Fetal cardiac parameters for predicting postnatal operation type of fetuses with tetralogy of Fallot.

BACKGROUND: To assess fetal cardiac parameters predictive of postnatal operation type in fetuses with tetralogy of Fallot (TOF). METHODS: Echocardiographic data obtained in the second and third trimesters were retrospectively reviewed for fetuses diagnosed with TOF between 2014 and 2018 at Asan Medical Center. The following fetal cardiac parameters were analyzed: 1) pulmonary valve annulus (PVA) z-score, 2) right pulmonary artery (RPA) z-score, 3) aortic valve annulus (AVA) z-score, 4) pulmonary valve peak systolic velocity (PV-PSV), 5) PVA/AVA ratio, and 6) RPA/descending aorta (DAo) ratio. These cardiac parameters were compared between a primary corrective surgery group and a palliative shunt operation followed by complete repair group. RESULTS: A total of 100 fetuses with TOF were included. Only one neonatal death occurred. Ninety patients underwent primary corrective surgery and 10 neonates underwent a multistage surgery. The PVA z-score, RPA z-score, and RPA/DAo ratio measured in the second trimester and the PVA z-score, RPA z-score, and PVA/AVA raio measured in the third trimester were significantly lower in the multistage surgery group, while the PV-PSV as measured in both trimesters were significantly higher in the multistage surgery group. CONCLUSION: Fetal cardiac parameters are useful for predicting the operation type necessary for neonates with TOF.

Paediatric heart transplantation recipients ≥7 years of age receiving donors with pre-existing coronary atherosclerosis showed progressive coronary artery disease.

BACKGROUND: This study aimed to determine the effect of donor-transmitted atherosclerosis on the late aggravation of cardiac allograft vasculopathy in paediatric heart recipients aged ≥7 years. METHODS: In total, 48 patients were included and 23 had donor-transmitted atherosclerosis (baseline maximal intimal thickness of >0.5 mm on intravascular ultrasonography). Logistic regression analyses were performed to identify risk factors for donor-transmitted atherosclerosis. Rates of survival free from the late aggravation of cardiac allograft vasculopathy (new or worsening cardiac allograft vasculopathy on following angiograms, starting 1 year after transplantation) in each patient group were estimated using the Kaplan-Meier method and compared using the log-rank test. The effect of the results of intravascular ultrasonography at 1 year after transplantation on the late aggravation of cardiac allograft vasculopathy, correcting for possible covariates including donor-transmitted atherosclerosis, was examined using the Cox proportional hazards model. RESULTS: The mean follow-up duration after transplantation was 5.97 ± 3.58 years. The log-rank test showed that patients with donor-transmitted atherosclerosis had worse survival outcomes than those without (p = 0.008). Per the multivariate model considering the difference of maximal intimal thickness between baseline and 1 year following transplantation (hazard ratio, 22.985; 95% confidence interval, 1.948-271.250; p = 0.013), donor-transmitted atherosclerosis was a significant covariate (hazard ratio, 4.013; 95% confidence interval, 1.047-15.376; p = 0.043). CONCLUSION: Paediatric heart transplantation recipients with donor-transmitted atherosclerosis aged ≥7 years had worse late cardiac allograft vasculopathy aggravation-free survival outcomes.

Clinical implication of intraoperative ventricular-arterial coupling in pediatric patients undergoing ventricular septal defects repair: A retrospective cohort study.

