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The optimal strategy for pulmonary atresia with ventricular septal defect (PA/VSD) without major aortopulmonary collateral arteries (MAPCAs) is yet to be defined. It remains unclear how primary complete repair compares with staged repair.We reviewed the records of 107 patients with PA/VSD managed with systemic-to-pulmonary shunts from February 1989 to November 2019. Tables presenting summary statistics of patient and surgical data and Kaplan-Meier curves presenting repair, mortality and reoperation were created. Logistic regression analysis was performed to identify risk factors for repair, morbidity and mortality.Ninety-one (85%) patients achieved complete repair at a median age of 1 year (interquartile range [IQR] 8-20 months). Survival was 97 (90%) patients at 6 months, 91 (85%) at 20 months and 87 (81%) at 10 years. Shunt diameter of 3.5 mm or less (p = 0.006) and shunt after 2009 (p0.001) were associated with earlier progression to complete repair. Shunt diameter of 3.5 mm or less was an independent risk factor for interstage morbidity (hazard ratio 4.5 [IQR 1.1-18.5], p = 0.039), but was not associated with complete repair (p = 0.75) or mortality (p = 0.62). The median follow-up period was 10.5 years (IQR 3.6-18.8 years) from birth.The complete repair and long-term survival rates of staged PA/VSD repair are high. The use of smaller shunts has resulted in earlier complete repair and is associated with increased risk of morbidity but not mortality.

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.We conducted a retrospective study of 1703 patients in the Australia and New Zealand Fontan Registry, who survived Fontan operation between 1987 and 2021.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).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.

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.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.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.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.

Atrioventricular valve (AVV) regurgitation is increasingly prevalent in patients with a Fontan circulation.We sought to determine the impact of ventricular dominance and AVV operation on outcomes in patients with a Fontan circulation and ≥moderate AVV regurgitation.We conducted a retrospective study, including propensity score matching analysis, of 1,703 patients who survived Fontan operation in Australia and New Zealand from 1987 to 2021.Patients undergoing AVV operation were more likely to have right ventricular (RV) dominance or an atrioventricular septal defect. In the entire cohort, death or transplantation after Fontan operation was significantly higher in patients who underwent AVV operation before or at Fontan completion compared with those who did not (20 years: 18%; 95% CI: 8%-26% vs 13%; 95% CI: 10%-15%; P = 0.03). After propensity score matching, including for RV dominance, there was no significant difference in death or transplantation between the groups (20 years: 18%; 95% CI: 8%-26% vs 16%; 95% CI: 10%-22%; P = 0.41). Only patients with RV dominance who developed ≥moderate AVV regurgitation after Fontan operation were at increased risk of death or transplantation (HR: 2.8; 95% CI: 1.4-5.3; P 0.01). In patients with left ventricular dominance, there was no significant difference in death or transplantation between patients with ≥moderate AVV regurgitation compared with those with moderate regurgitation (P = 0.8).RV dominance, but not AVV surgery itself, was associated with poor outcomes. Moderate or greater AVV regurgitation after Fontan operation is associated with a significantly increased risk of death or transplantation, only in patients with RV dominance.

Repair of complete atrioventricular septal defect (cAVSD) is achieved with low mortality. However, there is a high rate of reoperation on the left atrioventricular valve (LAVV), which is often attributed to nonclosure of the cleft. Although nonclosure of the cleft has been reported to be a risk factor for reoperation, no randomized controlled or propensity-matched trials have ever been performed. We investigated the effect of cleft closure on outcomes after cAVSD repair.We reviewed 455 patients who underwent cAVSD repair between 1990 and 2019. To determine the effect of cleft closure, propensity score matching was performed on risk factors for reoperation after cAVSD repair.Median age was 3.6 months (mean, 9.6 ± 20.4 months), median weight was 4.3 kg (mean, 4.7 ± 4.3 kg) and 41.9% (191 of 455) were male. Early mortality was 2.9% (13 of 455), and survival was 89.8% ± 1.9% at 20 years. Early reoperation was a risk factor for mortality (P = .004). Freedom from reoperation was 72.5% ± 4.0% at 20 years. Freedom from LAVV reoperation was 74.1% ± 4.0% at 20 years. Preoperative severe LAVV regurgitation (P.001) and early postoperative moderate or greater LAVV regurgitation (P = .007) were risk factors for reoperation, while trisomy 21 (P = .03) and recent era of surgery (P = .02) were protective. Propensity score matching yielded 106 pairs. There were no differences in long-term survival (P = .71) or reoperation (P = .26) between the 2 groups.Repair of cAVSD can be achieved with low mortality and good long-term survival, although the reoperation rate remains high. Similar freedom from reoperation can be achieved with or without closure of the LAVV cleft.

