Background The exercise capacity long after repair of tetralogy of Fallot, when performed exclusively with a transatrial repair, is unclear. It is also unknown whether echocardiography and cardiopulmonary exercise testing can predict the risk of reoperation in this patient group. Method We retrospectively reviewed the clinical records of 59 patients who underwent cardiopulmonary exercise testing after transatrial Fallot repair at a single centre. Patients underwent cardiopulmonary exercise testing at a mean age of 16.6 +/- 4.4 +/- 4.4 years, and at 15.3 +/- 4.1 +/- 4.1 years after Fallot repair. Results At testing, the volume of oxygen consumption at maximal exercise (VO2 2 max) was 71%+/- 13% +/- 13% and the oxygen pulse was 80%+/- 17% +/- 17% of predicted values. Seventeen (17) patients (29%) had a VO2 2 max superior to 80% of the predicted value. Thirty-two (32) patients (56%) had severe pulmonary regurgitation, three (5%) had moderate pulmonary regurgitation, and 12 (21%) had mild pulmonary regurgitation. After a mean of 7.8 +/- 3.9 +/- 3.9 years following cardiopulmonary exercise testing (23 +/- 5.3 +/- 5.3 years after the repair), 21 (40%) patients underwent reoperation. Right ventricular dilation and systolic function on echocardiography were both significantly associated with subsequent reoperation rates. Patients who had severe right ventricular dilation were eight times more likely to undergo subsequent reoperation (hazard ratio 8.67; 1.82-41.3; p=0.007). No cardiopulmonary exercise testing variable independently predicted reoperation. Conclusions The exercise capacity at adolescence following transatrial repair of tetralogy of Fallot is maintained at around 70% of predicted values. Only the patients with normal right ventricular size and normal right ventricular function seemed to be protected from reoperation over the subsequent decade. We found no exercise variables which predicted reoperation.

BACKGROUND During hypoxia or acidosis, S-nitrosoglutathione (GSNO) has been shown to protect the cardiomyocyte from ischemia-reperfusion injury. In a randomized double-blinded control study of a porcine model of paediatric car-diopulmonary bypass (CPB), we aimed to evaluate the effects of 2 different doses (low and high) of GSNO.METHODS Pigs weighing 15-20 kg were exposed to CPB with 1 hour of aortic cross-clamp. Prior to and during CPB, animals were randomized to receive low-dose (up to 20 nmol/kg/min) GSNO (n = 8), high-dose (up to 60 nmol/kg/min) GSNO (n = 6), or normal saline (n = 7). Standard cardiac intensive care management was continued for 4 hours post -bypass.RESULTS There was a reduction in myocyte apoptosis after administration of GSNO (P = .04) with no difference between low-and high-dose GSNO. The low-dose GSNO group had lower pulmonary vascular resistance post-CPB (P = .007). Mitochondrial complex I activity normalized to citrate synthase activity was higher after GSNO compared with control (P = .02), with no difference between low-and high-dose GSNO.CONCLUSIONS In a porcine model of CPB, intravenous administration of GSNO limits myocardial apoptosis through preservation of mitochondrial complex I activity, and improves pulmonary vascular resistance. There appears to be a dose-dependent effect to this protection.(Ann Thorac Surg 2022;114:1468-74)(c) 2022 by The Society of Thoracic Surgeons

BACKGROUND:Berlin Heart EXCOR Pediatric Ventricular Assist Device (BHE) (Berlin Heart AG, Berlin, Germany, BHE) is used worldwide for mechanical circulatory support as a bridge to transplantation or recovery for children with end-stage heart failure. The study aim was to evaluate morbidity and mortality of children less than one year old supported with BHE to identify predictors of adverse outcomes.METHODS:Data of all children aged less than one year supported with BHE between 2005 and 2018 at the Royal Children's Hospital, Melbourne were reviewed. Adverse events were defined using PediMACS criteria.RESULTS:Fourteen (14) children under 1 year of age were implanted with BHE at a median age and weight of 0.37 years (IQR 0.09-0.7) and 5.7 kg (IQR 3.5-7.95) respectively. Four (4) patients were neonates, and 10 were older infants. Twelve (12) patients had cardiomyopathy and two, myocarditis. Preoperative extracorporeal membrane oxygenation (ECMO) support was required in six patients for a mean of 9 days (IQR 6-13). Sepsis occurred in five patients (36%) and thromboembolic stroke in two patients (14%). Survival to bridge to transplantation (11) and recovery (1) was achieved in 12 patients (86%). Mortality was 14%. The median duration of BHE support was 110 days (IQR 40-161). Both patients who died were neonates with myocarditis and required surgical re-intervention during BHE support.CONCLUSIONS:BHE provides excellent support as a bridge to transplantation or recovery in infants, with a low incidence of neurological dysfunction. Neonates with myocarditis may be at greater risk for death after BHE implantation.