BACKGROUND: Ventricular-arterial coupling is the ratio of arterial elastance to ventricular end-systolic elastance. AIMS: The objective of this study was to determine the clinical implication of intraoperative ventricular-arterial coupling derived from the pressure-area relationship using transesophageal echocardiography. METHODS: This retrospective study reviewed the medical records of 72 pediatric patients with ventricular septal defects who underwent corrective surgery with cardiopulmonary bypass. The single-beat modified method was used to assess ventricular-arterial coupling. Logistic regression analyses were performed to determine the correlation between ventricular-arterial coupling and early postoperative outcomes, including the maximum vasoactive-inotropic score, length of mechanical ventilation, and length of hospital stay. RESULTS: Ventricular-arterial coupling after cardiopulmonary bypass significantly increased (from 1.0 ± 0.4 to 1.4 ± 0.8, p < .001), indicating a disproportionate increase in the arterial elastance index (from 11.5 ± 5.1 to 19.8 ± 7.5 mmHg/cm2 /m2 , p < .001) compared with the ventricular end-systolic elastance index (from 13.0 ± 6.9 to 16.9 ± 9.0 mmHg/cm2 /m2 , p < .001). Logistic regression analyses revealed that high postoperative ventricular-arterial coupling was independently associated with higher postoperative maximum vasoactive-inotropic score (>10; odds ratio [OR], 8.04; 95% confidence interval [CI], 1.38-46.85, p = .020), longer postoperative mechanical ventilation (>15 h; OR: 11.00; 95% CI: 1.26-96.45, p = .030), and longer postoperative hospital stay (>7 days; OR: 2.98; 95% CI: 1.04-8.58, p = .043). CONCLUSIONS: Ventricular-arterial coupling can be easily obtained from the intraoperative transesophageal echocardiography in pediatric patients undergoing ventricular septal defects repair. High postoperative ventricular-arterial coupling is strongly associated with worse early postoperative outcomes. Ventricular-arterial coupling shows promise as an intraoperative analysis tool that can provide insight into the impact of interventions on cardiovascular performance and identify potential targets for treatment in this population.

Predictive Parameters of Decreased Left Ventricular Global Longitudinal Strain at 1 Month After Pediatric Heart Transplantation.

Previous reports indicate that the decreased left ventricular global longitudinal strain (LVGLS) seen in the early postoperative period of pediatric heart transplant patients generally recovers over the course of 1-2 years. In this study, we investigate the predictive capacity of preoperative parameters on the LVGLS decline seen at 1 month post transplant. Forty-six transplant subjects with 2D echocardiographic images sufficient for speckle tracking echocardiography were enrolled. We excluded patients diagnosed with cardiac allograft vasculopathy or with an episode of rejection 1 month before or after their echocardiographic examinations. The mean LVGLS was significantly reduced at 1 month when compared to 1 year following transplant (- 15.5% vs. - 19.4%, respectively, p < 0.001). The predictors of LVGLS that decline at 1 month were the LV mass z-score [odds ratio (OR) 1.452; 95% confidence interval (CI) 1.007-2.095, p = 0.046], recipient age (OR 1.124; 95% CI 1.015-1.245, p = 0.025), and donor age (OR 1.081; 95% CI 1.028-1.136, p = 0.002) in the univariate logistic regression analyses. Although multivariate analysis yielded no significant predictors, higher LV mass z-scores showed a trend associated with the decline of LVGLS (p = 0.087). The donor/recipient weight ratio was associated with the LV mass z-score (R2 = 0.412, p < 0.001).

Acute Change in Ventricular Contractility-Load Coupling After Corrective Surgery for Congenital Heart Defect: A Retrospective Cohort Study.

