Early outcomes after post-cardiotomy extracorporeal membrane oxygenation in paediatric patients: a contemporary, binational cohort study.

OBJECTIVES: The aim of this study was to assess the early outcomes and risk factors of paediatric patients requiring extracorporeal membrane oxygenation after cardiac surgery (post-cardiotomy). METHODS: Retrospective binational cohort study from the Australia and New Zealand Congenital Outcomes Registry for Surgery database. All patients younger than 18 years of age who underwent a paediatric cardiac surgical procedure from 1 January 2013 to 31 December 2021 and required post-cardiotomy extracorporeal membrane oxygenation (PC-ECMO) in the same hospital admission were included in the study. RESULTS: Of the 12 290 patients included in the study, 376 patients required post-cardiotomy ECMO (3%). Amongst these patients, hospital mortality was 35.6% and two-thirds of patients experienced a major complication. Hypoplastic left heart syndrome was the most common diagnosis (17%). The Norwood procedure and modified Blalock-Taussig shunts had the highest incidence of requiring PC-ECMO (odds ratio of 10 and 6.8 respectively). Predictors of hospital mortality after PC-ECMO included single-ventricle physiology, intracranial haemorrhage and chylothorax. CONCLUSIONS: In the current era, one-third of patients who required PC-ECMO after paediatric cardiac surgery in Australia and New Zealand did not survive to hospital discharge. The Norwood procedure and isolated modified Blalock-Taussig shunt had the highest incidence of requiring PC-ECMO. Patients undergoing the Norwood procedure had the highest mortality (48%). Two-thirds of patients on PC-ECMO developed a major complication.

CardioCel® for repair of congenital heart defects: nationwide results of over 1000 implants.

OBJECTIVES: To assess the mid-term performance of CardioCel for the repair of congenital heart defects. METHODS: Data were retrospectively collected from databases and hospital records in 3 congenital cardiac surgery centres in Australia. Kaplan-Meier curves and log-rank tests were used to test for associations between patient age, gender, patch type and site of implantation. Multivariable Cox regression was used to test whether any specific implantation site was associated with reintervention risk, after adjusting for age group, gender and patch type. RESULTS: A total of 1184 CardioCel patches were implanted in 752 patients under the age of 18 years. Median age at implant was 12 months [interquartile range (IQR) 3.6-84]. Median follow-up was 2.1 years (IQR 0.6-4.6). Probability of freedom from CardioCel-related reintervention was 93% [95% confidence interval (CI) 91-95] at 1 year, 91% (95% CI 88-93) at 3 years and 88% (95% CI 85-91) at 5 years, respectively. On multivariable regression analysis, aortic valve repair had a higher incidence of reintervention [hazard ratio (HR) = 7.15, P = 0.008] compared to other sites. The probability of reintervention was higher in neonates (HR = 6.71, P = 0.0007), especially when used for augmentation of the pulmonary arteries (HR = 14.38, P = 0.029), as compared to other age groups. CONCLUSIONS: CardioCel can be used for the repair of a variety of congenital heart defects. In our study, in patients receiving a CardioCel implant, reinterventions were higher when CardioCel was used to augment the pulmonary arteries in neonates and for aortic valve repair as compared to other sites.

Utility of routine chest radiographs after chest drain removal in paediatric cardiac surgical patients-a retrospective analysis of 1076 patients.