Atrioventricular valve (AVV) dysfunction is a common cause of reoperation and morbidity in patients with atrioventricular septal defects (AVSDs). In the setting of AVSD with single ventricle physiology, the risk of AVV failure is particularly high, and repair of the common AVV in these patients is notoriously challenging. Furthermore, patients with significant residual AVV regurgitation, after attempted repair, are at particularly high risk of mortality. One of the key factors which contributes to regurgitation in patients with a common AVV is the lack of a normal fibrous skeleton of the heart. We report a repair technique which utilizes a bridging annuloplasty in order to emulate the normal fibrous skeleton of the heart.

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.

OBJECTIVES Left ventricular outflow tract obstruction (LVOTO) is a recognized complication after complete repair of atrioventricular septal defect (AVSD). This study reviewed the incidence and management of LVOTO following AVSD repair at a single institution. METHODS From 1975 to 2019, 24 patients (3.3%, 24/730) underwent reoperation due to LVOTO following partial AVSD (pAVSD) and complete AVSD (cAVSD) repair. The data were retrospectively reviewed. RESULTS The incidence of LVOTO following pAVSD and cAVSD repair was 4.4% (12/275) and 2.6% (12/455). Freedom from LVOTO reoperation following pAVSD and cAVSD repair at 25 years was 94.3% [95% confidence interval (CI); 89.7–96.7] and 95% (95% CI; 91.1–97.3). The median time from complete repair of pAVSD and cAVSD to LVOTO reoperation was 4.4 years [interquartile range (IQR): 3.4–6.7] and 2.6 years (IQR: 2.2–4.7). Freedom from second LVOTO reoperation at 5, 10 and 15 years was 83.7% (95% CI; 57.2–98.2), 59.2% (95% CI; 28.7, 80.3) and 39.5% (95% CI; 13.2–65.3). The median time between the first and the second LVOTO reoperation in the groups of pAVSD and cAVSD was 6.1 years (IQR: 3.4–8.9) and 8.6 years (IQR: 5.7–9.8). There was no significant difference regarding the first (P = 0.7406) and subsequent LVOTO (P = 0.7153) following complete repair of pAVSD and cAVSD. Combined access to the left ventricular outflow tract was not protective regarding LVOTO reoccurrence. Survival for both groups after LVOTO reoperation at 15 years was 95.6% (95% CI 99.4–72.9). CONCLUSIONS Incidence of LVOTO after AVSD repair is low but the reoccurrence rate is high. Standard subaortic resection does not always provide definitive LVOTO relief. The survival after LVOTO reoperation is excellent.

