CHD care pathways to inform counselling and equitable service planning.

Heart defects are the second most common congenital anomaly in babies born in the UK and standards state families should have access to a children's cardiac nurse specialist telephone advice service. However, there is little published information to describe the nature of calls and the workload associated with telephone support. We conducted a prospective service evaluation of telephone calls received at one UK specialist children's cardiac surgical center from parents/carers (April-June 2019). All inpatient cardiac teams (cardiology secretaries, inpatient cardiac wards, outpatient department and Clinical Nurse Specialist team) were asked to record calls on a purpose-designed template. This included recording of call duration and reason (from pre-defined categories), with the aim to identify the volume and nature of phone calls. Actions and time taken to resolve issues were not recorded. Data was entered into Excel and analyzed using descriptive statistics. In a seven-week period, 204 telephone calls were received; 41% (n = 83) to the clinical nurse specialist team, 25% (n = 51) to medical secretaries, 20% (n = 42) to cardiac ward staff and 14% (n = 28) to the cardiac outpatient department. The mean length of calls was 6.08 mins (SD 5.07 mins). Across all groups phone call duration totaled 20.8 hours. Sixty-two calls (31% of all calls) reflected 70 concerns/queries about a current health issue. The most common reasons included respiratory (n = 13), heart rate or rhythm (n = 12) and chest pain/pallor (n = 11). Sixty-five calls (32% of all calls) related to issues surrounding medications, with 23% (n = 15) related to dose queries and 49% (n = 32) related to repeat prescription requests or challenges obtaining prescriptions. The results demonstrated a high telephone support need for current health issues or medication queries which required timely assessment and support from health care professionals working across cardiac services. Further research is required to identify the time implications of dealing with phone calls and interventions to support parent/guardian assessment and communication about their concerns and medication management.

Limited data exist on the implementation of current fetal cardiology training and practice guidelines, how trainees are assessed, and how trained fetal cardiologists maintain their skills among countries affiliated with the Association of European Paediatric and Congenital Cardiology (AEPC). A structured questionnaire was sent to fetal cardiologists or national delegates from 44 centers in 33 European countries. Responses were obtained from 37 centers in 29 European countries with 31 responses from fetal cardiologists. Fetal echocardiography was equally performed in maternal (18) and pediatric (16) hospitals with median 3 (range 0–6) fetal cardiologists per center and > 4 fetal cardiologists in 13 centers. Core and advanced fetal cardiology training was offered in 17 (46

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.

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.

Homozygous plakophilin-2 (PKP2) variants have been identified as a cause of a lethal form of dilated cardiomyopathy with excessive trabeculations (DCM-ET) in three cases. We report three more cases from two families with homozygous pathogenic PKP2 variants and perinatal-onset, lethal DCM-ET. Identification of the genetic abnormalities played a key role in decision-making and family counselling in these cases. This case series supports the published evidence that biallelic loss of function PKP2 variants cause a lethal, perinatal-onset cardiomyopathy.

Congenitally corrected transposition of the great arteries (ccTGA) is a rare malformation with diverse morphology. We assessed features of fetuses with ccTGA and evaluated neonatal and pediatric outcomes. This was a retrospective review of fetuses with ccTGA at Birmingham Women’s and Children’s Hospital born from 2005 to 2019. Of thirty-six fetuses identified, six had unavailable prenatal data, one was postnatally diagnosed with isomerism and 29 fetuses were evaluated. ccTGA without associated cardiac lesions was found in 28

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.

Objective: Surgical outcome data differs from overall outcomes of prenatally diagnosed fetuses with hypoplastic left heart syndrome (HLHS). Our aim was to describe outcome of prenatally diagnosed fetuses with this anomaly. Methods: Retrospective review of prenatally diagnosed classical HLHS at a tertiary hospital over a 13-year period, estimated due dates 01/08/2006 to 31/12/2019. HLHS-variants and ventricular disproportion were excluded. Results: 203 fetuses were identified with outcome information available for 201. There were extra-cardiac abnormalities in 8 What this study adds: How this study might affect practice:

Abstract 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.

