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Background: Single ventricle palliation may be performed in patients with congenitally corrected transposition of the great arteries due to hypoplasia of 1 ventricle or anatomic complexity rendering biventricular repair unfeasible. There have been only a few small studies of the outcomes of single ventricle palliation in the setting of congenitally corrected transposition of the great arteries., Methods: A multicenter, international, retrospective cohort study of patients with congenitally corrected transposition of the great arteries undergoing single ventricle palliation was conducted in 29 tertiary hospitals in 6 countries from 1990 to 2018., Results: A total of 194 patients with congenitally corrected transposition of the great arteries underwent single ventricle palliation. A functionally univentricular heart was present in 62.4% (121/194). Patients with 2 good-sized ventricles had more laterality defects (50.7% vs 28.1%, P = .002). Fontan completion was achieved in 80.3% (155/194). A tricuspid valve surgery was performed in 9.3% (18/194). Survival was 97.4% (95% CI, 92.1-99.2) at 15 years in the univentricular cohort and 89.8% (95% CI, 78.0-95.5) at 15 years in those with 2 adequate ventricles (P = .05). At last follow-up, 11.5% of patients had heart failure, whereas 6.0% had moderate or greater systemic right ventricle dysfunction and 8.4% exhibited moderate or greater tricuspid regurgitation. In multivariable analysis, aortic coarctation or hypoplasia (hazard ratio, 7.7; P = .005) was associated with mortality., Conclusions: Single ventricle palliation in patients with congenitally corrected transposition of the great arteries is associated with excellent long-term survival and low rates of heart failure and atrioventricular valve failure. In patients who would require complex surgery to achieve a biventricular repair, single ventricle palliation appears to be a good alternative., 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., (Copyright © 2024 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

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: In adults, the Ross procedure provides an excellent alternative to prosthetic valves, but it is underutilized because of concerns about technical complexity, durability, and perceived high late reoperation rates. The inclusion technique stabilizes the aortic root, prevents dilatation, and respects the dynamic root physiology. Long-term outcomes of the Ross procedure with the inclusion cylinder technique (1992-2022) are reported., Methods: Long-term (12.2 years; 95% CI, 11.5-12.8) single institution results, with more than 25 years of follow-up in 44 patients. A total of 516 patients aged 39 ± 13 years underwent Ross procedure with inclusion cylinder and annular reduction. All had yearly clinical review and biannual transthoracic echocardiogram. The etiology of valve disease comprised aortic stenosis (AS) (n = 233 [44.8%]), aortic regurgitation (AR) (n = 179 [34.4%]), and mixed AS/AR (n = 108 [20.8%]). The primary end points were 25-year survival and freedom from reoperation, with degree of autograft regurgitation in all morphologies (AS, AR, and mixed AR/AS) as the secondary end point., Results: Total reoperation rate was 8.3% after 25 years (n = 43; aortic valve = 28, pulmonary valve 15), with early reoperation (<1 year) in 0.6% and early postoperative death in 0.2% following myocardial infarction. Overall, 25-year survival was 85.3%, and 25-year freedom from autograft reoperation was 89.5% (AS = 95.0%, AR = 78.0%, and mixed AS/AR = 94.3%) (P = .01). Freedom from redo pulmonary valve replacement was 92.7%., Conclusions: The Ross procedure with inclusion cylinder technique provides excellent hemodynamics. Autograft inclusion in the native aorta minimizes prosthetic material and respects physiological root changes, reducing shear stress on neoaortic cusps and increasing durability. Long-term reoperation rate is low in experienced centers., 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., (Copyright © 2025 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Coarctation of the aorta (CoA) is one of the most common types of congenital heart disease. Unfortunately, there is a high prevalence of hypertension and late cardiovascular mortality in patients with CoA despite successful repair. The growing impact of acquired cardiovascular disease remains a significant concern as the adult congenital heart disease population continues to rapidly expand and age. This review aims to explore (1) the determinants of vascular health and atherosclerosis including endothelial dysfunction and vascular wall abnormalities, (2) the prevalence of atherosclerosis and associated sequelae in repaired CoA including coronary artery disease, coronary artery calcium, aortic calcium, stroke, and peripheral artery disease, and (3) the contributing factors specific to CoA. This review aims to guide optimization of long-term cardiovascular health to ultimately reduce mortality and morbidity in this young high-risk population., Competing Interests: The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Melissa G. Y. Lee reports financial support was provided by 10.13039/501100000925National Health and Medical Research Council. Melissa G. Y. Lee reports financial support was provided by 10.13039/501100001232The Royal Australasian College of Physicians. Melissa G. Y. Lee reports financial support was provided by Avant Mutual Group Ltd. Anoop N. Koshy reports financial support was provided by 10.13039/501100000925National Health and Medical Research Council. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors.)

