Clinical Guidelines for Diagnosis and Management of Cowden Syndrome/PTEN Hamartoma Tumor Syndrome in Children and Adults-Secondary Publication.

Cowden syndrome (CS)/PTEN hamartoma tumor syndrome (PHTS) is a rare autosomal dominantly inherited condition caused by germline pathogenesis. It is associated with multiple hamartomatous lesions occurring in various organs and tissues, including the gastrointestinal tract, skin, mucous membranes, breast, thyroid, endometrium, and brain. Macrocephaly or multiple characteristic mucocutaneous lesions commonly develop in individuals in their 20s. This syndrome is occasionally diagnosed in childhood due to the occurrence of multiple gastrointestinal polyps, autism spectrum disorders, and intellectual disability. CS/PHTS can be diagnosed taking the opportunity of multigene panel testing in patients with cancer. Appropriate surveillance for early diagnosis of associated cancers is required because patients have a high risk of cancers including breast, thyroid, colorectal, endometrial, and renal cancers. Under these circumstances, there is growing concern regarding the management of CS/PHTS in Japan, but there are no available practice guidelines. To address this situation, the guideline committee, which included specialists from multiple academic societies, was organized by the Research Group on Rare and Intractable Diseases granted by the Ministry of Health, Labour, and Welfare, Japan. The present clinical guidelines explain the principles in the diagnosis and management of CS/PHTS, together with four clinical questions and the corresponding recommendations, incorporating the concept of the Grading of Recommendations Assessment, Development, and Evaluation system. Herein, we present an English version of the guideline, some of which have been updated, to promote seamless implementation of accurate diagnosis and appropriate management of pediatric, adolescent, and adult patients with CS/PHTS.

The degree of satisfaction and level of learning in male and female surgical residents: a nationwide questionnaire survey of graduating residents in Japan.

PURPOSE: Since 2002, the Japan Surgical Society has established a board certification system for surgeons to be certified for a specialty. Surgery remains a male-dominated field in Japan. This study aimed to clarify if the Japanese surgical residency training system is equally suitable for female and male residents. METHODS: The Japan Surgical Society conducted the first questionnaire survey regarding the system of surgical training for the residents in 2016. The questionnaire included the degree of satisfaction with 7 aspects of the training system, including the number and variety of cases experienced and duration and quality of instruction, and the learning level for 31 procedures. The degree of satisfaction and level of learning were compared between female and male residents. RESULTS: The degree of satisfaction was similar for all items between female and male residents. Female residents chose breast surgery as their subspecialty more frequently than male residents and were more confident in breast surgery procedures than male residents. Conversely, fewer female residents chose gastrointestinal surgery and were less confident in gastrointestinal surgery procedures than male residents. CONCLUSION: Female residents were as satisfied with the current surgical training system as male residents. However, there may be room for improvement in the surgical system, considering that fewer applications for gastrointestinal surgery come from female residents than from males.

A Cross-sectional Study of Regret in Cancer Patients After Sharing Test Results for Pathogenic Germline Variants of Hereditary Cancers With Relatives.

BACKGROUND: Research on whole genome/exome sequencing is increasing worldwide. However, challenges are emerging in relation to receiving germline pathogenic variant results and sharing them with relatives. OBJECTIVE: The aim of this study was to investigate the occurrence of and reasoning related to regret among patients with cancer who shared single-gene testing results and whole exome sequencing with family members. METHODS: This was a single-center, cross-sectional study. The Decision Regret Scale was administered, and descriptive questionnaires were used with 21 patients with cancer. RESULTS: Eight patients were classified as having no regret, 9 patients were classified as having mild regret, and 4 patients were classified as having moderate to strong regret. Reasons patients felt that sharing was the right decision included the following: to allow relatives and children to take preventive measures, the need for both parties to be aware of and ready for the hereditary transmission of cancer, and the need to be able to discuss the situation with others. On the other hand, some patients did not think it was a good decision to share the information because of the associated anxiety. CONCLUSIONS: Regret over sharing test results for pathogenic germline variants of hereditary cancers with relatives tended to be low. The main reason was that patients believed that they were able to benefit others by sharing. IMPLICATIONS FOR PRACTICE: Healthcare professionals need to understand the postsharing perceptions and experiences of patients and support them throughout the sharing process.

Impact of the Axillary Approach on Epicardial Pacing Lead Durability in Children.

