A comparative study using gastric ultrasound to evaluate the safety of shortening the fasting time before pediatric echocardiography: a randomized controlled non-inferiority study.

PURPOSE: The objective of this study was to demonstrate that the gastric cross-sectional area (CSA) in the right lateral decubitus position (RLDP) during a 2-h fasting period is not larger than that during a conventional 4-h fasting period prior to pediatric echocardiography. METHODS: 93 patients aged under 3 years scheduled for echocardiography under sedation were enrolled and randomly allocated into two groups; 2-h fasting vs 4-h fasting. For group 4 h (n = 46), the patients were asked to be fasted for all types of liquid for more than 4 h, while group 2 h (n = 47) were asked to be fasted for all types of liquid for 2 h before echocardiography. Gastric ultrasound was performed before echocardiography, and CSARLDP was measured. We compared CSARLDP, incidence of at-risk stomach, fasting duration, and the incidence of major (pulmonary aspiration, aspiration pneumonia) and minor complications (nausea, retching, and vomiting, apnea, and bradycardia) between two groups. RESULTS: The mean difference of CSARLDP (group 2 h-group 4 h) was 0.49 (- 0.18 to 1.17) cm2, and it was within the non-inferiority margin (Δ = 2.1 cm2). There was no difference in the incidence of at-risk stomach (P = 0.514). There was no significant difference in the incidence of major and minor complications between the two groups. CONCLUSION: Two-hour fasting in pediatric patients who need an echocardiography did not increase major and minor complications and CSA significantly.

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

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

Treatment of Primary Cardiac Tumors in Children: A Single Center 18-Year Experience.

This study aimed to compare the clinical characteristics and courses of pediatric patients with cardiac tumors in nonoperative and operative groups to help guide treatment decisions. We reviewed the medical records of patients diagnosed with primary pediatric cardiac tumors at our institution between 2003 and 2020. Demographic data, clinical characteristics, and follow-up data between the operation and nonoperation groups were compared. A total of 56 patients were included in the study. Thirteen patients underwent surgery. The median age was 1.4 months (range, 1 to 18 years). The patients in the operation group had more frequent symptoms or signs, such as desaturation, respiratory difficulty, murmur, a higher mass area/chamber area (MC) ratio, decreased ventricular contractility, and significant ventricular outflow tract obstruction (VOTO). An MC ratio of 0.568 was the cutoff value for differentiating patients with symptoms or signs of heart failure and decreased ventricular contractility. At the last follow-up, all patients had good ventricular contractility except one patient in the operative group with fibroma. In the non-operative group, rhabdomyomas often regressed spontaneously, while fibromas often increased in size. Two patients in the nonoperative group died. In the operative group, there was no early or late mortality or tumor recurrence. In this study, patients had good outcomes with or without surgery, even when the tumor was large, or surgery was performed in early infancy.

Long-Term Outcomes of Modified Cone Reconstruction for Ebstein's Anomaly in Pediatric Patients in a Single Center.

BACKGROUND AND OBJECTIVE: We aimed to investigate long-term clinical and echocardiographic outcomes, including tricuspid valve durability, annular growth, and left ventricular reverse remodeling, after modified cone reconstruction in patients with Ebstein's anomaly. METHODS: This was a retrospective analysis of all pediatric patients who underwent modified cone reconstruction for Ebstein's anomaly at a single tertiary center between January 2005 and June 2021. RESULTS: A total of 14 pediatric patients underwent modified cone reconstruction for Ebstein's anomaly; the median age was 5.8 years (range, 0.01-16.6). There were three patients (21.4%) with Carpentier type B, ten patients with Carpentier type C (71.4%), and one patient with Carpentier type D (7.1%). There was no early or late mortality, arrhythmia, or readmission for heart failure at a 10-year follow-up. There were no cases of more than mild tricuspid stenosis or more than moderate tricuspid regurgitation during the study period, except for one patient with severe tricuspid regurgitation who underwent reoperation. The z value for tricuspid valve annular size significantly decreased immediately after the operation (2.46 vs. -1.15, p<0.001). However, from 1 year to 7 years after surgery, the z values were maintained between -1 and +1. Left ventricular end-systolic volume, end-diastolic volume, and stroke volume increased after surgery and remained elevated until seven years postoperatively. CONCLUSIONS: Ebstein's anomaly in children can be repaired by modified cone reconstruction with low mortality and morbidity, good tricuspid valve durability, and annular growth relative to somatic growth.

