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.

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.

Management of Cannula Wound Problems with Vacuum-Assisted Therapy for a Child Who Received Berlin Heart EXCOR Implantation.

A child being supported with an extracorporeal ventricular assist device, such as the Berlin Heart EXCOR (Berlin Heart GmbH, Berlin, Germany), must have at least 2 large cannulae for a long period. Management of cannula wounds is crucial since a cannula forms a track of prosthetic material passing the mediastinum to the heart. Deep wound complications, if they occur, can be troublesome and difficult to control with conventional methods. We applied vacuum-assisted closure to a patient who had Berlin-Heart EXCOR and a gap at the cannulation site. Herein, we describe the technical aspects of management in detail.

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.

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.

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.

Impact of age on the outcomes of extracorporeal cardiopulmonary resuscitation: analysis using inverse probability of treatment weighting.

OBJECTIVES: Patient selection is crucial to improving the outcomes of extracorporeal cardiopulmonary resuscitation (ECPR). However, ECPR's efficacy with older patients remains unknown. METHODS: We reviewed patients who underwent extracorporeal membrane oxygenation (ECMO) for refractory cardiac arrest from January 2006 to December 2018. Exclusion criteria were age <18 years, cannulation failure and ECMO applied at another hospital. We divided patients into 2 groups with an age cut-off of 66 years, using the Contal and O'Quigley method for overall survival. We performed inverse probability of treatment weighting (IPTW) between the 2 groups and set the primary outcome as overall survival. RESULTS: We included 318 patients in our study (≤66 years, n = 199; >66 years, n = 119). Before IPTW, we observed that the older group had a higher frequency of diabetes, hypertension, and myocardial infarction. In the young group, more patients had out-of-hospital cardiac arrest as compared with the older group. The hospital mortality rate was 55% (48.7% in the young group, 65.5% in the older group; P = 0.004). In the multivariable analysis after IPTW, the older group showed worse outcomes in overall survival [hazard ratio (HR) = 2.02; 95% confidence interval (CI), 1.50-2.70; P < 0.001] and neurological outcomes at discharge (odds ratio = 2.95; 95% CI, 1.69-5.14; P = <0.001). ECMO insertion during catheterization (HR = 0.57; 95% CI, 0.36-0.90; P = 0.015) and recovery of spontaneous circulation before pump-on (HR = 0.67; 95% CI, 0.50-0.89; P = 0.007) were positive predictors, but initial asystole rhythm, non-cardiac cause (HR = 2.39; 95% CI, 1.59-3.61; P < 0.001), out-of-hospital cardiac arrest (HR = 1.86; 95% CI, 1.24-2.79; P = 0.003) and prolonged cardiopulmonary resuscitation to pump-on time (HR = 1.01; 95% CI, 1.01-1.02; P < 0.001) were negative predictors for overall survival. CONCLUSIONS: Older patients who had ECPR had significantly worse survival and neurological outcomes. For patients older than age 66 years, more careful patient selection is critically important for improving the efficacy of ECPR.

Clinical Outcomes of Implantable Cardioverter-Defibrillator in Pediatric Patients　- A Korean Multicenter Study.

BACKGROUND: Implantable cardioverter-defibrillator (ICD) therapy is important for the prevention of sudden cardiac death, but data on clinical outcomes of ICD therapy in Asian pediatric patients are scarce. The aim of this Korean multicenter study was to evaluate the current state and elucidate the clinical outcomes of ICD therapy in children.Methods and Results:Data from 5 pediatric cardiology centers were retrospectively collected from 2007 to 2019. Altogether, 99 patients were enrolled (mean age 13.9±4.1 years). The most common underlying disease was a primary electrical disease (56%). An ICD was implanted for primary prevention in 19%. Appropriate shock occurred in 44% of patients at a median of 1.6 years after implantation. There was no significant difference in the appropriate shock rate between patients with primary and secondary prevention indications (32% vs. 48%, respectively). A total of 33 patients (33%) experienced inappropriate shock, which was associated with primary electrical disease and follow-up duration on multivariate analysis. 17% of patients had ICD-related complications. CONCLUSIONS: The utilization rate of ICD for primary prevention was still low in the pediatric population in Korea, but there was a substantial rate of appropriate shock in these patients. Efforts to increase ICD usage to save the lives of high-risk patients and reduce the incidence of inappropriate shock are required.