BACKGROUND: To avoid lead failure and pocket infection in neonates and infants requiring pacemakers, we used the axillary approach of placing the generator in the axilla and the leads in the intrathoracic space. We describe the technical details of the axillary approach and evaluate the efficacy of this method. METHODS: We assessed 21 patients (7 male) weighing 8 kg or less who underwent epicardial pacemaker implantation with the axillary approach between 2004 and 2018. The axillary approach entails (1) positioning the pacemaker generator in the axilla to avoid local skin and pocket complications due to tissue compression by the generator, and (2) making a double loop in the pleural space to reduce stress on the pacemaker leads caused by somatic growth. This approach can be combined with median sternotomy for simultaneous intracardiac repair. RESULTS: The patients' median age at pacemaker implantation was 6 months; 16 patients (76%) were aged less than 12 months. The median body weight was 4.5 kg (interquartile range, 3 to 7). In all 5 patients requiring simultaneous cardiac repair, a median sternotomy was performed to access the heart. Sixteen patients required only pacemaker implantation: left thoracotomy was performed in 10 patients, right thoracotomy in 5, and subxiphoid approach in 1. The 5-year and 10-year freedom from pacemaker-related adverse events was 89.4% and 79.5%, respectively. CONCLUSIONS: The axillary approach using intrathoracic double-loop routing of leads to position the generator in the axilla for pacemaker implantation can be a valuable alternative for neonates/infants weighing 8 kg or less with or without complex congenital heart disease.

Sharing genetic test results of germline pathogenic variants of hereditary cancer with relatives: A single-center cross-sectional study.

OBJECTIVE: This study aimed to determine whether Japanese cancer patients share test results of germline pathogenic variants of hereditary cancer with their relatives. METHODS: This single-center cross-sectional study enrolled 21 Japanese patients who received results of germline pathogenic variants of hereditary cancer at least 6 months prior. RESULTS: All patients shared their test results with at least one relative, with the following sharing rates: 85.7% for first-degree relatives, 10% for second-degree relatives and 8.3% for third-degree relatives. Patients most commonly shared the information with their children aged >18 years (86.7%), followed by their siblings (73.6%), spouses (64.7%) and parents (54.5%). Three categories were extracted from qualitative analysis: 'characteristics of my cancer', 'knowledge and caution about inheritability' and 'utilization of medical care.' CONCLUSIONS: The rate of test result sharing with first-degree relatives was comparable with those in Europe and the USA. Patients with germline pathogenic variants also tended to share their test results more with their children and siblings than with their parents. Informing their relatives of the results was suggestive of the motivation to influence their relatives' health outcome and contribute to the well-being of their children and siblings.

Is there disparity between regions and facilities in surgical resident training in Japan? Insights from a national survey.

PURPOSE: This study sought to assess the disparity between regions and facilities in surgical resident training in Japan via a national level needs-assessment. METHODS: A survey was sent to all 909 graduating residents of 2016. Residents trained in the six prefectures with a population of 7 million or more were included in the large prefecture (LP) group. Residents trained in the other 41 prefectures were included in the small prefecture (SP) group. Each group was further divided into a university hospital (UH) group and a non-university hospital (NUH) group. RESULTS: The response rate was 56.3% (n = 512). Excluding nine residents who did not report their prefectures and facilities, surveys from 503 residents were analyzed. The UH group received significantly more years of training. In the SP and UH groups, there were significantly fewer residents who had performed 150 procedures or more under general anesthesia in comparison to the LP and NUH groups, respectively. Self-assessed competencies for several procedures were significantly lower in the SP and UH groups. CONCLUSION: Disparity in surgical resident training was found between regions and facilities in Japan. The surgical residency curriculum in Japan could be improved to address this problem.

Rapid two-stage Starnes approach in high-risk neonates with Ebstein anomaly.

OBJECTIVES: The purpose of this study is to review the short- and long-term outcomes of high-risk neonates with Ebstein anomaly treated with a newly developed rapid 2-stage Starnes procedure, which is aimed at reducing the size of the enlarged right side of the heart. METHODS: Fifty-two foetuses with Ebstein anomaly were analysed in this study and divided into 2 groups. The control group comprised 25 foetuses, referred to us before 2008, and the study group was composed of 27 foetuses, referred to us after 2009. The right atrial area index was defined as high risk when it was >1.5. We applied our management approach to 6 high-risk neonates in the study group. This approach consisted of reducing the size of the right side of the heart through a 2-stage process: (i) right atrial plication without the use of a bypass and (ii) a Starnes procedure. Cox proportional hazards models were used to evaluate the effects of our management approach on the survival rates of the neonates. RESULTS: The mean follow-up period was 7.5 ± 3.3 years. All 6 high-risk neonates in the study group survived. The overall hazard ratio was 0.12 (95% confidence interval of 0.03-0.43) in the study group as compared with the control group (P = 0.0007). A Fontan operation was completed in all but 1 case, with the remaining case awaiting a Fontan operation. CONCLUSIONS: We suggest that a rapid 2-stage Starnes approach can be effective in the treatment of high-risk neonates with Ebstein anomaly.

