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Pulmonary atresia with an intact ventricular septum (PAIVS) is a rare congenital cardiac disease with various structural abnormalities. For patients with moderate right ventricular (RV) hypoplasia for whom the decision has not been made between biventricular and single ventricular repair, if possible, the ideal treatment choice is biventricular repair, by promoting the growth of the underdeveloped RV. Since RV sinus myectomy has been proposed, various surgical strategies have been introduced to promote RV growth. Herein, we described instances of RV sinus myectomy and the accompanying techniques for “RV overhaul.” Furthermore, we introduced a current approach for small patients with PAIVS within an uncertain area.

Atrioventricular valve regurgitation (AVVR) is associated with increased morbidity and mortality in patients with single ventricle physiology. The purpose of this study was to evaluate the long-term results of the surgical management of AVVR and to analyze the effects of AV valve replacement. The medical records of 38 single ventricle patients who underwent atrioventricular valve surgery more than once between January 2001 and March 2018 were retrospectively reviewed. We analyzed and compared clinical data of patients who underwent valve replacement as an initial treatment (n = 8) for AVVR with patients who initially underwent valve repair (n = 30). The median follow-up duration was 98.1 months (range, 0.9–209.6 months). There was one early mortality and seven late mortalities. Freedom from reoperation between the two groups at 15 years of follow-up was significantly different: 18.3% in the repair group and 100% in the replacement group (p = 0.013). The replacement group showed a better overall survival rate (100%) at 15 years than the repair group (68.5%) without statistical significance (p = 0.097). All mortalities occurred in the repair group. Nine patients in the repair group (30%) and one patient in the replacement group (12.5%) showed preoperative ventricular dysfunction. RV-type single ventricle with atrioventricular (AV) valve annular dilatation was found out as a risk factor of AVV replacement both in univariate (p = 0.04) and multivariate (p = 0.004) analysis. AV valve replacement might be considered as a primary treatment option for patients who have an annular dilation with an RV-type single ventricle rather than repeated valvuloplasty.

Background:Although coronary artery bypass grafting is not frequently performed in children, Kawasaki disease is one of the most common indications for coronary artery bypass grafting in children. Here, we reviewed the long-term clinical outcomes including graft patency after coronary artery bypass grafting.Methods:Between March 2004 and March 2013, six patients with Kawasaki disease underwent coronary artery bypass grafting. All patients were male. Their median age was 13.0 years (interquartile range, 7.8–17.8 years) at the timing of coronary artery bypass grafting, and the median age at the onset of Kawasaki disease was 3.3 years (interquartile range, 1.0–7.0 years). Four patients presented with multiple lesions including aneurysms.Results:The median follow-up duration was 12.1 years (interquartile range, 9.5–13.1 years), and there were no operative complications or overall mortality. One patient had pre-operative symptoms such as exertional chest pain and dyspnoea on exertion, whereas one patient had ventricular tachyarrhythmia. There was an improvement in subjective symptoms after surgery in two patients. The left internal thoracic artery, right internal thoracic artery, and saphenous vein were used in five (83.3%), one (16.7%), and two (33.3%) cases, respectively. In all six patients, post-operative single-photon emission CT findings showed improved perfusion compared with pre-operative single-photon emission CT. All grafts were patent as confirmed by coronary angiography or CT angiography.Conclusions:Coronary artery bypass grafting could be a good surgical option in children with coronary lesions caused by Kawasaki disease in terms of graft patency and myocardial perfusion.

We encountered siblings with familial Majewski osteodysplastic primordial dwarfism type II (MOPD II) with acute myocardial infarction in adolescence and in their early 20s. We successfully performed percutaneous and surgical coronary interventions. From these cases, we were able to better understand coronary artery disease of MOPD II and provide better management. (Level of Difficulty: Intermediate.), Central Illustration

OBJECTIVES We compared the clinical outcomes between tricuspid valve detachment (TVD) and non-TVD for ventricular septal defect (VSD) closure in infants METHODS From January 2004 to April 2020, 462 infants RESULTS The median age and body weight at operation were 1.9 months [interquartile range(IQR), 1.4–2.5] and 4.2 kg (IQR, 3.7–4.6). The median follow-up duration was 83.4 months (IQR, 43.5–130.4). After matching, 44 pairs were extracted from each group. There were no significant differences in all-cause mortality (P = 0.176), reoperation (P = 0.172), postoperative morbidities, including residual VSD, aortic regurgitation, atrioventricular block and significant tricuspid regurgitation (TR) (P = 0.346) between group D and group N. However, group D showed significantly less TR progression during follow-up (P = 0.019). CONCLUSIONS In infants

Since the first reported case of coronavirus disease 2019 (COVID-19) in December 2019, the numbers of confirmed cases and deaths have continued to increase exponentially despite multi-factorial efforts. Although various attempts have been made to improve the level of evidence for extracorporeal membrane oxygenation (ECMO) treatment over the past 10 years, most experts still hesitate to take an active position on whether to apply ECMO in COVID-19 patients. Several ECMO management guidelines have been published recently, but they reflect some important differences from the Korean medical system and aspects of real-world medical practice in Korea. We aimed to find evidence on the efficacy of ECMO for COVID-19 patients by reviewing the published literature and to propose expert recommendations by analyzing the Korean COVID-19 ECMO registry data.

Background Nepal has a high prevalence of congenital anomaly contributing to high infant mortality. Ultrasound, an important tool to detect congenital anomalies and manage maternity-related risk factors, is not properly used in Nepal because Nepali doctors have limited opportunities for learning ultrasound techniques. Hence, we developed and implemented an ultrasound education program from 2016 to 2018. The objective of this study is to evaluate the education program using the Reach, Effectiveness, Adoption, Implementation, and Maintenance (RE-AIM) framework. Methods We conducted a mixed-method study to evaluate each component of RE-AIM. The team collected quantitative data from administrative records, tests, surveys, and an online follow-up survey. Qualitative data were collected from individual in-depth interviews at least a year after the program. The proportions, means, and t-tests were used for quantitative data, and thematic coding for qualitative data. Results A total of 228 healthcare workers representing 27.3% of the districts of Nepal were reached from 2016 to 2018. The program improved participants’ knowledge (29.3, 8.7, and 23.8 increases out of 100, each year, pn=85) and self-confidence (0.6, 0.3, 1.3 increases out of 4.0, pn=111). The participants were highly satisfied with the program (4.2, 4.1, and 4.0 out of 5.0, n=162). Among the respondents of the online follow-up survey (n=28), 60.7% had used ultrasound in their daily practice after the education program, and a medical institution established an ultrasound training center. The absence of clear accreditation and practical guidelines in ultrasound use were presented as barriers for adoption and maintenance. Conclusion The program was successful in improving participant’s knowledge and self-confidence in ultrasound techniques and showed great potential for the adoption and maintenance of the techniques in their practice. Continuous implementation of the program and institutional policy changes to facilitate ultrasound use may increase the ultrasound use and improve ultrasound service quality in Nepal.