Extracorporeal Membrane Oxygenation for Fulminant Myocarditis: Increase of Cardiac Enzyme and SOFA Score Is Associated with High Mortality.

We aimed to evaluate the outcomes of patients with fulminant myocarditis and investigate the factors associated with mortality. This is a retrospective single-center cohort study that included adult and pediatric patients with fulminant myocarditis treated at Samsung Medical Center between September 2004 and December 2019. The primary outcome was in-hospital mortality. Among 100 patients, 71 underwent veno-arterial extracorporeal membrane oxygenation (ECMO) (ECMO group). Comorbidities were not significantly different between the ECMO and non-ECMO groups. Cardiac enzymes, creatinine, and median sequential organ failure assessment (SOFA) score at intensive care unit admission were significantly different between the groups. Twenty patients (28.7%) in the ECMO group and two (6.9%) in the non-ECMO group died in-hospital (p = 0.02). The significant risk factors of in-hospital mortality were creatine kinase MB fraction (CK-MB) and SOFA score (p = 0.009 and p = 0.001, respectively) in the ECMO group. In the receiver-operating characteristic curve analysis, the cutoffs of SOFA score and CK-MB were 12 and 94.74 ng/mL, respectively. The patients with both variables above the cutoffs showed significantly worse outcomes (p < 0.001). ECMO can be an effective treatment option for fulminant myocarditis. SOFA score and CK-MB are significant risk factors for in-hospital mortality.

Successful Modified Nikaidoh Procedure (Pivot Rotation) in a Patient with Double Outlet Right Ventricle and Pulmonary Atresia: Case Report.

Double outlet right ventricle (DORV) with pulmonary atresia (PA) is an uncommon congenital disease. Because of anatomical and physiological anomalies in the systemic and pulmonary circulation, corrective surgery may be challenging. We present the case of a patient with DORV and PA. This condition was successfully corrected using a modified Nikaidoh procedure, resulting in reduced obstruction of the left ventricular outflow tract.

Impact of a Multidisciplinary Team Approach on Extracorporeal Circulatory Life Support-Bridged Heart Transplantation.

BACKGROUND: The number of heart transplantations (HTx) is increasing annually. Due to advances in medical and surgical support, the outcomes of HTx are also improving. Extracorporeal circulatory life support (ECLS) provides patients with decompensated heart failure a chance to undergo HTx. A medical approach involving collaboration among experienced experts in different fields should improve the outcomes and prognosis of ECLSbridged HTx. METHODS: From December 2003 to December 2018, 1,465 patients received ECLS at Samsung Medical Center. We excluded patients who had not undergone HTx or underwent repeated transplantations. Patients younger than 18 years were excluded. We also excluded patients who received an implantable durable left ventricular assist device before HTx. In total, 91 patients were included in this study. A multidisciplinary team approach began in March 2013 at our hospital. We divided the patients into 2 groups depending on whether they were treated before or after implementation of the team approach. RESULTS: The 30-day mortality rate was significantly higher in the pre-ECLS team group than in the post-ECLS team group (n=5, 18.5% vs. n=2, 3.1%; p=0.023). The 1-year survival rate was better in the post-ECLS team group than in the pre-ECLS team group (n=57, 89.1% vs. n=19, 70.4%; p=0.023). CONCLUSION: We found that implementing a multidisciplinary team approach improved the outcomes of ECLS-bridged HTx. Team-based care should be adapted at HTx centers that perform high-risk HTx.

Use of extracorporeal membrane oxygenation in postpartum patients with refractory shock or respiratory failure.