OBJECTIVES: Chest drains are routinely placed in children following cardiac surgery. The purpose of this study was to determine the incidence of a clinically relevant pneumothorax and/or pleural effusion after drain removal and to ascertain if a chest radiograph can be safely avoided following chest drain removal. METHODS: This single-centre retrospective cohort study included all patients under 18 years of age who underwent cardiac surgery between January 2015 and December 2019 with the insertion of mediastinal and/or pleural drains. Exclusion criteria were chest drain/s in situ ≥14 days and mortality prior to removal of chest drain/s. A drain removal episode was defined as the removal of ≥1 drains during the same episode of analgesia ± sedation. All chest drains were removed using a standard protocol. Chest radiographs following chest drain removal were reviewed by 2 investigators. RESULTS: In all, 1076 patients were identified (median age: 292 days, median weight: 7.8 kg). There were 1587 drain removal episodes involving 2365 drains [mediastinal (n = 1347), right pleural (n = 598), left pleural (n = 420)]. Chest radiographs were performed after 1301 drain removal episodes [mediastinal (n = 1062); right pleural (n = 597); left pleural (n = 420)]. Chest radiographs were abnormal after 152 (12%) drain removal episodes [pneumothorax (n = 43), pleural effusion (n = 98), hydropneumothorax (n = 11)]. Symptoms/signs were present in 30 (2.3%) patients. Eleven (<1%) required medical management. One required reintubation and 2 required chest drain reinsertion. CONCLUSIONS: The incidence of clinically significant pneumothorax/pleural effusion following chest drain removal after paediatric cardiac surgery is low (<1%). Most patients did not require reinsertion of a chest drain. It is reasonable not to perform routine chest radiographs following chest drain removal in most paediatric cardiac surgical patients.

Mid- and long-term outcomes after surgical correction of subaortic stenosis: a 27-year experience.

OBJECTIVES: We reviewed the mid- and long-term surgical outcomes of patients with subaortic stenosis (SAS). METHODS: Patients operated for SAS from April 1990 to August 2016 were reviewed retrospectively. Patients with major associations such as aortic arch obstruction were excluded. Time to reintervention and predictors of recurrence were assessed using Kaplan-Meier analysis, log-rank test and uni/multivariable Cox regression. RESULTS: 120 patients at a median age of 4.7 years (interquartile range 2.9, 8.1) underwent primary operation (median peak preoperative left ventricular outflow tract gradient 52.5 mmHg, interquartile range 40, 70) involving fibrous tissue excision (n = 120) with septal myectomy (93%; n = 112) as the procedure of choice.At median follow-up of 13 years (interquartile range 7, 18), freedom from reintervention at 1, 3, 5 and 10 years was 99% (95% confidence interval 94%, 99%), 94% (87%, 97%), 93% (86%, 96%) and 90% (82%, 94%), respectively. Recurrence occurred in 18% (n = 20) with 15 patients undergoing reinterventions, 13 of whom required radical reoperation. Multivariable analysis revealed higher preoperative peak left ventricular outflow tract gradient (hazard risk 1.06, confidence interval 1.03, 1.09, P < 0.001), and presence of bicuspid aortic valve (hazard risk 14.13, confidence interval 3.32, 60.1, P < 0.001) as predictors for reintervention. Mild/moderate aortic regurgitation occurred in 49% (n = 55) of patients at the most recent follow-up. CONCLUSIONS: Reintervention for recurrent SAS is common, predicted by higher preoperative peak left ventricular outflow tract gradient, and presence of bicuspid aortic valve, and frequently involves a radical procedure. Aortic regurgitation is a major consequence of SAS, but its severity usually remains low. CLINICAL REGISTRATION NUMBER: SCHN HREC reference number 2019/ETH02729, approved on 09 July 2019.

A machine learning approach to predicting 30-day mortality following paediatric cardiac surgery: findings from the Australia New Zealand Congenital Outcomes Registry for Surgery (ANZCORS).

OBJECTIVES: We aim to develop the first risk prediction model for 30-day mortality for the Australian and New Zealand patient populations and examine whether machine learning (ML) algorithms outperform traditional statistical approaches. METHODS: Data from the Australia New Zealand Congenital Outcomes Registry for Surgery, which contains information on every paediatric cardiac surgical encounter in Australian and New Zealand for patients aged <18 years between January 2013 and December 2021, were analysed (n = 14 343). The outcome was mortality within the 30-day period following a surgical encounter, with ∼30% of the observations randomly selected to be used for validation of the final model. Three different ML methods were used, all of which employed five-fold cross-validation to prevent overfitting, with model performance judged primarily by the area under the receiver operating curve (AUC). RESULTS: Among the 14 343 30-day periods, there were 188 deaths (1.3%). In the validation data, the gradient-boosted tree obtained the best performance [AUC = 0.87, 95% confidence interval = (0.82, 0.92); calibration = 0.97, 95% confidence interval = (0.72, 1.27)], outperforming penalized logistic regression and artificial neural networks (AUC of 0.82 and 0.81, respectively). The strongest predictors of mortality in the gradient boosting trees were patient weight, STAT score, age and gender. CONCLUSIONS: Our risk prediction model outperformed logistic regression and achieved a level of discrimination comparable to the PRAiS2 and Society of Thoracic Surgery Congenital Heart Surgery Database mortality risk models (both which obtained AUC = 0.86). Non-linear ML methods can be used to construct accurate clinical risk prediction tools.