Ventricular performance and its loading condition change drastically after surgical correction of congenital heart defect. Pressure-volume loops analysis can provide quantitative information about ventriculo-arterial coupling (VAC) indicating the interaction between ventricular contractility and loading condition. Therefore, we investigated changes in VAC after corrective surgery for ventricular septal defect (VSD)/tetralogy of Fallot (TOF), and implication of ventriculo-arterial decoupling as a prognostic factor of post-operative outcomes. In children with VSD/TOF, pre- and post-operative arterial elastance (Ea), end-systolic ventricular elastance (Ees) and VAC (Ea/Ees) were non-invasively estimated using echocardiographic parameters. Post-operative outcomes included maximum vasoactive-inotropic score, the duration of mechanical ventilation and hospital stay. Preoperatively, patients with VSD had significantly lower Ea and Ees than patients with TOF; however, VAC were preserved in both. In patients with VSD, post-operative Ea increased disproportionately to change in Ees, resulting in increased VAC. Post-operative higher VAC in patients with VSD was independently associated with maximum vasoactive-inotropic score (odds ratio [OR] 63.9; 95% Confidence Interval [CI] 4.0-553.0; P = 0.003), prolonged mechanical ventilation (OR 6.3; 95% CI 1.1-37.8; P = 0.044) and longer hospitalization (OR 17.6; 95% CI 1.6-187.0; P = 0.018). In patients with TOF, Ea and Ees reduced post-operatively; however, VAC remained unchanged and was not associated with post-operative outcomes. Despite of different loading condition, preoperative VAC maintained in both of VSD and TOF. However, particularly in VSD patients, abrupt increase in post-operative loading state induces contractility-load decoupling, which leads to worse post-operative outcomes.

Is there an optimal organ acceptance rate for pediatric heart transplantation: "A sweet spot"?

Despite a limited supply of donors, potential donor hearts are often declined for subjective concerns regarding organ quality. This analysis will investigate the relationship between donor heart AR and patient outcome at pediatric transplant centers. The UNOS database was used to identify all match runs for pediatric candidates (age < 18 years) from 2008 through March 2015 in which a heart offer was ultimately placed. Centers which received ≥10 offers/y were included (10 634 offers, 38 centers). Transplant centers were stratified based on their AR: low (<20%, n = 13), medium (20%-40%, n = 16), or high (>40%, n = 9). Low AR centers experienced worse negative WL outcome compared with medium (P = .022) and high (P = .004) AR centers. Low AR centers had similar post-transplant graft survival to medium (P = .311) or high (P = .393) AR centers; however, medium AR centers had better post-transplant graft survival than high AR centers (P = .037). E-F survival from listing regardless of transplant was worse for low AR centers compared with medium (P < .001) or high (P = .001) AR centers. Low AR centers experience worse WL outcomes without improvement in post-transplant outcomes. High AR centers experience higher post-transplant graft failure than medium AR centers. AR of 20%-40% appears to have optimal WL and post-transplant outcomes.

Restoration of Tricuspid Valve Mechanism at the Level of Displaced Septal and Posterior Leaflets in Ebstein's Anomaly.

BACKGROUND: Surgical techniques currently used for the repair of Ebstein's anomaly comprise reconstruction of the tricuspid valve mechanism at the level of the true annulus with or without plication of the atrialized right ventricle. However, performing this procedure for patients with a dysmorphic anterior leaflet (i.e., insufficient leaflet tissue and decreased mobility due to tethering) may necessitate technical modifications. METHODS: A retrospective review was performed of 31 patients (seven males and 24 females, median age at operation 31 years) with Ebstein's anomaly, who underwent tricuspid valve repair between March 2002 and December 2014. The original Hetzer technique (annulus to annulus approximation) was employed for six patients with a well-formed anterior leaflet. In 25 patients, the tricuspid valve mechanism was restored at the displaced septal leaflet by approximating the anterior leaflet attachment in the true annulus to the displaced septal leaflet attachment in the mid-septum. A bidirectional superior cavopulmonary anastomosis was added in 27 of 31 (87%) patients. RESULTS: No early or late death occurred during the median follow-up of 66 months (1-138 months). Immediate postoperative tricuspid regurgitation was trivial to mild in 22 patients, and the median preoperative, immediate postoperative, and last follow-up tricuspid regurgitation jet areas in 21 adult patients were 23.3 cm2, 10.4 cm2, and 7.0 cm2, respectively. Two patients underwent reoperation at 81 and 119 months postoperatively. Five-year freedom from severe tricuspid regurgitation or reoperation was 93.2%. CONCLUSION: Restoration of the tricuspid valve mechanism at the level of displaced septal leaflet leads to excellent long-term outcomes. The addition of the bidirectional superior cavopulmonary anastomosis has contributed to the success of this technique.