Although extracorporeal membrane oxygenation (ECMO) is increasingly utilized, only a limited level of experience has been reported in postpartum cardiopulmonary failure. Ten critically ill postpartum patients who received ECMO were included between January 2010 and December 2018 in this retrospective observational study. The main indication for ECMO support was peripartum cardiomyopathy (n = 5), followed by postpartum hemorrhage (n = 2). Nine patients initially received veno-arterial ECMO, and one patient received veno-venous ECMO. Major bleeding occurred in six patients. The median number of units of red blood cells (RBC) transfused during ECMO was 14.5 units (interquartile range 6.8-37.8 units), and most RBC transfusions occurred on the first day of ECMO. The survival-to-discharge rate was 80%. Compared to the survival outcomes in female patients of similar age who received ECMO, the survival outcomes were significantly better in the study population (56% versus 80%, P = 0.0004). Despite the high risk of major bleeding, ECMO for patients with postpartum cardiac or respiratory failure showed excellent survival outcomes. ECMO is feasible in these patients and can be carried out with good outcomes in an experienced centre.

Surgical Repair of a Sinus of Valsalva Aneurysm: A 22-Year Single-Center Experience.

BACKGROUND: Several reports described the repair of sinus of Valsalva aneurysms (SVAs); however, there is still debate regarding the optimal method of operation. We investigated the determinants of the development of significant aortic regurgitation (AR) and long-term survival after surgical repair. METHODS: Between January 1995 and December 2016, 71 patients (31 females; median age: 33.3 years) underwent surgical SVA repair with (n = 60) or without (n = 11) rupture. Aortic valvuloplasty (AVP) was performed using Trusler's technique in 28 patients (39.4%), and 11 patients (15.5%) underwent aortic valve replacement during the first operation. RESULTS: There was no early mortality, and three deaths occurred during follow-up (median: 65.4 months). Patients with grade II preoperative AR who underwent AVP tended to develop significant postoperative AR, but freedom from significant AR did not differ statistically (p = 0.387). Among patients who underwent AVP, freedom from significant AR did not differ statistically between those with grades I and II and those with grades III and IV (p = 0.460). CONCLUSION: Surgical repair of SVA with or without rupture can be performed safely using the dual approach technique. Concomitant aortic valve repair can be performed without difficulty and should be recommended not only for patients with moderate or severe preoperative AR (grades III and IV) but also for those with minimal or mild preoperative AR (grades I and II), whose aortic valve geometry needs correction.

Surgical Outcomes for Obstructive Total Anomalous Pulmonary Venous Return of the Non-Infracardiac Type Compared with the Infracardiac Type.

BACKGROUND: Infracardiac obstructive total anomalous pulmonary venous return (TAPVR) has a poor outcome following surgical correction. We compared the surgical outcomes of obstructive TAPVR between non-infracardiac and infracardiac types. METHODS: Among 51 patients who underwent surgical repair for obstructive TAPVR, 23 with infracardiac type and 28 with non-infracardiac type were included in this investigation. The study compared the immediate postoperative courses in the intensive care unit and long-term mortality and pulmonary vein stenosis. The risk factors for long-term survival in obstructive TAPVR also were investigated. RESULTS: The postoperative follow-up period was 79.8 ± 81.5 months. Immediate major operative complications were observed in 22 patients (43.1%); 10 patients (19.6%) died, and eight patients (15.7%) experienced pulmonary vein stenosis during the follow-up period. The Kaplan-Meier curve showed better cumulative survival in patients with infracardiac TAPVR (P = 0.308). The significant factors for survival after surgical repair of obstructive TAPVR did not include anatomical type but instead were postoperative course of ventilator care and lengths of intensive care unit and hospital stays. CONCLUSION: Patients with non-infracardiac TAPVR with obstruction had a longer postoperative course and experienced more complications. Their survival rate was poorer, and postoperative pulmonary vein stenosis was more frequent in those patients compared with infracardiac TAPVR patients. However, a large-scale study is mandatory to gather more data and confirm our findings.

Left Ventricular Assist Device Implantation via Dual Left Thoracotomy in an Adult Patient with Congenitally Corrected Transposition of the Great Arteries.