Aim: Heart transplantation (HT) in patients with failing univentricular circulation is often challenging. This is compounded by the ever-increasing number of patients with prior Norwood-type reconstruction of the aorta, large aortic root, and often dense adhesions from multiple prior operations. We aimed to elucidate differences in outcomes of HT in patients with prior univentricular palliations, with and without prior Norwood-type aortic arch reconstruction (ArchRec)., Methods: All patients who underwent HT for failed univentricular palliation during the 1990-2022 period were included in the study., Results: Of 45 patients, 18 had undergone ArchRec. Hospital mortality improved in the recent era (17.4% before 2006 vs 0% after 2006; p=0.11), despite a higher proportion of patients with ArchRec (17.4% before 2006 vs 60.8% after 2006, p=0.002). Patients with ArchRec had a higher number of prior cardiac surgeries (4.1±1.5 vs 3.2±1.3, p=0.04), longer cardiopulmonary bypass time (320±23 vs 242±21 min, p=0.02), more concomitant arch reconstruction (33.3% vs 0%, p=0.02), greater need for post-HT extracorporeal membrane oxygenation (33.3% vs 3.7%; p=0.01) and longer hospital stay (37.1±30.5 days vs 23.6±11.8 days, p=0.04). Freedom from death or retransplantation for all patients was 91%, 73%, 67%, and 53% at 1, 5, 10, and 15-years, respectively. Prior ArchRec, Fontan procedure, and earlier eras were not risk factors for death., Conclusions: The outcomes of HT after univentricular palliation have improved in recent times and low operative mortality can be achieved. Despite increased complexity, good similar outcomes can be achieved in patients with and without prior arch reconstruction regardless of the palliation stage., Competing Interests: Conflict of Interest Statement The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Crown Copyright © 2024. Published by Elsevier B.V. All rights reserved.)

Objectives: The optimal treatment strategy for symptomatic young infants with tetralogy of Fallot (TOF) is unclear. We sought to compare the outcomes of staged repair (SR) (shunt palliation followed by second-stage complete repair) versus primary repair (PR) at 2 institutions that have exclusively adopted each strategy., Methods: We performed propensity score-matched comparison of 143 infants under 4 months of age who underwent shunt palliation at one institution between 1993 and 2021 with 122 infants who underwent PR between 2004 and 2018 at another institution. The primary outcome was mortality. Secondary outcomes were postoperative complications, durations of perioperative support and hospital stays, and reinterventions. Median follow-up was 8.3 years (interquartile range, 8.1-13.4 years)., Results: After the initial procedure, hospital mortality (shunt, 2.8% vs PR, 2.5%; P  = .86) and 10-year survival (shunt, 95%; 95% confidence interval [CI], 90%-98% vs PR, 90%; 95% CI, 81%-95%; P  = .65) were similar. The SR group had a greater risk of early reinterventions but similar rates of late reinterventions. Propensity score matching yielded 57 well-balanced pairs. In the matched cohort, the SR group had similar freedom from reintervention (55%; 95% CI, 39%-68% vs 59%; 95% CI, 43%-71%; P  = .85) and greater survival (98%; 95% CI, 88%-99.8% vs 85%; 95% CI, 69%-93%; P  = .02) at 10 years, as the result of more noncardiac-related mortalities in the PR group., Conclusions: In symptomatic young infants with TOF operated at 2 institutions with exclusive treatment protocols, the SR strategy was associated with similar cardiac-related mortality and reinterventions as the PR strategy at medium-term follow-up., Competing Interests: Dr Brizard 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. on behalf of The American Association for Thoracic Surgery.)

Objective: Use of a valved Sano during the Norwood procedure has been reported previously, but its impact on clinical outcomes needs to be further elucidated. We assessed the impact of the valved Sano compared with the nonvalved Sano after the Norwood procedure in patients with hypoplastic left heart syndrome., Methods: We retrospectively reviewed 25 consecutive neonates with hypoplastic left heart syndrome who underwent a Norwood procedure with a valved Sano conduit using a femoral venous homograft and 25 consecutive neonates with hypoplastic left heart syndrome who underwent a Norwood procedure with a nonvalved Sano conduit between 2013 and 2022. Primary outcomes were end-organ function postoperatively and ventricular function over time. Secondary outcomes were cardiac events, all-cause mortality, and Sano and pulmonary artery reinterventions at discharge, interstage, and pre-Glenn time points., Results: Postoperatively, the valved Sano group had significantly lower peak and postoperative day 1 lactate levels (P = .033 and P = .025, respectively), shorter time to diuresis (P = .043), and shorter time to enteral feeds (P = .038). The valved Sano group had significantly fewer pulmonary artery reinterventions until the Glenn operation (n = 1 vs 8; P = .044). The valved Sano group showed significant improvement in ventricular function from the immediate postoperative period to discharge (P < .001). From preoperative to pre-Glenn time points, analysis of ventricular function showed sustained ventricular function within the valved Sano group, but a significant reduction of ventricular function in the nonvalved Sano group (P = .003). Pre-Glenn echocardiograms showed competent conduit valves in two-thirds of the valved Sano group (n = 16; 67%)., Conclusions: The valved Sano is associated with improved multi-organ recovery postoperatively, better ventricular function recovery, and fewer pulmonary artery reinterventions until the Glenn procedure., (Copyright © 2023 The American Association for Thoracic Surgery. 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.)