Objectives: Long-term survival is an important metric for health care evaluation, especially in functionally single-ventricle (f-SV) congenital heart disease (CHD). This study's aim was to evaluate the relationship between center volume and long-term survival in f-SV CHD within the centralized health care service of England and Wales.Methods: This was a retrospective cohort study of children born with f-SV CHD between 2000 and 2018, using the national CHD procedure registry, with survival ascertained in 2020.Results: Of 56,039 patients, 3293 (5.9%) had f-SV CHD. Median age at first intervention was 7 days (interquartile range [IQR], 4, 27), and median follow-up time was 7.6 years (IQR, 1.0, 13.3). The largest diagnostic subcategories were hypoplastic left heart syndrome, 1276 (38.8%); tricuspid atresia, 440 (13.4%); and double inlet left ventricle, 322 (9.8%). The survival rate at 1 year and 5 years was 76.8% (95% confidence interval [CI], 75.3%-78.2%) and 72.1% (95% CI, 70.6%- 73.7%), respectively. The unadjusted hazard ratio for each 5 additional patients with f-SV starting treatment per center per year was 1.04 (95% CI, 1.02-1.06), P <.001. However, after adjustment for significant risk factors (diagnostic subcategory; antenatal diagnosis; younger age, low weight, acquired comorbidity, increased severity of illness at first procedure), the hazard ratio for f-SV center volume was 1.01 (95% CI, 0.99-1.04) P = .28. There was strong evidence that patients with more complex f-SV (hypoplastic left heart syndrome, Norwood pathway) were treated at centers with greater f-SV case volume (P < .001).Conclusions: After adjustment for case mix, there was no evidence that f-SV center volume was linked to longer-term survival in the centralized health service provided by the 10 children's cardiac centers in England and Wales. (J Thorac Cardiovasc Surg 2023;166:306-16)

Objective: To investigate UK variability in prenatal and postnatal management strategy of right aortic arch and double aortic arch (RAA/DAA). Methods: Online surveys were sent to senior physicians (consultants) of the National Fetal Cardiology Working Group regarding prenatal diagnosis, counselling, and perinatal management of antenatally diagnosed RAA/DAA and to the British Congenital Cardiovascular Association regarding postnatal management strategies. Results: There were 28 prenatal and 90 postnatal surveys completed. Prenatally, there was consensus for potential associated chromosomal/genetic anomalies, but there was variation in the risk quoted. Confidence in defining aortic arch morphology was reported by 43% (12/28) of fetal cardiologists. There was variation in what was felt to be possible symptoms/signs of a compressive vascular ring, postnatal investigation, postnatal management, follow-up duration of asymptomatic patients, and indications for surgical intervention. Conclusion: This study has highlighted important areas for future research: improving accuracy of prenatal diagnosis, clarification of potential symptoms, optimal investigation strategies, and indications for surgery.