Objective: To determine the outcomes of patients with a quadricuspid truncal valve (TV) and durability of TV repair. Method: We reviewed 56 patients with truncus arteriosus and a quadricuspid TV who underwent complete repair between 1979 and 2018. Results: TV insufficiency was present in 39 patients (mild, n = 22; moderate, n = 14; and severe, n = 3). Fourteen patients had concomitant TV surgery. Early mortality in patients who had concomitant TV surgery was 14% (2 out of 14 patients) and overall survival was 77.1% +/- 11.7% at 15 years. Freedom from TV reoperation was 30.3% +/- 14.6% at 15 years. Early mortality in patients who did not undergo concomitant TV surgery was 9.5% (4 out of 42 patients) and overall survival was 74.9% +/- 6.9% at 15 years. Progression of TV insufficiency requiring TV surgery occurred in 16.7% (7 out of 42 patients). Freedom from TV reoperation was 77.1% +/- 7.8% at 15 years. The most common method of repair was tricuspidization of the TV. Freedom from TV reoperation was 64.3% +/- 21.0% at 10 years after tricuspidization and 0% at 6 years after other types of TV surgery. Overall follow-up was 97.6% (41 out of 42 patients) complete for survivors with median follow-up of 16.6 years. At last follow-up there was no TV insufficiency in 16 patients, mild insufficiency in 24 patients, and moderate insufficiency in 1 patient. Conclusions: More than one-third of patients with a quadricuspid TV require TV surgery. Tricuspidization of the quadricuspid TV appears to be a durable repair option with good long-term outcomes.

OBJECTIVES: Management of patients with left ventricular inflow and outflow stenotic lesions can be challenging. Our purpose was to characterize such patients and review the long-term outcomes of those requiring mitral valve (MV) surgery.METHODS: We performed a retrospective study of 40 patients with subaortic, aortic and/or arch stenotic lesion(s) who underwent MV surgery between 1985 and 2016.RESULTS: Associated left-sided stenotic lesions included aortic valve stenosis in 20 patients (50%), subaortic stenosis in 19 (47.5%) patients, coarctation in 23 (57.5%) patients and hypoplastic aortic arch in 16 (40%) patients. Nineteen patients (47.5%) had a supravalvular mitral ring and 15 (37.5%) patients had a parachute MV. The overall mortality rate was 32.5% (13 patients) with a mean follow-up of 16.3 +/- 1.8 years. Being <6 months of age at the time of MV surgery (P = 0.02) and having had previous neonatal aortic valve and/or arch surgery (P = 0.01) were associated with death. The incidence of reoperation (95% confidence interval) at 1, 5, 10 and 15 years was 38% (2353%), 54% (38-70%), 68% (53-84%) and 85% (72-98%), respectively.CONCLUSIONS: Results after MV surgery for children with associated left-sided stenotic lesions are closely age-related. The need for mitral intervention shortly after the initial aortic valve and/or arch intervention was a predictor of dismal outcomes