Individual Pulmonary Veins Outgrow Somatic Growth After Primary Sutureless Repair for Total Anomalous Pulmonary Venous Drainage.

Indications of sutureless repair (SR) for pulmonary vein anomalies have evolved from re-operational SR for pulmonary vein stenosis after the repair of total anomalous pulmonary venous drainage (TAPVD) to primary SR for TAPVD associated with right atrial isomerism or isolated TAPVD with small individual pulmonary veins (IPVs) and an unfavorable pulmonary vein anatomy. We sought to determine whether small IPVs outgrow somatic growth after primary SR. Between 2004 and 2013, 21 children underwent primary SR for TAPVD: 13 with a functionally single ventricle, 11 with right atrial isomerism, six with isolated TAPVD, and 13 with a pulmonary venous obstruction. TAPVD types were supracardiac in nine, infracardiac in 10, and mixed in two. Utilizing cardiac computed tomography (CT), the maximal diameter of each IPV was measured, and pulmonary vein index (PVI, summation of cross-sectional areas of all four IPVs divided by body surface area) was calculated. There were five early deaths after SR. Among survivors, 10 had both preoperative and postoperative cardiac CT at a 3.6-month median interval. On postoperative cardiac CT, IPVs were patent in all patients except one who developed a left lower pulmonary vein obstruction. There was a 71 ± 48 % postoperative increase in the actual diameter of all four IPVs, and PVI increased significantly from 215 ± 55 to 402 ± 117 mm(2)/m(2) (P value = 0.005). IPVs outgrew somatic growth after primary SR of TAPVD. Primary SR may be a useful measure in TAPVD patients whose IPVs are small.

Ligation of the left hepatic vein for dilated intrahepatic collaterals late after fontan operation.

A 17-year-old male presented with two episodes of syncope, cyanosis, and exercise intolerance 13 years after the Fontan operation. Echocardiography and magnetic resonance imaging showed dilated intrahepatic collaterals, which drained into the atrium through the left hepatic vein, and 24-hour Holter monitoring revealed sinus node dysfunction. We performed ligation of the left hepatic vein using intrahepatic collaterals as channels draining hepatic venous blood into the Fontan pathway, and implanted an epicardial dual chamber pacemaker. At one-year follow-up, the patient remained asymptomatic and his cardiac performance was much improved.

Outcomes after Biventricular Repair Using a Conduit between the Right Ventricle and Pulmonary Artery in Infancy.

Background: This study investigated the outcomes of biventricular repair using right ventricle to pulmonary artery (RV-PA) conduit placement in patients aged <1 year. Methods: Patients aged <1 year who underwent biventricular repair using an RV-PA conduit between 2011 and 2020 were included in this study. The outcomes of interest were death from any cause, conduit reintervention, and conduit dysfunction (peak velocity of ≥3.5 m/sec or moderate or severe regurgitation). Results: In total, 141 patients were enrolled. The median age at initial conduit implantation was 6 months. The median conduit diameter z-score was 1.3. The overall 5-year survival rate was 89.6%. In the multivariable analysis, younger age (p=0.006) and longer cardiopulmonary bypass time (p=0.001) were risk factors for overall mortality. During follow-up, 61 patients required conduit reintervention, and conduit dysfunction occurred in 68 patients. The 5-year freedom from conduit reintervention and dysfunction rates were 52.9% and 45.9%, respectively. In the multivariable analysis, a smaller conduit z-score (p<0.001) was a shared risk factor for both conduit reintervention and dysfunction. Analysis of variance demonstrated a nonlinear relationship between the conduit z-score and conduit reintervention or dysfunction. The hazard ratio was lowest in patients with a conduit z-score of 1.3 for reintervention and a conduit z-score of 1.4 for dysfunction. Conclusion: RV-PA conduit placement can be safely performed in infants. A significant number of patients required conduit reintervention and had conduit dysfunction. A slightly oversized conduit with a z-score of 1.3 may reduce the risk of conduit reintervention or dysfunction.