HomeCirculationVol. 148, No. 17Risk Factors for Reintervention With Functionally Single-Ventricle Disease Undergoing Staged Palliation in England and Wales: A Retrospective Cohort Study Open AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toOpen AccessResearch ArticlePDF/EPUBRisk Factors for Reintervention With Functionally Single-Ventricle Disease Undergoing Staged Palliation in England and Wales: A Retrospective Cohort Study Qi Huang, Deborah Ridout, Victor Tsang, Nigel E. Drury, Timothy J. Jones, Hannah Bellsham-Revell, Elena Hadjicosta, Anna N. Seale, Chetan Mehta, Christina Pagel, Sonya Crowe, Ferran Espuny-Pujol, Rodney C.G. Franklin and Kate L. Brown Qi HuangQi Huang Correspondence to: Qi Huang, PhD, Clinical Operational Research Unit, University College London, London, WC1H 0BT. Email E-mail Address: [email protected] https://orcid.org/0000-0003-4456-2999 Clinical Operational Research Unit, Department of Mathematics (Q.H., E.H., C.P., S.C., F.E.-P. , Deborah RidoutDeborah Ridout Population, Policy and Practice Programme, Great Ormond Street Institute of Child Health (D.R.), University College London. , Victor TsangVictor Tsang Institute of Cardiovascular Science (V.T., K.L.B.), University College London. Great Ormond Street Hospital Biomedical Research Centre, London (V.T., K.L,B,). , Nigel E. DruryNigel E. Drury https://orcid.org/0000-0001-9012-6683 Paediatric Cardiology and Cardiac Surgery, Birmingham Children's Hospital, Birmingham (N.E.D., T.J.J., A.N.S., C.M.). Institute of Cardiovascular Sciences, University of Birmingham (N.E.D., T.J.J., A.N.S.). , Timothy J. JonesTimothy J. Jones https://orcid.org/0000-0001-8870-3496 Paediatric Cardiology and Cardiac Surgery, Birmingham Children's Hospital, Birmingham (N.E.D., T.J.J., A.N.S., C.M.). Institute of Cardiovascular Sciences, University of Birmingham (N.E.D., T.J.J., A.N.S.). , Hannah Bellsham-RevellHannah Bellsham-Revell https://orcid.org/0000-0002-0360-944X Paediatric Cardiology, Evelina London Children's Hospital (H.B.-R.). , Elena HadjicostaElena Hadjicosta Clinical Operational Research Unit, Department of Mathematics (Q.H., E.H., C.P., S.C., F.E.-P. , Anna N. SealeAnna N. Seale Paediatric Cardiology and Cardiac Surgery, Birmingham Children's Hospital, Birmingham (N.E.D., T.J.J., A.N.S., C.M.). Institute of Cardiovascular Sciences, University of Birmingham (N.E.D., T.J.J., A.N.S.). , Chetan MehtaChetan Mehta https://orcid.org/0000-0002-4527-5265 Paediatric Cardiology and Cardiac Surgery, Birmingham Children's Hospital, Birmingham (N.E.D., T.J.J., A.N.S., C.M.). , Christina PagelChristina Pagel https://orcid.org/0000-0002-2857-1628 Clinical Operational Research Unit, Department of Mathematics (Q.H., E.H., C.P., S.C., F.E.-P. , Sonya CroweSonya Crowe https://orcid.org/0000-0003-1882-5476 Clinical Operational Research Unit, Department of Mathematics (Q.H., E.H., C.P., S.C., F.E.-P. , Ferran Espuny-PujolFerran Espuny-Pujol https://orcid.org/0000-0001-9085-7400 Clinical Operational Research Unit, Department of Mathematics (Q.H., E.H., C.P., S.C., F.E.-P. , Rodney C.G. FranklinRodney C.G. Franklin Paediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London (R.C.G.F.). and Kate L. BrownKate L. Brown https://orcid.org/0000-0002-0729-4959 Institute of Cardiovascular Science (V.T., K.L.B.), University College London. Great Ormond Street Hospital Biomedical Research Centre, London (V.T., K.L,B,). Originally published23 Oct 2023https://doi.org/10.1161/CIRCULATIONAHA.123.065647Circulation. 2023;148:1343–1345Our study aimed to evaluate population-based rates of, and risk factors for, cardiac reinterventions in children with functionally single-ventricle (f-SV) congenital heart disease. A retrospective cohort study was undertaken, including all children born in England and Wales with f-SV congenital heart disease1 between 2000 and 2018 who underwent any initial or staged palliative procedures.1,2 The National Congenital Heart Diseases Audit (registry) was used, with National Health Service Research Ethics Committee approval; the study dataset is available only on this basis. Five-year survival, as ascertained in 2020, was 72.1% (95% CI, 70.6%–73.7%).1 The study outcome was any cardiac operation or interventional catheter undertaken in addition to the planned treatment pathway. The association between prespecified risk factors (see Table) and the cumulative incidence of additional procedures was investigated using multivariable Fine-Gray regression. Competing events were death and next staged treatment completion or heart transplant without additional procedures.Table. Adjusted