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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.

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).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.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/mPAB 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.

Upper-extremity deep vein thrombosis (DVT) accounts for 1% to 4% of DVT cases. Brachial plexus injury can result from venous distention due to thrombosis in the upper extremity, leading to neurologic deficits. We report a patient diagnosed with brachial plexopathy caused by venous thrombosis in the upper extremity. A 66-year-old female patient with a medical history of Parkinson disease and dementia presented to the emergency department with drowsy mental status. She was diagnosed with pre-renal acute kidney injury with multi-organ dysfunction. On the second hospital day, right upper extremity edema and muscle weakness were observed. On the sixth hospital day, the patient’s overall medical condition improved after conservative treatment, but severe edema was noticed, and muscle weakness did not show significant improvement in the right upper extremity. Computed tomography showed multifocal pulmonary thromboembolism and DVT in the pulmonary arteries, superior vena cava, and popliteal veins. After anticoagulant administration, the swelling subsided, but the weakness in the entire right upper extremity did not improve. Electromyography demonstrated right brachial pan-plexopathy involving the upper, middle, and lower trunks. A follow-up examination showed no significant improvement of muscle strength and function in the right upper extremity at 3 months after the first hospital day.

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.

초록 전체 아래 내용으로 교체해주세요.Purpose: To compare the impact of hospital avoidance on the department of hand surgery at a tertiary hospital in Daejeon in the wake of the Middle East respiratory syndrome (MERS-CoV) and COVID-19 outbreaks.Methods: This is a retrospective comparative study of patients who were treated at the department of hand surgery in a tertiary hospital of a local community. We analyzed patients who were treated during the MERS-CoV and COVID-19 outbreaks. Controls included patients who were treated in the previous year during the same months. The number of outpatient visits and surgeries were compared to examine the difference from the previous year during the epidemic period of each infectious disease, and changes in demographic factors, the ratio of first and second visits, and the ratio of scheduled and emergent surgeries.Results: Under MERS-CoV, outpatient visits decreased by 9.7% and surgeries by 26.5% for three months. In June 2015, when MERS-CoV was widespread in Daejeon, the number of outpatients and surgeries dropped by 25.3% and 42.8%, respectively. During COVID-19, outpatient visits decreased by 4.3% and surgeries increased by 2.9%. However, when COVID-19 was most prevalent in Daejeon, the number of outpatients and surgeries increased by 36.6% and 31.1%, respectively.Conclusion: The impact of the COVID-19 outbreak in 2020 on the department of hand surgery at a tertiary hospital in Daejeon was limited, compared to the MERS-CoV epidemic in 2015, which can be attributed to improved infectious disease response capabilities.

This study aimed to investigate surgical outcomes of pulmonary artery (PA) sling without tracheoplasty.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.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.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.

This study aimed to investigate how long it takes for the dorsally displaced distal radial epiphysis to achieve realignment. We retrospectively reviewed 56 patients with dorsally displaced Salter-Harris type II distal radial epiphyseal fractures who were aged ≤15 years at the time of injury. All fractures were treated with closed reduction and immobilised using a sugar tong splint for 6 weeks. We evaluated the change in the displaced epiphysis position (%) until 12 weeks and the long-term clinical and radiological outcomes. We analysed significant differences in demographic factors and epiphyseal displacement according to the required period for epiphyseal realignment. The estimated area of the receiver operating characteristics (ROC) curve was calculated, and cut-off values were suggested to predict the required period for epiphyseal realignment. Sixteen (28.6%) and 42 (75%) patients achieved realignment of the epiphysis within 8 and 12 weeks, respectively. The cut-off values of 13.1 and 22.9% displacement at the 1-week follow-up were the best predictors of epiphyseal realignment within 8 and 12 weeks, respectively. Patients with a residual displacement of up to 51.3% in the sagittal plane at the 1-week follow-up achieved complete realignment of the epiphysis at the 6-month follow-up. From this study, we could predict the timing of epiphyseal realignment, and expect epiphyseal realignment even if re-displacement occurred up to 51.3% at the 1-week follow-up.