Peripheral veno-artertial extracorporeal membrane oxygenation (VA-ECMO) is commonly used in the paediatric population for intractable respiratory and cardiac failure. One of the devastating complications of VA-ECMO is severe brain damage due to ischemia or haemorrhage. We describe a case of peripheral cervical VA-ECMO complicated by evolving right cerebral ischemia which was rescued with rapid conversion from peripheral to central VA-ECMO support. Notably, the patient had a complete circle of Willis. Following conversion, we observed complete resolution of neurological symptoms with full functional recovery., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: The Bex-Nikaidoh operation can effectively relieve left ventricular outflow tract obstruction. However, if a conduit is used for right ventricular outflow tract reconstruction, a late reoperation can be anticipated. We examined the impact of double root translocation on outcomes., Methods: We performed a retrospective single-centre study of patients who underwent aortic root translocation between 2006 and 2019., Results: Aortic root translocation was performed in 23 patients at a median age of 1.6 years [interquartile range (IQR) 0.9-2.5]. Concomitant repairs were done in 52.2% of patients (12/23) including the Senning atrial switch in 34.8% (8/23). The right ventricular outflow tract was reconstructed with valved conduits in 39.1% (9/23), direct anastomoses in 4.35% (1/23) and pulmonary autografts in 56.5% of patients (13/23). Aortic cross-clamp time was significantly longer in patients with double root translocation [308 min (IQR 270-259) vs 209 min (IQR 179-281), P = 0.02]; 2 patients in this group required temporary mechanical circulatory support. There were no early deaths. Median follow-up time was 7.5 years (IQR 3.3-10.5). The estimated 10-year survival was 90% [95% confidence interval (CI): 47.3%, 98.5%]. There was no recurrent left ventricular outflow tract obstruction. Freedom from any reoperation was 64.2% (95% CI: 40.8%, 80.3%) at 3 years and 44.5% (95% CI: 21.2%, 65.5%) at 6 years. The main indication for late reoperation was conduit degeneration. Freedom from a right ventricular outflow tract reoperation was significantly higher, and the number of reoperations per patient was lower when a double root translocation had been performed (P = 0.03)., Conclusions: The Bex-Nikaidoh operation effectively relieved left ventricular outflow tract obstruction. A double root translocation further increased procedural complexity but was associated with better mid-term freedom from a right ventricular outflow tract reoperation. It should be considered in suitable patients., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objective: This study evaluates the long-term results of tetralogy of Fallot repair and assesses the risk factors for adverse outcomes., Methods: This retrospective study included 960 patients who underwent transatrial transpulmonary tetralogy of Fallot repair between 1990 and 2020., Results: A transannular patch was placed in 722 patients, and pulmonary valve preservation was achieved in 233 patients. The median age at tetralogy of Fallot repair was 9.4 (interquartile range, 6.2-14.2) months. The median follow-up duration was 10.6 (interquartile range, 5.4-16.3) years. There were 8 early deaths (0.8%) and 20 late deaths (2.1%). Genetic syndrome and pulmonary valve annulus Z score less than -3 were risk factors for mortality. The survival was 97.7% (95% confidence interval, 96.4-98.5) and 94.5% (95% confidence interval, 90.9-96.7) at 10 and 30 years, respectively. Freedom from any reoperation was 86.4% (95% confidence interval, 83.6-88.7) and 65.4% (95% confidence interval, 59.8-70.4) at 10 and 20 years, respectively. Postoperative right ventricular outflow tract peak gradient of 25 mm Hg or greater correlated with reoperation. Propensity score-matched analysis demonstrated that freedom from pulmonary valve replacement at 15 years was higher in the pulmonary valve preservation group compared with the transannular patch group (98.2% vs 78.4%, P = .004). Freedom from reoperation for right ventricular outflow tract obstruction at 15 years was lower in the pulmonary valve preservation group compared with the transannular patch group (P = .006)., Conclusions: The long-term outcomes of tetralogy of Fallot repair are excellent. A postoperative right ventricular outflow tract peak gradient less than 25 mm Hg appears to be optimal to prevent reoperation. If the pulmonary valve size is suitable, pulmonary valve preservation reduces the risk of pulmonary valve replacement, yet increases the reoperation rate for right ventricular outflow tract obstruction., (Crown Copyright © 2023. Published by Elsevier Inc. All rights reserved.)

