Search

Infant long-segment congenital tracheal stenosis (LTS) is rare and presents a challenging clinical scenario. We describe the management of a child who required extracorporeal membrane oxygenation following a respiratory arrest and underwent slide tracheoplasty in infancy for severe LTS and required repeated bronchoscopic reinterventions for recurrent tracheal granulations. At 9 years of age, the child has normal pulmonary function testing and a normal exercise tolerance., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.

Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.

Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.

Objective: The study objective was to evaluate the outcomes of the extracardiac Fontan operation at a single institution., Methods: We conducted a retrospective study of 398 patients from a single institution who underwent their initial extracardiac Fontan operation between 1997 and 2020. We determined the incidence of and risk factors for failure of the Fontan circulation, which includes death, Fontan takedown, heart transplantation, protein-losing enteropathy, plastic bronchitis, and functional status at the last follow-up., Results: The median follow-up time was 10.3 years (interquartile range, 6.4-14.6). The overall survival was 96% and 86% at 10 and 20 years after extracardiac Fontan operation, respectively. There were 6 early deaths (6/398, 1.5%) and 15 late deaths (15/398, 3.8%). Forty-nine patients (12.5%) developed failure of the Fontan circulation. Freedom from the failure of Fontan circulation was 88% at 10 years and 76% at 20 years. Risk factors for failure of the Fontan circulation were right ventricular dominance (hazard ratio, 4.7; P < .001; 95% CI, 2.1-10.5), aortic atresia (hazard ratio, 5.5; P < .001; 95% CI, 2.3-12.8), and elevated mean pulmonary artery pressure (hazard ratio, 2.3; P = .002; 95% CI, 1.2-6.7)., Conclusions: Rates of failure of the Fontan circulation are low after the contemporary extracardiac Fontan operation. Risk factors for failure of the extracardiac Fontan circulation include right ventricular dominance, aortic atresia, and elevated pulmonary artery pressures., Competing Interests: Conflict of Interest Statement C.P.B. has served on the advisory board of Admedus. All other authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Crown Copyright © 2024. Published by Elsevier Inc. All rights reserved.)

Background: Neo-aortic root dilatation and valve regurgitation are emerging problems late after arterial switch operation (ASO). We sought to evaluate the prevalence and outcomes of neo-aortic root or valve reoperation after ASO., Methods: All patients with biventricular circulation who underwent an ASO between 1983 and 2015 were included at a single institution., Results: In our cohort of 782 late ASO survivors, the median duration of follow-up was 18.1 years (interquartile range [IQR], 11.3-25.6 years). During follow-up, 47 patients (6.0%) underwent 60 reoperations on the neo-aortic valve/root. The first neo-aortic valve/root reoperation occurred at a median of 15.2 years (IQR, 7.8-18.4 years) after ASO. Operations included mechanical Bentall (31.9%; n = 15), aortic valve repair (25.5%; n = 12), mechanical aortic valve replacement (AVR) (21.3%; n = 10), valve-sparing root replacement (19.1%; n = 9), and the Ross procedure (2.1%; n = 1). There was 1 late death (2.1%). Multivariable predictors of neo-aortic valve/root reoperation were bicuspid valve (hazard ratio [HR], 4.8; 95% confidence interval [CI], 2.1-10.7; P < .001), Taussig-Bing anomaly (HR, 3.0; 95% CI, 1.2-7.4; P < .02), previous pulmonary artery band (HR, 2.8; 95% CI, 1.2-6.3; P < .01) and left ventricular outflow tract obstruction before ASO (HR, 2.4; 95% CI, 1.0-5.8; P < .04). Freedom from neo-aortic valve or root reoperation was 98.0% (95% CI, 96.7%-98.8%) at 10 years, 93.3% (95% CI, 90.8%-95.2%) at 20 years, and 88.5% (95% CI, 84.1%-91.8%) at 30 years after ASO. Among the 47 patients who underwent neo-aortic reoperation, freedom from AVR was 82.3% (95% CI, 67.7%-90.7%) at 10 years, 58.0% (95% CI, 41.8%-71.2%) at 20 years, and 43.2% (95% CI, 27.0%-58.3%) at 25 years after ASO., Conclusions: The need for neo-aortic valve or root reoperation surpasses 10% by 30 years post-ASO. Evolving understanding of the mechanisms of neo-aortic valve insufficiency and techniques of neo-aortic valve repair may decrease the need for AVR., Competing Interests: Conflict of Interest Statement Dr Brizard serves on the advisory board of Admedus. All other authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Crown Copyright © 2023. Published by Elsevier Inc. All rights reserved.)