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 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

Myotonic dystrophy is a systemic disorder associated with progressive muscle weakness of the extremities and multi-organ dysfunction. This condition commonly affects the levator palpebrae superioris muscle, which leads to ptosis and eventually affects patients' activities of daily living. The effects of ptosis on activities of daily living are usually overlooked; therefore, the importance of treatment is underestimated, and surgical procedures are considered infeasible in patients with pre-existent levator palpebrae superioris muscle weakness. This case report describes the case of a middle-aged man with myotonic dystrophy type 1, who presented with ptosis, which significantly restricted his participation in rehabilitation and activities of daily living secondary to a limited visual field. He was evaluated by an ophthalmologist; however, surgery was contraindicated in view of the high risk of complications, such as exposure keratitis and corneal ulcer. Double eyelid tapes were used as a non-surgical intervention, which significantly increased the palpebral fissure height and improved the visual field, with a higher modified functional index score. Double eyelid tapes may serve as an effective, safe, and reliable therapeutic option for severe ptosis in patients with myotonic dystrophy.

Background Osteoarthritis (OA) and osteoporosis (OP) are the 2 most common bone disorders associated with aging. We can simply assume that older patients have a higher incidence of OA and OP with more severity. Although several papers have conducted studies on the relationship between OA and OP, none of them has demonstrated a conclusive link. In this study, we used radiological knee OA and bone mineral density (BMD; T-score of the total hip and lumbar spine) to analyze the incidence of OA and OP in a large population. We aimed to determine the relationship between OA and OP and investigate the associated risk factors Methods This cross-sectional study used data extracted from the 2010–2012 Korea National Health and Nutrition Examination Survey. We evaluated a total of 4,250 participants aged ≥ 50 years who underwent knee radiography and dual-energy X-ray absorptiometry and their laboratory results. The relationship between radiological knee OA and BMD was assessed. The generalized linear model was used to evaluate the relationship between BMD and Kellgren-Lawrence (KL) grade. Results The higher KL grade was associated with older age, higher body mass index (BMI), female sex, and lower hemoglobin level (p < 0.001). No significant association was found between OA and the following variables: white blood cell, platelet, total cholesterol, vitamin D, alkaline phosphatase, parathyroid hormone, hypertension, diabetes, asthma, dyslipidemia, smoking status, alcohol consumption, and regular exercise (p > 0.05). After adjusting for confounding factors (age, BMI, diabetes, hypertension, smoking, and alcohol consumption), the average T-scores of total hip and lumbar spine were the highest in the mild OA group with KL grade 2 (−0.22 ± 1.08 and −0.89 ± 1.46, respectively, p < 0.001). The average T-scores of the total hip and lumbar spine significantly decreased as OA progressed from moderate (KL grade 3; −0.49 ± 1.05 and −1.33 ± 1.38, respectively, p < 0.001) to severe (KL grade 4; −0.73 ± 1.13 and −1.74 ± 1.75, respectively, p < 0.001). T-scores of the moderate-to-severe OA group were significantly lower than those of the non-OA group (KL grades 0 and 1, p < 0.001). Conclusions Compared with the non-OA group, BMD (T-scores of the total hip and lumbar spine) was higher in the mild OA group and lower in the moderate-to-severe OA group.

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

Purpose Prompt diagnosis and treatment of septic arthritis of the knee is crucial. Nevertheless, the quality of evidence for the diagnosis of septic arthritis is low. In this study, the authors developed a machine learning-based diagnostic algorithm for septic arthritis of the native knee using clinical data in an emergency department and validated its diagnostic accuracy. Methods Patients (n = 326) who underwent synovial fluid analysis at the emergency department for suspected septic arthritis of the knee were enrolled. Septic arthritis was diagnosed in 164 of the patients (50.3%) using modified Newman criteria. Clinical characteristics of septic and inflammatory arthritis were compared. Area under the receiver-operating characteristic (ROC) curve (AUC) statistics was applied to evaluate the efficacy of each variable for the diagnosis of septic arthritis. The dataset was divided into independent training and test sets (comprising 80% and 20%, respectively, of the data). Supervised machine-learning techniques (random forest and eXtreme Gradient Boosting: XGBoost) were applied to develop a diagnostic model using the training dataset. The test dataset was subsequently used to validate the developed model. The ROC curves of the machine-learning model and each variable were compared. Results Synovial white blood cell (WBC) count was significantly higher in septic arthritis than in inflammatory arthritis in the multivariate analysis (P = 0.001). In the ROC comparison analysis, synovial WBC count yielded a significantly higher AUC than all other single variables (P = 0.002). The diagnostic model using the XGBoost algorithm yielded a higher AUC (0.831, 95% confidence interval 0.751–0.923) than synovial WBC count (0.740, 95% confidence interval 0.684–0.791; P = 0.033). The developed algorithm was deployed as a free access web-based application (www.septicknee.com). Conclusion The diagnosis of septic arthritis of the knee might be improved using a machine learning-based prediction model. Level of evidence Diagnostic study Level III (Case–control study).