There is limited data on the outcomes of children who undergo surgery for aortic valve infective endocarditis (IE), and the optimal surgical approach remains controversial. We investigated the long-term outcomes of surgery for aortic valve IE in children, with a particular focus on the Ross procedure. A retrospective review of all children who underwent surgery for aortic valve IE was performed at a single institution. Between 1989 and 2020, 41 children underwent surgery for aortic valve IE, of whom 16 (39.0%) underwent valve repair, 13 (31.7%) underwent the Ross procedure, 9 (21.9%) underwent a homograft root replacement, and 3 (7.3%) underwent a mechanical valve replacement. Median age was 10.1 years (interquartile range, 5.4-14.1). The majority of children (82.9%, 34/41) had underlying congenital heart disease, while 39.0% (16/41) had previous heart surgery. Operative mortality was 0.0% (0/16) for repair, 15.4% (2/13) for the Ross procedure, 33.3% (3/9) for homograft root replacement, and 33.3% (1/3) for mechanical replacement. Survival at 10 years was 87.5% for repair, 74.1% for Ross, and 66.7% for homograft (P > 0.05). Freedom from reoperation at 10 years was 30.8% for repair, 63.0% for Ross, and 26.3% for homograft (P = 0.15 for Ross vs repair, P = 0.002 for Ross vs homograft). Children undergoing surgery for aortic valve IE have acceptable long-term survival, although the need for long-term reintervention is significant. The Ross procedure appears to be the optimal choice when repair is not feasible., (Crown Copyright © 2023. Published by Elsevier Inc. All rights reserved.)

When the options of aortic valve repair or the Ross procedure are not feasible or have been exhausted, mechanical aortic valve replacement (AVR) may provide a reliable and structurally durable alternative, but with the limitations of long-term anticoagulation, thrombosis risk and lack of valve growth potential. In this article, we review the longitudinal outcomes of mechanical AVR in children in our institution and compare them to those recently reported by others. From 1978 to 2020, 62 patients underwent mechanical AVR at a median age of 12.4 years (interquartile range (IQR): 8.6-16.8 years). The most common underlying diagnoses were: conotruncal anomalies (40%, 25/62), congenital aortic stenosis (16%, 10/62), rheumatic valve disease (16%, 10/62), connective tissue disease (8.1%, 5/62) and infective endocarditis (6.5%, 4/62). Thirty-two patients (52%, 32/62) had at least 1 prior aortic valve surgery prior to mechanical AVR. Early death was 3.2% (2/62). Median follow-up was 14.4 years (IQR: 8.4-28.2 years). Kaplan-Meier survival was 96.8%, 91.9%, 86.3%, and 81.9% at 1, 5, 10, and 20 years. On competing risk analysis, the proportion of patients alive without aortic valve reoperation at 1, 5, 10, and 20 years was 95.2%, 87.0%, 75.5% and 55.4%, respectively, while the proportion of patients that had aortic valve reoperation (with death as a competing event) at 1, 5, 10, and 20 years was 1.6%, 4.9%, 12.8%, and 28.5%, respectively. In conclusion, when the options of aortic valve repair or the Ross procedure are not feasible in children, mechanical AVR is an alternative, yet the long-term rates of mortality and need for aortic valve reoperation are of concern., Competing Interests: Declaration of competing interest A/Prof Brizard serves on the advisory board of Admedus., (Crown Copyright © 2023. Published by Elsevier Inc. All rights reserved.)

Background: Aortic valve repair and the Ross procedure are widely used in children; however, it is unclear which provides the best outcomes., Methods: Patients who underwent primary aortic valve surgery from 1980 to 2018 were included. Propensity score matching was performed to adjust for baseline differences., Results: Of 415 children, 82.7% (343/415) underwent repair and 17.3% (72/415) underwent the Ross procedure. At 15 years, survival was higher for aortic valve repair (93.9% ± 1.8% vs 80.9% ± 6.4%, P = .04); freedom from reoperation (45.7% ± 4.9% vs 48.5% ± 9.0%, P = .29) did not differ, and freedom from aortic valve reoperation was higher in the Ross procedure group (45.7% ± 4.9% vs 70.7% ± 8.0%, P < .001). When analyzed by quality of repair, acceptable repair provided the highest survival (P = .01). Acceptable repair and the Ross procedure had similar freedom from reoperation at 15 years, whereas suboptimal repair performed worse (acceptable: 54.9% ± 6.7%; Ross procedure: 48.5% ± 9.0%; suboptimal: 27.0% ± 7.7%, P < .001). Acceptable repair and the Ross procedure had similar freedom from aortic valve reoperation at 15 years, whereas suboptimal repair showed worse results (acceptable: 54.9 ± 6.7; Ross procedure: 70.7% ± 8.0%; suboptimal: 27.0% ± 7.7%, P < .001). Propensity score matching paired 66 patients who underwent the Ross procedure with 198 patients who underwent repair. At 15 years, repair was associated with better survival (98.0% ± 1.2% vs 78.5% ± 7.2%, P = .03), whereas freedom from reoperation was similar (42.6% ± 7.6% vs 50.7% ± 9.8%, P = .50). However, the Ross procedure was associated with higher freedom from aortic valve reoperation (42.6% ± 7.6% vs 72.3% ± 8.5%, P = .002)., Conclusions: Primary aortic valve repair was associated with better survival than the Ross procedure, whereas overall freedom from reoperation was similar. When an acceptable intraoperative result was achieved, outcomes of repair were favorable. However, when the intraoperative result of repair was suboptimal, the Ross procedure showed better results., (Crown Copyright © 2023. Published by Elsevier Inc. All rights reserved.)