Subdistribution Hazard Ratios (With 95% CIs) for the Occurrence of Additional Procedures in Children With Functionally Single-Ventricle Disease at the 3 Stages of Interventional TreatmentRisk factor, by total patients in study (N=3307)Additional off-pathway surgeriesAdditional off-pathway catheter interventionsStage 1 to the next staged treatment (n=2916)Stage 2 to stage 3 (n=2435)Post stage 3† (n=1592)Stage 1 to the next staged treatment (n=2916)Stage 2 to stage 3 (n=2435)Post–stage 3† (n=1592)Noncardiac variables Male, 1937 (58.6%; Ref: female)0.98 (0.83–1.15)0.95 (0.74–1.22)0.95 (0.67–1.35)1.03 (0.87–1.22)1.05 (0.89–1.24)0.96 (0.78–1.18) Prematurity, 199 (6%; Ref: Born >37 wk gestation)1.18 (0.87– 1.61)1.00 (0.59–1.72)2.60 (1.35–4.99)**0.96 (0.67–1.37)1.52 (1.08–2.14)*0.91 (0.51–1.62) Congenital noncardiac comorbidity, 553 (16.7%; Ref: none)1.09 (0.89–1.34)1.31 (0.97–1.77)1.96 (1.30–2.97)**1.18 (0.96–1.45)1.34 (1.10–1.64)**1.66 (1.26–2.17)*** Low weight at first procedure, 339 (10.3%; Ref: >2.5 kg)‡1.05 (0.82–1.33)1.08 (0.68–1.70)1.24 (0.69–2.22)1.09 (0.83–1.43)0.81 (0.59–1.10)1.07 (0.73–1.56) Acquired comorbidity at first procedure, 159 (4.8%; Ref: none)1.09 (0.77–1.54)0.64 (0.31–1.32)N/A§1.12 (0.81–1.53)0.87 (0.57–1.32)0.94 (0.44–2.04) Increased severity of illness at first procedure, 384 (11.6%; Ref: none)1.50 (1.19–1.89)***1.13 (0.74–1.72)0.99 (0.45–2.21)1.09 (0.86–1.37)1.22 (0.94–1.59)1.62 (1.04–2.52) Age (y) at first procedure, median 6 days (interquartile range: 4–27)0.39 (0.13–1.11)0.97 (0.84–1.11)0.78 (0.58– 1.04)0.79 (0.32–1.99)1.04 (0.91–1.18)0.94 (0.79–1.13) Born after April 2009, 1697 (51.3%; Ref: born before April 2009)‖1.03 (0.86–1.22)1.07 (0.83–1.40)0.94 (0.62–1.42)1.55 (1.29–1.87)***1.31 (1.09–1.56)**0.73 (0.56–0.95)*** Additional cardiac risk factor (at any time, 234 (7.1%; Ref: none)1.56 (1.20–2.03)***2.51 (1.77–3.55)***2.36 (1.26–4.42)*1.23 (0.94–1.62)1.22 (0.89–1.67)1.38 (0.89–2.14)Stage 1 subtypes at first stage 2 (Ref: Norwood type, 1368 [46.9%]) No stage 1 procedure, 391 (11.8%)–1.52 (0.96–2.41)1.38 (0.74–2.55)–0.65 (0.46–0.93)*0.56 (0.36–0.86)** Isolated arch repair, 188 (6.5%)3.32 (2.51–4.39)***0.77 (0.35–1.71)0.77 (0.35–1.71)0.87 (0.61–1.23)0.70 (0.45–1.09)0.54 (0.32–0.92)* Hybrid procedure, 137 (4.7%)3.45 (2.56–4.65)***1.26 (0.59–2.69)0.44 (0.10–2.05)2.84 (2.13–3.79)***1.83 (1.14–2.93)*0.47 (0.14–1.53) Procedures to secure pulmonary blood flow, 829 (28.4%)2.12 (1.71–2.64)***0.84 (0.59–1.18)0.71 (0.45–1.12)0.97 (0.68–1.38)0.85 (0.69–1.04)0.62 (0.48–0.80)*** Pulmonary artery band, 394 (13.5%)2.02 (1.51–2.70)***1.19 (0.79–1.80)0.68 (0.38–1.24)0.40 (0.28–0.59)***0.57 (0.42–0.79)***0.74 (0.53–1.03)Surgical variables Pre–stage 1 procedure (any type), 339 (10.7%; Ref: none)0.95 (0.70–1.30)1.07 (0.70–1.98)0.94 (0.52–1.69)0.97 (0.68–1.38)1.36 (1.05–1.78)*1.19 (0.83–1.69) Stage 1 with concurrent surgery 157 (5.4%; Ref: none)0.89 (0.61–1.30)1.35 (0.79–2.32)0.81 (0.25–2.61)0.90 (0.65–1.24)1.21 (0.83–1.77)0.82 (0.44–1.51) No stage 2 procedure, 391 (11.8%; Ref: Glenn)––2.88 (1.30–6.39)**––1.25 (0.63–2.48) Comprehensive stage 2, 165 (5.0%; Ref: Glenn)–1.18 (0.70–1.98)2.02 (0.89–4.58)–1.15 (0.76–1.73)0.64 (0.37–1.13) Stage 2 with concurrent surgery, 951 (39.1%; Ref: none)–1.69 (1.29–2.21)***0.65 (0.42–1.01)–1.62 (1.36–1.93)***1.68 (1.35–2.08)*** Stage 3 with concurrent surgery, 254 (16.4%; Ref: none)––1.72 (1.15–2.59)**––1.38 (1.06–1.79)* Previous additional procedure (any type; Ref: none)–1.16 (0.86–1.55)1.31 (0.91–1.89)–1.40 (1.17–1.68)***1.06 (0.86–1.30)We performed 6 models (2 intervention types ×3 surgical stages) to investigate the relationship between the cumulative incidence of additional procedures and risk factors. In each model, only those patients who completed the staged procedure were included. The most common concurrent procedure was pulmonary arterioplasty at all 3 stages. The most common additional procedures were (1) stage 1 to next staged treatment: redo systemic-to-pulmonary arterial shunt (surgery, 4.1%) and balloon dilation of aortic re-coarctation (catheter, 5.4%); (2) stage 2 to 3: operative pulmonary arterial repair (1.2%) and transcatheter pulmonary artery stenting (5.5%); (3) post–stage 3: operative pacemaker placement (2%) and transcatheter Fontan fenestration closure (7.9%). Significance level (P value): *0.05, **0.01, ***0.001. N/A indicates not available; and Ref., reference.† In the post–stage 3 analysis, we censored patients who underwent competing events due to limited sample size (death or heart transplant after Fontan without additional surgeries 3.0% and catheter interventions 2.3%). We used a conventional multivariable Cox regression model and presented the adjusted cause-specific hazard ratio as the results.