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.

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.

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.

Extracorporeal Cardiopulmonary Resuscitation in Infants: Outcomes and Predictors of Mortality.

Background: Extracorporeal cardiopulmonary resuscitation (E-CPR) plays an indispensable role when resuscitation fails; however, extracorporeal life support (ECLS) in infants is different from that in adults. The objective of this study was to evaluate the outcomes of E-CPR in infants. Methods: A single-center retrospective study was conducted, analyzing 51 consecutive patients (age <1 year) who received E-CPR for in-hospital cardiac arrest between 2010 and 2021. Results: The median age and body weight was 51 days (interquartile range [IQR], 17-111 days) and 3.4 kg (IQR, 2.9-5.1 kg), respectively. The cause of arrest was cardiogenic in 45 patients (88.2%), and 48 patients (94.1%) had congenital cardiac anomalies. The median conventional cardiopulmonary resuscitation (C-CPR) time before the initiation of ECLS was 77 minutes (IQR, 61-103 minutes) and duration of ECLS was 7 days (IQR, 3-12 days). There were 36 in-hospital deaths (70.6%), and another patient survived after heart transplantation. In the multivariate analysis, single-ventricular physiology (odds ratio [OR], 5.05; p=0.048), open sternum status (OR, 8.69; p=0.013), and C-CPR time (OR, 1.47 per 10 minutes; p=0.021) were significant predictors of in-hospital mortality. In a receiver operating characteristic curve, the optimal cut-off of C-CPR time was 70.5 minutes. The subgroup with early E-CPR (C-CPR time <70.5 minutes) showed a tendency for lower in-hospital mortality tendency (54.5% vs. 82.8%, p=0.060), albeit not statistically significant. Conclusion: If resuscitation fails in an infant, E-CPR could be a life-saving option. It is crucial to improve C-CPR quality and shorten the time before ECLS initiation.

Characteristics of Patients with Surgical Closure of an Atrial Septal Defect during Infancy.

Background: Surgical closure of an atrial septal defect (ASD) is infrequently indicated during infancy. We evaluated the clinical characteristics and outcomes of patients who underwent surgical ASD closure during infancy. Methods: A single-center retrospective review was performed for 39 patients (19 males) who underwent surgical ASD closure during infancy between 1993 and 2020. The median body weight percentile at the time of operation was 9.3. Results: During a median follow-up of 60.9 months, 4 late deaths occurred due to chronic respiratory failure. A preoperative history of bronchopulmonary dysplasia (BPD) was the only risk factor for late mortality identified in Cox regression (hazard ratio, 3.54; 95% confidence interval [CI], 1.75-163.04; p=0.015). The 5-year survival rate was significantly lower in patients with preoperative history of BPD (97.0% vs. 50.0%, p<0.001) and preoperative ventilatory support (97.1% vs. 40.4%, p<0.001). There were significant postoperative increases in left ventricular end-diastolic (p=0.017), end-systolic (p=0.014), and stroke volume (p=0.013) indices. A generalized estimated equation model showed significantly better postoperative improvement in body weight percentiles in patients with lower weight percentiles at the time of operation (<10th percentile, p=0.01) and larger indexed ASD diameter (≥45 mm/m2, p=0.025). Conclusion: Patients with ASD necessitating surgical closure during infancy are extremely small preoperatively and remain small even after surgical closure. However, postoperative somatic growth was more prominent in smaller patients with larger defects, which may be attributable to an increase in postoperative cardiac output due to changes in ventricular septal configuration. The benefits of ASD closure in patients with BPD are undetermined.

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 Arterial Switch Operation With Aortic Arch Reconstruction.