We sought to determine the impact of juxtaductal stenosis (JDS) on branch pulmonary artery (PA) growth after systemic-to-pulmonary shunt (SPS) placement without pulmonary arterioplasty (ie the ‘shunt-only’ strategy). This was a retrospective review of 91 patients, 54 (59.3%) with pulmonary atresia and 27 (29.7%) with a functionally single ventricle, who underwent neonatal SPS placement without pulmonary arterioplasty between 2008 and 2017. The median age and body weight at SPS procedure were 16 day's (interquartile range [IQR], 11–22) and 3.10 kg (IQR: 2.85–3.40), respectively. All patients had pre-SPS computed tomography (CT) followed by post-SPS CT at a median interval of 5.8 months’ (IQR: 4.5–7.2). The ratio of the diameters of the juxtaductal PA over the non−SPS−side hilar PA (JD and/or PA) on preoperative CT—a surrogate for JDS severity—was 0.93 (IQR: 0.67–1.09). The median diameter (Z) of the SPS−side and non−SPS-side PA on postoperative CT were 1.0 (IQR: −0.07–1.73) and 0.99 (IQR: −0.45–1.70), respectively. The pulmonary artery index (Nakata index) increased significantly from 124.0 ± 50.2 mm2/m2 to 240.8 ± 88.7 mm2/m2 (P

Introduction Bethlem myopathy is a kind of collagen VI related myopathy which affects proximal skeletal muscles and leads to gait disturbance and multiple joint contractures with an onset in the first two decades of life. Lung function impairment (respiratory muscle and diaphragmatic weakness, ventilatory restriction, hypoxaemia and hypercapnia) and respiratory failure are part of the clinical spectrum and can occur in ambulatory patients. Methodology We carried out whole exome sequencing (WES) in combination with neuromuscular diseases-associated genes-filtering to detect the possible causative mutation(s) in a Korean family with Bethlem myopathy. An electrodiagnostic study showed myopathic pattern (normal nerve conduction study, and early recruitment and short amplitude muscle unit action potentials) in the proband. Results Coexistence of digenic mutations in the collagen VI genes (COL6A1 and COL6A3) was identified by WES in the proband only: heterozygous missense mutations of the COL6A1 (NM_001848.2: c.823G > T, p.Gly275Trp; rs1556425467) and of the COL6A3 genes (NM_004369.3: c.9349G > A, p.Asp3117Asn; rs1226664855). COL6A3 mutation may be candidate as disease-associated variant, as far as it was found only in the proband harboring another heterozygous mutation in COL6A1 gene, previously reported as different pathogenic mutations (p.Gly275Arg and p.Gly275Glu) at the same codon in Bethlem myopathy. Conclusion Our findings suggest that the coexistence of these digenic mutations is rare, but it may be used for the risk evaluation of individuals with a possible susceptibility to Bethlem myopathy. Taken together, genetic diagnosis using WES is a useful approach for the identification of pathogenic mutations associated with Bethlem myopathy.

Introduction Emery-Dreifuss muscular dystrophy (EDMD) also known as humeroperoneal muscular dystrophy, is a skeletal myopathy characterized by the clinical triad of progressive muscular weakness, joint contractures, and cardiac disease. Methodology Herein, we reported a family including two patients (the proband and his son) affected with progressive muscular dystrophy manifested by joint contractures without cardiac involvement (“EDMD-like” phenotype). Interestingly, electodiagnostic study results of the proband showed a neuropathic pattern different from the myopathic pattern in most muscular dystrophy patients. To identify the underlying genetic cause, genomic DNA of the proband was analyzed by WES using Agilent’s SureSelect XT Human All Exon v5. Results A novel de novo pathogenic heterozygous missense mutation (NM_182914.2: c.4858G > A; p.Ala1620Thr) of the SYNE2 gene, which had not been previously reported was identified by whole exome sequencing in the proband and by Sanger sequencing in his son. Conclusion To the best knowledge, SYNE2 mutation was reported first by whole exome sequencing in a Korean family with EDMD-like features. We emphasized the role of genetic analysis using whole exome sequencing, which allows the correct recognition of this molecular diagnosis and brings together the neuromuscular spectrum of this complex clinical scenario, leading to proper clinical management.