Are graduating residents sufficiently competent? Results of a national gap analysis survey of program directors and graduating residents in Japan.

PURPOSE: To evaluate the self-assessed competency of graduating residents (GRs) in Japan upon completion of their residency and to identify the gap between their competency and the competency expected by their program directors (PDs). METHOD: A list of 31 essential surgical procedures was compiled according to the consensus of surgical educators from around the country. A survey with this list was sent to all 909 GRs and their 611 PDs in 2016. The GRs rated their competency to perform these procedures and the PDs were asked to evaluate the expected competency of their GRs using the Zwisch Scale. RESULT: The response rate was 56.3% for the GRs and 76.8% for the PDs. Fewer than half of the GRs who responded felt confident performing ten (32%) of the surgical procedures evaluated. For most procedures, the GRs' self-reported competency was lower than the expectation reported by their PDs. This gap was more than 10% for 13 of the procedures. CONCLUSION: More than half of the GRs in Japan lacked the confidence in their skill to perform one-third of the surgical procedures selected for evaluation in this study. These findings should be used to update the surgical education curriculum in Japan.

Aortic erosion occurring in over 5 years after Amplatzer septal Occluder implantation for secundum atrial septal defect: a case report.

BACKGROUND: Aortic erosion is a serious complication that usually occurs shortly after Amplazter Septal Occluder (ASO) implantation for atrial septal defect (ASD). CASE PRESENTATION: A seven-year-old girl was diagnosed with secundum ASD without symptoms. Transesophageal echocardiography (TEE) showed a defect of 20 mm in diameter in the fossa ovalis without aortic rim. An ASO device of 24 mm in diameter was selected and electively implanted. The "A-shape" of the device was confirmed by intraoperative TEE, a landmark finding indicating the proper implantation of ASO in patients without aortic rim. After an uneventful postoperative course of 5 years and 10 months, she was transferred to our unit due to cardiogenic shock. Her echocardiogram in emergency room showed pericardial effusion with collapsed right ventricle. Given her history of ASO and the observation of the sequentially increasing pericardial effusion, we diagnosed her with acute cardiac tamponade due to aortic erosion. Emergency pericardiotomy was then performed to improve the hemodynamic condition. Fresh clots were found, so we immediately prepared the cardiopulmonary bypass circuit and explored the damage to the aorta, in which the clots had accumulated. Bleeding suddenly started when the clots were removed. We then inserted the cannulae for perfusion and venous drainage. The clots were removed, and tears were found in both the lateral side of the ascending aorta and the right atrial wall. Intraoperative TEE showed that an edge of the ASO device was directly touching the aortic wall and the Doppler color-flow imaging showed blood flow through this lesion. The erosive lacerations of both the ascending aorta and right atrium were detected from the inside after achieving cardioplegic cardiac arrest. The ascending aorta was obliquely incised, and the laceration was closed from inside the aortic root. The postoperative course was uneventful. She has been doing well for 5 years since the surgery. CONCLUSIONS: We experienced and successfully treated a rare case of acute cardiac tamponade caused by aortic erosion 5 years and 10 months after ASO implantation.

A snapshot of surgical resident training in Japan: results of a national-level needs assessment survey.

PURPOSE: To evaluate the status of surgical training in Japan through a national-level needs assessment. METHODS: A survey was sent to all 909 graduating residents (GRs) and their 611 program directors (PDs) for the year 2016. A working group of surgical educators from around the country was formed under the education committee of the Japan Surgical Society. The survey items were developed by consensus of this working group. The survey investigated the knowledge and problems of the current curriculum, and the status of the current residency training. RESULTS: The response rates were 56.3% of the GRs and 76.8% of the PDs. Among the participants, 47.6% of the GRs and 29.4% of the PDs believed that the residency curriculum did not match the clinical experience. Over 80% of the GRs and PDs agreed on the importance of training outside of the OR, whereas only 13% of the GRs had received such training regularly. Trainees also reported a lower satisfaction rate about the opportunity to train outside of the OR. CONCLUSION: This national-level needs assessment of surgical training in Japan identified several gaps in the curriculum. These results provide valuable data to assist the ongoing efforts for surgical residency curriculum improvement.