Surgical Management of Coronary Artery Fistulas in Children.

Background: This study investigated the surgical outcomes associated with coronary artery fistulas (CAFs) in children. Methods: We retrospectively reviewed the medical records of 23 pediatric patients who underwent surgical closure of CAFs between 1995 and 2021. At presentation, 7 patients (30.4%) exhibited symptoms. Associated cardiac anomalies were present in 8 patients. Fourteen fistulas originated from the right coronary artery and 9 from the left. The most common drainage site was the right ventricle, followed by the right atrium and the left ventricle. The median follow-up duration was 9.3 years (range, 0.1-25.6 years). Results: The median age and body weight at repair were 3.1 years (range, 0-13.4 years) and 14.4 kg (range, 3.1-42.2 kg), respectively. Cardiopulmonary bypass was used in 17 cases (73.9%), while cardioplegic arrest was employed in 14 (60.9%). Epicardial CAF ligation was utilized in 10 patients (43.5%), the transcoronary approach in 9 (39.1%), the endocardial approach in 2 (8.7%), and other methods in 2 patients (8.7%). The application of cardioplegic arrest during repair did not significantly impact the duration of postoperative intensive care unit stay or overall hospital stay. One in-hospital death and 1 late death were recorded. The overall survival rate was 95.7% at 10 years and 83.7% at 15 years. A residual fistula was detected in 1 patient. During the follow-up period, no surviving patient experienced cardiovascular symptoms or coronary events. Conclusion: Surgical repair of CAF can be performed safely with or without cardioplegic arrest, and it is associated with a favorable prognosis in children.

The long-term effects of the fenestration in patients with extracardiac Fontan circulation-a multicenter Korean cohort study based on national Fontan registry.

Introduction: The long-term effects of fenestration in patients with Fontan circulation remain unclear. We aim to evaluate the fenestration impact on early and late outcomes in patients with extracardiac Fontan (ECF) using a propensity score matching analysis. Methods: We performed an extensive retrospective multicenter clinical data review of the Korean Fontan registry and included 1,233 patients with surgical ECF (779 fenestrated, 454 non-fenestrated). Demographics, baseline, and follow-up data were collected and comprehensively analyzed. Patients were divided into two groups according to the baseline presence or absence of surgical fenestration. Subsequently, patients were sub-divided according to the fenestration status at the last follow-up. Propensity-score matching was performed to account for collected data between the 2 groups using a multistep approach. The primary outcomes were survival and freedom from Fontan failure (FFF). We also looked at postoperative hemodynamics, cardiopulmonary exercise test results, oxygen saturations, and functional status. Results: After propensity-score matching (454 matched pairs), there was no difference in survival or FFF between the 2 groups. However, ECF patients with baseline fenestration had significantly lower oxygen saturation (p = 0.001) and lower functional status (p < 0.001). Patients with fenestration had significantly longer bypass times, higher postoperative central venous pressure, higher postoperative left atrial pressure, and less prolonged pleural effusion in the early postoperative period. The propensity score matching according to the fenestration status at the last follow-up (148 matched pairs) showed that patients with a persistent fenestration had significantly lower oxygen saturation levels (p < 0.001). However there were no intergroup differences in the functional status, survival and FFF. Conclusions: Our results showed no long-term benefits of the Fenestration in terms of survival and FFF. Patients with persistent fenestration showed oxygen desaturation but no difference in exercise intolerance was shown between the 2 groups.

Anti-alpha-Gal antibody response following xenogeneic heart valve implantation in adults.