Background: Despite advances in neonatal intensive care and surgical procedures, perinatal mortality rates for premature infants with congenital heart disease (CHD) remain relatively high.Purpose: We aimed to describe the outcomes of premature infants with critical CHD and identify the risk factors including the new modified version of the Risk Adjustment for Congenital Heart Surgery (M-RACHS) category associated with in-hospital mortality in a Korean tertiary center.Methods: This was a retrospective cohort study of premature infants with critical CHD admitted to the neonatal intensive care unit from January 2005 to December 2016.Results: A total of 78 premature infants were enrolled. The median gestational age (GA) at birth was 34.9 weeks (range, 26.7–36.9 weeks), and the median birth weight was 1.91 kg (range, 0.53–4.38 kg). Surgical or percutaneous intervention was performed in 68 patients with a median GA at birth of 34.7 weeks (range, 26.7–36.8 weeks) and a median birth weight of 1.92 kg (range, 0.53–4.38 kg). The in-hospital survival rate was 76.9% among all enrolled preterm infants and 86.8% among patients who received an intervention. Very low birth weight (VLBW), persistent pulmonary hypertension of the newborn (PPHN), bronchopulmonary dysplasia (BPD), and M-RACHS category 5 or higher (more complex CHD) were independently associated with in-hospital mortality. For the 68 premature infants undergoing cardiac interventions, independent risk factors for mortality were VLBW, BPD, and CHD complexity. Late preterm infant and age at intervention were not associated with patient survival.Conclusion: For premature infants with critical CHD, VLBW, PPHN, BPD, and M-RACHS category ≥5 were risk factors for mortality. A careful approach to surgical intervention and prenatal care should be taken according to CHD type and neonatal condition.

Although the clinical outcomes of truncus arteriosus (TA) repair have been improving, few data are available on long-term outcomes after truncus arteriosus repair in the current era. This study evaluated long-term outcome after repair of TA. Fifty-one patients underwent total correction from April 1982 to June 2018. Since 2003, perioperative strategy has changed to minimal priming volume, modified ultrafiltration, and early total repair (n = 26). Mortality and reoperation rates were analyzed before and after 2003. There were 8 hospital deaths after initial operation, all before 1997. During the mean follow-up of 9.8 years, there were 2 deaths. The Kaplan-Meier estimate of survival among all hospital survivors was 94.7% at 5 years and 88.0% at 20 years. A significant independent risk factor for early mortality was operation before 2003 (Hazard ratio (HR) 9.710, p = 0.041) and REV operation (HR 8.000, p = 0.028). Freedom from reoperation for conduit change and TV repair were 88.3% and 41% at 1 and 5 years, and 96.2% and 85.4% at 1 and 5 years, respectively. After 2003, younger age and conduit choice were risk factors for conduit-related reoperation. Initial preoperative TV regurgitation was independent risk factor for sequential TV repair. Patients with TA can undergo total repair of TA with excellent results, especially in current era. Most of the patients require conduit-related reoperations. Younger age and the methods of RVOT reconstruction were risk factors for conduit-related reoperations. TV repair is necessary in limited patients, and initial regurgitation was a risk factor.

Background Perioperative dexmedetomidine use has been reported to reduce the incidence of postoperative acute kidney injury after adult cardiac surgery. However, large-scale randomized controlled trials evaluating the effect of dexmedetomidine use on acute kidney injury in pediatric patients are lacking. Aims We investigated whether intraoperative dexmedetomidine could reduce the incidence of acute kidney injury in pediatric cardiac surgery patients. Methods In total, 141 pediatric patients were randomly assigned to dexmedetomidine or control groups. After anesthetic induction, patients in the dexmedetomidine group were administered 1 µg/kg of dexmedetomidine over 10 minutes and an additional 0.5 µg/kg/h of dexmedetomidine during surgery. Additionally, 1 µg/kg of dexmedetomidine was infused immediately after cardiopulmonary bypass was initiated. The incidence of acute kidney injury was defined following Kidney Disease Improving Global Outcomes guidelines. Results The final analysis included 139 patients. The incidence of acute kidney injury did not differ between dexmedetomidine and control groups (16.9% vs 23.5%; odds ratio 0.661; 95% CI 0.285 to 1.525; P = .33). Similarly, neither the incidence of abnormal postoperative estimated glomerular filtration rate values (P = .96) nor the incidence of arrhythmia, mechanical ventilation duration, length of stay in the intensive care unit, and hospitalization differed between the two groups. Conclusions Intraoperative dexmedetomidine did not reduce acute kidney injury incidence in pediatric cardiac surgery patients.

Though ventricular assist devices (VADs) are an important treatment option for acute heart failure, an extracorporeal membrane oxygenator (ECMO) is usually used in pediatric patients for several reasons. However, a temporary centrifugal pump-based Bi-VAD might have clinical advantages versus ECMO or implantable VADs. From January 2000 to July 2018, we retrospectively reviewed 36 pediatric patients who required mechanical circulatory support (MCS) for acute heart failure. Cases with postoperative MCS were excluded. Since 2016, we have tried to immediately add a right VAD rather than ECMO, when the patients begin to present features of right heart failure after left VAD support started in cases that the patients’ respiratory function did not require an oxygenator. Original diagnoses included dilated cardiomyopathy (n = 18), myocarditis (n = 11), and others (n = 7). Eleven patients were supported by Bi-VAD, and 25 patients were supported by ECMO; of these. Four patients were successfully weaned from VAD, and 10 patients were weaned from ECMO. Eleven patients underwent heart transplantation. Overall, we have 15 (41.7%) early mortalities. There were no significant differences in early mortality, morbidity, and weaning rate between the Bi-VAD group and the ECMO group. During the support, patients with Bi-VADs significantly required fewer platelets and showed less hemolysis than ECMO patients. Patients with myocarditis were successfully weaned from Bi-VAD support and bridged to transplantation thereafter. A temporary centrifugal pump-based Bi-VAD was clinically comparable to ECMO for pediatric patients with acceptable pulmonary function.

The purpose of this study was to develop a diagnostic tool to automatically detect temporomandibular joint osteoarthritis (TMJOA) from cone beam computed tomography (CBCT) images with artificial intelligence. CBCT images of patients diagnosed with temporomandibular disorder were included for image preparation. Single-shot detection, an object detection model, was trained with 3,514 sagittal CBCT images of the temporomandibular joint that showed signs of osseous changes in the mandibular condyle. The region of interest (condylar head) was defined and classified into 2 categories—indeterminate for TMJOA and TMJOA—according to image analysis criteria for the diagnosis of temporomandibular disorder. The model was tested with 2 sets of 300 images in total. The average accuracy, precision, recall, and F1 score over the 2 test sets were 0.86, 0.85, 0.84, and 0.84, respectively. Automated detection of TMJOA from sagittal CBCT images is possible by using a deep neural networks model. It may be used to support clinicians with diagnosis and decision making for treatments of TMJOA.