Background: We aimed to assess the long-term outcomes of children in whom the aortic valve could be repaired without the use of patch material. We hypothesized that if the aortic valve is of sufficiently good quality to perform repair without patches, a durable repair could be achieved. Methods: All children (n = 102) who underwent aortic valve repair without the use of a patch between 1980 and 2016 were reviewed. Results: The median patient age at operation was 2 years (interquartile range, 1 month to 9.6 years). There were 25 neonates and 17 infants. There was no operative mortality. Mean overall survival at 10 years was 97.7% +/- 0.01% (95% confidence interval, [CI] 91.0%-99.4%). Forty-three patients (42.2%) required 56 aortic valve reoperations, including 24 redo aortic valve repairs, 22 Ross procedures, 8 mechanical aortic valve replacements, and 2 homograft aortic valve replacements. Mean freedom from aortic valve reoperation at 10 years was 57.4% +/- 0.06% (95% CI, 44.9%-68.1%), and freedom from aortic valve replacement at 10 years was 74.5% +/- 0.05% (95% CI, 63.0%-82.9%) at 10 years. Freedom from aortic valve reoperation at 10 years was 33.1% +/- 0.1% (95% CI, 14.5%-53.2%) in neonates and 68.9% +/- 0.06% (95% CI, 54.5%-79.6%) in older children (P<.01). Conclusions: In approximately one-third of children undergoing aortic valve repair, the repair could be achieved without patches. In these children, aortic valve repair was achieved without operative mortality. Infants and older children have low reoperation rates, whereas reoperation rates in neonates are higher. Initial repair allows valve replacement to be delayed to later in childhood, when a more durable result may be achieved.

Background: The association of atrioventricular septal defect and transposition of the great arteries is very rare. As a rule, these patients have unbalanced ventricles. However, there have been no studies describing the results of single-ventricle palliation in these children. Methods: All children who underwent surgery with a diagnosis of atrioventricular septal defect and transposition of the great arteries were included in the study. Data were obtained from medical records. Results: A total of 38 patients with atrioventricular septal defect and transposition of the great arteries underwent single-ventricle palliation at the study institution between 1971 and 2016. The mean follow-up was 12.4 years (median: 14.6 years, range 2-43.3 years). Most children had unbalanced atrioventricular septal defect (94.7%, 36/38). Survival was 67.6% (95% confidence interval [CI]: 50.0-80.2%) at 10 years and 57.8% (95% CI: 38.0-73.4%) at 20 years. By 10 years, 58.6% (95% CI: 40.8-72.7%) had progressed to Fontan completion, while 32.5% (95% CI: 18.2-47.6%) had died. In patients achieving Fontan completion, 20-year event-free survival was 73.3% (95% CI: 34.8-91.3%), while 5.0% (95% CI: 0.4-20.5%) had undergone cardiac transplantation and 21.7% (95% CI: 3.2-50.8%) had undergone takedown of the Fontan circulation. Freedom from atrioventricular valve surgery was 57.0% (95% CI: 37.2-72.7%) at 10 and 20 years. Conclusions: The association of atrioventricular septal defect and transposition of the great arteries is very rare, and most of these children have unbalanced ventricles. Single-ventricle palliation results in 25-year overall survival of 50%. However, in patients, who had Fontan completion, survival was 75% at 25 years after Fontan operation.

Background. To evaluate the outcomes after surgical repair of interrupted aortic arch in patients with biventricular circulation. Methods. We reviewed all children from a single institution (N = 177) who had interrupted aortic arch repair between 1978 and 2018. Patients were separated into simple (n = 122) and complex (n = 55) group based on their concomitant anomalies. Results. Median age at repair was 6 days (range, 1-298 days) and median weight was 3.1 kg (range, 0.95-5.1 kg). Median follow-up time was 11.5 years (mean 12.6 years; range, 0.1-35.9 years). Overall early mortality was 11.9% (21 of 177) and there were 5 late deaths. Era of surgery did not impact on overall survival (P = .37). Between 2000 and 2018, there was a significant difference in early mortality between the simple and complex group (3.2% [2 of 62] vs 24.1% [7 of 29], P = .002). There was an improvement in mortality in the simple group over time (P = .03). Competing risks analysis showed at 15 years after the initial operation 14% had died without arch reoperation, 15.2% had undergone aortic arch reoperation, and 70.8% were alive without arch reoperation. Reoperation on the aortic arch was more common in the complex group compared to the simple group (20.0% [11 of 55] vs 9.0% [11 of 122], P< .001). Conclusions. Survival of patients with interrupted aortic arch and associated simple anomalies has improved over time, although mortality in patients with complex congenital cardiac lesions remains high. (C) 2021 by The Society of Thoracic Surgeons