Performance of CardioCel in Cardiac Surgery: A Systematic Review.

A systematic review was performed for evaluation of the performance of CardioCel® in cardiac surgery. The review included all studies published from January 2013 to December 2020. We conclude that CardioCel is a strong, flexible tissue substitute with good handling characteristics and a low incidence of thrombosis, aneurysm formation, infection, or structural degeneration. It can be used for a variety of intracardiac and extracardiac repairs of congenital heart defects in all age groups with good durability at mid-term follow-up. However, the use of CardioCel in certain positions requires caution. Information on the long-term performance of CardioCel is lacking.

Clinical stakeholder preferences for paediatric cardiac surgery outcome reporting in Australia and New Zealand.

BACKGROUND: Outcome reporting is an essential element of quality assurance. Evaluation of the information needs of stakeholders of outcome reporting is limited. This study aimed to examine stakeholder preferences for the content, format, and dissemination of paediatric cardiac surgery performance data in Australia and New Zealand. METHODS: Semi-structured interviews were completed with a purposive sample of Queensland stakeholders to evaluate their attitudes and expectations regarding reporting of paediatric cardiac surgery outcomes. The interviews were audio-recorded and transcribed. Two researchers used an interpretive description approach to analyse the transcripts qualitatively. RESULTS: Nineteen stakeholders were interviewed including fifteen clinicians, four parents, one hospital administrator, and one consumer advocate were interviewed. Mortality was highlighted as the area of greatest interest in reports by clinical and consumer groups. The majority preferred hospital rather than individual/clinician-level reporting. Annual reports were preferred by clinicians who requested reports be distributed electronically. CONCLUSIONS: The evidence generated from outcome reporting in paediatric cardiac surgery is highly desired by clinicians, administrators, parents, families, and advocacy groups. Clinical users prefer information to assist in clinical decision-making, while families seek personalised information at crucial time points in their clinical journey.

Feeding Neonates and Infants Prior to Surgery for Congenital Heart Defects: Systematic Review and Meta-Analysis.

BACKGROUND: Necrotising enterocolitis (NEC) is a significant cause of mortality and morbidity in neonates requiring cardiac surgery. Feeding practices vary significantly across institutions and remain controversial. We conducted a systematic review of the literature and a meta-analysis to identify associations between feeding practices and necrotising enterocolitis. METHODS: This study was carried out in accordance with the PRISMA guidelines. A literature search was performed in November 2022 using the Cochrane Central Register, Embase, and Pubmed. Two investigators then independently retrieved eligible manuscripts considered suitable for inclusion. Data extracted included gestational age, birth weight, sex, nature of congenital heart lesion, type of operation performed, time on ventilator, ICU stay, hospital stay, post-operative feeding strategy, and complications. The methodological quality was assessed using the Downs and Black score for all randomised control trials and observational studies. RESULTS: The initial search yielded 92 studies. After removing duplicates, there were 85 abstracts remaining. After excluding ineligible studies, 8 studies were included for the meta-analysis. There was no significant risk of NEC associated with pre-operative feeding [OR = 1.22 (95% CI 0.77,1.92)] or umbilical artery catheter placement [OR = 0.91 (95% CI 0.44, 1.89)] and neither outcome exhibited heterogeneity [I2 = 8% and 0%, respectively]. There was a significant association between HLHS and NEC [OR = 2.56 (95% CI 1.56, 4.19)] as well as prematurity and NEC [OR 3.34 (95% CI 1.94, 5.75)] and neither outcome exhibited heterogeneity [I2 = 0% and 0%, respectively]. CONCLUSIONS: There was no association between NEC and pre-operative feeding status in neonates awaiting cardiac surgery. Pre-operative feeding status was not associated with prolonged hospital stay or need for tube assisted feeding at discharge. HLHS and prematurity were associated with increased incidence of NEC.