Prognosis of Chronic Kidney Disease and Metabolic Syndrome in Adults With Congenital Heart Disease.

BACKGROUND: Few studies have examined the incidence of chronic kidney disease (CKD) and metabolic syndrome (MS) and their combined prognostic effects in adult congenital heart disease (ACHD). Our aims were to identify the incidence and prognostic implications of CKD and MS in ACHD. METHODS: This is retrospective cohort study. We included 2,462 ACHD ≥ 20 years of age who were treated at a tertiary hospital in Korea from 2006 to 2018. CKD was defined as an estimated glomerular filtration rate < 60 mL/min/1.73m². MS was diagnosed based on the presence of abnormal metabolic parameters: blood sugar level, obesity, dyslipidemia, and hypertension. The primary outcome was all-cause mortality from 2006 through 2019 using data from the Ministry of the Interior and Safety in Korea. RESULTS: The incidence of CKD and MS in ACHD was 7.6% and 35.9%, respectively. The coexistence rate of CKD and MS was 4.6%. Although MS was not independently associated with mortality in the multiple analysis (adjusted hazard ratio [aHR], 1.07; 95% confidence interval [CI], 0.79-1.46), it was closely related to the presence of CKD (adjusted odds ratio, 2.62; 95% CI, 1.89-3.63). ACHD patients with CKD had a significantly increased risk of mortality compared with those without CKD (aHR, 2.84; 95% CI, 2.00-4.04). CONCLUSIONS: In patients with ACHD, the distribution of MS is higher, and both MS and its components were associated with CKD. Given the CKD was independently associated with mortality, close monitoring and management of renal dysfunction and metabolic parameters in ACHD patients is needed.

Valve-sparing root replacement in children with connective tissue disease: Long-term risk of aortic events.

BACKGROUND: Cardiovascular involvement in pediatric patients with connective tissue disease (CTD) is life-threatening, with aortic root dilatation being the most prevalent cardiovascular abnormality. We attempted to determine long-term outcomes of valve-sparing root replacement (VSRR) in this group, including cardiovascular reoperations for aortic aneurysm and dissection. METHODS: We conducted a retrospective analysis of pediatric patients with CTD who received VSRR in a single center from 2002 to 2021. The primary end point was a composite event of all-cause death and cardiovascular reoperations. The median follow-up duration was 8.3 years, with a maximum of 20.7 years. RESULTS: The median age of 24 pediatric patients who had VSRR was 14.4 years. Marfan syndrome and Loeys-Dietz syndrome affected 19 (79.2%) and 5 (20.8%) patients, respectively. There was no early death. The 15-year survival rate was 91.7%. At 10 years after VSRR, the cumulative incidence of reoperation for aortic regurgitation was 15.6%, and for aortic aneurysm or dissection, it was 29.1%. The 10-year rate of freedom from the primary end point was 53.1%. The Cox multivariable analysis revealed younger age at surgery (hazard ratio, 1.279; 95% confidence interval, 1.086-1.505; P = .003) and VSRR before 13 years of age (hazard ratio, 5.005; 95% confidence interval, 1.146-21.850; P = .032) as independent prognostic factors for the primary endpoint. CONCLUSIONS: VSRR for aortic root dilatation in pediatric patients with CTD demonstrated good long-term survival and low reoperation rates for aortic regurgitation. However, several patients developed later aortic aneurysm or dissection, and careful surveillance may be required, particularly in those who received VSRR at younger age.

Effects of time of bed rest on vascular complications after cardiac catheterization in pediatric patients with congenital heart disease: A randomized controlled trial.

BACKGROUND: Pediatric cardiac catheterization, which is performed by accessing the femoral vessel, requires immobilization and bed rest for 4-6 h to prevent vascular complications. Studies in adults suggest that the immobilization time for the same access can be safely reduced to approximately 2 h after catheterization. However, it is unclear whether bed-rest time can be safely decreased after catheterization in children. OBJECTIVE: To assess the effects of bed-rest duration on bleeding, vascular complications, pain level, and the use of additional sedatives after transfemoral cardiac catheterization in children with congenital heart disease. METHODS: This study was an open-label, randomized, controlled, posttest-only design, including 86 children who underwent cardiac catheterization. Children were allocated to receive either 2 h of bed rest (n = 42) in the experimental group or 4 h of bed rest (n = 42) in the control group following catheterization. RESULTS: The mean age of children was 3.93 (±3.82) years in the experimental group and 5.63 (±3.97) years in the control group. There was no difference in site bleeding incidence (P = 0.214), vascular complication score (P = 0.082), pain level (P = 0.445), or additional sedation use (P = 1.000) between the two groups. CONCLUSIONS: There were no significant hemostatic complications after 2 h of bed rest following pediatric catheterization; therefore, 2 h of bed rest was as safe as 4 h of bed rest. (Trial registration: KCT0007737).

