Outcomes of extracardiac Fontan operation: A single institution experience with 398 patients.

OBJECTIVE: To evaluate the outcomes of the extracardiac (EC) Fontan operation at a single institution. METHODS: We conducted a retrospective study of 398 patients from a single institution who underwent their initial EC 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 (IQR 6.4-14.6) years. The overall survival rate was 96% and 86% at 10 and 20 years after EC Fontan operation. There were 6 early (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 at 10 years was 88% and 76% at 20 years. Risk factors for failure of the Fontan circulation were right ventricular (RV) dominance (hazard ratio [HR], 4.7; P < 0.001; 95% confidence interval [CI], 2.1-10.5), aortic atresia (HR 5.5; P < 0.001; 95% CI, 2.3-12.8) and elevated mean pulmonary artery (PA) pressure (HR, 2.3; P = 0.002; 95% CI, 1.2-6.7). CONCLUSIONS: Rates of failure of the Fontan circulation are low following the contemporary EC Fontan operation. Risk factors for failure of the EC Fontan circulation include RV dominance, aortic atresia and elevated PA pressures.

Heart Transplantation after Univentricular Palliation: Improved Outcomes and Increased Complexity.

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.

Exercise Capacity and Reoperation Late After Transatrial Fallot Repair.

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

Long-Term Outcomes of Mechanical Aortic Valve Replacement in Children.

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.

Clinical assessment can be lifesaving: Salvage of catastrophic cerebral ischemia by conversion to central extracorporeal membrane oxygenation.

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.

Bex-Nikaidoh operation and the impact of double root translocation on outcomes.

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.

Outcomes of neo-aortic valve and root surgery late after arterial switch operation.

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.

Long-term outcomes of tetralogy of Fallot repair: A 30-year experience with 960 patients.

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.

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

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

Potential benefits of prenatal diagnosis of TGA in Australia may be outweighed by the adverse effects of earlier delivery: likely causation and potential solutions.

OBJECTIVE: Prenatal diagnosis of transposition of great arteries (TGA) is expected to improve postoperative outcomes after neonatal arterial switch operation (ASO); however, published reports give conflicting results. We aimed to determine the association between prenatal diagnosis and early postoperative outcomes after neonatal ASO. METHODS: Cohort study involving 243 newborns who underwent ASO (70% prenatally diagnosed) between 2010 and 2019. Multivariable regression was used to determine the association between prenatal diagnosis and (a) birth characteristics and (b) postoperative outcomes. RESULTS: Gestational age and birthweight centile were lower and small-for-gestational-age more common (11.8% vs 1.4%) in those diagnosed prenatally. Among births which followed labour induction or prelabour caesarean, prenatal diagnosis was associated with earlier gestation at birth (mean (SD), 38.5 (1.6) vs 39.2 (1.4), p=0.01). Among births which followed spontaneous labour, prenatal diagnosis was associated with earlier gestation at labour onset (38.2 (1.8) vs 39.2 (1.4), p=0.01). Prenatal diagnosis was associated with longer postoperative mechanical ventilation (incidence rate ratio 1.74, 95% CI 1.37 to 2.21), intensive care (1.70, 1.31 to 2.21) and hospital length of stay (1.37, 1.14 to 1.66) after ASO. Gestational age mediated up to 60% of the effect of prenatal diagnosis on postoperative outcomes. CONCLUSION: Among newborns undergoing ASO for TGA, prenatal diagnosis is associated with poorer early postoperative outcomes. In addition to minimising iatrogenic factors (such as planned births) resulting in earlier births, evaluation of other dynamics following a prenatal diagnosis which may result in poor fetal growth and earlier onset of spontaneous labour is important.

Safety and feasibility of adjunct autologous cord blood stem cell therapy during the Norwood heart operation.