Background The double-lumen cannula (DLC) has begun to be used worldwide for venovenous (VV) extracorporeal membrane oxygenation (ECMO). We aimed to examine whether the DLC could be an effective tool in the treatment of pediatric respiratory failure in Korea. Methods We reviewed the records of patients weighing under 15 kg who underwent ECMO due to respiratory failure between January 2017 and December 2018. Outcomes of ECMO using a DLC and conventional ECMO using central method or 2 peripheral cannulas were compared. Results Twelve patients were treated with ECMO for respiratory failure. Among them, a DLC was used in 5 patients, the median age of whom was 3.8 months (interquartile range, 0.1-49.7 months). In these patients, the median values of pH, partial pressure of carbon dioxide, and partial pressure of oxygen were 7.09, 74 mm Hg, and 37 mm Hg before ECMO and corrected to 7.31, 44 mm Hg, and 85 mm Hg, respectively, after ECMO cannulation. Median blood flow rate in the patients treated with ECMO using a DLC was slightly higher than that in the conventional ECMO group, but this difference was not statistically significant (86.1 mL/kg/min and 74.3 mL/kg/min, respectively; p=1.00). One patient from the DLC group and 3 patients from the conventional group were weaned off ECMO. Conclusion VV ECMO using a DLC provided adequate oxygenation, ventilation, and blood flow rate in Korean pediatric patients with respiratory failure. Further prospective and randomized studies are warranted.

Background As a result of advances in pediatric care and diagnostic testing, there is a growing population of adults with congenital heart disease (ACHD). The purpose of this study was to better define the epidemiology and changes in the trend of hospitalizations for ACHD in Korean society. Methods We reviewed outpatient and inpatient data from 2005 to 2017 to identify patient ≥18 years of age admitted for acute care with a congenital heart disease (CHD) diagnosis in the pediatric cardiology division. We tried to analyze changes of hospitalization trend for ACHD. Results The ratio of outpatients with ACHD increased 286.5%, from 11.1% (1748/15,682) in 2005 to 31.8% (7795/24,532) in 2017. The number of ACHD hospitalizations increased 360.7%, from 8.9% (37/414) in 2005 to 32.1% (226/705) in 2017. The average patient age increased from 24.3 years in 2005 to 27.4 in 2017. The main diagnosis for admission of ACHD is heart failure, arrhythmia and Fontan-related complications. The annual ICU admission percentage was around 5% and mean length of intensive care unit (ICU) stay was 8.4 ± 14.6 days. Mean personal hospital charges by admission of ACHD increased to around two times from 2005 to 2017. (from $2578.1 to $3697.0). Total annual hospital charges by ACHD markedly increased ten times (from $95,389.7 to $831,834.2). Conclusions The number of hospital cares for ACHD dramatically increased more than five times from 2005 to 2017. We need preparations for efficient healthcare for adults with CHD such as a multi-dimensional approach, effective communication, and professional training.

Insertion of an epicardial pacemaker is a useful treatment for pediatric patients with an abnormal heart rhythm. However, there are limitations and concerns when implanting epicardial pacemakers in infants and neonates due to their small body size. We report a pa-tient who experienced rare complications after implantation of a permanent pacemaker.

Premature fusion of the cranial suture and midface hypoplasia are common features of syndromic craniosynostosis caused by mutations in the FGFR2 gene. The only treatment for this condition involves a series of risky surgical procedures designed to correct defects in the craniofacial bones, which must be performed until brain growth has been completed. Several pharmacologic interventions directed at FGFR2 downstream signaling have been tested as potential treatments for premature coronal suture fusion in a mouse model of Apert syndrome. However, there are no published studies that have targeted for the pharmacologic treatment of midface hypoplasia. We used Fgfr2S252W/+knock-in mice as a model of Apert syndrome and morphometric analyses to identify causal hypoplastic sites in the midface region. Three-dimensional geometric and linear analyses of Fgfr2S252W/+mice at postnatal day 0 demonstrated distinct morphologic variance. The premature fusion of anterior facial bones, such as the maxilla, nasal, and frontal bones, rather than the cranium or cranial base, is the main contributing factor toward the anterior-posterior skull length shortening. The cranial base of the mouse model had a noticeable downward slant around the intersphenoid synchondrosis, which is related to distortion of the airway. Within a skull, the facial shape variance was highly correlated with the cranial base angle change along Fgfr2 S252W mutation–induced craniofacial anomalies. The inhibition of an FGFR2 downstream signaling enzyme, PIN1, via genetic knockdown or use of a PIN1 inhibitor, juglone, attenuated the aforementioned deformities in a mouse model of Apert syndrome. Overall, these results indicate that FGFR2 signaling is a key contributor toward abnormal anterior-posterior dimensional growth in the midface region. Our study suggests a novel therapeutic option for the prevention of craniofacial malformations induced by mutations in the FGFR2 gene.

Vaccine hesitancy is the primary barrier to controlling the COVID-19 pandemic in South Korea. We used logistic multivariate regression modeling to investigate (1) the prevalence and reasons for COVID-19 vaccine hesitancy, (2) sub-groups that had higher rates of vaccine hesitancy, and (3) vaccine hesitancy predictors. We used a national survey of representatively sampled households (n = 13,021 adults) from October to December 2020. A self-report questionnaire asked about vaccination intention and reasons for hesitancy and gathered data on socio-demographic, demographic, psychological, and experiential factors. Our study indicated that 39.8% of the participants answered that they hesitated or refused to be vaccinated. The most common reason for vaccine hesitancy was a lack of confidence in the COVID-19 vaccine (77.9%). Less or no fear of COVID-19 (OR = 2.08, 95% CI = 1.92-2.26; OR = 1.79, 95% CI = 1.54-2.08), unstable job status (OR = 1.42, 95% CI = 1.18-1.70), decreased family income (OR = 1.40, 95% CI = 1.21-1.61), and worsening health status (OR = 1.38, 95% CI = 1.13-1.68) were predictors of vaccine hesitancy. Younger age, no religious affiliation, political conservatism, and lower family income were also significantly associated with vaccine hesitancy. Effective health communication and policies need to consider the target subgroup population and predictors of vaccine hesitancy to attain herd immunity at an early stage.