‡ Low weight includes imputed data in 64 patients (2.1%).§ Not included due to nonoccurrence.‖ Externally validated national capture of all cardiac procedures for National Audit in England and Wales occurred from the year 2000, but procedures for capture of noncardiac variables were improved from 2009; hence, we added an era variable (born after vs before 2009) to the models.Among 3307 children with f-SV there were 1266 (38.3%) children with hypoplastic left heart syndrome; 448 (13.6%) with tricuspid atresia; 328 (9.9%) with double-inlet left ventricle; 243 (7.4%) with f-SV with atrial isomerism; 231 (7.0%) with unbalanced atrioventricular septal defect; 138 (4.2%) with pulmonary atresia; 112 (3.4%) with mitral atresia without hypoplastic left heart syndrome; and 541 (16.4%) with other f-SV. Median first procedure age was 6 days (interquartile range [first and third quartiles], 4–27); at this timepoint 384 (11.6%) had increased severity of illness. Congenital comorbidity was present in 553 (16.7%) patients, and 199 (6.0%) had premature birth.We identified the following cardiac procedures as on the planned treatment pathway2 (not the study outcome): interventions before stage 1 in 339 (10.3%) patients (eg, balloon atrial septostomy 195 [5.8%]); first stage 1 procedure in 2916 (88.2%) patients (subtypes in Table); second stage in 2435 (73.6%) patients (Glenn 2270 [68.6%]) and comprehensive stage 2 operation in 165 (5.0%); third stage (Fontan-type) operation in 1592 (48.1%) patients; and heart transplant in 47 (1.4%) patients.Over a median follow-up of 5.4 years (interquartile range [first and third quartiles], 0.8–10.8) of 3307 patients, 1730 (52.3%) patients had at least 1 additional procedure (ie, the study outcome), and 887 (26.8%) patients had multiple additional procedures. Of 3427 additional procedures, 1289 (37.4%) were cardiac surgery in 921 patients, and 2138 (62.4%) were interventional catheters in 1293 patients. In the Table, we show the adjusted subdistribution hazard ratios with each risk factor for additional procedures, from the first stage 1 procedure to the next stage, whichever stage occurred next (additional surgery in 596 [20.4%], and catheter intervention in 596 [20.4%] patients); from stage 2 to 3 (265 [10.9%] and 616 [25.3%]); and after stage 3 (135 [8.5%] and 387 [24.3%]).Between stage 1 and the next stage that occurred, all stage 1 subtypes were associated with higher risk of additional surgery, compared with the reference category of Norwood, most notably hybrid (adjusted subdistribution hazard ratio, 3.45 [95% CI, 2.56–4.65]; P<0.001). The hybrid was also associated with higher risk of additional catheter intervention after stage 1 (2.84 [2.13–3.79]; P<0.001); and between stages 2 and 3 (1.83 [1.14–2.93]; P<0.05). Nonhybrid stage 1 subtypes were all associated with lower risk than the Norwood, of additional catheter procedures at later stages.Increased severity of illness (ie, ventilation, shock) before the first procedure was associated with higher risk of additional surgery after stage 1 (1.50 [1.19–1.89]; P<0.001).When children underwent more interventions than the 3 planned palliative stages, subsequent additional procedures were more likely, as shown by increased risk of additional catheter interventions between stages 2 and 3, after a pre–stage 1 procedure (1.36 [1.05–1.78]; P<0.05); or after an additional procedure between stages 1 and 2 (1.40 [1.17–1.68]; P<0.001). Concurrent surgery with stage 2, was associated with increased risk for additional surgery (1.69 [1.29–2.21]; P<0.001), and additional catheters between stages 2 and 3 (1.62 [1.36–1.93]; P<0.001); and after stage 3 (1.68 [1.35–2.08]; P<0.001). Concurrent surgery with stage 3 was associated with higher risk of subsequent additional surgery (1.72 [1.15–2.59]; P<0.01) and catheter intervention (1.38 [1.06–1.79]; P<0.05).Children with complex features3 were overrepresented; with acquired cardiac risk factors (eg, impaired ventricular function, raised pulmonary vascular resistance), there was increased risk of additional surgery after stage 1 (1.56 [1.20–2.03]; P<0.001), between stages 2 and 3 (2.51 [1.77–3.55]; P<0.001), and after stage 3 (2.36 [1.26–4.42]; P<0.05). With congenital comorbidity, there was increased risk of additional catheter interventions after stage 2 (1.34 [1.10–1.64]; P<0.01) and stage 3 (1.66 [1.26–2.17]; P<0.001); and additional surgery after stage 3 (1.96 [1.30–2.97]; P<0.01). With