BACKGROUND: We conducted this phase I, open-label safety and feasibility trial of autologous cord blood (CB) stem cell (CBSC) therapy via a novel blood cardioplegia-based intracoronary infusion technique during the Norwood procedure in neonates with an antenatal diagnosis of hypoplastic left heart syndrome (HLHS). CBSC therapy may support early cardiac remodeling with enhancement of right ventricle (RV) function during the critical interstage period. METHODS: Clinical grade CB mononucleated cells (CBMNCs) were processed to NetCord-FACT International Standards. To maximize yield, CBSCs were not isolated from CBMNCs. CBMNCs were stored at 4 °C (no cryopreservation) for use within 3 days and delivered after each cardioplegia dose (4 × 15 mL). RESULTS: Of 16 patients with antenatal diagnosis, 13 were recruited; of these 13 patients, 3 were not treated due to placental abruption (n = 1) or conditions delaying the Norwood for >4 days (n = 2) and 10 received 644.9 ± 134 × 106 CBMNCs, representing 1.5 ± 1.1 × 106 (CD34+) CBSCs. Interstage mortality was 30% (n = 3; on days 7, 25, and 62). None of the 36 serious adverse events (53% linked to 3 deaths) were related to CBMNC therapy. Cardiac magnetic resonance imaging before stage 2 (n = 5) found an RV mass index comparable to that in an exact-matched historical cohort (n = 22), with a mean RV ejection fraction of 66.2 ± 4.5% and mean indexed stroke volume of 47.4 ± 6.2 mL/m2 versus 53.5 ± 11.6% and 37.2 ± 10.3 mL/m2, respectively. All 7 survivors completed stage 2 and are alive with normal RV function (6 with ≤mild and 1 with moderate tricuspid regurgitation). CONCLUSIONS: This trial demonstrated that autologous CBMNCs delivered in large numbers without prior cryopreservation via a novel intracoronary infusion technique at cardioplegic arrest during Norwood palliation on days 2 to 3 of life is feasible and safe.

Longitudinal cohort study investigating neurodevelopmental and socioemotional outcomes in school-entry aged children after open heart surgery in Australia and New Zealand: the NITRIC follow-up study protocol.

INTRODUCTION: Despite growing awareness of neurodevelopmental impairments in children with congenital heart disease (CHD), there is a lack of large, longitudinal, population-based cohorts. Little is known about the contemporary neurodevelopmental profile and the emergence of specific impairments in children with CHD entering school. The performance of standardised screening tools to predict neurodevelopmental outcomes at school age in this high-risk population remains poorly understood. The NITric oxide during cardiopulmonary bypass to improve Recovery in Infants with Congenital heart defects (NITRIC) trial randomised 1371 children <2 years of age, investigating the effect of gaseous nitric oxide applied into the cardiopulmonary bypass oxygenator during heart surgery. The NITRIC follow-up study will follow this cohort annually until 5 years of age to assess outcomes related to cognition and socioemotional behaviour at school entry, identify risk factors for adverse outcomes and evaluate the performance of screening tools. METHODS AND ANALYSIS: Approximately 1150 children from the NITRIC trial across five sites in Australia and New Zealand will be eligible. Follow-up assessments will occur in two stages: (1) annual online screening of global neurodevelopment, socioemotional and executive functioning, health-related quality of life and parenting stress at ages 2-5 years; and (2) face-to-face assessment at age 5 years assessing intellectual ability, attention, memory and processing speed; fine motor skills; language and communication; and socioemotional outcomes. Cognitive and socioemotional outcomes and trajectories of neurodevelopment will be described and demographic, clinical, genetic and environmental predictors of these outcomes will be explored. ETHICS AND DISSEMINATION: Ethical approval has been obtained from the Children's Health Queensland (HREC/20/QCHQ/70626) and New Zealand Health and Disability (21/NTA/83) Research Ethics Committees. The findings will inform the development of clinical decision tools and improve preventative and intervention strategies in children with CHD. Dissemination of the outcomes of the study is expected via publications in peer-reviewed journals, presentation at conferences, via social media, podcast presentations and medical education resources, and through CHD family partners. TRIAL REGISTRATION NUMBER: The trial was prospectively registered with the Australian New Zealand Clinical Trials Registry as 'Gene Expression to Predict Long-Term Neurodevelopmental Outcome in Infants from the NITric oxide during cardiopulmonary bypass to improve Recovery in Infants with Congenital heart defects (NITRIC) Study - A Multicentre Prospective Trial'. TRIAL REGISTRATION: ACTRN12621000904875.

Aortic valve repair versus the Ross procedure in children.

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.

The Ross Procedure in Children: Defining the Optimal Age.

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.

Aortic Valve Surgery in Children With Infective Endocarditis.

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.

Congenital Aortic Valve Repair When the Options aren't Good: Truncus Arteriosus and Transposition of the Great Arteries.

Patients with truncus arteriosus and transposition of great arteries are prone to neo-aortic valve insufficiency. Although presenting at opposite ends of the age spectrum, both conditions tend to be commonly associated with neo aortic root dilatation. In patients with truncus arteriosus there is an additional complexity of quadricuspid valve morphology, which make up the majority of valves requiring repair. A unified approach to all these patients would include reduction and stabilization of the annulus and sinotubular junction, as well as achieving equal and symmetrical coaptation of the valve leaflets. By systematically employing these techniques, valve replacement should be avoidable in most children.