Background: Various surgical techniques are utilized for reconstructing hypoplastic pulmonary arteries (PAs) in patients with conotruncal anomalies and at times, may be susceptible to restenosis and reoperation. We reviewed our experience with a simple technique of T-shaped remodeling of the PA bifurcation., Methods: Between 2005 and 2019, 31 patients underwent T-remodeling of central PAs by a single cardiac surgeon. The PA bifurcation was opened cranially, and the opening was augmented with an oval-shaped patch effectively transforming the V-shaped bifurcation into a T-shaped bifurcation. Both origins of the PAs were enlarged, even in the instance of single PA origin stenosis., Results: Median age at time of T-remodeling was 17 months (range: 7 weeks to 14 years). The following cardiac morphologies were observed: tetralogy of Fallot (n = 12, 39%), pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (n = 8, 26%), truncus arteriosus (n = 6, 19%), pulmonary atresia with VSD (n = 3, 9.7%), and transposition of the great arteries (n = 2, 6.5%). Thirteen patients (42%) had previous central shunt, and eight patients (26%) had previous modified Blalock-Taussig shunt. There were no operative mortalities. Immediately after T-remodeling, echocardiographic estimates of right ventricle to PA gradient decreased from 42 [interquartile range 28-58] mm Hg to 20 [12-36] mm Hg ( P  = .03). Freedom from reoperation on the PA bifurcation for the entire cohort was 100% at one year, 88% (95% CI 68%-96%) at five years and 82% (57%-93%) at ten years., Conclusions: T-remodeling for PA origin stenosis is a safe procedure with excellent freedom from reoperation that is easily reproducible and applicable to patients with all cardiac morphologies., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: To investigate the outcomes of surgery in children with paravalvular abscess at our institution., Methods: A retrospective review of all patients who underwent surgery for paravalvular abscess was performed., Results: Between 1989 and 2020, 30 patients underwent surgery for paravalvular abscess, of whom 5 (16.7%) had an intracardiac fistula and 6 (20.0%) had a pseudoaneurysm. Aortic annulus abscesses were most common, occurring in 23 patients (76.7%). Aortic root replacement was performed in 17 patients (56.7%), root reconstruction was performed in 4 (13.3%), and reconstruction of the central fibrous body was required in 5 (16.7%). Postoperatively, 7 patients (23.3%) required extracorporeal membrane oxygenation (ECMO) support, and 1 patient (3.3%) required permanent pacemaker insertion. There were 6 early deaths, 5 of whom were on ECMO, and no late deaths, with a 15-year survival of 79.7% (95% confidence interval [CI], 60.2%-90.3%). Deaths were from sudden cardiac arrest resulting in brain death in 3 patients, inability to wean from ECMO due to severe cardiac dysfunction in 2 patients, and cerebral mycotic aneurysm and hemorrhage in 1 patient. Freedom from reoperation was 40.0% (95% CI, 17.0%-62.3%) at 15 years Reoperation due to recurrence was rare, occurring in only 2 patients (6.7%). Streptococcus pneumoniae (hazard ratio [HR], 9.2; 95% CI, 1.6-51.7) and preoperative shock (HR, 6.4; 95% CI, 1.3-32.0) were associated with mortality. Central fibrous body reconstruction was associated with reoperation (HR, 4.4; 95% CI, 1.2-16.1)., Conclusions: Although paravalvular abscess in children is associated with high early mortality, hospital survivors have good long-term survival. Reoperation is frequent, but is rarely due to recurrence of endocarditis., Competing Interests: 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., (© 2023 Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery.)

Repair of tetralogy of Fallot (TOF) can be complicated by the presence of an anomalous coronary artery (ACA) crossing the right ventricular outflow tract (RVOT). This study sought to evaluate the late outcomes of a policy of transatrial-transpulmonary repair for this condition. The transatrial-transpulmonary approach was used in 864 consecutive TOF repairs between 1993 and 2018 at a single institution, of which 55 (6%) patients had an ACA. Nineteen (35%,19/55) patients underwent prior palliation. Late survival and freedom from reoperations were compared with the general cohort of 809 patients who underwent complete repair during the same period. Early mortality was 2% (1/55). Median follow-up was 15.6 years. Late mortality was 6% (3/54). Absence of a preoperative diagnosis of ACA was not a risk factor for worse outcomes in terms of late re-interventions, acute coronary syndrome, residual RVOT gradient, and late mortality. Survival was 91% (95% confidence interval [CI]: 77-96%) at 20 years and was comparable to the general TOF cohort (95%, 95% CI: 90-98%, P = 0.12). Actuarial freedom from any re-intervention was 46% (95% CI: 27-62%) at 20 years, which was also comparable to the general cohort (31%, 95% CI: 20-42%, P = 0.19). The presence of an ACA does not appear to affect late survival or re-intervention rates in patients undergoing transatrial-transpulmonary repair of TOF., (Crown Copyright © 2022. Published by Elsevier Inc. All rights reserved.)