OBJECTIVES: The aim of this study was to study the impact of a decision-making protocol for shunt type in the Norwood procedure for hypoplastic left heart syndrome. Our cohort extends from 2004 to 2016. In era 1 (pre-2008), there was no policy for the choice of Norwood shunt. In era 2 (post-2008), a standard protocol was implemented. The right ventricle (RV)-to-pulmonary artery conduit was utilized for low-birth weight patients (<2.5kg). The right modified Blalock-Taussig Shunt (RBTS) was constructed for normal birth weight patients. METHODS: The records of 133 consecutive operative patients with hypoplastic left heart syndrome anatomy between 2004 and 2016 were retrospectively reviewed. Survival risk factors were analysed using the Cox proportional hazards risk model. RESULTS: The Norwood procedure was performed at a mean age of 2.91.9days. Bidirectional cavopulmonary shunt was performed at a median age of 99days (interquartile range 91-107). In era 1, 38.6% (22/57) of patients received the RBTS and 61.4% (35/57) of patients received the RV-to-pulmonary artery conduit. In era 2, 86.8% (66/76) of patients received the RBTS and 13.2% (10/76) of patients received the RV-to-pulmonary artery conduit. The actuarial survival to Fontan was 72.2% (96/133). Era 1 patients were more likely to die within the 1st year (hazard ratio=2.310, P=0.025). CONCLUSIONS: The shunt protocol may improve outcomes in high-risk patients, and we have demonstrated the reliability of the RBTS in low-risk patients. The short- and mid-term outcomes of our Norwood population justify the continued efforts to improve surgical and perioperative management.

OBJECTIVES:Having an anomalous right subclavian artery has been quoted to be a risk factor for early and late adverse events. We wanted to determine the rate of adverse outcomes in patients who have undergone arch repair with an associated anomalous right subclavian artery. METHODS:The follow-up of 76 patients, with an anomalous right subclavian artery, who underwent arch repair at a single institution for various indications between 1981 and 2017 was reviewed. RESULTS:There were 12 patient deaths. Twenty-three patients required an aortic arch reintervention (17 surgeries, 2 of which were indicated for bronchial obstruction). At last follow-up, 8 of 54 surviving patients (15%) had arch reobstruction (peak gradient >25 mmHg or reintervention). Freedom from aortic arch obstruction at 10 and 15 years was 51% [95% confidence interval (CI) 36-65%] and 35% (95% CI 19-51%), respectively. Neither the complete resection of the adjacent ridge nor the detachment and reimplantation of the anomalous subclavian vessel seemed to have an impact on the rate of reobstruction [hazard ratio (HR) 1.6, 95% CI 0.77-3.5; P = 0.2 and HR 0.61, 95% CI 0.083-4.5; P = 0.6, respectively]. CONCLUSIONS:Patients with an anomalous right subclavian artery are at risk of arch reobstruction necessitating reintervention but long-term follow-up was unable to demonstrate the mechanism of this obstruction in patients with this anomaly.

OBJECTIVES: Pulmonary artery (PA) sling is a rare vascular anomaly often associated with congenital tracheal stenosis. We describe the long-term outcomes with repair of this condition. METHODS: A retrospective study was conducted at 2 institutions. From 1984 to 2018, 33 patients with PA sling underwent repair. RESULTS: The median age at the time of surgery was 5.9 months (quartile 1-3: 2.5-12 months). Concomitant tracheal surgery was required in 21 patients (64%) where slide tracheoplasty was used in 11 patients (52%). There were no early deaths in patients who did not require tracheal surgery (n = 12). Operative mortality was 22% (2 of 9 patients) between 1984 and 1993, 11% (1 of 9 patients) between 1994 and 2003 and 6.7% (1 of 15 patients) between 2004 and 2018. The 15-year probability of survival for patients who had PA sling repair alone was 100%, and for patients who required PA sling and tracheal repair was 76 +/- 10% (95% confidence interval 51-89%) (P = 0.08). The mean follow-up for survivors was 14 +/- 9.8 years (3 months-33 years). All survivors were in the New York Heart Association functional class I/II at the last follow-up. Spirometry performed at a median age of 10.4 years after PA sling and tracheal surgery demonstrated obstructive lung defects with median forced expiratory volume in 1 s of 1.0 l (48% predicted), forced vital capacity of 1.5 l (74% predicted) and forced expiratory volume in 1 s/forced vital capacity of 0.69 (78% predicted). CONCLUSIONS: Early mortality after PA sling repair is determined by the need for tracheal surgery. Though late survival was excellent, and the majority of survivors remained asymptomatic, long-term respiratory assessment and follow-up is warranted for these patients.