Background: Patients with dextrocardia and d-transposition of the great arteries (d-TGA) present a technical challenge when performing the arterial switch operation (ASO). We sought to determine the long-term outcomes of this rare presentation., Methods: All patients who underwent the ASO with d-TGA and dextrocardia were identified from the hospital database., Results: There were four patients with dextrocardia (4 of 844, 0.5%) patients; two patients had situs solitus and two patients had situs inversus. Three patients had a ventricular septal defect. Left ventricular outflow tract obstruction was present in one patient and one patient had a hypoplastic arch with severe coarctation of aorta. Anomalous coronary anatomy occurred in two patients. There was one early mortality and the cause of death was sepsis. One patient required late reoperation for severe neo-pulmonary valve regurgitation at 23 years after the ASO. At last follow-up, all three surviving patients were in New York Heart Association Class I., Conclusions: Patients with d-TGA and dextrocardia who undergo the ASO are extremely rare and require challenging operative management., Competing Interests: Conflicts of Interest There are no conflicts of interest to disclose., (Copyright © 2023 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Objective: Patients undergoing the arterial switch operation (ASO) with inverted coronary anatomy represent a technical challenge. We sought to determine the long-term outcomes of patients with inverted coronary anatomy who underwent an ASO., Methods: A retrospective analysis of patients who underwent an ASO with inverted coronary anatomy at a single institution was performed and comparison was made between patients with inverted and normal coronary anatomy., Results: There were 43 (5.1% [43 out of 844]) patients with inverted coronary anatomy. Twenty patients (46.5% [20 out of 43]) had a right coronary artery that arises from sinus 1 and the left anterior descending and circumflex coronaries arise from sinus 2 anatomy and 23 (53.5% [23 out of 43]) patients with right coronary artery that arises from sinus 1 and gives rise to the left anterior descending with the circumflex coronaries arising from sinus 2. Median follow-up was 17 years (interquartile range, 12-21 years). Overall early mortality was 7.0% (3 out of 43) compared with 3.1% (17 out of 556) in patients with normal coronary anatomy undergoing operation over the same time period (P = .17). There were 2 (10% [2 out of 20]) early deaths in the right coronary artery that arises from sinus 1 and gives rise to the left anterior descending with the circumflex coronaries arising from sinus 2 group and 1 (4.3% [1 out of 23]) early death in the right coronary artery that arises from sinus 1 and the left anterior descending and circumflex coronaries arise from sinus 2 group. There was 1 late death occurring at 40 days after ASO. Nine (23% [9 out of 39]) patients had 15 reinterventions occurring at median 3.6 years (interquartile range, 1.1-8.2 years). The most common cause of reintervention was main or branch pulmonary artery stenosis (15% [6 out of 39]). Freedom from reintervention was 78% (95% CI, 68%-93%) and 75% (95% CI, 56%-86%) at 10 and 15 years, respectively. At final follow-up, all surviving local patients except 1 was in New York Heart Association functional class I., Conclusions: Patients with inverted coronary anatomy who underwent an ASO had a higher mortality but this was not statistically significant. However, there were no coronary reinterventions in survivors., (Crown Copyright © 2022. Published by Elsevier Inc. All rights reserved.)

Background: Arterial switch operation (ASO) in children with Taussig-Bing anomaly and associated aortic arch obstruction (AAO) is technically demanding. This study sought to determine outcomes of patients with Taussig-Bing anomaly and AAO who underwent ASO at a single institution., Methods: From 1983 to 2015, 844 patients underwent an ASO for biventricular repair at The Royal Children's Hospital. Twenty-eight patients (3.3%) had Taussig-Bing anomaly and AAO., Results: Of 28 patients, 21 (75%) underwent ASO and AAO repair as a single-stage procedure and 7 (25%) had arch repair before ASO. There were no early or late deaths. Follow-up after hospital discharge was available for all patients. Median follow-up was 17.5 years (interquartile range, 9.2-22.3; range, 4.0-31.5). All patients were in New York Heart Association class I at last follow-up. Freedom from reintervention was 61% (95% confidence interval, 40-76) and 56% (95% confidence interval, 36-73) at 5 and 15 years, respectively, after ASO. The most common reinterventions were repair of recurrent AAO, branch pulmonary artery stenosis, and neoaortic valve repair. Four of 28 patients (14%) required 5 reinterventions (3 reoperations and 2 catheter reintervention) for recurrent AAO at a median of 2.2 years after ASO (interquartile range, 2.1-2.3)., Conclusions: Patients with Taussig-Bing anomaly and AAO can undergo ASO with excellent survival. Reintervention is equally common for both left- and right-sided obstruction., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: The arterial switch operation (ASO) has excellent early outcomes in the modern era. We sought to determine the long-term outcomes in patients who underwent an ASO at a single institution., Methods: Patients who underwent an ASO between 1983 and 2015 were identified from the hospital database and retrospectively reviewed using hospital records., Results: From 1983 to 2015, 844 patients with a biventricular circulation underwent an ASO. There were 28 (3.3%, 28/844) early deaths. Follow-up was available for 94% (729/774) of local patients after hospital discharge. Median follow-up was 15 years (interquartile range, 8-20 years). There were 187 (26%, 187/729) patients with more than 20 years of follow-up and 95 (13%, 95/729) patients with more than 25 years of follow-up. Overall survival was 95% (95% confidence interval [CI], 94%-97%) at 10 and 25 years after the ASO. At 25 years after ASO, freedom from overall reintervention was 77% (95% CI, 73%-81%), freedom from reoperation on the neoaortic root or neoaortic valve was 92% (95% CI, 88%-95%), and freedom from coronary reoperation was 99% (95% CI, 98%-99.7%). Left ventricular (LV) systolic function was normal in 595 of 609 (98%) of patients who had LV function quantified at latest follow-up. Of the 95 patients with more than 25 years of follow-up after ASO, 6 (6.3%) had at least moderate neoaortic valve regurgitation (AR) and 8 (8.4%) had undergone replacement of the neoaortic valve., Conclusions: Overall, survivors of ASO have excellent late survival and normal LV systolic function into adult life. However, AR and reoperation on the neoaortic valve remains an issue for older patients., (Crown Copyright © 2021. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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., (Crown Copyright © 2020. Published by Elsevier Inc. All rights reserved.)