The effects of music intervention on anxiety and stress responses in adults with CHD undergoing cardiac catheterisation.

INTRODUCTION: This study evaluated the effect of music intervention on the anxiety and stress responses of patients who underwent an interventional cardiac catheterisation. METHODS: The study design was a pre- and post-test randomised controlled trial that included 94 patients who underwent a transcatheter atrial septal defect closure. Patients were allocated to receive either music intervention (n = 47) or usual care (n = 47) during the interventional cardiac catheterisation. Music intervention effectiveness was examined in terms of anxiety, salivary cortisol level, and heart rate variability. RESULTS: The average age of participants was 45.40 years (±16.04) in the experimental group and 47.26 years (±13.83) in the control group. Two-thirds (66.0%) of the participants in each group were women. State anxiety (F = 31.42, p < 0.001), anxiety-numerical rating scale (F = 20.08, p < 0.001), salivary cortisol levels (F = 4.98, p = 0.021), and low-frequency component/high-frequency component ratio (F = 17.31, p < 0.001) in the experimental group were significantly reduced compared with those in the control group at the end of the music intervention. CONCLUSION: This study provides practical evidence of a reduction in anxiety and stress response from music intervention preceding an interventional cardiac catheterisation, indicating that this intervention should be considered in clinical management.

Long-Term Observational Outcomes after Total Correction of Congenital Heart Disease in Korean Patients with Down Syndrome: A National Cohort Study.

Background: In the present study, the population prevalence and postoperative morbidity and mortality in Down syndrome patients who underwent total correction for congenital heart disease were investigated using data from a large national cohort. Methods: Retrospective administrative data from 2,395,966 participants born between 2008 and 2012 were acquired from the National Investigation of Birth Cohort in Korea. Among Down syndrome patients, 58.3% had congenital heart disease and 32.3% underwent total correction. Propensity score matching (maximum 1:1) and stabilized inverse probability treatment weighting (IPTW) were performed for each group (153 Down syndrome patients and 4482 non-Down syndrome patients). Results: T late mortality rate was significantly higher in the Down syndrome group than in the non-Down syndrome group (8.1% vs. 3.8%). No differences were observed in postoperative heart failure and arrhythmias, but pulmonary hypertension was significantly greater in the Down syndrome group than in the non-Down syndrome group (26.9% vs. 7.0%). The length of hospitalization was longer in the Down syndrome group than in the non-Down syndrome group (14 days vs. 11 days; interquartile range (IQR): 10−25 vs. 6−19; p < 0.0001). After total correction, readmission frequency for any reason was minimally but statistically significantly higher in the Down syndrome group compared to the non-Down syndrome group (5 times vs. 5 times; IQR: 3−8 vs. 4−9; p < 0.0001). However, the number of emergency room visits was minimally but significantly lower in the Down syndrome group compared to the non-Down syndrome group (2 visits vs. 2 visits (IQR): 2−7 vs. 1−4; p = 0.016). Conclusions: Down syndrome patients with congenital heart disease undergoing total correction showed pulmonary hypertension after surgery, longer length of hospitalization, frequent hospitalization after surgery, and a higher rate of late mortality.

Outcomes After Transcatheter Closure of Atrial Septal Defect Without Using a Balloon-Sizing Technique: A Randomized Controlled Comparison with Closures Using a Balloon-Sizing Technique.

BACKGROUND: The transcatheter closure of atrial septal defect could be completed without the balloon-sizing technique, so we evaluated long-term outcomes compared with closure using balloon sizing, which was the conventional method. Even without using the balloon-sizing technique, transcatheter closure of atrial septal defect might be safe and effective. METHODS: We included 124 patients with isolated atrial septal defects who underwent device closure without balloon sizing between 2012 and 2016, and we further included 257 patients as a control group. Patients who received closure with multiple devices or who experienced postoperative residual defects were excluded. Immediate procedural results, as well as long-term outcomes for closure without balloon sizing, were investigated and compared with the control group. RESULTS: The procedural success rate was 96.7%, and there were no mortalities. No embolization or cardiac erosions were observed; however, one patient experienced residual shunt, and another developed progressed mitral regurgitation during the follow-up period (983±682 days). Newly onset persistent atrial fibrillation developed in one patient (1.0%). There were no significant differences in procedures or follow-up between the study and control groups. Despite the shorter procedural time in the study group, fluoro time was not different. Atrial arrhythmias were more frequently observed in the control group, but the difference was not significant. Persistent atrial fibrillation was observed in two patients in the control group (0.8%). CONCLUSIONS: Transcatheter closure of atrial septal defect can be performed safely and effectively without using the balloon-sizing technique. The long-term outcomes were similar to outcomes with balloon sizing.