Physical Activity Among Postoperative Esophageal Cancer Patients.

BACKGROUND: Previous literature has reported that regular physical activity enhances health-related quality of life for cancer patients. However, there is a lack of studies that focus on physical activity among postoperative esophageal cancer patients. OBJECTIVE: The aims of this study were to (1) describe the prevalence of physical activity among postoperative esophageal cancer patients, (2) explore variables related to physical activity (demographics, nutrition, dysphagia, and health-related qualify of life), and (3) examine potential reasons for inactivity among participants who scored low using case studies. METHODS: In this cross-sectional design, physical activity was evaluated by the Japanese version of the International Physical Activity Questionnaire. Variables were analyzed using Spearman rank correlation coefficients, Mann-Whitney U tests, or Fisher exact tests. RESULTS: Fifty-eight patients participated in this study; 79% met the recommended physical activity guidelines. Present occupational status and past leisure-time physical activity behavior before the cancer diagnosis were related to current leisure-time physical activity and meeting the guidelines postoperatively. Participants who scored 0 (little or no activity) in the questionnaire also scored low in health-related quality of life. CONCLUSIONS: Participants performed especially well in physical activity related to leisure time and transportation. Positive reinforcement is needed for patients who perform adequate levels of physical activity, and reviewing the benefits of regular physical activity is encouraged for all. IMPLICATIONS FOR PRACTICE: Occupational status and past leisure-time physical activity before diagnosis can be indicators for promoting physical activity among postoperative esophageal cancer patients. Inactive participants should be supported to promote both physical activity and health-related qualify of life.

Mindfulness-based cognitive therapy for Japanese breast cancer patients-a feasibility study.

OBJECTIVES: Mindfulness-based intervention has been receiving growing attention in cancer care. This study aimed to examine feasibility and to preliminary explore effectiveness of mindfulness-based cognitive therapy (MBCT) in Japanese breast cancer patients, and to explore possible modification of the program so that it fits better with this population. METHODS: Twelve participants with diagnosis of Stage I-III breast cancer received an eight session, weekly MBCT intervention in a group therapy format. The participants were followed up until 3 months after the completion of the program. RESULTS: All the participants completed the program with high attendance rate (mean number of attended sessions = 7.7). Significant improvement in anxiety (Hospital Anxiety and Depression Scale (HADS) - anxiety subscale; effect size Cohen's d = 0.88, P < 0.05), trauma-related psychological symptoms (Impact of Event Scale-revised; d = 0.64, P < 0.01) and quality of life (Functional Assessment of Cancer Therapy-Breast Cancer: FACT-B; d = 0.72, P < 0.01), and trend-level improvement in depression (HADS - depression subscale; d = 0.53, P = 0.054) were observed. Qualitative analyses suggested the program may be beneficial for alleviating fear of cancer recurrence and for increasing spiritual well-being. Some recommended modification of the program was indicated from the post-intervention interviews. CONCLUSIONS: Mindfulness-based cognitive therapy was well accepted by Japanese breast cancer patients and yielded favorable effect on their psychological status and quality of life. Further effectiveness study in a randomized-control design is warranted.

Surgical approach for systemic-pulmonary shunt in neonates with functionally univentricular heart: comparison between sternotomy and thoracotomy.

OBJECTIVE: The preferred surgical approach for systemic-pulmonary shunts has changed from thoracotomy to sternotomy in our institution, to concomitantly manage the ductus arteriosus during surgery. The purpose of this study was to compare the outcomes of systemic-pulmonary shunts for neonates with functionally univentricular hearts based on surgical approach. METHODS: Fifty-two neonates with functionally univentricular hearts underwent systemic-pulmonary shunt via sternotomy (n = 28) or thoracotomy (n = 24). Patient characteristics, achievement rates of right heart bypass, and survival rates were compared for the different approaches. RESULTS: Prenatal diagnosis was made more common in the sternotomy group (p = 0.006). The shunt was placed more centrally in the sternotomy group. The ductus arteriosus was ligated or banded in most patients in the sternotomy group (26/28) and in a few patients in the thoracotomy group (6/24). Frequency of ductal management in the early postoperative phase was not different between the groups (21 vs 25 %), but three new incisions had to be made in the thoracotomy group. No differences were seen in the achievement rates of bidirectional cavopulmonary shunts (86 vs 87 % at 10 months of age) and total cavopulmonary connection (81 vs 81 % at 2 years of age), or in the survival rates (92 vs 96 % at 8 years). CONCLUSIONS: There were no differences in short- and long-term outcomes between the groups. The sternotomy approach might be preferable in the current era of prenatal diagnosis, to allow simultaneous duct management during systemic-pulmonary shunt surgery, particularly in patients with large ducts associated with functionally univentricular hearts.