Background: The Ross procedure in children is performed either as a primary operation, or a secondary operation after initial aortic valve surgery., Objectives: The study aimed to determine whether the outcomes of primary and secondary Ross procedure are similar., Methods: All patients who underwent Ross procedure between 1995 and 2018 were included in the study. Outcomes were compared between those who had primary Ross procedure and those who had secondary Ross procedure, after aortic valve surgery. Propensity score matching for baseline characteristics and risk factors for death and reoperation was performed., Results: Of 140 Ross procedures, 51.4% (n = 72 of 140) were primary operations, while 48.6% (n = 68 of 140) were secondary operations. Patients undergoing primary Ross procedure tended to be older (median age 8.6 years vs. 7.0 years; p = 0.10) and have a higher weight (28.9 kg vs. 19.4 kg; p = 0.07). There were no significant differences in survival or freedom from reoperation in the unmatched cohort. Propensity score matching resulted in 50 well-matched pairs. In the matched cohort, survival at 10 years was 90.0% (95% confidence interval [CI]: 77.5% to 95.7%) in the primary Ross group, compared with 96.8% (95% CI: 79.2% to 99.5%) in the secondary Ross group (p = 0.04). Freedom from autograft reoperation at 10 years was 82% (95% CI: 64.1% to 91.5%) in the primary Ross group, compared with 97.0% (95% CI: 80.4% to 99.6%) in the secondary Ross group (p = 0.03)., Conclusions: Secondary Ross procedure performed after initial aortic valve surgery achieves superior long-term survival and freedom from autograft reoperation compared with primary Ross procedure. A strategy of initial aortic valve repair followed by delayed Ross procedure may provide better long-term survival and freedom from autograft reoperation., (Crown Copyright © 2020. Published by Elsevier Inc. All rights reserved.)

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.

Objective: We sought to determine the influence of coronary artery anatomy on mortality in more than 1000 children undergoing the arterial switch operation., Methods: All patients who underwent an arterial switch operation were identified from 2 hospital databases and reviewed retrospectively. Coronary anatomy was recorded from operative reports using the Leiden classification., Results: An arterial switch operation was performed in 1033 children between 1983 and 2013. Coronary anatomy was normal in 697 patients (67%). The most common type of anomalous coronary anatomy was the circumflex coronary artery arising from sinus 2 (in 152 patients [15%]). Forty-seven patients (4.5%) had all coronary arteries arising from a single sinus. Of these 47 patients, 34 patients (3.3%) had a true single coronary artery. Fifty-two patients (5.0%) had an intramural coronary artery. Overall early mortality was 3.3% (34 out of 1033 patients) over the 30-year period. Early mortality was 3.0% (21 out of 697) for patients with normal coronary anatomy and 3.9% (13 out of 336) for any type of anomalous coronary anatomy. Early mortality was 3.3% (5 out of 152) for patients with the circumflex coronary artery arising from sinus 2, 6.4% (3 out of 47) for patients with all coronary arteries arising from a single sinus, and 5.9% (2 out of 34) for patients with a true single coronary artery. Early mortality for patients with intramural coronaries was 1.9% (1 out of 52). No coronary pattern was found to be a risk factor for mortality., Conclusions: Patients with anomalous coronary artery anatomy had higher rates of early mortality after the arterial switch operation but this was not statistically significant. Coronary artery reoperations were rare., (Crown Copyright © 2020. Published by Elsevier Inc. All rights reserved.)