BACKGROUND: This study investigated the outcomes and factors associated with reintervention or development of significant pulmonary stenosis (PS) after repair of transposition of the great arteries (TGA) or Taussig-Bing anomaly (TBA) with aortic arch obstruction. METHODS: A total of 51 patients with TGA or TBA who underwent an arterial switch operation and aortic arch reconstruction between 2004 and 2020 were included. The outcomes of interest were all-cause death, including heart transplantation, all-cause reintervention, right-sided reintervention, and development of significant PS. RESULTS: The median age and body weight at repair were 9 days and 3.2 kg, respectively. Forty-nine patients (96.1%) underwent 1-stage repair. A total of 28 patients (54.9%) had TBA, and 8 patients (15.7%) had interrupted aortic arch. There were 5 early deaths (9.8%) and 2 late deaths during a median follow-up duration of 59 months. The transplant-free survival rate 10 years after repair was 82.6%. A total of 21 reinterventions were required in 10 patients. The significant PS-free survival rate 10 years after repair was 68.8%. In univariable analysis, a higher ratio of the diameter of the main pulmonary artery to the ascending aorta was associated with all-cause reintervention (P = .007) and right-sided reintervention (P = .002). A smaller aortic annulus z-score at the pulmonary position was associated with the development of significant PS (P = .049). CONCLUSIONS: The rates of overall mortality and reintervention after repair were not negligible. A higher degree of size discrepancy between the 2 great arteries was associated with all-cause or right-sided reintervention. A smaller aortic annulus z-score at the pulmonary position was associated with the development of significant PS.

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 of pulmonary artery sling repair without tracheoplasty.

OBJECTIVE: This study aimed to investigate surgical outcomes of pulmonary artery (PA) sling without tracheoplasty. METHODS: From 2001 through 2020, among 22 patients who underwent PA sling repair, all but 1 patient who underwent concomitant tracheal surgery were analyzed. The outcomes of interest were all-cause death, PA reintervention, tracheal intervention, and readmission for respiratory symptoms. Computed tomography was used to measure the narrowest tracheal diameter. RESULTS: The median age and weight at repair were 7.6 months and 7.7 kg, respectively. Most patients (20 out of 21, 95.2%) had preoperative respiratory symptoms. Associated airway anomalies included tracheal ring in 12 (57.1%), bridging bronchus in 8 (38.1%), and tracheal bronchus in 2 patients (9.5%). There was 1 in-hospital death (4.8%). The median ventilator time and intensive care unit stay were 23 hours and 3 days, respectively. There was neither late death nor tracheal intervention during follow-up. Five patients (25.0%) underwent reintervention for left PA stenosis. Hospital readmission for respiratory symptom was required in 7 patients and was associated with the narrowest preoperative tracheal diameter (P = .025) and cardiopulmonary bypass time (P = .040) in univariable analysis. The narrowest tracheal diameter of 3.4 mm was identified as a cutoff value for readmission for respiratory symptom. Freedom from readmission for respiratory symptom was 63.3% at 10 years. CONCLUSIONS: PA sling repair without tracheal surgery might be a reasonable surgical option with rare need for tracheal intervention. Hospital readmissions for respiratory symptoms are more frequently required in patients with smaller tracheal diameter and all readmissions were limited to within 2 years after repair.

Risk factors for early adverse outcomes after bovine jugular vein conduit implantation: influence of oversized conduit on the outcomes.