BACKGROUND AND AIM OF THE STUDY: The study aim was to investigate the xenoreactive immune response directed at Galalpha1, 3-Galbeta1-4GlcNAc-R (alpha-Gal) which is known to be a major barrier in xenotransplantation, and to identify factors such as age, gender, ABO group and type of implanted tissue that might affect the anti-alpha-Gal immune response in adults subjected to bioprosthetic heart valve (BHV) implantation. METHODS: A total of 103 early survivors aged > 20 years who underwent cardiac surgery using cardiopulmonary bypass was enrolled. Among the patients (45 males, 58 females; mean age 62.8 years), 66 who underwent BHV implantation were assigned as a study group, while the remainder were assigned to a control group. Serum samples were obtained from all patients on three occasions: before surgery (TO); on postoperative day 1 (T1); and on postoperative day 14 or at discharge (T2). A serum sample was also obtained from 31 patients in the study group at the out-patient clinic (T3) at a mean of 38 days after surgery. RESULTS: Anti-alpha-Gal antibody reactivity at TO was higher in patients aged < 65 years. Anti-alpha-Gal IgM and IgG reactivity at T2 was higher in the study group when compared to that in controls. In the study group, anti-alpha-Gal IgM and IgG reactivities were decreased at T1, but then increased at T2 when compared to that at TO. Anti-alpha-Gal IgG reactivity remained elevated at T3, but the IgM reactivity declined in the study group. None of the factors, including age, gender, ABO group and type of implanted tissue, had any effect on the anti-alpha-Gal immune response after BHV implantation. CONCLUSION: BHV implantation in adults elicits an increased formation of anti-alpha-Gal antibodies, with different patterns for each isotype. Based on the study results, host factors including age, gender and blood type might be less important in the anti-alpha-Gal immune response following BHV implantation in adults.

Volumetric and functional assessment using cardiac magnetic resonance imaging in young children exposed to acute pulmonary regurgitation: is pulmonary regurgitation just a long-term matter?

BACKGROUND: The early effect of pulmonary regurgitation (PR) on both ventricular volume and function has not been well established in children. We evaluated the early effect of PR on both ventricular volume and function in young children. METHODS: Among patients who underwent total repair of pulmonary atresia with ventricular septal defect between January 2007 and December 2008, cardiac magnetic resonance imaging (CMRI) was performed in 12 patients at a median interval of 15.6 months (6-22 months). Valveless right ventricular outflow tract (RVOT) reconstruction was performed in five patients (valveless group) and RVOT reconstruction using valved conduit in seven patients (valve group). Age and weight at operation, and the interval between the operation and CMRI were not different between the groups. RESULTS: We observed a higher pulmonary regurgitant fraction (p = 0.003), a higher right ventricular end-diastolic volume index (RVEDVI) (p = 0.003), a higher right ventricular end-systolic volume index (p = 0.003), a higher left ventricular end-diastolic volume index (p = 0.010), a higher left ventricular end-systolic volume index (p = 0.018), and a lower left ventricular ejection fraction (LVEF; p = 0.048) in the valveless group. Right ventricular ejection fraction (RVEF) was not different between two groups. The RVEDVI was negatively correlated with RVEF (rho = -0.601, p = 0.039) and LVEF (rho = -0.580, p = 0.048). CONCLUSIONS: Both ventricular volumes increased and left ventricular function was compromised, but right ventricular function was preserved early after the exposure to PR in children. Right ventricular volume was associated with both ventricular functions.

Outcome of neonates with Ebstein's anomaly in the current era.

Ebstein's anomaly is frequently detected before birth, with prenatal detection accounting for the majority of cases in the current population. This study aimed to identify the outcome variables among these infants. The medical records of 59 patients with neonatal Ebstein's anomaly managed at the Asan Medical Center between January, 2001 and June, 2012 were investigated retrospectively. In 46 cases, the diagnosis was made prenatally. Surgical/interventional procedures were performed for 27 of the analyzed patients. Biventricular repair was successful for 12 patients but not for 9 patients with pulmonary atresia. The median follow-up period was 1.96 years (range 0.0-10.4 years). The overall mortality rate was 23.7 % (14/59). Of the 14 deaths, 5 occurred within several hours after birth. The 1- and 5-year survival rates were 78.6 and 76.3 %, respectively. Univariate analysis identified several variables related to the time to death: fetal distress (p = 0.002), prematurity (p = 0.036), low birth weight (p = 0.003), diameter of the atrial septal defect (p = 0.022), and pulmonary stenosis/atresia (p = 0.001). Neither the Carpentier classification (p = 0.175) nor the Celermajer index (p = 0.958) was a significant variable. According to the multivariate analysis, fetal distress (p = 0.004) and pulmonary atresia/stenosis (p < 0.001) were significant determinants of outcome. In conclusion, fetal distress and pulmonary atresia/stenosis are significant predictors of mortality in the current population of patients with neonatal Ebstein's anomaly. A close cooperation of associated clinicians is required for an improvement in outcome. To establish a better surgical strategy for patients with Ebstein's anomaly and pulmonary atresia, studies of larger populations are required.