Development of necrotizing enterocolitis in full-term infants with duct dependent congenital heart disease.

BACKGROUND: Although many studies have described an increased risk of necrotizing enterocolitis in duct dependent congenital heart diseases, very few have investigated its occurrence in full-term infants with duct dependent congenital heart diseases. METHODS: To evaluate the characteristics and risk factors of necrotizing enterocolitis, we performed a retrospective review of 355 full-term infants with duct dependent congenital heart diseases who received prostaglandin E1 therapy from April 2000 to May 2020. RESULTS: Necrotizing enterocolitis was observed in 10 patients (3.0%). Their average gestational age and birth weight were 38.2 weeks and 2783.5 g, respectively. The median age at diagnosis was 8.0 days (2-70 days). One patient was diagnosed with necrotizing enterocolitis stage IIA, five with stage IIB, two with stage IIIA, and two with stage IIIB; two (20%) received surgical treatment. The duct dependent pulmonary circulation group had higher frequencies of necrotizing enterocolitis (4.4%) than the duct dependent systemic circulation (2.0%) and parallel circulation (1.3%) groups. The necrotizing enterocolitis and the other groups had significantly different birth weight (2783.5 g vs 3170.9 g, respectively) and gestational age (38.2 weeks vs 39.1 weeks, respectively). Gestational age under 38 weeks (OR 8.87, p = 0.002), birth weight of < 2500 g (OR 5.1, p = 0.042), need for mechanical ventilation (OR 4.6, p = 0.021), parenteral nutrition (OR 107.7, p < 0.001), and functional single ventricle (OR 5.8, p = 0.009) were significant risk factors. The case-fatality rate was higher in the necrotizing enterocolitis (40.0%) than in the other group (8.3%, p = 0.009). CONCLUSIONS: Three percent of full-term infants with duct dependent congenital heart diseases developed necrotizing enterocolitis. Neonates with low birth weight, gestational age less than 38 weeks, functional single ventricle, or receiving assisted mechanical ventilation or parenteral nutrition are at increased risk.

A Retrospective Population-Based Survival Study of Idiopathic Pulmonary Arterial Hypertension in Korea.

BACKGROUND: Few studies used nationwide data to assess the survival rates (SRs) and death risk for idiopathic pulmonary arterial hypertension (IPAH; ICD-10 I27.0) in Korea. METHODS: IPAH data (N = 9,017; female:male = 6:4) were collected from the National Health Insurance Service in Korea, from 2006 through 2017. The data consisted of primary diagnoses related to IPAH. The Kaplan-Meier method and Cox proportional-hazards analyses were carried out. RESULTS: The mean age was 62.3 (± 19.4) years, 64.2 (± 18.9) years in female and 59.4 (± 19.8) years in male (P < 0.001). The one-, three-, five- and 10-year SRs for IPAH were 89.0%, 79.8%, 72.3% and 57.0%, respectively. The adjusted hazard ratio (HR) of IPAH was 1.81 (95% confidence interval [CI], 1.26-2.59) in 60-69 age group, 3.42 (95% CI, 2.40-4.87) in 70-79, and 7.73 (95% CI, 5.43-11.0) in 80s. Other risk factors were male, low-income status, diabetes, myocardial infarction, atrial fibrillation, ischemic stroke, hemorrhagic stroke, and malignant neoplasm. CONCLUSION: The 10-year SR of IPAH was 57% in Korea. The HR for IPAH was significantly high in patients with older age and other risk factors.

Midterm results of arch augmentation with autologous vascular patch in interrupted aortic arch.