A 59-year-old man presented for possible durable ventricular assist device (VAD) implantation. He had previously been diagnosed with congenitally corrected transposition of the great arteries, a ventricular septal defect, an atrial septal defect, pulmonary valve stenosis, and aortic valve regurgitation. In the previous 22 years, he had undergone palliative cardiac surgery 3 times. VAD implantation as a bridge to transplantation was planned. Owing to severe adhesions, mesocardia, a left ascending aorta, and moderate aortic regurgitation, we performed VAD implantation and aortic valve closure via a dual left thoracotomy and partial sternotomy.

The progression of an acceptable pulmonary stenosis immediately after total correction of tetralogy of Fallot.

BACKGROUND: A certain degree of pulmonary stenosis after total correction of tetralogy of Fallot has been considered acceptable. But the long-term outcomes are not well understood. We observed the natural course of immediate pulmonary stenosis and investigated related factors for progression. METHODS: Fifty-two patients with acceptable pulmonary stenosis immediately after operation were enrolled. Acceptable pulmonary stenosis was defined as peak pressure gradient between 15 and 45 mmHg by Doppler echocardiography. Latent class linear mixed model was used to differentiate patients with progressed pulmonary stenosis, and the factors related to progression were analysed. RESULTS: Pulmonary stenosis progressed in 14 patients (27%). Between the progression group and no progression group, there were no significant differences in operative age, sex, and the use of the transannular patch technique. However, immediate gradient was higher in the progression group (32.1 mmHg versus 25.7 mmHg, p = 0.009), and the cut-off value was 26.8 mmHg (sensitivity = 65.3%, specificity = 65.8%). Main stenosis at the sub-valve was observed more frequently in the progression group (85.7% versus 52.6%, p = 0.027). Despite no difference in the preoperative pulmonary valve z value, the last follow-up pulmonary valve z value was significantly lower in the progression group (-1.15 versus 0.35, p = 0.002). CONCLUSIONS: Pulmonary stenosis immediately after tetralogy of Fallot total correction might progress in patients with immediate pulmonary stenosis higher than ≥26.8 mmHg and the main site was sub-valve area.

Left heart decompression at venoarterial extracorporeal membrane oxygenation initiation in cardiogenic shock: prophylactic versus therapeutic strategy.

BACKGROUND: We aimed to investigate the effects and proper timing of left heart decompression (LHD) on the clinical outcomes of patients on venoarterial extracorporeal membrane oxygenation (VA ECMO). METHODS: Among 335 adult patients who underwent VA ECMO from January 2013 to December 2016, 50 who underwent LHD were analyzed. The patients were divided into two groups according to the timing of LHD: those in whom percutaneous LHD was performed at ECMO initiation (prophylactic LHD group, n=18) and those who required LHD to treat complications of impaired left ventricle (LV) unloading (therapeutic LHD group, n=32). RESULTS: Thirty-day mortality rates were 34.4% and 5.6% in the therapeutic and prophylactic LHD groups, respectively (P=0.036). The rate of successful weaning from ECMO (62.5% vs. 83.3%, P=0.123) and duration of ECMO support (10.5 vs. 15.4 days, P=0.332) were not significantly different between the groups. However, the rate of bridging to cardiac replacement therapy, such as heart transplantation or left ventricular assist device, was significantly higher in the prophylactic LHD group (66.7%) than in the therapeutic LHD group (37.5%) (P=0.048). CONCLUSIONS: Prophylactic percutaneous transseptal left atrial (LA) drainage at ECMO initiation appeared to be associated with a lower early mortality rate and a higher likelihood of successful bridging to cardiac replacement therapy than therapeutic LHD to treat complications of left ventricular distension.

The Effect of Multidisciplinary Approach on the Birth Rate of Fetuses with Prenatally Diagnosed Congenital Heart Disease.

BACKGROUND: This study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs). METHODS: Among the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results. RESULTS: Among the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035). CONCLUSION: When a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.