BACKGROUND:Data on the long-term outcomes in children after the Ross operation are limited. This study aimed to assess the long-term outcomes in children who underwent the Ross operation at a single institution. METHODS:The study reviewed all children (n = 140) who underwent the Ross operation at the Royal Children's Hospital in Melbourne, Australia between 1995 and 2018. RESULTS:Mean follow-up time was 8.9 years. Median age at operation was 7.4 years. The root replacement (n = 120, Ross-Konno; n = 38), root inclusion (n = 17), and subcoronary implantation (n = 3) techniques were used. Operative mortality was 5.0% (7 of 140; 3 neonates and 4 infants). There were 6 late deaths. Overall survival at 10 years was 96.2% in children older than 1 year of age and 78.9% in children younger than 1 year of age at operation (P = .003). Freedom from autograft reoperation was 86.0% at 10 years. Age younger than 1-year at operation was a risk factor for autograft reoperation (P = .02). Patients younger than 1 year of age at operation experienced a higher incidence of moderate or greater aortic insufficiency compared with patients who were older than 1 year of age (P = .006). In patients who had a poly-(p-dioxanone)-filament band placed around the sinotubular junction, freedom from moderate or greater aortic insufficiency at 10 years was 100%, compared with 83.1% in patients with no band (P = .09). CONCLUSIONS:In children older than 1 year of age, the Ross operation has excellent outcomes with no operative mortality and a low incidence of aortic insufficiency. In children younger than 1 year of age, the Ross operation is associated with higher operative mortality and a higher incidence of aortic insufficiency. Where possible, the Ross operation should be delayed beyond infancy. Poly-(p-dioxanone)-filament banding may reduce the incidence of aortic insufficiency after the Ross operation.

Objectives: Cost data for pediatric heart transplantation are scarce. We examined hospital cost of the national pediatric heart transplantation program in Australia and assessed factors associated with increased costs. Methods: The hospital cost of all children who underwent heart transplantation at a national referral center between January 2003 and June 2015 and were followed more than 1 year was retrospectively analyzed. Lifetime follow-up costs were adjusted for quality of life and projected to life expectancy. All costs were reported in 2016 US dollars. Results: Of 70 children who underwent heart transplantation in the study period, 61 were followed more than 1 year after transplantation (mean, 4.3 +/- 2.5 years). Mean cost of primary heart transplantation was $278,480 (95% confidence interval, 219,282-337,679) and did not change over time. Pretransplant mechanical circulatory support was required in 36% (22/61) of children. On multivariable analysis, greater admission costs were associated with ventricular assist device and pretransplant length of stay. Mean annual follow-up cost after discharge was $55,823 (95% confidence interval, 47,631-64,015) in the first year and $12,119 (95% confidence interval, 8578-15,661) thereafter. Increased first-year follow-up costs were associated with endomyocardial biopsies and length of readmissions. Cost per quality-adjusted life-year gained varied from $29,161 to $44,481 on sensitivity analysis. Freedom from treated rejections was 65.5% at 1 year, 63.2% at 3 years, and 59.5% at 5 years. Endomyocardial biopsies contributed to 52% of first-year follow-up costs. Conclusions: Primary pediatric heart transplantation in Australia is cost-effective for long-term survivors, even for those supported by ventricular assist device. Surveillance endomyocardial biopsy was a major contributor to post-transplantation costs. Selective targeting of surveillance biopsies may be cost-saving.