The surgical treatment for young patients with aortic valve diseases has not been standardized because of differences in the patients’ growth. We aimed to investigate short-term results of aortic valve repair with leaflet extension techniques. From October 2017 to June 2020, we performed aortic valve repair with leaflet extension technique in 15 consecutive patients with a median age of 13 years (range, 33 months–27 years). Among them, all had moderate or severe aortic regurgitation (AR), eight had concomitant aortic stenosis, and 12 underwent prior cardiac operations. The leaflet patch design has been modified from separate patch design to single patch design. No early and late deaths were reported, no re-operations were performed, and temporary ectopic atrial arrhythmia was the only early complication noted. The patients were discharged with less than moderate AR after a median postoperative period of 5 (range, 3–7) days. All the patients were New York Heart Association class I after a median follow-up period of 17.3 (range, 4.4–34.6) months. However, two patients progressed to moderate AR postoperatively at 6 and 30 months, respectively, after which one was treated with single patch technique. Among the remaining patients, six had trace, six had mild, and one had mild-to-moderate AR. Aortic valve repair with leaflet extension is acceptable in young patients. Our single patch design was easy to manipulate and showed good short-term results. Long-term follow-up is required to further confirm the efficacy of this technique.

Because Fontan circulation does not have a subpulmonary ventricle, the preload is limited. In Fontan circulation with extracardiac conduit, the size of conduit could be an important factor in determining the preload. We compared exercise capacity with each conduit size and tried to search for optimal conduit size in Fontan circulation. We reviewed the medical record of 677 patients with Fontan circulation. Patients who had other type Fontan circulation (Kawashima, atriopulmonary, lateral tunnel), SpO2

Introduction:This research investigated patients who underwent surgery for a dilated aorta associated with a connective tissue disease or inflammatory vasculitis in children and adolescents.Materials and Methods:The medical records of 11 patients who underwent aortic surgery for dilatation resulting from a connective tissue disease or inflammatory vasculitis between 2000 and 2017 were retrospectively reviewed.Results:The median age and body weight of the patients were 9.6 years (range 5.4 months–15.5 years) and 25.8 kg (range 6.8–81.5), respectively. The associated diseases were Marfan syndrome (n = 3), Loeys-Dietz syndrome (n = 3), Kawasaki disease (n = 1), Takayasu arteritis (n = 1), PHACE syndrome (n = 1), tuberous sclerosis (n = 1), and unknown (n = 1). The most common initially affected area was the ascending aorta. During the 66.4 ± 35.9 months of follow-up, two Marfan syndrome patients died, and four patients (one Marfan syndrome and three Loeys-Dietz syndrome) had repeated aortic operation. Except for one patient, the functional class was well maintained in all patients who were followed up.Conclusion:Cases of surgical treatment for a dilated aorta associated with a connective tissue disease and inflammatory vasculitis are rare in children and adolescents at our institution. Most of the patients in this study showed a tolerable postoperative course. However, the aorta showed progressive dilation over time even after surgical treatment, especially in patients with Loeys-Dietz syndrome. In these patients, close and more frequent regular follow-up is required.

Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.

Pulmonary valve replacement (PVR) is often performed in patients with repaired tetralogy of Fallot (TOF). Concomitant tricuspid valvuloplasty (TVP) in those with tricuspid regurgitation (TR) at the time of PVR is still controversial. We retrospectively reviewed clinical records of patients who underwent PVR between 2001 and 2012. We analyzed the impact of concomitant TVP on the tricuspid valve function and right ventricle function and size in mid-term. 119 patients with mild to moderate TR at the time of PVR were enrolled. 33 patients underwent concomitant TVP (TVP group) and 86 patients underwent PVR alone (no-TVP group). There was a significant reduction of TR (p

OBJECTIVES We compared the surgical outcomes of infective endocarditis (IE) between early surgery and non-early surgery groups in children. METHODS From January 2000 to April 2020, we retrospectively reviewed 50 patients RESULTS The median age and body weight at operation were 7.7 years [interquartile range (IQR), 2.3–13.2] and 23.7 kg (IQR, 10.3–40.7), respectively. The median follow-up duration was 9.5 years (IQR, 4.0–14.5). In 28 patients with native valve endocarditis, the native valve was preserved in 23 (82.1%). The most common causative microorganism was Streptococcus viridans (32.0%). The operative mortality was 2.0%, and 13 (26.0%) patients required reoperation most commonly for prosthesis failure (n = 7). There were no significant differences in patient characteristics and perioperative data between early surgery (n = 9) and non-early surgery (n = 36) groups, except for the interval between diagnosis and surgery (early surgery < non-early surgery, P CONCLUSIONS Surgical outcomes for IE in children were acceptable irrespective of the time of surgery. Our results suggest that it may not be required to delay surgery for IE and the potential benefit of early surgery could be expected in children.

Background Surgery can correct congenital heart defects, but disease management in low- and middle-income countries can be challenging and complex due to a lack of referral system, financial resources, human resources, and infrastructure for surgical and post-operative care. This study investigates the experiences of caregivers of children with CHD accessing the health care system and pediatric cardiac surgery. Methods A qualitative study was conducted at a teaching hospital in Ethiopia. We conducted semi-structured interviews with 13 caregivers of 10 patients with CHD who underwent cardiac surgery. We additionally conducted chart reviews for triangulation and verification. Interviews were conducted in Amharic and then translated into English. Data were analyzed according to the principles of interpretive thematic analysis, informed by the candidacy framework. Results The following four observations emerged from the interviews: (a) most patients were diagnosed with CHD at birth if they were born at a health care facility, but for those born at home, CHD was discovered much later (b) many patients experienced misdiagnoses before seeking care at a large hospital, (c) after diagnosis, patients were waiting for the surgery for more than a year, (d) caregivers felt anxious and optimistic once they were able to schedule the surgical date. During the care-seeking journey, caregivers encountered financial constraints, struggled in a fragmented delivery system, and experienced poor service quality. Conclusions Delayed access to care was largely due to the lack of early CHD recognition and financial hardships, related to the inefficient and disorganized health care system. Fee waivers were available to assist low-income children in gaining access to health services or medications, but application information was not readily available. Indirect costs like long-distance travel contributed to this challenge. Overall, improvements must be made for district-level screening and the health care workforce.