Background: Pregnancy outcomes after the arterial switch operation (ASO) are rare. We sought to determine outcomes of ASO survivors who underwent pregnancy., Methods: Female patients who had an ASO and underwent pregnancy were identified from the congenital heart disease pregnancy clinic at The Royal Melbourne Hospital. All follow-up data were collected retrospectively by medical record review., Results: Eleven (11) women were identified as having undergone medical care during pregnancy, from the adult congenital database, at The Royal Melbourne Hospital. There were 17 successful pregnancies, and nine women have been followed post pregnancy. Of the 17 successful deliveries, eight were delivered by Caesarean section, seven were vaginal deliveries and two were instrumented vaginal deliveries. Of the eight Caesarean sections, five were emergency and three were elective. The indications for emergency Caesarean section were obstructed labour (n = 2), abnormal cardiotocography (n = 1), obstructed labour and abnormal cardiotocography (n = 1) and congestive cardiac failure (n = 1). There was one neonatal complication (respiratory distress requiring intubation) in a child born at 31 weeks. There were maternal obstetric complications in 10 patients. There were two maternal cardiac complications during pregnancy (heart failure and rapid atrial fibrillation/flutter). There was no change in left ventricular function post-pregnancy. There was progression of severity of neo-aortic valve regurgitation in two patients post pregnancy (trivial to mild and moderate-severe to severe respectively)., Conclusion: Pregnancy post ASO appears to be safe in the majority of women. Maternal cardiac complications are uncommon in patients without residual significant haemodynamic lesions, although maternal obstetric complications may be common., (Crown Copyright © 2019. Published by Elsevier B.V. All rights reserved.)

Objective: To examine the long-term surgical outcomes of patients who underwent truncus arteriosus (TA) repair., Methods: Between 1979 and 2018, a total of 255 patients underwent TA repair at 3 Australian hospitals. Data were obtained by review of medical records from initial admission until last cardiology follow-up., Results: At the time of TA repair, the median patient age was 44 days, and median weight was 3.5 kg. Early mortality was 13.3% (34 of 255), and overall survival was 76.8 ± 2.9% at 20 years. Neonatal surgery and low operative weight were risk factors for early mortality. Most deaths (82.5%; 47 of 57) occurred within the first year following repair. A coronary artery anomaly and early reoperation were identified as risk factors for late mortality. A total of 175 patients required at least 1 reoperation, with overall freedom of reoperation of 2.9 ± 1.5% at 20 years. Follow-up of survivors was 96% complete (191 of 198). The median duration of follow-up was 16.4 years. At the last follow-up, 190 patients were categorized as New York Heart Association class I/II, and 1 patient was class III., Conclusions: TA repair during the neonatal period presents significant surgical challenges. Neonates with signs of overcirculation should be operated on promptly. A coronary artery anomaly is a risk factor for late mortality. Survival beyond the first year following repair is associated with excellent outcomes., (Crown Copyright © 2020. Published by Elsevier Inc. All rights reserved.)