Background: Infective endocarditis (IE) is rare in children. Limited data have been reported on long-term outcomes of children who undergo surgery for IE. Methods: Data were retrospectively obtained from medical records for all children who underwent surgery for IE. Results: Between 1987 and 2017, 138 children with IE required surgery (mean age, 8.3 +/- 6.5 years). The majority of children (80.4% [111 out of 138]) had underlying cardiac structural anomalies. Prior heart surgery was performed in 50.7% of patients (70 out of 138), including 19.6% (27 out of 138) who had valve replacement. Operative mortality was 5.8% (8 out of 138). Mean follow-up time was 9.7 +/- 7.6 years. Long-term survival at 5 and 25 years was 91.5% (95% confidence interval, 85.1% -95.2%) and 79.1% (95% confidence interval, 66.3%-87.5%), respectively. Risk factors associated with death were: age (hazard ratio [HR], 0.88; P = .015), prosthetic valve IE (HR, 3.86; P = .02), coagulase-negative staphylococci (HR, 4.52; P = .015), increased duration of preoperative antibiotic therapy (HR, 1.02; P = .009), shock (HR, 3.68; P = .028), and aortic valve replacement (HR, 3.22; P = .044). In patients with left-sided IE, risk factors independently associated with death were heart failure (HR, 18.8; P = .025) and vegetation size adjusted to body surface area (HR, 1.06; P = .008). Freedom from recurrent endocarditis was 94.7% (95% confidence interval, 87.7%-97.8%) at 25 years. Conclusions: Children undergoing surgery for IE had good long-term survival and recurrence of IE was uncommon. Surgery during the active phase of endocarditis did not increase risk of mortality or reoperation. In patients with left-sided IE, vegetation size adjusted for patient body surface area was identified as a risk factor for death, and a useful indicator of prognosis.

AbstractBackground:Paediatric heart transplantation in Australia is centralised at The Royal Children’s Hospital, Melbourne. Survival to adulthood is improving but the ongoing need for complex medical therapy, surveillance, and potential for late complications continues to impact on quality of life. Quality of life in adults who underwent heart transplantation in childhood in Australia has not been assessed.Methods:Cross-sectional quality of life data were collected from paediatric heart transplant survivors >18 years of age using Rand 36-Item Health Survey. Self-reported raw scores were transformed to a 0–100 scale with higher scores indicating better quality of life. Mean scores were compared to National Health Survey Short Form-36 Population Norms data using the independent sample t-test.Results:A total of 64 patients (64/151) who underwent transplantation at The Royal Children’s Hospital between 1988 and 2016 survived to adulthood. In total 51 patients (51/64, 80%) were alive at the time of the study and 27 (53%) responded with a mean age of 25 ± 6 years, being a median of 11 years (interquartile range 7–19) post-transplantation. Most self-reported quality of life subscale scores were not significantly different from the Australian normative population data. However, self-reported ‘General Health’ was significantly worse than normative data (p = 0.02). Overall, 93% (25/27) reported their general health as being the same or better compared to 1-year ago.Conclusion:Adult survivors after paediatric heart transplantation in Australia report good quality of life in multiple domains and demonstrate independence in activities of daily living and employment. However, lifelong medical treatment may affect perceptions of general health.

Introduction: Myocardial dysfunction remains a major contributor of morbidity and mortality in children after cardiopulmonary bypass (CPB). During hypoxia or acidosis, S-nitrosoglutathione (GSNO) h...

BACKGROUND:We sought to determine the long-term quality of life after repair of anomalous pulmonary venous drainage using the Short Form (SF)-36 questionnaire in adult survivors. METHODS:All patients who underwent repair of partial or total anomalous pulmonary venous drainage (PAPVD or TAPVD) and were 18 years of age or older with a current contact number were identified from the hospital database. The mean age of the 101 patients was 26 ± 7 years (range, 18-49) old. Patients completed the SF-36 quality of life questionnaire via telephone. The results of the 8 domains of the SF-36 questionnaire and the derived health state summary score (SF-6-Dimension) were compared against an age-matched Australian population data. RESULTS:Compared with Australian population age-matched data, the 18- to 24-year-old TAPVD/PAPVD patients ranked their health higher in 1 of 8 domains; however the SF-6-Dimension scores were similar (0.75 for TAPVD and PAPVD patients vs 0.77 for the Australian population, P = .2). In the 25-50 age group TAPVD/PAPVD patients ranked their health higher in 3 of 8 domains. However the SF-6-Dimension scores were similar to Australian age-matched population (0.78 for TAPVD and PAPVD patients vs 0.77 for the Australian population, P = .51). CONCLUSIONS:Young adult survivors after anomalous pulmonary venous drainage repair have similar quality of life outcomes as age-matched Australian control subjects as measured by SF-6-Dimension.