Background The occurrence of coronary artery disease (CAD) is unknown to date in patients who have undergone the right ventricular outflow tract (RVOT) reconstruction surgery for congenital heart disease using a bioprosthetic valve or conduit. Purpose We present the incidence and outcomes of CAD in patients who underwent RVOT reconstruction surgery in a single tertiary center. Method From April 1986 to February 2019, 544 patients underwent Rastelli operation or pulmonary valve replacement for conotruncal anomalies at the Seoul National University Children's Hospital in Korea. The patient's medical records and imaging findings were analyzed retrospectively. Results Significant coronary stenosis or occlusion were observed in 19 patients (3.5%) after RVOT reconstruction surgery. Their underlying diseases were pulmonary atresia in 8, tetralogy of Fallot in 3, double outlet right ventricle with pulmonary stenosis in 3, truncus arteriosus in 2, congenitally corrective transposition of great artery in 2, and congenital aortic stenosis in 1. Mechanism of CAD was compression of coronary artery by artificial conduit or valve in 10, intraoperative coronary injury in 7, and intramural course of coronary artery in 2 patients. The median number of RVOT reconstruction surgery before the diagnosis of CAD was 2 times (interquartile range: 2–3 times). The median age at diagnosis of CAD was 15 years (interquartile range: 9.5 - 25 years). The median time to detection of the CAD from the last RVOT reconstruction surgery was 9 years (interquartile range: 3.5–12.5 years). Involved coronary artery was right coronary artery (RCA) in 10, left anterior descending coronary artery (LAD) in 7, and left main coronary artery in 2 patients. Seven (36.8%) patients had underlying congenital coronary anomalies, such as single coronary artery, intramural course of the coronary artery, and anomalous origin of the coronary artery from an abnormal sinus. One patient died due to brain injury on extracorporeal membrane oxygenator after intraoperative coronary injury. Two patients with intramural course of coronary artery and one patient with high take-off of RCA which was compressed by adjacent calcified conduit underwent surgical repair of the CAD. One patient with recurrent ventricular tachycardia needed an implantable cardioverter-defibrillator. Nine patients are on medication for heart failure. Three patients with total occlusion of RCA or LAD showed well-developed collaterals from another normal coronary artery. Conclusion CAD after RVOT reconstruction surgery are not common but can be fatal. Because the clinical significance of CAD and long-term prognosis are not known yet, we need to closely track the patient after RVOT reconstruction surgery. Indications for the management of the CAD should be prepared to prevent sudden cardiac death, arrhythmia, and ventricular dysfunction in these patients. Funding Acknowledgement Type of funding source: None

OBJECTIVES Clinically, tetralogy of Fallot (TOF) patients who underwent repair late (older than 2 years) appears to have worse outcomes after pulmonary valve replacement than patients who underwent repair early. We proceeded to review the clinical features of late-repaired TOF patients who required pulmonary valve replacement. METHODS Fifty patients who underwent pulmonary valve replacement after TOF repair over the age of 2 years from 2000 to 2018 were retrospectively reviewed. Pre- and postoperative cardiac magnetic resonance imaging, cardiopulmonary exercise tests, and cardiac catheterization were analysed. RESULTS The median age of patients at the time of TOF repair and pulmonary valve replacement was 3.6 and 23.4 years, respectively. The median interval from TOF repair to pulmonary valve replacement was 20.5 years. Cardiac magnetic resonance imaging and cardiopulmonary exercise tests were performed at a median of 5.9 and 3.7 years after pulmonary valve replacement, respectively. Cardiac magnetic resonance revealed that there were significant changes in the indexed values of the right ventricle end-diastolic volume (164.7–106.9 ml/m2, P CONCLUSIONS Although pulmonary valve replacement after late TOF correction improves right ventricular volume status, only a minority of patients achieve normalization of right ventricular end systolic volume and a normal functional status.

Background: Most children who have congenital heart disease in low- and middle-income countries (LMICs), including Uzbekistan, do not receive adequate and timely pediatric cardiac surgical care. To strengthen the surgical capacity of a local pediatric cardiac surgery team in Tashkent, Uzbekistan, the JW LEE Center for Global Medicine at Seoul National University College of Medicine has developed a team-based training program and has been collaboratively conducting surgeries and care in order to transfer on-site knowledge and skills from 2009 to 2019. Objectives: To evaluate the long-term effects of the collaborative program on the cardiac surgical capacity of medical staff (teamwork, surgical complexity, and patients’ pre-surgical weights) as well as changes in the lives of the patients and their families. To derive lessons and challenges for other pediatric cardiac surgical programs in LMICs. Methods: To assess the effects of this ten-year long program, a mixed-methods design was developed to examine the trend of surgical complexity measured by Risk Adjustment for Congenital Heart Surgery 1 score (RACHS-1) and patients’ pre-surgical weights via medical record review (surgical cases: n = 107) during the decade. Qualitative data was analyzed from in-depth interviews (n = 31) with Uzbek and Korean medical staff (n = 10; n = 4) and caregivers (n = 17). Findings: During the decade, the average RACHS-1 of the cases increased from 1.9 in 2010 to 2.78 in 2019. The average weight of patients decreased by 2.8 kg from 13 kg to 10.2 kg during the decade. Qualitative findings show that the surgical capacity, as well as attitudes toward patients and colleagues of the Uzbek medical staff, improved through the effective collaboration between the Uzbek and Korean teams. Changes in the lives of patients and their families were also found following successful surgery. Conclusions: Team-based training of the workforce in Uzbekistan was effective in improving the surgical skills, teamwork, and attitudes of medical staff, in addition, a positive impact on the life of patients and their families was demonstrated. It can be an effective solution to facilitate improvements in pediatric cardiovascular disease in LMICs if training is sustained over a long period.

BACKGROUND AND OBJECTIVES We reviewed the long-term outcomes after tetralogy of Fallot (TOF) repair with trans-annular incision; and evaluated the effectiveness of pulmonary valve replacement (PVR) on outcomes. METHODS This was a retrospective review of clinical outcomes of 180 of 196 TOF patients who underwent total correction with trans-annular incision from 1991 to 1997 (PVR group: 81; non-PVR group: 99). RESULTS The median age of the patients was 14.0 months (interquartile range [IQR], 10.7-19.8 months) at TOF repair. Ten in-hospital deaths (5.1%) occurred. During the follow-up, 81 patients underwent PVR at the median age of 13.5 years (IQR, 11.2-17.1 years). The patients in PVR group showed better outcomes than non-PVR group in overall survival rate (100% in PVR vs. 88.7% in non-PVR, p=0.007), in all adverse events (arrhythmia, neurologic complications, 95.5% in PVR vs. 74.6% in non-PVR, p=0.024) at 20 years. Age at TOF repair younger than 1 year (hazard ratio [HR], 2.265; p=0.01) and previous shunt history (HR, 2.195; p=0.008) were predictive for requiring PVR. During follow-up, 10 late deaths (5 sudden deaths) occurred in the non-PVR group, mainly due to ventricular arrhythmia and right ventricular failure; there was 1 late death (not a sudden death) in the PVR group. CONCLUSIONS Long-term survival after repair of TOF with trans-annular incision were acceptable. However, arrhythmias were frequently observed during 20 years of follow-up. The patient age

One of the complications of permanent pacemaker implantation is unintended phrenic nerve stimulation. A 15-year-old boy with a permanent pacemaker presented with chest discomfort due to synchronous chest wall contraction with pacing beats. Even after reprogramming of the pacemaker, diaphragmatic stimulation persisted. Therefore, we performed thoracoscopic phrenic nerve insulation using a Gore-Tex patch to insulate the phrenic nerve from the wire. A minimally invasive approach using a thoracoscope is a feasible option for retractable phrenic nerve stimulation after pacemaker implantation.