OBJECTIVES: We investigated potential risk factors for early failure of bovine jugular vein conduit (Contegra®) implantation for right ventricular outflow tract (RVOT) reconstruction. METHODS: A single-centre retrospective review of 115 consecutive patients (54 males) who underwent RVOT reconstruction with Contegra between 2016 and 2019 was performed. Overall survival, explantation-free survival and freedom from significant RVOT lesions (valve regurgitation ≥ moderate or flow velocity ≥3.5 m/s) were investigated. RESULTS: Median age, body weight and Contegra diameter were 10.3 months [interquartile range (IQR) 5.7-26.9 months], 7.8 kg (IQR 6.3-12.4 kg) and 14 mm (IQR 12-16 mm), respectively. During the median follow-up duration of 25.1 months, there were 7 deaths, 34 significant RVOT lesions, 10 endocarditis episodes and 15 explantations. Overall survival and explantation-free survival at 3 years were 94.8% and 78.4%, respectively. Significant RVOT lesions (n = 34) comprised 20 stenoses, 8 regurgitations and 6 combined lesions. Freedom from significant RVOT lesions at 3 years was 62.6%. Cox regression identified higher indexed Contegra size (Contegra diameter/body weight, mm/kg) as the only risk factor for decreased time to explantation or death (hazard ratio 2.32, P < 0.001) and time to significant RVOT lesions development (hazard ratio 2.75, P < 0.001). The cut-off value of indexed Contegra size for significant RVOT lesions at 12 months was 1.905 mm/kg (sensitivity, 0.75; specificity, 0.78; area under the curve, 0.82). CONCLUSIONS: Outcomes of RVOT reconstruction using Contegra were acceptable. However, oversized Contegra should be avoided when possible. IRB APPROVAL DATE: 26 October 2020. IRB REGISTRATION NUMBER: S2020-2505-0001.

Contemporary Outcomes After Pulmonary Artery Banding in Complete Atrioventricular Septal Defect.

BACKGROUND: This study investigated the clinical outcomes and the effect of band tightness on outcome after pulmonary artery banding (PAB) in patients with complete atrioventricular septal defect (AVSD). METHODS: From 2000 through 2019, among 133 patients with isolated complete AVSD pursuing biventricular repair, 34 patients (25.6%) who underwent PAB were included in this study. Factors associated with adverse outcome, which was defined as prolonged stay in the intensive care unit (>10 days), were analyzed using a multiple logistic regression model. Receiver operating characteristic analysis was performed to identify a threshold band tightness for adverse outcome. RESULTS: The median age and weight were 43 days and 3.6 kg, respectively. There were 4 early deaths. The median intensive care unit stay was 8 days. Twenty-eight patients (28/34; 82.4%) underwent definitive repair 10 months (interquartile range, 7-12 months) after PAB. In multivariable analysis, indexed band diameter was identified as a factor associated with adverse outcome (odds ratio, 1.60; 95% CI, 1.03-2.48; P = .035). Receiver operating characteristic analysis indicated 22.2 mm/m2 of indexed PAB diameter measured at discharge as a threshold band tightness for adverse outcome (area under curve, 0.871; P < .001). The level of B-type natriuretic peptide similarly decreased after PAB regardless of band tightness, although the probability of worsening in atrioventricular valve regurgitation was significantly decreased in patients with a tighter band (P = .027). CONCLUSIONS: PAB is a viable option for early-presenting patients with complete AVSD. Tighter PAB might be beneficial for early postoperative outcomes and preventing progression of atrioventricular valve regurgitation in complete AVSD.

The impact of a bicuspid pulmonary valve in the aortic position after arterial switch for transposition of the great arteries on neoaortic root dimension and function: a propensity score matched analysis.

OBJECTIVES: This study investigated the effect of a bicuspid pulmonary valve on neoaortic root morphology, function, and the clinical outcomes of early survivors after the arterial switch operation using propensity score matching. METHODS: From 1997 to 2018, a total of 442 patients underwent the arterial switch operation for transposition of the great arteries. After exclusion of patients who underwent a staged repair, were repaired beyond 1 year of age, died before discharge and who lacked echocardiographic data at discharge, propensity score matching was used for analysis. RESULTS: Among 352 eligible patients, 18 patients (5.1%) had a bicuspid pulmonary valve. After propensity score matching (1:4), 15 patients with bicuspid valve (bicuspid group) and 60 patients with tricuspid valve (tricuspid group) were enrolled. The median follow-up duration was 9.9 years (4 months∼22.3 years). All-cause reoperation-free survival at 10 years was 93.3% in the bicuspid group and 87.0% in the tricuspid group (P = 0.839), and reoperation for neoaortic valve or root was required in 2 patients in the bicuspid group and 1 in the tricuspid group without intergroup difference. The z-score of the neoaortic annulus did not change in either group, although there was an increasing tendency in the z-score of the neoaortic sinus without intergroup difference (P = 0.690). Deterioration in neoaortic valve function was more prominent in the bicuspid group (p = 0.028). CONCLUSIONS: The neoaortic sinus might outgrow the norm regardless of the number of neoaortic valve cusps, whereas the neoaortic annulus remained unchanged. Deterioration of valve function was more prominent in the bicuspid group, which suggests that a bicuspid valve might play a significant role in deterioration of neoaortic valve function, without an additional effect on root pathology.