OBJECTIVES: Aortic arch reconstruction of interrupted aortic arch remains challenging, and subsequent problems, including arch and airway stenosis, may occur. Thus, we investigated midterm results of an augmentation technique using autologous vascular patch. METHODS: This retrospective study included 24 patients who underwent arch reconstruction with an autologous vascular patch for interrupted aortic arch with biventricular physiology from 2006 to 2018. The median age and body weight at operation were 10 days (range 4-77 days) and 3 kg (range 2.5-5.1 kg), respectively. The reconstructed arch was supplemented in the lesser curvature with an autologous vascular patch that was harvested from main pulmonary artery (n = 19), left subclavian artery (n = 3) or aberrant right subclavian artery (n = 1). One patient used patches from both the main pulmonary and left subclavian artery. RESULTS: There was 1 early death due to right heart failure. All survivors were discharged 15 days (range 9-58 days) after surgery without residual arch stenosis. Late death occurred in 1 patient with Cri-du-chat syndrome and airway stenosis. Two reoperations and 1 intervention for arch stenosis were performed. The 1-, 5- and 10-year survival was 92%. Freedom from reoperation or intervention for arch stenosis was 86% 1, 5 and 10 years after surgery. No occurrence of arch aneurysm formation, left main bronchial stenosis and significant hypertension was found during a median follow-up period of 5.5 years (range 0.3-13.3 years). CONCLUSIONS: Augmenting the lesser curvature with an autologous vascular patch during arch reconstruction resulted in reasonable midterm outcomes.

How can stretched ductus diameter be predicted from angiographic diameter?

BACKGROUND: An appropriate size of device for patent ductus arteriosus (PDA) could be chosen by a stretched PDA size. We propose prediction of stretched size from intact PDA size. METHODS: A total of 361 patients was enrolled. Intact size was measured on angiography before closure, and stretched size was the narrowest diameter immediately after device implantation. RESULTS: The median patient age was 2.8 years. Intact diameter and stretched diameter were 3.6±1.4 mm and 5.1±1.3 mm. The difference and difference ratio were 1.5±0.8 mm and 51.3±38.1% and had negative linear correlations with age (P<0.001). Patients were divided into four groups: <2 years old (159), 2-15 years old (68), 15-40 years old (72), and ≥40 years old (62). Among groups, difference and difference ratio were significantly different. Stretched diameter was inferred by the following formulas: stretched diameter = 0.660 × intact diameter + 2.791 (<2 years old); = 0.971×intact diameter + 0.058 × age + 1.131 (2-15 years old); = 0.790 × intact diameter + 2.263 (15-40 years old); and = 0.837 × intact diameter + 1.353 × BSA - 0.096 (≥40 years old). CONCLUSIONS: Stretched PDA diameter can be predicted from intact diameter and might be helpful for safe PDA closure especially in small infants.

The Waiting List Mortality of Pediatric Heart Transplantation Candidates in Korea before the Pediatric Ventricular Assist Device Era.

BACKGROUND: Despite advancements in heart transplantation for pediatric patients in Korea, the waiting list mortality has not been reported. Therefore, we investigated the waiting list mortality rate and factors associated with patient mortality. METHODS: We reviewed the medical records of pediatric patients who were registered for heart transplantation at three major hospitals in Korea from January 2000 to January 2020. All patients who died while waiting for heart transplantation were investigated, and we identified the waiting list mortality rate, causes of mortality and median survival periods depending on the variable risk factors. RESULTS: A total of 145 patients received heart transplantations at the three institutions we surveyed, and the waiting list mortality rate was 26%. The most common underlying diseases were cardiomyopathy (66.7%) and congenital heart disease (30.3%). The leading causes that contributed to death were heart failure (36.3%), multi-organ failure (27.2%), and complications associated with extracorporeal membrane oxygenation (ECMO) (25.7%). The median survival period was 63 days. ECMO was applied in 30 patients. The different waiting list mortality percentages according to age, cardiac diagnosis, use of ECMO, and initial Korean Network of Organ Sharing (KONOS) level were determined using univariate analysis, but age was the only significant factor associated with waiting list mortality based on a multivariate analysis. CONCLUSION: The waiting list mortality of pediatric heart transplantation candidates was confirmed to be considerably high, and age, underlying disease, the application of ECMO, and the initial KONOS level were the factors that influenced the survival period.

The effects of rational emotive behavior therapy for depressive symptoms in adults with congenital heart disease.