Background: Limited data exist on long-term pulmonary valve function after the Ross procedure. This study sought to determine the long-term function of the pulmonary valve in 443 consecutive adult patients who underwent a Ross procedure., Methods: All 443 patients who underwent a Ross procedure between November 1992 and March 2018 were reviewed retrospectively. All underwent pulmonary valve replacement using a cryopreserved pulmonary allograft. Freedom from the study's outcomes were calculated using Kaplan Meier survival. Risk factors for valve failure were analyzed using Cox regression., Results: Mean age at time of operation was 39 years (range: 15-66 years). There was 1 (0.2%, 1 of 443) operative mortality. Nine patients required reintervention on the pulmonary allograft at a mean 6.1 years (range: 1-12 years) after Ross procedure. Patients required pulmonary allograft reintervention for infective endocarditis (n = 4), severe pulmonary stenosis (n = 4), or severe pulmonary regurgitation (n = 1). Freedom from pulmonary allograft reintervention was 98.9% (95% confidence interval [CI] 97.1%-99.6%), 97.7% (95% CI 95.1%-98.9%), 96.6% (95% CI 93.3%-98.3%), and 96.6% (95% CI 93.3%-98.3%) at 5, 10, 15, and 20 years, respectively. Freedom from pulmonary allograft dysfunction (at least moderate pulmonary regurgitation and/or mean systolic gradient ≥ 25 mm Hg and/or reintervention) was 94.5% (95% CI 91.6%-96.4%), 88.1% (95% CI 83.6%-91.4%), 84.9% (95% CI 79.6%-88.9%), and 78.3% (95% CI 69.5%-84.9%) at 5, 10, 15, and 20 years, respectively. No risk factors were identified to influence pulmonary valve durability., Conclusions: The pulmonary valve allograft gives excellent long-term function when used in adults undergoing the Ross procedure. Reintervention on the pulmonary valve is rare and significant pulmonary allograft dysfunction is uncommon., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: We aim to evaluate the long-term outcomes following repair of truncus arteriosus with an interrupted aortic arch., Methods: We reviewed all children (n = 24) who underwent repair of truncus arteriosus and an interrupted aortic arch between 1979 and 2018 in a single institution. The morphology of the interrupted aortic arch was type A in 5, type B in 18 and type C in 1., Results: The median age at repair was 10 days and the median weight was 3.1 kg. Direct end-to-side anastomosis of the ascending and descending aorta was performed in 16 patients (67%, 16/24), patch augmentation in 5 patients (21%, 5/24) and direct anastomosis with the use of an interposition graft to the descending aorta in 2 patients (8%, 2/24). One patient, the first in the series, underwent interrupted aortic arch repair via subclavian flap aortoplasty prior to truncus repair. A period of deep hypothermic circulatory arrest was used in 16 patients, and isolated cerebral perfusion was used in 8 patients. The early mortality rate was 17% (4 out of 24 patients). There were no late deaths and overall survival was 83 ± 8% [95% confidence interval (CI) 61-93] at 20 years. Freedom from any reoperation was 33 ± 11% (95% CI 14-54) at 5 years and 13 ± 9% (95% CI 2-34) at 10 years. Six patients underwent 10 aortic reoperations. Freedom from aortic arch reoperation was 69 ± 11% (95% CI 42-85) at 10 and 20 years. Follow-up was 95% complete (19/20), with a median follow-up time of 20 years. At last follow-up, no clinically significant aortic arch obstruction was identified in any patient, and all patients were in New York Heart Association Class I/II., Conclusions: Repair of truncus arteriosus with an interrupted aortic arch with direct end-to-side anastomosis results in good survival beyond hospital discharge. Although the long-term functional state of patients is good, reoperation rates are high., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objective: Transposition of the great arteries or Taussig-Bing anomaly with concomitant aortic arch obstruction is uncommon, with limited data on long-term outcomes after arterial switch operation and aortic arch obstruction repair. This study sought to determine outcomes of patients undergoing arterial switch operation and aortic arch obstruction repair at a single institution., Methods: From 1983 to 2015, 844 patients underwent an arterial switch operation for biventricular repair at The Royal Children's Hospital. Eighty-three (9.8%, 83/844) patients underwent an arterial switch operation and aortic arch obstruction repair., Results: Fifty-five (66%, 55/83) patients had transposition of the great arteries. and 28 (34%, 28/83) patients had Taussig-Bing anomaly. Fifty-nine (71%, 59/83) patients underwent arterial switch operation and aortic arch obstruction repair as a single-stage procedure, and 24 (29%, 24/83) patients underwent arterial switch operation and aortic arch obstruction repair as a 2-stage procedure. There were 5 early deaths (6.0%, 5/83). Follow-up was available for 74 (95%) of the 78 survivors. Median follow-up was 13.3 years (interquartile range, 7.3-19.3 years; range, 1-30 years). There were no late deaths. Freedom from reintervention was 77%, 71%, and 68% at 5, 10, and 20 years, respectively. Reintervention was more common compared with patients without aortic arch obstruction (P < .001). Reintervention for right-sided obstruction was more common compared with patients without aortic arch obstruction (P = .006)., Conclusions: Patients with transposition of the great arteries or Taussig-Bing anomaly with associated aortic arch obstruction have a higher reintervention rate, especially for right-sided obstruction. Closer monitoring of this subgroup of patients is warranted., (Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.)

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., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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., (Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.)