Children with SARS-CoV-2 infection generally present with milder symptoms or are asymptomatic in comparison with adults, however severe disease occurs in a subset of children. To date, the immune correlates of severe COVID-19 in young children have been poorly characterised. We report the kinetics of immune responses in relation to clinical and virological features in an infant with acute severe COVID-19 using high-dimensional flow cytometry and multiplex cytokine analysis. Systemic cellular and cytokine profiling show an initial increase in neutrophils and monocytes with depletion of lymphoid cell populations (particularly CD8 + T and NK cells) and elevated inflammatory cytokines. Expansion of memory CD4 + T (but not CD8 + T) cells occurred over time, with a predominant Th2 bias. Marked activation of T cell populations observed during the acute infection gradually resolved as the child recovered. Substantial in vitro activation of T-cell populations and robust cytokine production, in response to inactivated SARS-CoV-2 stimulation, was observed 3 months after infection indicating durable, long-lived cellular immune memory. These findings provide important insights into the immune response of a young infant with severe COVID-19 and will help to inform future research into therapeutic targets for high-risk groups. SARS-CoV-2 infection can cause COVID-19, which is usually a mild disease in children. However, severe illness requiring intensive care management can occur, particularly in younger children and those with chronic disease. The immune system likely plays an important role in susceptibility to severe disease, but few studies have examined the immune response in infants with severe COVID-19. Here, we provide an in-depth analysis of the clinical features and immune response over time in a 5-month-old infant with congenital heart disease and severe COVID-19. Robust immune responses were observed up to 3 months following infection, providing evidence of durable and long-lived immunity against SARS-CoV-2. These findings provide important insights into the immune responses of a young infant with severe COVID-19 and might help inform future research into therapeutic targets. Wurzel et al. describe the kinetics of the immune response in relation to clinical and virological features in a 5-month old infant with congenital heart disease and severe COVID-19. The immune response was characterised by an elevated inflammatory response in the acute phase of infection, followed by Th2 skewing and prolonged T cell activation.

Patients with aortic atresia (AA) have the worst prognosis in the spectrum of hypoplastic left heart syndrome (HLHS). It remains unknown if patients with AA continue to do poorly after Fontan operation. This study aimed to determine the association between AA and atrioventricular valve (AVV) function and clinical outcomes after Fontan operation in patients with HLHS.We performed a retrospective study of 1,731 patients who survived the Fontan operation from the Australia and New Zealand Fontan Registry between 1975-2020.We identified 188 patients with HLHS, including 99 (53%) with AA. 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%). The cumulative incidence of AVV operation at 15-years of age for patients with AA and aortic stenosis (AS) was 28% (95%CI, 19%-38%) and 14% (95%CI, 7%-22%, p=0.03). The cumulative incidence of AVV failure (≥moderate regurgitation or AVV operation) at 15-years of age for patients with AA and AS was 50% (95%CI, 37%-61%) and 30% (95%CI, 19%-42%, p=0.01). Patients with AVV failure were at increased risk of developing ≥moderately decreased systolic ventricular function (OR 6.7, 95%CI 1.7-33, p=0.01) and failure of Fontan circulation (HR 3.7, 95%CI 1.5-9.1, p0.01).In patients with HLHS, there is no significant difference in transplant-free survival after Fontan operation in patients with AA and AS. However, patients with AA have a much higher burden of AVV failure than those with AS. AVV failure is associated with failure of Fontan circulation.

This study assessed outcomes of neonatal aortic valve (AoV) repair in presumed high-risk patients with depressed left ventricular (LV) function. A retrospective analysis of all neonates who underwent isolated AoV repair for severe aortic stenosis (AS) was performed. Patients with moderate or severe LV dysfunction were compared to those with normal or mild LV dysfunction. From 1980-2021, 43 neonates underwent isolated AoV repair for AS. Of these, 16 patients (37.2%) had ≥moderate LV dysfunction. Mean LV ejection fraction (EF) was 32.8 ± 9.1%. Valve morphology was mostly unicuspid (68.75%, 11/16). Median age at surgery was 6.5 days (IQR 1-17.5). An optimal repair result with ≤mild AS or aortic regurgitation was achieved in 75% (12/16). There was no early death. One patient (6.25%) required postoperative extracorporeal membrane oxygenation (ECMO) support for 3 days. LVEF improved after surgery to 56.4 ± 12.6% before discharge (P0.0001) and normalized in 87.5% (14/16) with a median time of 6.4 days (IQR 3.4-39). Freedom from AoV reoperation was 45.1% (95%CI 17.9-69.3%) and 27.1% (95%CI 6.8-53%) at 5 and 10 years, respectively. Freedom from AoV replacement was 59% (95%CI 27-80.8%) and 31.4% (95%CI 6-62.2%) at 5 and 10 years, respectively. While survival was similar, freedom from AoV reoperation and replacement tended to be lower compared to neonates with preserved LVEF. AoV repair was associated with a low incidence of postoperative ECMO and mortality. LV function normalized after relief of obstruction in most patients before discharge. Late reoperation remained common for those with severely dysplastic valves.