premature birth, there was increased risk after stage 3 of additional surgery (2.60 [1.35–4.99]; P<0.01) and after stage 2 catheter intervention (1.52 [1.08–2.14]; P<0.05).Parents and clinicians should be prepared for additional procedures in the early years for most children with f-SV disease. Although it is infeasible to adjust for all aspects of case complexity, additional procedures could represent a disadvantage of the hybrid pathway. As previously reported,4,5 the pulmonary arteries most often require additional interventions in f-SV, especially transcatheter beyond stage 2. Strategies to minimize pulmonary arterial distortion, preserve ventricular function, and maintain lower pulmonary vascular resistance may protect children from additional procedures during childhood.ARTICLE INFORMATIONSources of FundingThis study was funded by the British Heart Foundation (Project Grant No. PG/17/88/33401). Drs Tsang and Brown received support from the NIHR Biomedical Research Centre at Great Ormond Street Hospital.Disclosures The study was approved by the UK National Health Service Stanmore Research Ethics Committee (Reference 18/LO/1688) and the need for patient consent was waived.FootnotesFor Sources of Funding and Disclosures, see page 1345.Circulation is available at www.ahajournals.org/journal/circCorrespondence to: Qi Huang, PhD, Clinical Operational Research Unit, University College London, London, WC1H 0BT. Email Qi_Huang@ucl.ac.ukREFERENCES1. Brown KL, Huang Q, Hadjicosta E, Seale AN, Tsang V, Anderson D, Barron D, Bellsham-Revell H, Pagel C, Crowe S, et al. Long-term survival and center volume for functionally single-ventricle congenital heart disease in England and Wales.J Thorac Cardiovasc Surg. 2023; 166:306–316.e3. doi: 10.1016/j.jtcvs.2022.11.018CrossrefMedlineGoogle Scholar2. Feinstein JA, Benson DW, Dubin AM, Cohen MS, Maxey DM, Mahle WT, Pahl E, Villafañe J, Bhatt AB, Peng LF, et al. Hypoplastic left heart syndrome: current considerations and expectations.J Am Coll Cardiol. 2012; 59:S1–S42. doi: 10.1016/j.jacc.2011.09.022CrossrefMedlineGoogle Scholar3. Brown KL, Rogers L, Barron DJ, Tsang V, Anderson D, Tibby S, Witter T, Stickley J, Crowe S, English K, et al. Incorporating comorbidity within risk adjustment for UK pediatric cardiac surgery.Ann Thorac Surg. 2017; 104:220–226. doi: 10.1016/j.athoracsur.2016.12.013CrossrefMedlineGoogle Scholar4. Shearer L, Justo RN, Marathe SP, Betts K, Venugopal P, Winlaw DS, Bullock A, Robertson T, Gentles TL, Celermajer D, et al. Augmentation of the pulmonary arteries at or prior to the Fontan procedure is not associated with worse long-term outcomes: a propensity-matched analysis from the Australia-New Zealand Fontan Registry.Eur J Cardiothorac Surg. 2019; 55:829–836. doi: 10.1093/ejcts/ezy376CrossrefMedlineGoogle Scholar5. Newburger JW, Sleeper LA, Gaynor JW, Hollenbeck-Pringle D, Frommelt PC, Li JS, Mahle WT, Williams IA, Atz AM, Burns KM, et al; Pediatric Heart Network Investigators. Transplant-free survival and interventions at 6 years in the SVR trial.Circulation. 2018; 137:2246–2253. doi: 10.1161/CIRCULATIONAHA.117.029375LinkGoogle Scholar eLetters(0) eLetters should relate to an article recently published in the journal and are not a forum for providing unpublished data. Comments are reviewed for appropriate use of tone and language. Comments are not peer-reviewed. Acceptable comments are posted to the journal website only. Comments are not published in an issue and are not indexed in PubMed. Comments should be no longer than 500 words and will only be posted online. References are limited to 10. Authors of the article cited in the comment will be invited to reply, as appropriate. Comments and feedback on AHA/ASA Scientific Statements and Guidelines should be directed to the AHA/ASA Manuscript Oversight Committee via its Correspondence page. Sign In to Submit a Response to This Article Previous Back to top Next FiguresReferencesRelatedDetails October 24, 2023Vol 148, Issue 17 Advertisement Article Information Metrics © 2023 The Authors. Circulation is published on behalf of the American Heart Association, Inc., by Wolters Kluwer Health, Inc. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution, and reproduction in any medium, provided that the original work is properly cited.https://doi.org/10.1161/CIRCULATIONAHA.123.065647PMID: 37871240 Originally publishedOctober 23, 2023 Keywordscardiac surgical proceduresheart defects, congenitalrisk factorsPDF download Advertisement Subjects Cardiovascular Surgery Catheter-Based Coronary and Valvular Interventions Congenital Heart Disease