BACKGROUND:In 2011 we reported unfavourable outcomes of second-run extracorporeal life support (ECLS) in children. We wanted to investigate whether this previous report affected our strategy and modified our long-term outcomes.METHODS:Between 1988 and 2015, 31 patients underwent a second-run ECLS. Median age at the time of first support was 9days (0-16 years). Median length of support for the first and second runs were 4.7days (0.1-10) and 3.6days (0.5-8.7) respectively, with an interval of 1.8days (0.1-66) between supports.RESULTS:There was an increasing trend in the number of patients undergoing second-run ECLS after our report: 21 patients between 1988 and mid-2010 (0.9 patients/year) and 10 between mid-2010 and 2015 (two patients/year) (p=0.06). However, among all the patients who underwent ECLS, the proportion of second-run ECLS was not different before and after 2010 (4% vs. 4.2% respectively, p=0.92). While 58% of patients (18/31) survived weaning of support, only 23% (7/31) survived to hospital discharge and 14% (5/31) were still alive after hospital discharge at a median of 6.5 years (1.2-11.6). The three patients who had positive long-term outcomes had the second-run ECLS instituted to allow for major cardiac operations.CONCLUSIONS:Compassionate use of second-run ECLS is difficult to refuse but one should be aware that its outcomes are dismal. In our centre, benefits seem to be limited to cases where the second-run ECLS allows for a major cardiac intervention.

OBJECTIVES: To date, few studies have assessed the quality of life following congenital cardiac surgery. In this study, we aimed at determining the quality of life after truncus arteriosus (TA) repair using the Short Form 36 questionnaire in adult survivors. METHODS: Seventy-three patients (age > 18 years) who underwent TA repair at the Royal Children's Hospital, Australia were identified for the study. Of these, 42 patients (58%, 42/73) participated in the study and completed the Short Form 36 questionnaire. The results of the 8 domains and the derived health state summary score (Short Form Six Dimension, SF-6D) were compared with age-matched Australian population controls, and with patients who underwent the arterial switch operation (ASO). RESULTS: Compared with the age-matched Australian population, 18- to 24-year-old TA patients (31%, 13/42) had lower scores in 6 of 8 domains; 25- to 34-year-old TA patients (36%, 15/42) scored lower in 5 of 8 domains; and 35- to 44-year-old TA patients (33%, 14/42) scored lower in 4 of 8 domains. SF-6D scores were not significantly different between TA patients and the age-matched Australian population. Compared with patients who underwent ASO, 18-to 24-year-old TA patients scored lower in 3 of 8 domains; and 25- to 34-year-old TA patients scored lower in 2 of 8 domains. There was no significant difference in SF-6D scores between TA and patients who underwent the ASO. CONCLUSIONS: Adult survivors of TA have similar quality of life compared with age-matched Australian controls measured by SF-6D. Despite a higher reoperation rate in TA patients, they have similar quality of life compared with ASO patients.

OBJECTIVE:The study objective was to identify determinants of adverse outcomes in patients undergoing univentricular palliation after the shunt procedure. METHODS:We performed a retrospective study of patients with univentricular lesions undergoing modified Blalock-Taussig shunt and central shunt placement, with or without concomitant Norwood/Damus-Kaye-Stansel procedures, between 2004 and 2014. RESULTS:Overall, 246 patients were included, with 150 patients undergoing concomitant Norwood/Damus-Kaye-Stansel procedure. The 30-day and in-hospital mortality were 7.3% and 14.6%, respectively. Progression to bidirectional cavopulmonary connection was achieved in 76% of patients. Acute events occurred in 66 patients (27%), with 42 having an acute event in the first 72 hours postoperatively. Of all in-hospital deaths, 75% had preceding acute events. On multivariable analysis, the risk factor for 30-day or in-hospital mortality was the incidence of an acute event (odds ratio [OR], 13.5; 95% confidence interval [CI], 5.51-36.4; P < .001). Postoperative pH was associated with fewer acute events (OR, 0.61 per 0.1 unit increase; 95% CI, 0.45-0.82; P = .002). Risk factors for shunt thrombosis associated with an acute event were increased postoperative hematocrit (OR, 2.12 per 0.1 unit increase; 95% CI, 1.01-4.58; P = .049) and 3.0-mm shunt size (OR, 3.78; 95% CI, 1.47-10.6; P = .007). Damus-Kaye-Stansel/Norwood procedure, shunt type, morphology, and extracardiac or genetic anomaly were not risk factors for mortality or acute events. CONCLUSIONS:More than one-fifth of patients shunted during univentricular palliation die before the second stage. The majority of these deaths are associated with acute events occurring early after surgery. Strategies to improve survival should focus on prevention and management of acute events.