Pulmonary vein stenosis (PVS) is still a frustrating disease with extremely high mortality, especially in children with multiple severe PVS. Hybrid pulmonary vein stenting (HPVS) is a rescue treatment for recurrent and malignant PVS. The aim of this study is to share our successful experience with intraoperative HPVS for recurrent PVS after total anomalous pulmonary venous connection (TAPVC) repair in infant. Six patients were identified between 2013 and January 2018, who were diagnosed with recurrent PVS and underwent HPVS in the operating room. The mean age at the time of the HPVS was 10.3 ± 2.7 months (range 7-14 months) and the mean body weight was 7.9 ± 2.6 kg (range 4.1-10.5 kg). Prior pulmonary vein surgery had been performed on average 2.7 times (range 2-3) in all patients. We used a bare-metal stent (BMS) of 6-8 mm diameter in 15 veins of five patients and a drug-eluting coronary stent (DES) in two veins of one patient. All patients had undergone several elective further pulmonary vein in-stent balloon dilatations or another stent insertion after HPVS. Over a mean follow-up of 17.3 ± 13.7 months (range 6-44 months), all patients maintained patency of stents although two patients died due to respiratory failure not associated with PVS. HPVS is a useful treatment modality for recurrent PVS patient that could save the life and achieve longer freedom from restenosis than repetitive surgical pulmonary vein widening only. Even though the prognosis of severe multiple PVS is very poor, planned HPVS could be a good palliation in this patients group.

Background: Early extubation after cardiovascular surgery has some clinical advantages, including reduced hospitalization costs. Herein, we review the results of ultra-fast-track (UFT) extubation, which refers to extubation performed on the operating table just after the operation, or within 1-2 hours after surgery, in patients with congenital cardiac disease. Methods: We performed UFT extubation in patients (n=72) with a relatively simple congenital cardiac defect or who underwent a simple operation starting in September 2016. To evaluate the feasibility and effectiveness of our recently introduced UFT extubation strategy, we retro-spectively reviewed 195 patients who underwent similar operations for similar diseases from September 2015 to September 2017, including the 1-year periods immediately before and after the introduction of the UFT extubation protocol. Propensity scores were used to assess the effects of UFT extubation on length of stay (LOS) in the intensive care unit (ICU), hospital LOS, and medical costs. Results: After propensity-score matching using logistic regression analysis, 47 patients were matched in each group. The mean ICU LOS (16.3±28.6 [UFT] vs. 28.0±16.8 [non-UFT] hours, p=0.018) was significantly shorter in the UFT group. The total medical costs (182.6±3.5 [UFT] vs. 187.1±55.6 [non-UFT] ×100,000 Korean won [KRW], p=0.639) and hospital stay expenses (48.3±13.6 [UFT] vs. 54.8±29.0 [non-UFT] ×100,000 KRW, p=0.164) did not significantly differ between the groups. Conclusion: UFT extubation decreased the ICU LOS and mechanical ventilation time, but was not associated with postoperative hospital LOS or medical expenses in patients with simple congenital cardiac disease.

Despite improvements in valve repair techniques, conditions in which infants and children need for mechanical valve replacement (MVR) are still present. We analyzed supra-annular MVR outcomes in infants and children with small annulus and compared them with conventional annular MVR outcomes. Data were collected retrospectively from medical records of infants and children (weighing

Objectives Coronary artery stenosis after an arterial switch operation (ASO) leads to subsequent reoperation. Therefore, we investigated the indications for reoperation and the results of reoperation to suggest methods to improve our management protocol for coronary artery stenosis after an ASO. Methods Between September 2003 and December 2016, 86 consecutive patients who underwent an ASO were included in the study. The indications for reoperation, reoperation techniques and postoperative results were investigated. Results There were 4 in-hospital deaths (4.7%). One late death occurred during the median follow-up period of 59.6 months. The 5-year overall survival rate was 94.2%. Seven reoperations were performed in 5 patients due to coronary artery stenosis. The indications for reoperation were severe coronary artery stenosis confirmed by computed tomography (CT) angiography or coronary angiography with or without symptoms. Patients with a coronary artery between the great arteries or a high take-off coronary artery frequently required reoperation due to coronary artery stenosis. None of the patients who underwent unroofing or cut-back angioplasty experienced complications during the median follow-up period of 52.0 months. However, 2 patients who underwent ostioplasty required an additional reoperation due to coronary artery restenosis. Conclusions A standardized follow-up protocol including CT angiography or coronary angiography after the ASO is required to address coronary artery stenosis. Good reoperation results were observed using the unroofing and cut-back angioplasty techniques.

Patients with double-inlet left ventricle usually have a small ascending aorta. In the Norwood procedure, which involves a staged operation, a neoaorta is constructed with a homograft, and the pulmonary artery plays a role in the systemic circulation. Dilatation or aneurysmal changes can occur over time due to the exposure o f the neoaorta t o systemic p ressure, w hich m ay i nduce a dverse effects on adj acent structures. We report a rare case of surgical repair for neoaortic root dilataiton with aortic regurgitation, compressing the left pulmohary artery, in a patient who underwent the Norwood procedure.

Introduction: To make more efficient use of limited resources, Vietnam incorporated health technology assessment (HTA) into the decision-making process for the health insurance benefit package in 2014. We evaluated progress in HTA institutionalization in Vietnam based on the theoretical framework developed by the National Institute for Health and Care Excellence and the Health Intervention and Technology Assessment Program, identified negative and conducive factors for HTA development, and finally suggested policy recommendations that fit the Vietnamese context. Methods: Semi-structured in-depth qualitative interviews were conducted between December 2017 and March and April 2018 with a purposive sample of 24 stakeholders involved in decision-making for health insurance reimbursement. We employed thematic analysis to examine themes within the data. Results: Despite a variety of activities (e.g., training and advising/mentoring) and a substantial level of output (e.g., policy statements, focal points assigned, and case studies/demonstration projects), Vietnam has not yet reached the policy decision stage based on HTA with scientific integrity and active stakeholder participation. Most respondents, except some clinicians, supported the use of HTA. The lack of capacity of human resources in the government sector and academia, the limited data infrastructure, the absence of guidelines, the government’s interest in immediate budget-saving, and public resistance were identified as barriers to the advancement of HTA. Conclusions: A structured data repository, guidelines based on the Vietnamese context for both policy decision-making at the central level and daily clinical decision-making at the micro-level, and integration of a participatory process into HTA are suggested as priorities for HTA institutionalization in Vietnam.