Objective: To describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD)., Methods: Retrospective chart review for all patients who underwent surgery for EA or TVD during the index hospitalization after birth at our institution from January 2005 to February 2023., Results: Fifteen symptomatic neonates and infants who had surgery for EA or TVD were included, 8 with EA and 7 with TVD. Eleven patients (73%) and 3 patients (20%) required preoperative inotropes and extracorporeal membrane oxygenation, respectively. Nine patients (60%) had a Starnes procedure and 6 patients (40%) had tricuspid valve repair (TVr). Mortality at last follow-up was 27% overall (n = 4/15), 22% after Starnes (n = 2/9) and 33% after TVr (n = 2/6), without a significant difference despite a greater-risk profile in the Starnes group. Postoperative day 1 lactate level was associated with mortality on Cox regression (hazard ratio, 1.45; P  = .01). Three of 9 patients who had a Starnes procedure were or will be converted to a cone repair (1.5/2-ventricle repair)., Conclusions: Mortality after surgery for EA or TVD during the index hospitalization after birth is still significant in the current era and is associated with a greater lactate level at postoperative day 1. The Starnes procedure and TVr had comparable outcomes despite a greater-risk profile in the Starnes group. An initial single-ventricle approach does not preclude conversion to biventricular or 1.5-ventricle repair., Competing Interests: 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., (© 2023 The Author(s).)

Background: The results of the Kawashima operation are incompletely defined. Furthermore, optimal timing of Kawashima operation, an important consideration when managing desaturated young infants awaiting surgery, remains unclear. We reviewed our outcomes of Kawashima operation, with a focus on the impact of age., Methods: We conducted a retrospective review of patients who underwent Kawashima operation from 1990 to 2020., Results: Thirty patients underwent Kawashima operation at a median age of 11.7 months (interquartile range, 4.4-27.4). Left isomerism was present in 27 patients (90%). There were no early deaths. There were 2 patients (6.7%) who had Kawashima takedown, both attributed to hypoxia. Fontan completion was achieved in 25 patients (83%). Overall freedom from death and transplantation at 20 years was 67% (95% CI, 32%-87%). Freedom from death and transplantation at 10 years was 100% for children 3 to 6 months of age at the time of Kawashima, compared with 86.4% for older children (P = 1.0). However, children aged 3 to 6 months had longer ventilation time (P = .01), intensive care stays (P = .03), and hospital stays (P = .05). Freedom from death or transplantation at 20 years among those who had concomitant common atrioventricular valve repair was 33%, vs 79% for those who did not undergo common atrioventricular valve repair (P = .02)., Conclusions: Kawashima operation can be performed with low operative risk and acceptable long-term outcomes. Performing Kawashima operation on a patient aged less than 6 months does not affect survival, but is associated with increased morbidity. Need for common atrioventricular valve operation carries significant risk of mortality, and more effective techniques for atrioventricular valve repair are required., (Crown Copyright © 2023. Published by Elsevier Inc. All rights reserved.)

Background: Patients with aortic atresia have the worst prognosis in the spectrum of hypoplastic left heart syndrome. It remains unknown whether patients with aortic atresia continue to do poorly after Fontan operation. This study aimed to determine the association between aortic atresia and atrioventricular valve (AVV) function and clinical outcomes after Fontan operation in patients with hypoplastic left heart syndrome., Methods: We performed a retrospective study of 1731 patients who survived the Fontan operation from the Australia and New Zealand Fontan Registry between 1975 and 2020., Results: We identified 188 patients with hypoplastic left heart syndrome, including 99 (53%) with aortic atresia. Overall transplant-free survival and freedom from failure of Fontan circulation at 15 years was 91% (95% CI, 86%-96%) and 79% (95% CI, 71%-88%), respectively. The cumulative incidence of AVV operation at 15 years of age for patients with aortic atresia and aortic stenosis was 28% (95% CI, 19%-38%) and 14% (95% CI, 7%-22%; P = .03), respectively. The cumulative incidence of AVV failure (moderate or greater regurgitation or AVV operation) at 15 years of age for patients with aortic atresia and aortic stenosis was 50% (95% CI, 37%-61%) and 30% (95% CI, 19%-42%; P = .01). Patients with AVV failure were at increased risk of having moderately, or worse, decreased systolic ventricular function (odds ratio 6.7; 95% CI, 1.7-33; P = .01) and failure of Fontan circulation (hazard ratio 3.7; 95% CI, 1.5-9.1; P < .01)., Conclusions: In patients with hypoplastic left heart syndrome, there is no significant difference in transplant-free survival after Fontan operation between patients with aortic atresia and patients with aortic stenosis. However, patients with aortic atresia have a much higher burden of AVV failure than patients with aortic stenosis. Atrioventricular valve failure is associated with failure of Fontan circulation., (Crown Copyright © 2023. Published by Elsevier Inc. All rights reserved.)