BACKGROUND: Over 30% of adults with congenital heart disease (CHD) experience depression. OBJECTIVES: To evaluate the effectiveness of Rational Emotive Behavior Therapy (REBT) in reducing depressive symptoms in adults with CHD. METHODS: Forty-two adults with CHD were recruited from an outpatient clinic and randomized to a study group (n = 21), which comprised 8-weekly group-based counseling sessions, or a control group (n = 21), which received usual care. REBT effectiveness was examined using the Hamilton Depression Rating Scale, Beck Depression Inventory, Shorten General Attitude and Belief Scale, and salivary cortisol levels before therapy, after the last session, and at follow-up 4 weeks later. RESULTS: Average participant ages were 30.1 ± 7.58 and 33.3 ± 7.1 years in study and control groups, respectively; 52.4% of participants in each group were female. After REBT, depression (p < 0.001), irrational beliefs (p < 0.001), and salivary cortisol levels (p = 0.006) were significantly lower in the study group than in the control group. Effects of REBT in the study group remained consistent at the 4-week follow-up. CONCLUSION: REBT may be effective in reducing depression in adults with CHD.

Mid-term outcomes of the Pulsta transcatheter pulmonary valve for the native right ventricular outflow tract.

OBJECTIVES: The aim of this study is to present the mid-term outcomes of Pulsta valve. BACKGROUND: The Pulsta valve is a Self-expandable knitted nitinol-wire stent mounted with a treated tri-leaflet α-Gal-free porcine pericardial valve for percutaneous pulmonary valve implantation (PPVI) in patients with native right ventricular outflow tract (RVOT) lesions. METHODS: A multi-center clinical trial using Pulsta valve® was designed for patients with severe pulmonary regurgitation (PR) in the native RVOT in multiple centers in South Korea and 25 patients were enrolled. Before PPVI, severe PR (mean PR fraction: 45.5 ± 6.9%) and enlarged RV volume (mean indexed RV end-diastolic volume; 169.7 ± 13.0 ml/m2 ) was present. The mean age was 21.6 ± 6.6 years old. RESULTS: All patients were successfully implanted with 26, 28, or 32 mm diameter of Pulsta valve loaded on the 18 or 20 French delivery catheters. At 6 months follow up, indexed RV end-diastolic volume was decreased to 126.9 ± 16.9 ml/m2 . At mean 33.1 ± 14.3 months follow-up, the mean value of mean pressure gradient in Pulsta valve was 6.5 ± 3.0 mmhg without significant PR. There was no serious device-related adverse event. CONCLUSIONS: A multi-center clinical trial was completed successfully with planned Pulsta valve implantation and demonstrated good mid-term effectiveness without device-related serious adverse events.

Percutaneous coronary intervention in a 4-month-old infant for stenosis of both coronary arteries after arterial switch operation for transposition of the great arteries.

We present a case of percutaneous coronary intervention in a 4-month-old infant with both severe coronary stenosis and acute heart failure after arterial switch operation for transposition of the great arteries. Under extracorporeal membrane oxygenation, balloon angioplasty of the left coronary artery with a 2.0 × 15-mm balloon and stent implantation on the right coronary artery with a 2.25 × 26-mm stent were performed successfully. Echocardiography after the intervention showed recovered cardiac function and no complications.

Prevalence of associated extracardiac anomalies in prenatally diagnosed congenital heart diseases.

OBJECTIVE: To investigate the prevalence of extracardiac anomalies (ECA) in prenatally diagnosed congenital heart diseases (CHD), and to provide more information for counseling of women with prenatally diagnosed fetal CHD. METHODS: This was a retrospective cohort study of 791 cases of fetal CHD diagnosed by prenatal ultrasound from January 2005 to April 2018. Associated ECAs included extracardiac structural malformation (ECM), chromosomal anomaly, and 22q11.2 microdeletion. CHD was classified into 10 groups according to a modified anatomic and clinical classification of congenital heart defects. RESULTS: The overall prevalence of ECA in our CHD cohort was 28.6% (226/791): ECM, 25.3%; chromosomal anomaly, 11.7%; and 22q11.2 microdeletion, 5.5%. For those with ECM, ventricular septal defect (VSD) had the highest prevalence (34.5%), followed by anomalies of atrioventricular junctions and valves (28.8%) and heterotaxy (26.9%). For those with chromosomal anomaly, anomalies of atrioventricular junctions and valves had the highest prevalence (37.5%), followed by anomalies of atria and interatrial communications (25.0%) and VSD (22.9%). 22q11.2 microdeletion was detected only in those with anomalies of extrapericardial arterial trunks (14.3%) or ventricular outflow tracts (6.4%). CONCLUSION: ECM, chromosomal anomaly, and 22q11.2 microdeletion have different prevalence according to the type of CHD.