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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.

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

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.

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.

This study investigated mortality and morbidity in patients requiring postcardiotomy extracorporeal membrane oxygenation (ECMO) support after operations for congenital heart disease (CHD).CHD patients requiring postoperative ECMO support between May 2011 and May 2021 were retrospectively reviewed. Patients were divided into non-survivors and survivors to hospital discharge. Survival outcomes and associations of various factors with in-hospital death were analyzed.Fifty patients required postoperative ECMO support. Patients' median age and weight at the time of ECMO insertion were 1.85 months (interquartile range [IQR], 0.23-14.5 months) and 3.84 kg (IQR, 3.08-7.88 kg), respectively. Twenty-nine patients (58%) were male. The median duration of ECMO support was 6 days (IQR, 3-12 days). Twenty-nine patients (58%) died on ECMO support or after ECMO weaning, and 21 (42%) survived to hospital discharge. Postoperative complications included renal failure (n=33, 66%), bleeding (n=11, 22%), and sepsis (n=15, 30%). Prolonged ECMO support (p=0.017), renal failure (p=0.005), continuous renal replacement therapy (CRRT) application (p=0.001), sepsis (p=0.012), bleeding (p=0.032), and high serum lactate (p=0.002) and total bilirubin (p=0.017) levels during ECMO support were associated with higher mortality risk in a univariate analysis. A multivariable analysis identified CRRT application (p=0.013) and a high serum total bilirubin level (p=0.001) as independent risk factors for death.Postcardiotomy ECMO should be considered as an important therapeutic modality for patients unresponsive to conventional management. ECMO implementation strategies and management in appropriate patients without severe complications, particularly renal failure and/or liver failure, are crucial for achieving positive outcomes.

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.

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

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.

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.

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.

Background We reviewed our revised surgical strategy for tetralogy of Fallot (TOF) total correction to minimize early exposure to significant pulmonary regurgitation (PR) and to avoid right ventriculotomy (RV-tomy). Methods and Results: Since February 2016, we have tried to preserve, first, pulmonary valve (PV) function to minimize PR by extensive commissurotomy with annulus saving; and second, RV infundibular function by avoiding RV-tomy. With this strategy, we performed total correction for 50 consecutive patients with TOF until May 2018. We reviewed the early outcomes of 27 of 50 patients who received follow-up for ≥3 months. Mean patient age at operation was 10.2±5.0 months, and mean body weight was 8.8±1.2 kg. The preoperative pressure gradient at the RV outflow tract and the PV z-score were improved at most recent echocardiography from 82.0±7.1 to 26.8±6.4 mmHg, and from -2.35±0.49 to -0.55±0.54, respectively, during 11.1±1.6 months of follow-up after operation. One patient required re-intervention for residual pulmonary stenosis. Twenty-two patients had less than moderate PR (none, 1; trivial, 8; mild, 13), and 5 patients had moderate PR. There was no free or severe PR. Conclusions At 1-year follow-up, the patients who underwent total TOF correction with our revised surgical strategy had acceptable results in terms of PV function. The preserved PV had a tendency to grow on short-term follow-up.

Severe tricuspid regurgitation resulting from a flail leaflet is a rare cause of neonatal cyanosis. We report a neonate with profound cyanosis and severe tricuspid regurgitation caused by a rupture of the papillary muscle supporting the anterior leaflet, without other structural heart defects. Ductal patency could not be established. The repair of the tricuspid valve was performed after initial stabilization by using extracorporeal membrane oxygenation.

Recurrent ventricular arrhythmia can be fatal and cause serious complications, particularly when it is caused immediately after an operation. Incorrect placement of a Swan-Ganz catheter can trigger life-threatening ventricular arrhythmia, but even intensive care specialists tend to miss this fact. Here, we report a case of recurrent ventricular arrhythmia causing a severe hemodynamic compromise; the arrhythmia was induced by a severely angulated Swan-Ganz catheter. The recurrent ventricular arrhythmia was not controlled by any measures including repositioning of the catheter, until the complete removal of the Swan-Ganz catheter. It is necessary to keep in mind that the position of the pulmonary artery catheter should be promptly checked if there is intractable recurrent ventricular arrhythmia.

Cardiac complications such as arrhythmia and heart failure are common in Graves disease. Early detection and proper treatment of hyperthyroidism are important because cardiac complications are reported to be reversible if the thyroid function is normalized by medical treatment. We report here a case of cardiac complication of Graves disease that was too late to reverse with medical treatment and required surgical treatment.

Background: We compared the mid-term results of the Cox maze IV procedure using argon-based cryoablation with a procedure using N 2 O-based cryoablation. Methods: From May 2006 to June 2012, 138 patients (mean age, 58.2±11.0 years) underwent the Cox maze IV procedure. Eighty-five patients underwent the maze procedure using an N 2 O-based cryoprobe (group N), and 53 patients underwent the maze procedure using an argon-based cryoprobe (group A). Bipolar radiofrequency ablation was concomitantly used in 131 patients. The presence of atrial fibrillation immediately, 6 months, 1 year, and 2 years after surgery was compared. Results: Early mortality occurred in 6 patients (4.3%). There were no differences in early mortality or postoperative complications between the two groups. Nineteen of 115 patients (16.5%) remained in atrial fibrillation at postoperative 12 months (14 of 80 patients (17.5%) in group N and 5 of 35 patients (14.3%) in group A, p=0.669). There were no differences in the number of patients who remained in atrial fibrillation at any of the time periods except in the immediate postoperative period. A multivariable analysis revealed that the energy source of cryoablation was not associated with the presence of atrial fibrillation at 1 year (p=0.862) and that a fine F wave (＜0.1 mV) was the only risk factor predicting the presence of atrial fibrillation at 1 year (p＜0.001, odds ratio=20.287). Conclusion: The Cox maze IV procedure using an argon-based cryoprobe was safe and effective compared with the maze procedure using an N 2 O-based cryoprobe in terms of operative outcomes and the restoration of sinus rhythm for up to 2 years after surgery.