Background: The optimal management strategy for symptomatic young infants with tetralogy of Fallot (TOF) is yet to be determined. We aimed to evaluate the long-term outcomes of a staged approach with initial shunt palliation followed by complete repair., Methods: Between January 1993 and July 2021, 160 children with TOF underwent a systemic-to-pulmonary shunt at our institution, including 65 neonates (41%). The mean duration of follow-up was 12.3 ± 8.1 years., Results: Hospital mortality was 3% (4 of 160), all occurring in patients with a shunt size-to-weight ratio ≥1.2 mm/kg. Composite morbidity-defined as cardiac arrest, postoperative mechanical circulatory support, or unplanned reoperation-occurred in 21% (33 of 160). On multivariable analysis, a shunt size-to-weight ratio ≥1.2 mm/kg and prematurity were independent predictors of composite morbidity. Interstage mortality was 3% (4 of 156). A limited transannular patch was used in 75% (113 of 150) of TOF repairs. Actuarial survival at 20 years after shunt was 90% (95% confidence interval [CI], 79%-95%). Actuarial freedom from reinterventions at 20 years after TOF repair was 40% (95% CI, 28%-52%). Neonates had comparable composite morbidity, mortality, and late risk of reinterventions to older children., Conclusions: Staged repair of TOF in symptomatic young infants results in low mortality but high rates of reinterventions at long-term follow-up. A shunt size-to-weight ratio ≥1.2 mm/kg is a significant risk factor for mortality and morbidity prior to complete repair. Neonates undergoing shunt insertion have comparable outcomes to older children., (Crown Copyright © 2022. Published by Elsevier Inc. All rights reserved.)

Background: It has been proposed that delaying the Ross procedure to later in childhood, allowing autograft stabilisation and placement of a larger pulmonary conduit, may improve outcomes. However, the effect of age at the time of Ross procedure on outcomes remains unclear., Methods: All patients who underwent the Ross procedure between 1995 and 2018 were included in the study. Patients were divided into four groups: infants, age 1 to 5 years, age 5 to 10 years and age 10 to 18 years., Results: A total of 140 patients underwent the Ross procedure in the study period. Early mortality was 23.3% (7/30) for infants compared to 0% for older children (p<0.001). Survival at 15 years was significantly lower in infants (76.3%±9.9%), compared to children aged 1 to 5 years (90.9%±20.1%), 5 to 10 years (94%±13.3%), and 10 to 18 years (86.7%±10.0%), p=0.01. Freedom from autograft reoperation at 15 years was significantly lower in infants (58.4%±16.2%), compared to children aged 1 to 5 years (77.1%±14.9%), 5 to 10 years (84.2%±6.0%) and 10 to 18 years (87.8%±9.0%), p=0.01. Overall freedom from reoperation at 15 years was 13.0%±6.0% for infants, 24.2%±9.0% for children aged 1 to 5 years, 46.7%±15.8% for children aged 5 to 10 years, and 78.4%±10.4%, p<0.001., Conclusions: The Ross procedure performed after 10 years of age appears to be associated with improved freedom from reoperation, primarily due to a reduction in reoperation on the pulmonary conduit., Competing Interests: Disclosures Professor Brizard is a consultant for Admedus., (Crown Copyright © 2023. Published by Elsevier B.V. All rights reserved.)

Mitral valve infective endocarditis (IE) in children is rare, and there are few reports on the outcomes of surgery in these patients. This study investigated the long-term outcomes of mitral valve repair in children with IE. Data were retrospectively obtained from medical records and correspondence. Univariable regression analyses were performed and outcomes including survival and freedom from reoperation were analyzed using the Kaplan-Meier method. Surgery for native mitral valve IE was performed in 39 patients between 1987 and 2020. Of these, 92.3% (36/39) of patients underwent mitral valve repair, while 7.7% (3/39) required replacement. Median age was 8 years. Preoperatively, 80.5% (29/36) of patients had moderate or greater mitral regurgitation. Congenital heart disease was present in 38.9% (14/36), while 11.1% (4/36) had rheumatic heart disease and 25.0% (9/36) had prior cardiac surgery. Postoperatively, only 1 patient (2.8%, 1/36) had moderate or greater residual mitral regurgitation. There were 2 early deaths (5.6%, 2/36), with survival being 94.1% (95%CI, 78.5-98.5) at 15 years. At 10 years, freedom from reoperation was 62.9% (95%CI, 41.0-78.5) while freedom from mitral valve replacement was 80.2% (95%CI, 55.5-92.3). Larger vegetation size was a risk factor for embolic events both pre- and postoperatively (OR, 1.15, P = 0.02). Mitral valve repair is feasible in the majority of children requiring surgery for mitral valve IE. Survival is excellent, and at 10 years, approximately two-thirds of patients are free from mitral reoperation, and 80% are free from replacement. Larger vegetation size is associated with an increased risk of embolic events., (Copyright © 2022. Published by Elsevier Inc.)