The occult rheumatic scourge: A clinicopathological analysis of missed rheumatic heart disease.

Aims: To study the clinical and pathological manifestations of missed cases of rheumatic heart disease (RHD) and postulate possible reasons behind a missed diagnosis. Materials and Methods: Retrospective 20-year (2000-2019) autopsy data of chronic RHD were reviewed and patients, in whom the valvular deformities had been incidental autopsy findings, were selected. The clinical details of these patients were correlated with the morphology of the affected valves. On this pathological analysis, the patients were assigned to a category of subtle or significant valvular deformity. By clinically correlating, the latter group was subdivided into clinically misdiagnosed, clinically undiagnosed, and sudden cardiac death. Statistical Analysis: Nil. Results: Among the 475 cases of chronic RHD identified at autopsy in the study period, the disease was diagnosed incidentally in 69 patients (14.5%). Significant valvular deformity was noted in 61 cases while the other 8 cases had subtle valvular deformity. The most common cause of death was cardiac failure in 39 out of 69 patients (56%). Eleven (16%) patients had experienced sudden cardiac death. Among the undiagnosed cases, 5 (7%) of them had a diagnosis of non-rheumatic cardiac disease, while the other 14 (20.5%) patients had overwhelming non-cardiac diseases. Conclusions: Our study indicates that mortality and morbidity due to RHD are underdetermined. The patients remain undiagnosed due to either insignificant valvular involvement, clinically silent in the presence of significant valvular deformity, presence of other overwhelming diseases or misdiagnosis partly due to the resemblance with the other pathologies.

The Australia and New Zealand Congenital Outcomes Registry for Surgery (ANZCORS): methodology and preliminary results.

BACKGROUND: Analysis of multi-institutional data and benchmarking is an accepted accreditation standard in cardiac surgery. Such a database does not exist for congenital cardiac surgery in Australia and New Zealand (ANZ). To fill this gap, the ANZ Congenital Outcomes Registry for Surgery (ANZCORS) was established in 2017. METHODS: Inclusion criteria included all cardiothoracic and extracorporeal membrane oxygenation (ECMO) procedures performed at five participating centres. Data was collected by data managers, validated by the surgical team, and securely transmitted to a central repository. RESULTS: Between 2015 and 2019, 9723 procedures were performed in 7003 patients. Cardiopulmonary bypass was utilized for 59% and 9% were ECMO procedures. Fifty-seven percent (n = 5531) of the procedures were performed in children younger than 1 year of age. Twenty-four percent of procedures (n = 2365) were performed in neonates (≤28 days) and 33% (n = 3166) were performed in children aged 29 days to 1 year (infants). The 30-day mortality for cardiac cases (n = 6572) was 1.3% and there was no statistical difference between the participating centres (P = 0.491). Sixty-nine percent of cases had no major post-operative complications (5121/7456). For cardiopulmonary bypass procedures (n = 5774), median stay in intensive care and hospital was 2 days (IQR 1, 4) and 9 days (IQR 5, 18), respectively. CONCLUSION: ANZCORS has facilitated pooled data analysis for paediatric cardiac surgery across ANZ for the first time. Overall mortality was low. Non-risk-adjusted 30-day mortality for individual procedures was similar in all units. The continued evaluation of surgical outcomes through ANZCORS will drive quality assessment in paediatric cardiac surgery across ANZ.

Contemporary outcomes of the double switch operation for congenitally corrected transposition of the great arteries.