The arterial switch operation has excellent early outcomes. Although the majority of patients are growing into healthy adults, a small subset require reoperation for pulmonary stenosis as well as neoaortic root dilatation and neoaortic valve regurgitation, especially as follow-up increases. Challenging subgroups include patients with anomalous coronary anatomy, concomitant aortic arch obstruction, Taussig-Bing anomaly, ventricular septal defect, and low operative weight, and patients with an intact interventricular septum who present late., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: We sought to determine the quality of life after the arterial switch operation (ASO) using the Short Form 36 questionnaire in adult survivors., Methods: All patients (n = 107) who underwent the ASO and were 18 years of age or older living in the Australian state of Victoria with a contact telephone number were identified from the hospital database. Fifty-one (48%) patients were 18-24 years old and 56 (52%) patients were 25-34 years old. Patients completed the Short Form 36 quality of life questionnaire via telephone. The results of the 8 domains of the Short Form 36 questionnaire and the derived health state summary score (Short Form 6-Dimension) were compared against mean scores from age-matched Australian population data., Results: Compared with the Australian population age-matched data, 18- to 24-year-old ASO patients ranked their health higher in 3 of the 8 domains (P < 0.01). The 25-34 age group ranked their health higher in 4 of the 8 domains (P < 0.01). No statistically significant differences in the mean Short Form 6-Dimension scores were observed in the 18-24 age group (0.769 for ASO patients vs 0.772 for Australian population, P = 0.85) or the 25-34 age group (0.795 for ASO patients vs 0.780 for Australian population, P = 0.33)., Conclusions: Young adult survivors of the ASO have similar outcomes to age-matched controls in quality of life measured by Short Form 6-Dimension.

Objectives: Preoperative moderate or greater truncal valve (TV) insufficiency is one of the most important factors influencing mortality in children with truncus arteriosus. We therefore sought to determine the impact of TV insufficiency and concomitant TV surgery on children who underwent truncus arteriosus repair at a single institution., Methods: We reviewed 180 patients who underwent truncus arteriosus repair between 1979 and 2016. Preoperative echocardiography demonstrated TV insufficiency in 80 patients (mild: 33.9%, 61/180; moderate: 9.4%, 17/180 and severe: 1.1%, 2/180)., Results: Twenty-one patients had concomitant TV surgery with an early mortality of 19% (4/21) and overall survival of 70.8 ± 10.1% at 25 years. There were 60 neonates, 11 of whom had concomitant TV surgery with an early mortality of 27% (3/11) and overall survival of 62.3 ± 15.0% at 20 years. Concomitant TV repair (P = 0.5) was not a risk factor for death. TV reoperation was common in those who had concomitant TV surgery, with freedom from reoperation of 19.2 ± 14.9% at 20 years. In the remaining 159 patients, 14 required subsequent TV surgery, and the freedom from TV surgery was 84.0 ± 4.6% at 20 years. At a median follow-up of 18.5 years, TV insufficiency was none or trivial in 79.6% (109/137) and mild or less in 98.5% (135/137) of patients., Conclusions: Most patients with mild TV insufficiency are free from TV surgery up to 25 years. The durability of TV repair is poor. Most patients with moderate or greater TV insufficiency and a quadricuspid TV will require TV surgery.