OBJECTIVES We evaluated the outcomes of patients who underwent extracardiac Fontan circulation procedures (ECFP) and received 16-mm polytetrafluoroethylene conduits. METHODS From June 1997 to May 2015, among the 408 patients who underwent ECFP, 66 patients (Group S) also received 16-mm polytetrafluoroethylene conduits. To compare this patient cohort with similarly sized patients who received larger conduits, a matched cohort (66 patients, Group L) was selected according to age and body weight. RESULTS The mean age, body weight and Nakata index at ECFP in Groups S and L were 2.9 ± 1.2 and 3.1 ± 1.2 years (P = 0.243), 13.0 ± 2.4 and 13.0 ± 2.3 kg (P = 0.101), 175.3 ± 59.0 and 236.1 ± 75.1 mm2/m2 (P = 0.005), respectively. The mean follow-up periods for Groups S and L were 7.8 ± 6.0 and 9.1 ± 4.9 years (P = 0.150), respectively. The conduit size of Group L was 19.2 ± 1.4 mm (P

OBJECTIVES Mitral valve (MV) repairs have been performed in paediatric patients with congenital MV stenosis. However, congenital MV stenosis lesions are a heterogeneous group of lesions, and their repair remains challenging. METHODS From March 1999 to September 2014, MV repair was performed in 22 patients with congenital MV stenosis. The median age was 10.3 months (ranging from 22 days to 9.1 years), and the mean body weight was 7.9 ± 4.0 kg at the time of the operation. Multiple-level left-side heart obstructions were present in 9 (45%) patients. RESULTS The main aetiology of the mitral stenosis was a supravalvular mitral ring in 8 patients, valvular stenosis in 4 patients, a parachute deformity of the papillary muscles in 4 patients and other abnormal papillary muscles in 6 patients. The mean MV pressure gradient improved from 10.4 ± 3.9 mmHg to 3.4 ± 1.7 mmHg after MV repair (n = 18, P

Pulmonary arteriovenous fistula (PAVF) is a complication of the Glenn shunt. A 57-year-old tetralogy of Fallot (TOF) patient, who had undergone a Glenn shunt and TOF total correction, complained of dyspnea and cyanosis. P AVFs w ere present i n the rig ht l ung, and rig ht l ung perfusion was nearly a bsent. A fter c oil embolization, t akedown of the G lenn s hunt, a nd r econstruction of t he rig ht pulmonary a rtery, the p atient’s s ymptoms were relieved. Extrapulmonary radioisotope uptake caused by the PAVFs shown in lung perfusion scans decreased, and right lung perfusion increased gradually. Although the development and resolution of PAVFs after a Glenn shunt have been reported in the pediatric population, this may be the first report on this change in old age.

OBJECTIVES This study aimed to compare the effects of hypothermic ventricular fibrillation and beating-heart techniques during cardiopulmonary bypass (CPB) on postoperative outcomes after simple pulmonary valve replacement in patients with repaired tetralogy of Fallot (TOF). METHODS We retrospectively reviewed the data of 47 patients with repaired tetralogy of Fallot at a single institution, who received pulmonary valve replacement under the ventricular fibrillation or beating-heart technique without cardioplegic cardiac arrest during CPB between January 2005 and April 2015. RESULTS The patients were divided into fibrillation (n = 32) and beating-heart (n = 15) groups. On comparing these groups, the fibrillation group had a larger sinotubular junction (27.1 ± 4.6 vs 22.1 ± 2.4 mm), had a longer operation duration (396 ± 108 vs 345 ± 57 min), required more postoperative transfusions (2.1 ± 2.6 vs 5.0 ± 6.3 units) and had a higher vasoactive-inotropic score at intensive care unit admission (8.0 vs 10, all P

Managing protein-losing enteropathy after Fontan surgery is challenging. We report a case of protein-losing enteropathy associated with Fontan circulation in a 14-year-old boy. He was treated medically for 2 years, without improvement until Fontan takedown and pulsatile bidirectional cavopulmonary shunting were performed.

Background/Introduction Lowering blood pressure is very beneficial in reducing the risk of cardiovascular morbidity and mortality. But, the extent of optimal target blood pressure in hypertensive patients is still controversial. Purpose The objectives of this study were to assess the level of proper systolic and diastolic blood pressure to prevent cardiovascular events in older and younger patients. Methods We used the National Sample Cohort from the National Health Insurance Service of 2007 to 2013 in Korea and analyzed data from 44,462 hypertensive patients aged from 20 to 84, treated with one or more antihypertensive agents and participated at least one general health examination. Achieved systolic and diastolic blood pressure (SBP/DBP) were categorized by exclusive average achieved SBP ( Results Of 44,462 patients, 5,478 (12.3%), 13,410 (30.2%), 15,021 (33.8%), 7,051 (15.9%), and 3,502 (7.9%) patients achieved SBP Conclusion In conclusion, an intensive lowering of blood pressure is more likely to increase the risk rather than to prevent major cardiovascular events and all-cause death, particularly in older than younger. Therefore, an intensive blood pressure lowering of SBP/DBP below 120/70 mm Hg in the elderly should be avoided. Acknowledgement/Funding The Korea National Institute of Health research grant 2017-NI63001-00

Objectives The long-term durability of the 2 most commonly used types of bioprosthetic valves in the pulmonic position, the porcine and pericardial valves, is unclear. We compared the long-term durability of the pericardial (Carpentier-Edwards PERIMOUNT [CE]) and porcine (Hancock II) valves in the pulmonic position in patients with congenital cardiac anomalies. Methods We retrospectively reviewed the medical records of 258 cases (248 patients) of pulmonary valve implantation or replacement using CE (129 cases, group CE) or porcine (129 cases, group H) valves from 2 institutions between 2001 and 2009. Results The patients' age at pulmonary valve implantation was 14.9 ± 8.7 years. No significant differences in perioperative characteristics were observed between groups CE and H. Follow-up data were complete in 219 cases (84.9%) and the median follow-up duration was 10.5 (interquartile range, 8.4∼13.0) years. Ten mortalities (3.9%) occurred. Sixty-four patients underwent reoperation for pulmonary valve replacement due to prosthetic valve failure; 10 of these 64 patients underwent reoperation during the study period. Patients in group CE were significantly more likely to undergo reoperation (hazard ratio, 2.17; confidence interval, 1.26-3.72; P = .005) than patients in group H. Patients in group CE showed had a greater prosthetic valve dysfunction (moderate-to-severe pulmonary regurgitation or pulmonary stenosis with ≥3.5 m/s peak velocity through the prosthetic pulmonary valve) rate (hazard ratio, 1.83; confidence interval, 1.07-3.14; P = .027) than patients in group H. Conclusions Compared with the pericardial valve, the porcine valve had long-term advantages in terms of reduced reoperation rate and prosthetic valve dysfunction in the pulmonic position in patients with congenital cardiac anomalies.