Hybrid Right Ventricular Outflow Stent Insertion in a Small Neonate with Muscular Pulmonary Atresia with Intact Ventricular Septum: A Case Report.

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital heart disease that often needs a critical decision on whether to open the right ventricular outflow tract (RVOT). Significant morbidity and considerable mortality might preclude the safe use of percutaneous or surgical right ventricular decompression in patients with muscular PAIVS. We report the case of a 21-day-old neonate weighing less than 3 kg who underwent hybrid RVOT stent insertion as initial palliation for muscular PAIVS and subsequent anatomical correction at 5 months of age, with 6 years of follow-up.

Outcomes of Surgical Repair for Truncus Arteriosus: A 30-Year Single-Center Experience.

Background: We investigated the long-term outcomes of truncus arteriosus repair at a single institution with a 30-year study period. Methods: Patients who underwent repair of truncus arteriosus between 1993 and 2022 were reviewed retrospectively. Factors associated with early mortality, overall attrition, and reintervention were identified using appropriate statistical methods. Results: In total, 42 patients were enrolled in this study. The median age and weight at repair were 26 days and 3.5 kg, respectively. Thirty patients (71.4%) underwent 1-stage repair. There were 8 early deaths (19%). In the univariable analysis, undergoing surgery before 2011 was associated with early mortality (p=0.031). The overall survival rate at 10 years was 73.8%. In the multivariable analysis, significant truncal valve (TrV) dysfunction (p=0.010), longer cardiopulmonary bypass time (p=0.018), and the earlier era of surgery (p=0.004) were identified as risk factors for overall mortality. During follow-up, 47 reinterventions were required in 27 patients (64.3%). The freedom from all-cause reintervention rate at 10 years was 23.6%. In the multivariable analysis, associated arch obstruction (p<0.001) and significant TrV dysfunction (p=0.011) were identified as risk factors for all-cause reintervention. Arch obstruction (p=0.027) and a number of TrV cusps other than 3 (p=0.014) were identified as risk factors for right ventricle to pulmonary artery (RV-PA) reintervention, and significant TrV dysfunction was identified as a risk factor for TrV reintervention (p=0.002). Conclusion: Despite recent improvements in survival outcomes after repair of truncus arteriosus, RV-PA or TrV reinterventions were required in a significant number of patients during follow-up.

The Usefulness of Computed Tomography in Predicting Left Ventricular Outflow Tract Obstruction After Neonatal Arch Repair.