BACKGROUND Scaphoid nonunion in adolescents is rare, and most treatments include prolonged immobilization and screw fixation. Many studies have shown that Kirschner wires (K-wires) show comparable outcomes in screw fixation in adult scaphoid nonunion. However, few studies have reported K-wire fixation results in the treatment of adolescent scaphoid nonunion. The purpose of this study was to evaluate the clinical and radiologic results after bone graft and K-wire fixation for scaphoid nonunion in adolescents. METHODS We retrospectively reviewed 12 adolescent patients, mean age 15.4 (12 to 17) years, who underwent surgical treatment of scaphoid nonunion. Autogenous bone grafts with K-wire fixation were performed for all patients. Radiologic results, including bone union and degenerative changes, were evaluated with serial radiographs. Clinical results, including range of motion, grip strength, and a visual analogue scale for pain, were assessed. RESULTS All patients received

Younger gestational age (GA) is known to be associated with worse outcomes after congenital cardiac surgery. We sought to determine the impact of GA on surgical outcomes of single-ventricle palliation.Among the 284 patients with functionally single ventricle who were born between January 2005 and December 2014, 50 neonates were born prematurely (GA37 weeks) and 113 neonates in the early term period (37 weeks ≤ GA39 weeks). Initial palliation was required in 251 patients, whereas 33 patients received primary bidirectional cavopulmonary anastomosis (BCPA).BCPA and the completion Fontan operation were performed in 200 and 169 patients, respectively. Overall survival at 5 years were 62.5% ± 2.9%. On Cox regression younger GA (hazard ratio, 1.14 per 1-week decrease; P = .007) was identified as a risk factor for increased interstage mortality (ISM) between initial palliation and BCPA. On subgroup analysis of the preterm or early-term patients with initial palliation (n = 145), younger postmenstrual age at initial palliation was associated with increased ISM before BCPA (hazard ratio, 1.18; P = .005). After BCPA, however, younger GA did not increase the risk of ISM between BCPA and the Fontan operation (P = .47).Younger GA is a risk factor for ISM between initial palliation and BCPA. Deferral of initial palliation may be beneficial to decrease the risk of ISM in patients who were born at preterm or early term. Adverse effects of younger GA on survival disappeared once BCPA was performed.

This retrospective study aimed to investigate the outcomes of allogeneic hematopoietic stem cell transplantation (HSCT) for childhood myelodysplastic syndrome (MDS). Thirty-six patients (low-grade MDS, 24; advanced MDS, 12) received HSCT at the Asan Medical Center over two decades (early period, 1997–2007; recent period, 2008–2017). The transplantation outcomes were analyzed according to disease status, conditioning regimen, various donor types, and period of HSCT. During a median follow-up of 5.6 (range, 1.4–21.1) years, the probability of overall survival (OS) and failure-free survival was 77% and 69%, respectively. The cumulative incidence of transplantation-related mortality (TRM) was 12%. Significantly reduced TRM and improved OS were observed in patients who received HSCT during the recent period vs. the early period (TRM, 4% vs. 30%, P = 0.021; OS, 87% vs. 50%, P = 0.006). Comparable outcomes were observed for HSCT from haploidentical family donors vs. HLA-identical donors (TRM, 10% vs. 14%, P = 0.837; OS, 86% vs. 79%, P = 0.625). This study identified the improved outcomes of allogeneic HSCT for childhood MDS over time, in addition, the feasible outcomes of haploidentical HSCT suggested its use as an attractive alternative in the future procedures.

OBJECTIVES The objectives of this study were to evaluate long-term outcomes of pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot (TOF) and to identify the factors associated with adverse clinical events (ACEs). METHODS A total of 190 patients who underwent PVR between 1998 and 2015 after repair of TOF were retrospectively analysed. ACE was defined as all-cause death, heart transplantation or new-onset sustained arrhythmia. Univariable Cox proportional hazards regression analysis was used to identify the factors associated with ACE after PVR. RESULTS The median age at PVR was 19 years. Preoperative magnetic resonance imaging (MRI) was performed in 143 (75%) patients, and the median right ventricular (RV) end-diastolic and end-systolic volume index was 164 and 82 ml/m2, respectively. The follow-up completeness was 94%, and the median follow-up duration was 9.8 years. The transplantation-free survival and freedom from ACE at 15 years was 95% and 90%, respectively. The factors associated with ACE were older age at PVR, older age at TOF repair, New York Heart Association functional class III or IV, presence of tachyarrhythmias, longer cardiopulmonary bypass time and concomitant arrhythmia surgery. In a subgroup analysis of 143 patients with preoperative MRI data, larger RV end-systolic volume index, larger left ventricular end-systolic volume index and lower left ventricular ejection fraction were associated with ACE. CONCLUSIONS Long-term outcomes of PVR in patients with repaired TOF were satisfactory. Proactive PVR before the onset of advanced symptoms, tachyarrhythmias and ventricular dysfunction may further improve the long-term survival of this patient population.