Towards clinical application of pronuclear transfer to prevent mitochondrial DNA disease.

Mitochondrial DNA (mtDNA) mutations are maternally inherited and are associated with a broad range of debilitating and fatal diseases. Reproductive technologies designed to uncouple the inheritance of mtDNA from nuclear DNA may enable affected women to have a genetically related child with a greatly reduced risk of mtDNA disease. Here we report the first preclinical studies on pronuclear transplantation (PNT). Surprisingly, techniques used in proof-of-concept studies involving abnormally fertilized human zygotes were not well tolerated by normally fertilized zygotes. We have therefore developed an alternative approach based on transplanting pronuclei shortly after completion of meiosis rather than shortly before the first mitotic division. This promotes efficient development to the blastocyst stage with no detectable effect on aneuploidy or gene expression. After optimization, mtDNA carryover was reduced to <2% in the majority (79%) of PNT blastocysts. The importance of reducing carryover to the lowest possible levels is highlighted by a progressive increase in heteroplasmy in a stem cell line derived from a PNT blastocyst with 4% mtDNA carryover. We conclude that PNT has the potential to reduce the risk of mtDNA disease, but it may not guarantee prevention.

Coronary angioplasty for congenital obstruction of the left main coronary artery.

We present three children who underwent coronary angioplasty for the surgical treatment of a congenital obstruction of the left main coronary artery. An azygos vein patch or graft was a feasible and useful material for reconstructing congenital ostial stenosis and atresia of the left main coronary artery.

Micro(mi) RNA-34a targets protein phosphatase (PP)1γ to regulate DNA damage tolerance.

The DNA damage response (DDR) triggers widespread changes in gene expression, mediated partly by alterations in micro(mi) RNA levels, whose nature and significance remain uncertain. Here, we report that miR-34a, which is upregulated during the DDR, modulates the expression of protein phosphatase 1γ (PP1γ) to regulate cellular tolerance to DNA damage. Multiple bio-informatic algorithms predict that miR-34a targets the PP1CCC gene encoding PP1γ protein. Ionising radiation (IR) decreases cellular expression of PP1γ in a dose-dependent manner. An miR-34a-mimic reduces cellular PP1γ protein. Conversely, an miR-34a inhibitor antagonizes IR-induced decreases in PP1γ protein expression. A wild-type (but not mutant) miR-34a seed match sequence from the 3' untranslated region (UTR) of PP1CCC when transplanted to a luciferase reporter gene makes it responsive to an miR-34a-mimic. Thus, miR-34a upregulation during the DDR targets the 3' UTR of PP1CCC to decrease PP1γ protein expression. PP1γ is known to antagonize DDR signaling via the ataxia-telangiectasia-mutated (ATM) kinase. Interestingly, we find that cells exposed to DNA damage become more sensitive - in an miR-34a-dependent manner - to a second challenge with damage. Increased sensitivity to the second challenge is marked by enhanced phosphorylation of ATM and p53, increased γH2AX formation, and increased cell death. Increased sensitivity can be partly recapitulated by a miR-34a-mimic, or antagonized by an miR-34a-inhibitor. Thus, our findings suggest a model in which damage-induced miR-34a induction reduces PP1γ expression and enhances ATM signaling to decrease tolerance to repeated genotoxic challenges. This mechanism has implications for tumor suppression and the response of cancers to therapeutic radiation.

Hybrid palliation for a neonate with functional single ventricle and restrictive atrial septal defect: a case report.

We present the case of a neonate who was successfully treated with emergency hybrid palliation soon after birth, including a stent insertion into a severely thickened atrial septum and bilateral pulmonary artery banding. The patient was prenatally diagnosed with severe pulmonary venous hypertension owing to mitral atresia and intact atrial septum associated with functional single ventricle and coarctation of the aorta.

[Improvement of outcomes in the surgical treatment of hypoplastic left heart syndrome (HLHS) with staged Norwood operation].