This study investigated the outcome after neonatal arch repair, and the usefulness of computed tomography (CT) in predicting the development of left ventricular outflow tract (LVOT) obstruction (LVOTO). A total of 150 neonates who underwent arch repair between 2008 and 2019 were included. The diameters of the aortic valve annulus (AVA) and LVOT in millimeters were measured with transthoracic echocardiography (TTE) or CT and indexed by subtracting body weight in kilograms. The outcomes of interest were the development of LVOTO (peak flow velocity > 3 m/s on TTE) and reintervention or reoperation for LVOTO. The median follow-up duration was 3.6 years. The rates of overall survival, freedom from reintervention for LVOTO, and freedom from the LVOTO development at 7 years were 93.7%, 88.2%, and 81.0%, respectively. In univariable Cox regression analysis, weight-indexed CT-measured LVOT diameter (concordance index [C-index] = 0.73, P = 0.002) and weight-indexed TTE-measured AVA diameter (C-index = 0.69, P = 0.001) were significant predictors of LVOTO. The maximal chi-square test identified the following cutoff values for predicting LVOTO: 1.4 for weight-indexed CT-measured LVOT diameter and 1.6 for weight-indexed TTE-measured AVA diameter. The high-risk group (both measures lower than the cutoff values) had a significantly lower rate of freedom from LVOTO development than the low-risk group (both measures higher than the cutoff values) (P < 0.001). The weight-indexed CT-measured LVOT diameter could be used to predict LVOTO development after neonatal arch repair, as an independent measure or complementary to traditional measures.

Long-term Surgical Outcomes of Supravalvar Aortic Stenosis: Modified Simple Sliding Aortoplasty.

This study investigated long-term outcomes and factors associated with reoperations in patients who underwent surgical repair of congenital supravalvar aortic stenosis (SVAS). A total of 39 consecutive patients who underwent congenital SVAS repair from 1999 through 2018 were included. Aortic root geometry was evaluated by measuring the ratio of the sinotubular junction diameter to the aortic annulus diameter (STJ/AVA) on echocardiography and proportion of intercommissural distance (ICD) of each sinus on computed tomography. The median age and weight at the time of operation were 4.3 years and 16.9 kg, respectively. Williams syndrome was associated in 25 patients (64.1%). Modified simple sliding aortoplasty (MSSA) was mostly used (n = 35, 89.7%). The median follow-up duration was 9.5 years. There were no early deaths and 1 late death. Overall survival rate was 97.0% at 15 years. There were 7 reoperations during follow-up. Freedom from reoperation for left ventricular outflow tract obstruction and all-cause reoperation were 91.9% and 80.4%, respectively. Age younger than 2 years at initial repair were associated with all-cause reoperation in the univariable analysis. In 35 patients who underwent MSSA, the degree of aortic regurgitation was equal to or less than mild in all patients during follow-up. Their median STJ/AVA on postoperative echocardiography was 0.95 (0.84-1.02). SVAS repair with MSSA provided excellent long-term survival with well-preserved aortic valve competence. Age younger than 2 years at initial repair might be associated with reoperation.

Outcomes after Mechanical Aortic Valve Replacement in Children with Congenital Heart Disease.

Background: The optimal choice of valve substitute for aortic valve replacement (AVR) in pediatric patients remains a matter of debate. This study investigated the outcomes following AVR using mechanical prostheses in children. Methods: Forty-four patients younger than 15 years who underwent mechanical AVR from March 1990 through March 2023 were included. The outcomes of interest were death or transplantation, hemorrhagic or thromboembolic events, and reoperation after mechanical AVR. Adverse events included any death, transplant, aortic valve reoperation, and major thromboembolic or hemorrhagic event. Results: The median age and weight at AVR were 139 months and 32 kg, respectively. The median follow-up duration was 56 months. The most commonly used valve size was 21 mm (14 [31.8%]). There were 2 in-hospital deaths, 1 in-hospital transplant, and 1 late death. The overall survival rates at 1 and 10 years post-AVR were 92.9% and 90.0%, respectively. Aortic valve reoperation was required in 4 patients at a median of 70 months post-AVR. No major hemorrhagic or thromboembolic events occurred. The 5- and 10-year adverse event-free survival rates were 81.8% and 72.2%, respectively. In univariable analysis, younger age, longer cardiopulmonary bypass time, and smaller valve size were associated with adverse events. The cut-off values for age and prosthetic valve size to minimize the risk of adverse events were 71 months and 20 mm, respectively. Conclusion: Mechanical AVR could be performed safely in children. Younger age, longer cardiopulmonary bypass time and smaller valve size were associated with adverse events. Thromboembolic or hemorrhagic complications might rarely occur.