OBJECTIVE: To determine the contemporary outcomes of the double switch operation (DSO) (ie, Mustard or Senning + arterial switch). METHODS: A single-institution, retrospective review of all patients with congenitally corrected transposition of the great arteries undergoing a DSO. RESULTS: Between 1999 and 2019, 103 patients underwent DSO with a Mustard (n = 93) or Senning (n = 10) procedure. Segmental anatomy was (S, L, L) in 93 patients and (I, D, D) in 6 patients. Eight patients had heterotaxy and 71 patients had a ventricular septal defect. Median age was 2.1 years (range, 1.8 months-40 years), including 34 patients younger than age 1 year (33%). Median weight was 10.9 kg (range, 3.4-64 kg). Sixty-one patients had prior pulmonary artery bands for a median of 1.1 years (range, 14 days-12.9 years; interquartile range, 0.7-3.1 years). Median intensive care unit and hospital lengths of stay were 5 and 10 days, respectively. Median follow-up was 3.4 years (interquartile range, 1-9.8 years) and 5.2 years (interquartile range, 2.3-10.7 years) in 79 patients with >1 year follow-up. At latest follow-up, aortic, mitral, tricuspid valve regurgitation, and left ventricle dysfunction was less than moderate in 96%, 98%, 96%, and 93%, respectively. Seventeen patients underwent reoperation: neoaortic valve intervention (n = 10), baffle revision (n = 5), and ventricular septal defect closure (n = 4). At latest follow-up, 17 patients (17%) had a pacemaker and 27 (26%) had cardiac resynchronization therapy devices. There were 2 deaths and 2 transplants. Transplant-free survival was 94.6% at 5 years. Risk factors for death or transplant included longer cardiopulmonary bypass time and older age at DSO. CONCLUSIONS: The outcomes of the DSO are promising. Earlier age at operation might favor better outcomes. Progressive neoaortic regurgitation and reinterventions on the neo-aortic valve are anticipated problems.

A single-centre, retrospective study of mid-term outcomes of aortic arch repair using a standardized resection and patch augmentation technique.

OBJECTIVES: The aim of this study was to evaluate the mid-term outcomes after the repair of aortic arch using a standard patch augmentation technique. METHODS: The study included all patients who underwent repair of a hypoplastic/interrupted aortic arch (IAA) in a single institute from June 2012 to December 2019 by a standardized patch augmentation (irrespective of concomitant intra-cardiac lesions). End points evaluated were reintervention for arch obstruction and persistent/new-onset hypertension. RESULTS: The study included 149 patients [hypoplastic aortic arch, n = 92 (62%), IAA, n = 9 (6%), Norwood procedure, n = 48 (32%)]. The patch material used for augmentation of the aortic arch included pulmonary homograft (n = 120, 81%), homograft pericardium (n = 18, 12%), CardioCel® (n = 9, 6%) and glutaraldehyde-treated autologous pericardium (n = 2, 1%). The median age and weight at surgery were 7 days [interquartile range (IQR) 5-17 days] and 3.5 kg (IQR 3-3.9 kg), respectively. The median follow-up was 3.27 years (IQR 1.28, 5.08), range (0.02, 8.76). Freedom from reintervention at 1, 3 and 5 years was 95% [95% confidence interval (CI) = 89%, 98%], 93% (95% CI = 86%, 96%) and 93% (95% CI = 86%, 96%) respectively. One patient (0.6%) had persistent hypertension 8 years after correction for interrupted arch with truncus arteriosus. CONCLUSIONS: Repair of hypoplastic/IAA by transection and excision of all ductal tissue and standardized patch augmentation provide good mid-term durability. The freedom from reintervention at 5 years is >90%. The incidence of persistent systemic hypertension following arch reconstruction is low. The technique is reproducible and applicable irrespective of underlying arch anatomy.

Perioperative Cefazolin Prophylaxis in Pediatric Cardiac Surgery: A Prospective, Cohort Study.