Background:Given their importance as a metric for health care evaluation, this study's aim was to evaluate the rates of surgical and catheter reinterventions for children with functionally single-ventricle (f-SV) congenital heart disease (CHD) undergoing staged palliation. Methods:We undertook a retrospective cohort study of children born with f-SV CHD between 2000 and 2018 in England and Wales, using the national registry, with survival ascertained in 2020. Competing risk analysis was used to describe the incidence of additional procedures that occurred first, during follow-up, accounting for competing events of death or transplantation. Results:Of 56,039 patients who received an intervention for CHD, 3307 (5.9%) had f-SV. The largest diagnostic subcategories were hypoplastic left heart syndrome (1266 [38.3%]), tricuspid atresia (448 [13.5%]), and double-inlet left ventricle (328 [9.9%]). During a median follow-up of 5.4 (interquartile range, 0.8-10.8) years, 921 (27.9%) patients had at least 1 additional interstage surgery and 1293 (39.1%) had at least 1 additional interstage catheter intervention. The cumulative incidence of additional surgery at 6 months after stage 1 was 17.6% (95% CI, 16.2%-19.0%); at 2 years after stage 2, 8.3% (7.2%-9.5%); and at 5 years after stage 3, 8.4% (7.0%-9.9%). The cumulative incidence of additional catheter at 6 months after stage 1 was 18.0% (16.6%-19.4%); at 2 years after stage 2, 14.7% (13.3%-16.2%); and at 5 years after stage 3, 23.7% (21.5%-26.0%). Conclusions:It is important to quantify additional procedures for children with f-SV disease to inform parents and health professionals, potentially facilitating the development of interventions that aim to reduce these important adverse outcomes.