Background: Patients with single ventricle (SV) without Fontan palliation are uncommon, and their long-term outcomes remain unclear., Methods: Retrospective study of 35 adult patients with SV without Fontan from two tertiary centers. Primary outcome was mortality., Results: Median age at first follow-up was 31 years (IQR: 20-40). Most common defect was double inlet left ventricle (34%), and 69% had left ventricular morphology. Patients were unoperated (46%), had systemic-to-pulmonary artery shunt (31%) or bidirectional cavopulmonary shunt (23%) as final palliation. Most common reasons for not progressing to Fontan palliation were pulmonary vascular disease (54%), patient refusal (17%), Fontan takedown (14%), and hypoplastic pulmonary arteries (11%). Baseline mean hemoglobin was 195 ± 29 g/L, mean O 2 saturation 83 ± 6.9%, and 4 patients in NYHA Class III‒IV. After a mean follow-up of 10 ± 8.3 years, there were 9 deaths with heart failure being the leading cause (n = 6). Age-adjusted survival of these adult SV survivors was 73% and 53% at 40 and 50 years of age, respectively. Deceased patients more frequently had renal impairment (50% vs 0%) and QRS prolongation (75% vs 16%) at baseline (all p < 0.05). During follow-up, 40% had a new arrhythmia (atrial: n = 14, ventricular: n = 3), 34% had one or more hospitalizations for heart failure, and 17% had a stroke. A greater proportion of patients with pre-existing or new atrial/ventricular arrhythmia died compared to those without (42% vs 6%, p = 0.02)., Conclusions: Patients with SV without Fontan have high mortality and a substantial burden of cardiovascular complications, particularly arrhythmia. QRS prolongation and renal impairment were associated with mortality., Competing Interests: The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Melissa Lee holds a Medical Research Future Fund (MRFF) Investigator Grant (1197307). Yves d’Udekem is a Deputy Editor, and David Celermajer and Igor Konstantinov are Associate Editors for IJC-CHD., (© 2023 The Authors.)

Cardio-cutaneous fistula is a very rare complication of cardiac surgery, and the optimal management strategy is unclear. We present a case of a right ventricle-to-pulmonary artery conduit (RV-PA) forming a cutaneous fistulate that was successfully surgically repaired. A 43-year-old male presented for an elective RV-PA conduit replacement with a cutaneous skin lesion and associated sub-sternal collection. The patient underwent redo-sternotomy for the previous surgical replacement RV-PA conduit, of pulmonary atresia, ventricular septal defect, and ligation of main aorto-pulmonary collateral arteries in childhood, with the subsequent upgrade of the RV-PA conduit using pulmonary homograft. Upon entry into the thoracic cavity, it was clear that there was a direct fistula formed from the RV-PA conduit that was responsible for the skin lesion and hence a direct communication to the PA. We discuss the surgical method and surrounding discussions regarding Cardio-cutaneous fistula in a successful surgical repair when the pathology is difficult to truly identify preoperatively., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objective: The effect of ventricular dominance and previous atrioventricular valve (AVV) surgery on patient outcomes after Fontan operation remains unclear. We sought to determine the effect of ventricular dominance and previous AVV surgery on transplantation-free survival and long-term AVV competency in patients with atrioventricular septal defect (AVSD) and Fontan circulation., Methods: We conducted a retrospective study of 1703 patients in the Australia and New Zealand Fontan Registry, who survived Fontan operation between 1987 and 2021., Results: Of 174 patients with AVSD, 60% (105/174) had right ventricular (RV) dominance and 40% (69/174) had left ventricular (LV) dominance. The cumulative incidence of moderate or greater AVV regurgitation at 25 years after Fontan operation in patients with LV dominance was 56% (95% CI, 35%-72%), compared with 54% (95% CI, 40%-67%) in patients with RV dominance (P = .6). Nonetheless, transplantation-free survival at 25 years in patients with LV dominance was 94% (95% CI, 86%-100%), compared with 67% (95% CI, 52%-87%) in patients with RV dominance (hazard ratio, 5.9; 95% CI, 1.4-25.4; P < .01). Of note, transplantation-free survival was not different in patients who underwent AVV surgery before or at Fontan completion compared with those who did not (15 years: 81% [95% CI, 62%-100%] vs 88% [95% CI, 81%-95%]; P = .3)., Conclusions: In patients with AVSD and Fontan circulation the rate of moderate or greater common AVV regurgitation is similar in those with LV and RV dominance. RV dominance, rather than previous AVV surgery, is a risk factor for death or transplantation., (Crown Copyright © 2022. Published by Elsevier Inc. All rights reserved.)