BACKGROUND: This study's objective was to determine the effect of age, prolonged bypass, and hypothermia on serum cefazolin concentrations in children undergoing cardiac surgery. METHODS: A prospective, single-center, observational study was conducted, examining children undergoing cardiac surgery. Participants received cefazolin intravenously approximately 1 hour before skin incision, 3 hourly intraoperatively, and 8 hourly postoperatively. Blood samples were collected at 6 to 8 time points intraoperatively and at 6 time points in the first 24 hours postoperatively. Target unbound serum cefazolin concentrations were 2 mg/L. RESULTS: Sixty-eight patients were enrolled in the study, and 64 were included in the analysis. All maintained concentrations ≥ 2 mg/L throughout the operation. Nineteen patients (30%) did not maintain concentrations ≥ 2 mg/L in the first 24 hours after surgery. Older, larger children (P < .0001) were significantly less likely to achieve target unbound serum cefazolin concentrations. CONCLUSIONS: Intraoperative cefazolin concentrations reached the target concentration in all pediatric cardiac surgical cases. Postoperative cefazolin dosing appears to be insufficient to achieve minimum inhibitory concentrations in many patients.

Major Aortopulmonary Collateral Arteries Requiring Percutaneous Intervention Following the Arterial Switch Operation: A Case Series and Systematic Review.

Background: Dextro transposition of the great arteries (d-TGA) is the most common critical congenital cardiac defect surgically treated in the neonatal period by arterial switch operation (ASO). Major aortopulmonary collaterals (MAPCAs) can be present in this population and may complicate the early postoperative period. Our aim was to review our institutional data and systematically review the available literature to provide further insight on the clinical significance of MAPCAs during the early postoperative course after ASO. Methods: This is a retrospective study of patients with simple d-TGA who underwent ASO between March 1998 and September 2020 at Boston Children's Hospital. The MEDLINE, Embase, and Cochrane databases were searched from inception to June 2020. Results: Of the 671 d-TGA patients who underwent ASO at our center, 13 (1.9%) were diagnosed with MAPCAs. Five were diagnosed before ASO, while eight were diagnosed after ASO. Of these, two patients required catheterization for MAPCAs coiling during the same hospitalization on the 2nd and 11th postoperative days. The systematic review retrieved a total of 34 articles after duplicates were removed. Finally, nine studies reporting on 23 patients were deemed eligible for our analysis. The average time to MAPCAs coiling was 12 days, while the mean hospital stay was 36 days. Conclusions: MAPCAs should be included in the differential diagnosis of ASO complicated by cardiac or respiratory failure, or pulmonary hemorrhage acutely postoperatively. Once managed, recovery of these patients is predictable, and mortality is low. Further studies investigating the diagnostic value of echocardiography and the long-term outcomes of these MAPCAs are necessary.

Comparison of homografts and bovine jugular vein conduits in the pulmonary position in patients <20 years of age.

OBJECTIVE: To compare the performance of homografts and bovine jugular vein (BJV) conduits in the pulmonary position. METHODS: All patients with congenital heart disease up to age 20 years who underwent pulmonary valve replacement with homografts or BJV at 3 centers in Australia were evaluated. There were 674 conduits, with 305 (45%) pulmonary homografts (PHs), 303 (45%) BJV conduits, and 66 (10%) aortic homografts (AHs). Endpoints were freedom from reintervention, structural valve degeneration (SVD), and infective endocarditis (IE). Propensity score matching was used to balance the comparison of PH and BJV conduits. RESULTS: The median follow-up was 6.4 years (interquartile range, IQR, 3.1-10.7 years). Freedom from reintervention at 5 and 10 years was 92% and 80%, respectively, for PH, 74% and 37% for BJV, and 75% and 47% for AH. BJV conduits had a higher risk of reintervention (P < .001) and SVD (P < .001) compared with PHs. These findings were confirmed with propensity score matching valid for conduit size >15 mm. AHs >15 mm had a higher risk of reintervention (P < .001) and SVD (P < .001) compared with PHs >15 mm. The performance of AHs and BJV conduits was similar across all sizes (reintervention, P = .94; SVD, P = .72). The incidence of IE was 1% for PH, 10% for BJV, and 1.5% for AH. CONCLUSIONS: In patients age <20 years with a conduit >15 mm, PHs outperformed BJV conduits and AHs in the pulmonary position. The performance of AH and BJV was comparable. Small conduits (≤15 mm) had similar performance across all conduit types.