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.

Surgical Outcomes of Cor Triatriatum Sinister: A Single-Center Experience.

BACKGROUND: We investigated surgical outcomes after the surgical repair of cor triatriatum sinister (CTS). METHODS: Thirty-two consecutive patients who underwent surgical repair of CTS from 1993 through 2020 were included in this study. The morphological characteristics, clinical features, and surgical outcomes were described and analyzed. RESULTS: The median age and body weight at operation were 9 months (interquartile range [IQR], 3-238 months) and 7.5 kg (IQR, 5.8-49.6 kg), respectively. There were 16 males (50%). According to the modified Lucas classification, type IA (classical CTS) was most common (n=20, 62.5%). Atrial septal defect was associated in 22 patients (68.8%) and anomalous pulmonary venous return in 8 patients (25%). Pulmonary hypertension was preoperatively suspected with a high probability in 18 patients (56.3%). There was 1 early death (3.1%) after emergent membrane excision and hybrid palliation in a high-risk hypoplastic left heart syndrome patient. There were no late deaths. The overall survival rate was 96.9% at 15 years post-repair. No early survivors required reoperation during follow-up. Most survivors (31 of 32 patients, 96.9%) were in New York Heart Association functional class I at a median follow-up of 74 months (IQR, 39-195 months). At the latest echocardiography performed at a median of 42 months (IQR, 6-112 months) after repair, no residual lesion was observed except in 1 patient who had moderate pulmonary hypertension (mean pulmonary arterial pressure of 36 mm Hg). CONCLUSION: Surgical repair of cor triatriatum could be performed safely and effectively with an extremely low risk of recurrence.

Resolution of Postnatal Shock Before Open Heart Surgery in Neonates With Prenatal Diagnoses.

BACKGROUND: The impact of a prenatal diagnosis (PreND) for congenital heart disease on outcomes after neonatal open heart surgery is undetermined. We hypothesized that PreND has a positive impact on surgical outcomes in terms of immediate postnatal intensive care, which may lead to a decreased risk of persistent shock before surgery. METHODS: Among the 949 neonates who underwent open heart surgery between January 2002 and December 2017, 655 patients (69.0%) were diagnosed prenatally (group-PreND) and 294 patients (31.0%) were diagnosed postnatally (group-PostND). Procedural complexity, incidence of postnatal shock (serum lactate >4.0 mmol/L or pH <7.2), hospitalization length of stay, duration of shock, resolution of shock, and in-hospital mortality were compared between the 2 groups. RESULTS: In group-PreND, the procedure-dependent comprehensive Aristotle score (10.8 vs 10.0, P < .001), incidence of extracardiac anomalies (13.0% vs 7.1%, P = .008), heterotaxy syndrome (3.8% vs. 1.0%, P = .021), and postnatal shock (244 of 655 [37.3%] vs 78 of 294 [26.5%], P = .001) were higher than in group-PostND. However, patients in group-PreND were hospitalized earlier after birth (0 day vs 5 days, P < .001), experiencing shorter duration of shock (5.3 hours vs 9.0 hours, P = .01), and, consequently, showing higher incidence of shock resolution (212 of 244 [87%] vs 52 of 78 [67%], P < .001). In-hospital mortality was comparable between the 2 groups (P = .070). CONCLUSIONS: Postnatal shock is more frequently observed in group-PreND. However, prenatal awareness of the disease leads to immediate postnatal initiation of intensive care with shorter exposure to shock, leading to higher probability of shock resolution.

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.