Background: In 2005 the Department of Cardiothoracic Surgery at The Royal Children's Hospital started an early academic mentoring program for medical students and junior doctors with the aim of fostering an interest in academic surgery., Methods: Between 2005 and 2015, 37 medical students and junior doctors participated in research in the Department of Cardiothoracic Surgery at The Royal Children's Hospital. Each was given an initial project on which to obtain ethics approval, perform a literature review, data collection, statistical analysis, and prepare a manuscript for publication. A search of the names of these former students and doctors was conducted on PubMed to identify publications., Results: A total of 113 journal articles were published in peer-reviewed journals with an average impact factor of 4.1 (range, 1.1 to 19.9). Thirty (30 of 37, 81%) published at least one article. A mean of 4.3 journal articles was published per student or junior doctor (range, 0 to 29). Eleven (11 of 37, 30%) received scholarships for their research. Nine (9 of 37, 24%) have completed or are enrolled in higher research degrees with a cardiothoracic surgery focus. Of these 9, 2 have completed doctoral degrees while in cardiothoracic surgery training. Five will complete their cardiothoracic surgery training with a doctoral degree and the other 2 are pursuing training in cardiology., Conclusions: A successful early academic mentoring program in a busy cardiothoracic surgery unit is feasible. Mentoring of motivated individuals in academic surgery benefits not only their medical career, but also helps maintain high academic output of the unit., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: The surgical indication, timing, strategy, and surgical technique for anatomic correction of congenitally corrected transposition of the great arteries are challenging. We evaluated the long-term results at The Royal Children's Hospital Melbourne., Methods: Review of 32 successive anatomic corrections between 1996 and 2015., Results: Twenty-one double-switch (66%), 6 Senning/Bex-Nikaidoh (19%), and 5 Senning/Rastelli (16%) procedures were performed (median age, 1.9 years). Median follow-up was 5.4 years with 4 deaths and 1 heart transplant. Cumulative incidence of late reoperation was 8%, 29%, and 59% at 1, 5, and 10 years, respectively. Twenty-six patients had full follow-up with native hearts. Nineteen had normal left ventricle (LV) function. Late LV dysfunction, mostly mild, was not related to needing a pacemaker (P = .4) or a pulmonary artery band (PAB) (P = .08). Previous PAB was linked to the need for aortic valve surgery or neoaortic regurgitation moderate or greater (P = .03). Six required Senning revision. The introduction of the Shumacker modification of the Senning has generated stable pulmonary venous pathways. Six patients developed postoperative iatrogenic atrioventricular block dependent on a permanent pacemaker., Conclusions: Anatomic correction is a surgical challenge. It provides excellent functional outcomes in survivors with a significant need for reoperation and a definite risk of death or transplantation. Normal LV function should be expected in most patients. LV dysfunction was not linked to PAB or pacemaker requirement but surgery without LV training had better long-term LV function. The Shumacker modification provided stable venous pathways. Iatrogenic atrioventricular block remains a challenge., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Children with body weight less than 2.5 kg who undergo the arterial switch operation (ASO) represent a challenging group. We sought to determine outcomes of patients with weight less than 2.5 kg at ASO at a single institution., Methods: All patients who underwent an ASO with biventricular repair and weighed less than 2.5 kg at time of surgery were identified from the hospital database and reviewed retrospectively., Results: From 1983 to 2014, 870 patients underwent an ASO with biventricular repair at our institution. At the time of ASO, 31 patients (3.6%, 31 of 870) weighed less than 2.5 kg (mean 2.1; median 2.1; range, 1.1 to 2.4). Twenty-nine patients underwent an ASO for d-transposition of the great arteries, and 2 patients had an ASO for Taussig-Bing anomaly. Mean age at operation was 16 days (median 11; range, 3 to 66). There were 6 hospital deaths (19%, 6 of 31) among patients weighing less than 2.5 kg compared with a hospital mortality of 1.9% (16 of 839) among patients weighing more than 2.5 kg (p < 0.0001). Mortality for children weighing 2.0 kg or less was 50% (5 of 10) compared with a mortality of 2.8% (1 of 21) for children weighing more than 2.0 kg but less than 2.5 kg. Four patients (13%, 4 of 31) required reoperation during hospital admission. Follow-up was available for 24 survivors (96%, 24 of 25). Mean follow-up was 13.2 years (median 11.9; range, 6 months to 25 years). There were no late deaths. Two patients (8%, 2 of 24) required late reoperation. No patient had more than mild neoaortic valve regurgitation, and all survivors were in New York Heart Association class I at last follow-up., Conclusions: Early mortality for children weighing less than 2.5 kg undergoing the ASO remains high; however, most of the mortality occurred in children weighing 2.0 kg or less. Long-term outcomes for survivors are excellent., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Complete vascular rings are rare and cause tracheoesophageal compression. Following surgical division, some patients have persisting tracheomalacia. We aim to assess the long-term outcomes of complete vascular ring division., Methods: All patients (n = 132) who underwent surgical division of a complete vascular ring between 1978 and 2014 were identified from the hospital database and retrospectively reviewed., Results: Complete vascular rings consisted of a double aortic arch (n = 80), right aortic arch with an aberrant subclavian artery and left ligamentum arteriosum (n = 50), right aortic arch with mirror image branching and left ligamentum arteriosum (n = 1), and a left aortic arch with right descending aorta and right ligamentum arteriosum (n = 1). Kommerell's diverticulum was identified in 10 patients. Preoperative tracheomalacia was identified via bronchoscopy in 25 patients. Concomitant tracheal reconstruction was not performed in any patient. Kommerell's diverticulum was resected in 1 patient. The hospital mortality rate was 1.5% (2/132). There were no late deaths. The overall survival rate was 98.3 ± 1.2% (95% CI: 93.4, 99.6) at 20 years. Postoperatively, persistent tracheal compression was reported in 3 patients, and tracheomalacia in 16 patients. The rate of freedom from reoperation was 88.6 ± 4.0% (95% CI: 77.9, 94.3) at 20 years. No patient required tracheal surgery during the follow-up period. Follow-up was 92% (121/132) complete, with a median follow-up of 11.4 years (range 44 days to 36 years). At the last follow-up, 7 patients had mild tracheomalacia., Conclusions: Outcomes of division of a complete vascular ring are excellent. Tracheomalacia often improves following division of the vascular ring. Respiratory symptoms following complete vascular ring division are uncommon., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