Biventricular conversion after Fontan completion: A preliminary experience.

OBJECTIVE: To assess the feasibility and outcomes of biventricular conversion following takedown of Fontan circulation. METHODS: Retrospective analysis of patients who had takedown of Fontan circulation and conversion to biventricular circulation at a single center from September 2007 to April 2020. Failing Fontan physiology was defined as Fontan circulation pressure >15 mm Hg and/or the presence of associated complications. RESULTS: Biventricular conversion was performed in 23 patients at a median age of 10.0 (7.5-13.0) years. Indications included failing Fontan physiology in 15 (65%) and elective takedown in 8 (35%) patients. A subset of patients (n = 6) underwent procedures for staged recruitment of the nondominant ventricle before conversion. Median z score of end-diastolic volume of borderline ventricle before takedown was -2.3 (-3.3, -1.3). Hypoplastic left heart syndrome (P < .01) and sub-/aortic stenosis (P < .01) were more common in these patients. Biventricular conversion with or without staged ventricular recruitment led to a significant increase in indexed end-diastolic volume (P < .01), indexed end-systolic volume (P < .01), and ventricular mass (P < .01) of the nondominant ventricle (14 right, 9 left ventricle). There were 5 (22%) deaths (1 [4%] early death). All who underwent elective biventricular conversion survived, whereas 2-year survival rate for patients with a failing Fontan circulation was 72.7% (95% confidence interval, 37%-90%). The overall, 3-year reoperation-free survival was 86.7% (95% confidence interval, 56%-96%). Left dominant atrioventricular canal defect (P < .01) and early era of biventricular conversion (P = .02) were significant predictors for mortality. CONCLUSIONS: A primary as well as a staged biventricular conversion is feasible in patients who have had previous Fontan procedure. Although this provides an alternative to transplantation in patients with failing Fontan, outcomes are worse in those with failing Fontan compared with elective takedown of Fontan circulation. Optimal timing needs further evaluation.

Fontan operation at less than 3 years of age is not a risk factor for long-term failure.

OBJECTIVES: The age at which the Fontan operation is performed varies globally. Over the last decade, the median age of patients having the Fontan in Australia and New Zealand has been 4.6 years, including 6% of patients younger than 3 years. Long-term outcomes of an early Fontan operation are unclear and are described in this study. METHODS: Patients from the Australian and New Zealand Fontan Registry were grouped by age at Fontan. A Fontan before 3 years (early Fontan) was compared to the combined second and third quartiles by age at surgery in the Registry (3.6-6.1 years; control). Outcomes included Fontan failure (death, transplant, New York Heart Association functional group III/IV heart failure, Fontan takedown or conversion, protein losing enteropathy and plastic bronchitis), arrhythmias, thromboembolism and reinterventions. RESULTS: A total of 191 patients who had early Fontan operations were compared to 781 controls. Profound or progressive cyanosis was noted more frequently in the early than in the control group (63% vs 23%; P < 0.001). The early group was followed up for a median 22.1 years. The incidence of long-term failure was similar between the 2 groups (early, 1.08 failures per 100 patient-years of follow-up vs control, 0.99; log-rank P = 0.79). Adjusted for risk factors, early age at Fontan was not a risk factor for long-term failure [hazard ratio (HR) 1.16, 95% confidence interval (CI) 0.77-1.76; P = 0.48], new-onset arrhythmia (HR 0.93, 95% CI 0.63-1.39; P = 0.73), thromboembolism (HR 0.50, 95% CI 0.28-0.91; P = 0.024) or reintervention (HR 1.08, 95% CI 0.80-1.45; P = 0.62). CONCLUSIONS: Having the Fontan operation at an early age was not a risk factor for short- or long-term adverse outcomes in our cohort.