On behalf of rapid progress of diagnostic technologies and new development of surgical technique or strategy, outcomes of surgical treatment of hypoplastic left heart syndrome (HLHS) has remarkably improved in the current practice. One of such approaches is the staged Norwood operation. We have reviewed our patients (n=54) between 2003 and 2013. A half of the patients with the staged group accomplished Norwood procedure and concomitant bidirectional cavopulmonary shunt at the mean age of 4 months old. An another half of the patients underwent secondary Norwood operation with Blalock-Taussig( BT) shunt or right-ventricle to pulmonary artery( RV-PA) conduit, because of ductal closure in spite of prostaglandin, or progressive reversed coarctation. Cardiac catheterization showed good results in both the primary (n=17) and the staged approach (n=37), partly because even in the primary Norwood group we used a smaller calibered graft for BT shunt or RV-PA conduit to unload the ventricle as much as possible. In conclusion, since the surgical mortality of staged group was superior to that of the primary group and the mid-term survival in the staged seemed to be better than that of primary, we would pursue our strategy of the staged approach in the surgical treatment of hypoplastic left heart syndrome.

Significance of the rapid increase in GSH levels in the protective response to cadmium exposure through phosphorylated Nrf2 signaling in Jurkat T-cells.

Although cadmium (Cd) is a redox system disruptor, the systematic defensive responses to Cd-induced oxidative stress remain unclear. In this study, we initially determined that when human T-cell-derived Jurkat cells were exposed to a low concentration of Cd, the glutathione (GSH) concentration rapidly increased via the transient nuclear accumulation of the transcription factor Nrf2. Therefore, we hypothesized that this increase in the GSH levels was a significant event that occurred in response to the Cd toxicity in the Jurkat T-cells. To test this hypothesis, the expression of Nrf2 in the cells was silenced using siRNA transfection. These restricted expression conditions demonstrated that the sensitivity of the Jurkat T-cells to Cd toxicity was significantly higher in the knockdown cells. Whereas we could not find differences in the metallothionein (MT) expression responses, accumulation of Nrf2 in the nuclei and the GSH increase after Cd exposure were clearly suppressed in the Nrf2 knockdown cells. These findings strongly suggest that the Cd-induced activation of GSH synthesis is initiated as an acute response for Cd detoxification. Furthermore, the Cd remaining in the Jurkat T-cells did not cause a significant inhibition of cell growth after the rapid and transient increase in the GSH concentration returned to its basal level. Additionally, we found that MT expression induced by Cd occurred much later, with the expression seen at least 12h or more after the Nrf2-dependent immediate responses were almost completed. These results indicate that the rapid increase in GSH is an essential defensive response, with the subsequent induction of MT potentially chelating the Cd retained in the cell, thereby leading to continued suppression of Cd toxicity.

Extracorporeal life support after cardiac surgery in children: outcomes from a single institution.

Extracorporeal life support (ECLS) is used after congenital heart surgery for several indications, including failure to separate from cardiopulmonary bypass, postoperative low cardiac output syndrome, and extracorporeal cardiopulmonary resuscitation. Here, we assessed the outcomes of ECLS in children after cardiac surgery at our institution. Medical records of all children who required postoperative ECLS at our institution were reviewed. Between 2003 and 2011, 36 (1.4%) of 2541 pediatric cardiac surgical cases required postoperative ECLS. Median age of patients was 64 days (range: 0 days-4.1 years). ECLS was in the form of either extracorporeal membrane oxygenation (ECMO; n = 24) or ventricular assist system (VAS; n = 12). Mean duration of ECLS was 4.9 ± 4.2 days. Overall, 21 patients (58%) were weaned off ECLS, and 17 patients (47%) were successfully discharged from the hospital. Patients with biventricular heart (BVH) had higher survival-to-hospital discharge rates compared with those with univentricular heart (UVH) (P = 0.019). Regarding ECLS type, UVH patients who received VAS showed higher rates of device discontinuation than UVH patients who received ECMO (P = 0.012). However, rates of hospital discharge were not significantly different between UVH patients who received VAS or ECMO. Surgical interventions, such as banding of Blalock-Taussig shunt to reduce pulmonary blood flow or placing bidirectional cavopulmonary shunt to minimize ventricular volume overload, were effective for weaning off ECLS in patients with UVH. ECLS is beneficial to children with low cardiac output after cardiac surgery. Rates of survival-to-hospital discharge were higher in BVH patients than UVH patients. Additional interventions to reduce ventricular volume load may be effective for discontinuing ECLS in patients with UVH.