We evaluated the long-term outcomes following repair of truncus arteriosus (TA) from a single institution. We conducted a retrospective review of children (n = 171) who underwent TA repair between 1979 and 2014. Early mortality rate was 11.7% (20/171). There were 19 late deaths. Most deaths (74%, 29/39) occurred within the first year following surgery. The 1-year mortality rate in 1979-2004 was 18% (25/136) and decreased to 11% (4/35) in 2005-2014. The overall survival rate was 73.6% at 30 years. Multivariate analysis identified postoperative extracorporeal membrane oxygenation (P = 0.003), operative weight <2.5kg (P = 0.012), prior surgical intervention (P = 0.018), and coronary artery anomaly (P = 0.037) as risk factors for early mortality. A Cox regression model identified DiGeorge syndrome (P = 0.008) as a risk factor for late mortality. Freedom from right ventricular outflow tract reoperation was 4.6% at 20 years. Concomitant truncal valve (TV) repair or replacement was undertaken in 20 patients. Additionally, 14 patients underwent late TV repair or replacement. The overall survival rate in patients who underwent TV operation was 76.9% at 20 years. A total of 19 patients had concomitant interrupted aortic arch with a survival rate of 89.5% at 20 years. Median follow-up was 19 years (mean = 17 years, range: 1-34 years). All patients were in New York Heart Association Class I/II at last follow-up. Following repair of TA, patients had good long-term functional status but had high reoperation rates. Repair of interrupted aortic arch and TV were not risk factors for mortality. Postoperative extracorporeal membrane oxygenation, operative weight <2.5kg, prior surgical intervention, and coronary artery anomaly were risk factors for early death. DiGeorge syndrome was associated with late death, most commonly from infection., (Crown Copyright © 2016. Published by Elsevier Inc. All rights reserved.)

Background: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect with limited data on long-term outcomes after surgical intervention., Methods: We conducted a retrospective review of all children (N = 42) who underwent surgical repair of ALCAPA between 1980 and 2014 at the Royal Children's Hospital, Melbourne., Results: Twenty-nine (69% [29 of 42]) patients underwent coronary reimplantation, 12 (29% [12 of 42]) had intrapulmonary baffle (Takeuchi) repair, and 1 (2% [1 of 42]) patient had ligation of the anomalous coronary artery. Nine (21%, 9 of 42) patients had concomitant mitral valve (MV) repair at the time of ALCAPA repair. A left ventricular assist device (LVAD) was used in 36% (15 of 42) of patients. Early mortality was 2.4% (1 of 42 patients). Median follow-up was 14 years (mean, 13 years; range, 4 months-31 years). There were no late deaths. Survival was 98% at 20 years. Freedom from reoperation was 81%, 81%, and 76% at 5, 10, and 20 years after operation, respectively. Eight patients underwent late MV repair or replacement at a median of 3 years (mean, 8 years; range, 2 months-25 years) after operation. Freedom from late MV repair or replacement was 86% at 5 and 10 years and 81% at 20 years after operation. Eleven (26% [11 of 42]) patients had severe mitral regurgitation (MR) preoperatively. Of those 11 patients, 5 (45% [5 of 11]) had concomitant MV repair at the time of ALCAPA repair, 3 (27% [3 of 11]) had late MV repair or replacement, and the remaining 3 (27% [3 of 11]) patients had mild MR at last follow-up. Thirty-six (90% [36 of 41]) patients had normal left ventricular function and 4 (10% [4 of 41]) patients had mildly reduced left ventricular (LV) function at last follow-up., Conclusions: ALCAPA can be operated on with good outcomes. Persistent MR and a moderate rate of late MV repair warrants close follow-up., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Intramural coronary arteries may complicate coronary artery transfer during the arterial switch operation. We sought to determine the long-term outcomes of 28 patients with intramural coronary arteries who underwent an arterial switch operation at a single institution., Methods: All patients who had intramural coronary arteries and underwent an arterial switch operation were identified from the hospital database and retrospectively reviewed., Results: From 1983 to 2009, 720 patients underwent an arterial switch operation at our institution. Twenty-eight (3.9%, 28 of 720) had intramural coronary arteries. Patients with intramural coronary arteries had transposition of the great arteries (96%, n = 27) or Taussig-Bing anomaly (4%, n = 1). There were no deaths. Follow-up was 100% complete. Mean follow-up was 16.3 years (median, 15.5 years; range, 5.6 to 26.9 years). No patient required reoperation or catheter reintervention on the coronary arteries. Freedom from reoperation was 93% at 10 years. No patient had more than mild aortic regurgitation at last follow-up. Nine (32%, 9 of 28) patients had coronary angiograms at median 16 months (range, 14 months to 17 years) after arterial switch operation. All patients were asymptomatic at the time of angiogram. One patient had mild stenosis of the circumflex coronary artery demonstrated on a routine coronary angiogram 14 months postoperatively. All 28 patients were asymptomatic and in New York Heart Association functional class I at last follow-up., Conclusions: Patients with intramural coronary arteries are not at increased risk of death or coronary reinterventions and have excellent late outcomes after the arterial switch operation., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)