Objective Given the paucity of long-term outcome data for complex congenital heart disease (CHD), we aimed to describe the treatment pathways and survival for patients who started interventions for functionally univentricular heart (FUH) conditions, excluding hypoplastic left heart syndrome. Methods We performed a retrospective cohort study using all procedure records from the National Congenital Heart Diseases Audit for children born in 2000-2018. The primary outcome was mortality, ascertained from the Office for National Statistics in 2020. Results Of 53 615 patients, 1557 had FUH: 55.9% were boys and 67.4% were of White ethnic groups. The largest diagnostic categories were tricuspid atresia (28.9%), double inlet left ventricle (21.0%) and unbalanced atrioventricular septal defect (AVSD) (15.2%). The ages at staged surgery were: initial palliation 11.5 (IQR 5.5-43.5) days, cavopulmonary shunt 9.2 (IQR 6.0-17.1) months and Fontan 56.2 (IQR 45.5-70.3) months. The median follow-up time was 10.8 (IQR 7.0-14.9) years and the 1, 5 and 10-year survival rates after initial palliation were 83.6% (95% CI 81.7% to 85.4%), 79.4% (95% CI 77.3% to 81.4%) and 77.2% (95% CI 75.0% to 79.2%), respectively. Higher hazards were present for unbalanced AVSD HR 2.75 (95% CI 1.82 to 4.17), atrial isomerism HR 1.75 (95% CI 1.14 to 2.70) and low weight HR 1.65 (95% CI 1.13 to 2.41), critical illness HR 2.30 (95% CI 1.67 to 3.18) or acquired comorbidities HR 2.71 (95% CI 1.82 to 4.04) at initial palliation. Conclusion Although treatment pathways for FUH are complex and variable, nearly 8 out of 10 children survived to 10 years. Longer-term analyses of outcome based on diagnosis (rather than procedure) can inform parents, patients and clinicians, driving practice improvements for complex CHD.

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.

To appreciate congenital heart disease fully, a detailed understanding of the anatomical presentation, as well as the physiology, is required. This is often introduced at an advanced stage of training. Professor Anderson has been influential in the Clinical Anatomy Intercalated BSc programme at the University of Birmingham, in particular in his teaching on Sequential Segmental Analysis. This article describes the experiences of the latest cohort of students on this programme, who undertook varying research projects using the Birmingham Cardiac Archive, with the guidance of Professor Anderson. The projects outlined include various aspects of isomerism, encompassing both the cardiac and abdominal manifestations, as well as details of congenitally corrected transposition of the great arteries and prenatally diagnosed right aortic arch and double arch. These studies all aimed to increase the knowledge base of their respective cardiac malformations and provide a basis for further research.