Background Patients with congenital complete atrioventricular block often require lifelong pacemaker therapy, which has a risk of left ventricular (LV) dysfunction. The aim of our study was to determine the long-term effects of epicardial pacing on LV function and dyssynchrony in children with congenital heart block. Methods We conducted a retrospective study at a single tertiary center in which 34 pediatric patients with isolated congenital complete heart block who underwent epicardial pacemaker implantation from 1987 to 2016 were enrolled. Results Median age during pacemaker insertion was 2.5 years (range, 0-16.7 years) and the median follow-up duration was 12.3 years. The initial pacing sites were the right ventricle (RV) free wall for 10 patients, RV apex for 16, and LV apex for eight. LV dysfunction developed in 7 patients, of whom 4 underwent lead relocation to the RV apex (n = 2) and cardiac resynchronization therapy upgrade (n = 2). RV free-wall pacing had a significantly higher risk of LV dysfunction than either LV or RV apical pacing (odds ratio 52.5; P = .003; 95% confidence interval 3.9-700). All 4 patients who underwent lead repositioning showed improvement of LV function (median ejection fraction, 24%-62.7%). Conclusions Our study suggests that RV free-wall pacing may be a significant risk factor for LV dysfunction. Pacemaker-induced LV dysfunction was improved via lead relocation to the RV apex or cardiac resynchronization therapy upgrade. Future studies with a larger sample size and longer-term follow-up are required to confirm our results.

OBJECTIVE Cardiovascular lesions are the leading cause of morbidity and mortality in patients with Williams syndrome. Recent studies have rebutted conventional reports about the natural course of cardiovascular anomalies in Williams syndrome. DESIGN Retrospective study. SETTING Single tertiary center. PATIENTS Eighty patients with Williams syndrome followed up for more than 5 years. INTERVENTIONS Not applicable. OUTCOME MEASURES Long-term outcome of cardiovascular lesions, peak velocity change in obstructive cardiovascular lesions over time, post-interventional courses of disease-specific intervention, and intervention-free survival of obstructive cardiovascular lesions. RESULTS The median follow-up duration was 11.0 (5.1-28.3) years. Among 80 patients, supravalvular aortic stenosis (87.5%) was the most common cardiovascular lesion, followed by branch pulmonary stenosis (53.8%), mitral valve prolapse (22.5%), and aortic arch hypoplasia/coarctation (5.0%). During the follow-up period, the peak flow velocity of supravalvular aortic stenosis did not change on peak Doppler echocardiography. Initially, severe supravalvular aortic stenosis was aggravated (P

In Ebstein's anomaly, maximal expansion in the atrialized right ventricle (RV) occurs during early diastole, whereas that of the functional RV occurs in late diastole, resulting in diastolic dyssynchronous expansion (DSE). We quantitatively assessed DSE and identified preoperative factors correlated with persistent DSE after surgery. Seventeen patients diagnosed with Ebstein's anomaly in whom transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) images available were retrospectively analyzed for quantitative DSE assessment and 10 patients who underwent surgery and postoperative TTE available were additionally analyzed for postoperative DSE. Severity of DSE was assessed by the time difference of maximal expansion between the atrialized and functional RV divided by the cardiac cycle length × 100 ("DSE index"). Relations between DSE and, clinical, electrophysiologic parameters and the severity of tricuspid valve (TV) tethering (the RV length / tethering height during diastole: "Tethering index") were assessed. In total patients, median DSE index and tethering index were 30.3 and 2.1 respectively, and the DSE index was correlated with tethering index (rs = 0.664, P = 0.004). In 10 patients who underwent surgery, this association remained after surgery and at 2-year follow up. Tethering index ≥2.5 separated patients with and without persistent DSE. In conclusion, DSE exists in Ebstein's anomaly. DSE index is related to the tethering index and DSE persists postoperatively if tethering index ≥ 2.5. As the persistent DSE might possibly impede the optimal recovery of RV function after surgery, severity of TV tethering should take into account in considering surgery.

Background Conduit survival without significant dysfunction is important when selecting the right ventricular outflow tract conduit. We made an expanded polytetrafluoroethylene tricuspid valved conduit using a simplified technique. We aimed to investigate the midterm functional results and longevity of this conduit. Methods Between November 2008 and December 2016, four hospitals in Korea implanted 145 valved conduits. We retrospectively analyzed their functional results and longevity. Results The patients' median age at operation was 36.6 months; the median body weight was 11.3 kg. The mean follow-up duration was 32.3 ± 24.5 months. There were four inhospital deaths and three late deaths, but there were no conduit-related deaths. The mean peak systolic pressure gradient across the conduit was 14.7 ± 8.3 mm Hg and 31.6 ± 17.7 mm Hg at discharge and last follow-up, respectively. Six patients (4.4%) had moderate or more conduit valve regurgitation at last follow-up. Conduit dysfunction was observed in 30 patients (21.9%), mainly caused by increased pressure gradient (24 of 30, 80%). Freedom from conduit dysfunction was 88.1% and 58.5% at 3 and 5 years, respectively. Lower freedom from conduit dysfunction was observed in small conduits. Eleven patients (7.8%) underwent conduit explantation, and freedom from explantation was 94.8% and 81.7% at 3 and 5 years, respectively. The main cause of explantation was conduit stenosis. Small conduits tended to have lower freedom from explantation. Conclusions Functional results and longevity of our expanded polytetrafluoroethylene tricuspid valved conduit are acceptable. Although our conduits tend to have increasing pressure gradient over time, especially in small conduits, they have low incidence of moderate or more regurgitation.

Mixing of autologous blood with priming volume has relatively significant effects on blood composition, especially in low-bodyweight neonates. In an effort to reduce these effects, mini-volume priming (MP) has been applied in cardiopulmonary bypass (CPB). The present study was designed to examine the effect of MP on clinical outcomes of low-bodyweight neonates undergoing open heart surgery.We retrospectively reviewed medical records of low-bodyweight (2.5 kg or less) neonates who underwent open heart surgery in our center from January 2000 to December 2014. A total of 64 patients were included. MP was introduced in 2007, and became a routine protocol in 2009. Preoperative and intraoperative characteristics included age, bodyweight, RACHS-1, priming volume, CPB time, and aortic cross-clamp time, transfusion, and hematocrit during CPB. Clinical outcomes included 30-day mortality, postoperative extracorporeal membrane oxygenation (ECMO) support, open sternum status, prolonged mechanical ventilation care (>7 days), and acute renal failure. MP was utilized in 39 patients and conventional priming (CP) was used in 25 patients. The priming volume decreased to 126.0 mL in the MP group compared with 321.6 mL in the CP group. Transfusion volume during CPB was 87.3 mL in the MP group versus 226.8 mL in the CP group, and the difference was statistically significant (P