Pulmonary valve replacement may not restore ventricular volume and functional status in patients with pulmonary regurgitation after late tetralogy of Fallot repair.

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 < 0.001), end-systolic volume (101.4-64.9 ml/m2, P < 0.001), stroke volume (66.8-48.0 ml/m2, P = 0.007) and cardiac output (5.1-3.6 l/m2, P = 0.040). Twenty-eight percentage of patients achieved normalization of the right ventricular volume after pulmonary valve replacement. In the exercise test, the maximum rate of oxygen consumption (72.5-69.5%) and oxygen pulse (95.0-83.0%) changed without statistical significance. 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.

Surgical outcomes of infective endocarditis in children: should we delay surgery for infective endocarditis?

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 <18years of age who underwent first surgery for IE. Early surgery was defined as that performed within 2 days for left-sided IE and 7 days for right-sided IE after diagnosis. 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 < 0.001) and preoperative negative blood culture conversion (early surgery < non-early surgery, P = 0.025). There were no significant differences in overall survival, recurrent IE, and reoperation rate between the groups. Early surgery and preoperative negative blood culture conversion were not found as significant factors for surgical adverse outcomes. 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.

Effect of Pulmonary Valve Replacement in the Repaired Tetralogy of Fallot Patients with Trans-annular Incision: More than 20 Years of Follow-up.

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 <1 year at the time of TOF repair and shunt implantation prior to TOF repair were predictive factors for requiring PVR.

Tricuspid valve detachment for ventricular septal defect closure in infants <5 kg: should we be hesitant?

OBJECTIVES: We compared the clinical outcomes between tricuspid valve detachment (TVD) and non-TVD for ventricular septal defect (VSD) closure in infants <5 kg. METHODS: From January 2004 to April 2020, 462 infants <5 kg with VSD without more complex intracardiac lesions and who had undergone VSD closure through the trans-atrial approach were enrolled. Propensity score-matching analysis was performed. Clinical outcomes were compared between the paired TVD group (group D) and paired non-TVD group (group N). 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 <5 kg, TVD can be a reasonable and valid option for successful VSD closure without morbidities, including TR progression if the indication exists.

Experience with Temporary Centrifugal Pump Bi-ventricular Assist Device for Pediatric Acute Heart Failure: Comparison with ECMO.

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.

Changes of hospitalization trend in the pediatric cardiology division of a single center by increasing adult with congenital heart disease.

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.

Outcomes of Supra-annular Mechanical Atrioventricular Valve Replacement with Polytetrafluoroethylene Graft in Infants and Children.

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 < 20 kg) who underwent atrioventricular valve replacement with mechanical valve in Seoul National University Children's Hospital between December 1984 and January 2019. We identified 8 patients (median age 20 months, median weight 10.2 kg) who underwent supra-annular MVR with polytetrafluoroethylene graft (supra-annular group). The patients were diagnosed with congenital mitral valve malformation (5 patients), complete atrioventricular septal defect (2 patients), and functional single ventricle (1 patient). The implanted mechanical valve size ranged from 16 to 23 mm. Thirty-three patients (median age 40 months, median weight 13 kg) underwent conventional annular MVR (annular group). The survival rate was not significantly different between the supra-annular and annular groups (75.0 vs 78.8%, P = 0.816). In patients with biventricular repair (7 patients with supra-annular MVR and 28 patients with annular MVR), mechanical valve-to-mitral valve annulus size ratio was higher in the supra-annular group than in the annular group (1.24 ± 0.30 vs 0.96 ± 0.22, P = 0.035). No coronary complication or heart block were observed in the supra-annular group. Supra-annular MVR with polytetrafluoroethylene graft may be a feasible surgical option in children with a small annulus when valve repair is unsuccessful.

Long-term Follow-up of Epicardial Pacing and Left Ventricular Dysfunction in Children With Congenital Heart Block.

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.

Reversibility of Pulmonary Hypertension Following Surgical Atrial Septal Defect Closure in Children with Down Syndrome.

BACKGROUND: Many Down syndrome (DS) patients have an atrial septal defect (ASD) and associated pulmonary hypertension (PH) from early childhood. ASD closure in DS patients with PH is often controversial due to concerns regarding exacerbation of PH. The aim of this study was to investigate the clinical outcome following surgical ASD closure in children with DS. METHODS: We retrospectively reviewed the medical records of DS patients who underwent surgical ASD patch closure from January 2000 to December 2016. RESULTS: A total of 15 patients underwent surgery for ASD. Prior to ASD patch closure, nine patients were diagnosed with PH, three of whom took medications for PH. The mean age of patients at ASD patch closure was 17.3 months, and the mean diameter of the ASD was 10.2 mm. Three patients who took medications for severe PH underwent ASD patch closure at ages 7, 12, and 25 months. Two patients continued medication for an additional 13 and 21 months, and one patient remained on medication 52 months after ASD closure. PH did not recur following discontinuation of selective pulmonary vasodilators in two patients. Although a moderate degree of PH remained in one patient due to a chronic lung problem, it was improved compared to before ASD closure. No PH was observed in the remaining 12 patients following ASD closure. CONCLUSIONS: A large ASD can be closed even in DS patients with severe PH during early childhood with the support of multiple selective pulmonary vasodilators.

Hybrid Pulmonary Vein Stenting in Infants with Refractory to Surgical Pulmonary Vein Stenosis Repair.

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.

Surgical results of mitral valve repair for congenital mitral valve stenosis in paediatric patients.

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 < 0.0001). The mean follow-up duration was 6.7 ± 5.4 years. One patient died postoperatively due to septic shock. Four patients required a second operation (2 patients for mitral stenosis, 1 patient for left ventricular outflow tract obstruction and mitral stenosis and 1 patient for mitral regurgitation). Among them, 2 patients died: 1 patient died due to cardiopulmonary bypass weaning failure and another patient died due to multiple cerebral infarcts. At the last follow-up, the mean MV pressure gradient was 4.5 ± 3.1 mmHg for all patients who did not have reoperation, and moderate or greater mitral insufficiency was detected in 3 patients. At 10 years, the survival rate was 85.9 ± 7.6%, and the freedom from reoperation rate was 77.5 ± 10.1%. In the log-rank test, MV repair in the neonate was associated with mortality (P = 0.010), and presentation of mitral insufficiency was associated with reoperation (P = 0.003). CONCLUSIONS: MV repair in paediatric patients with congenital mitral stenosis showed acceptable results. The follow-up echocardiogram also revealed satisfactory results. Close follow-up is necessary to detect the development of postoperative mitral stenosis or regurgitation.

Clinical Characteristics and Independent Factors Related to Long-Term Outcomes in Patients with Left Isomerism.

BACKGROUND AND OBJECTIVES: We studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis. SUBJECTS AND METHODS: We reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014. RESULTS: Of the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5.2%) had normal heart structure. Extracardiac anomalies were noted in 38.1% of the study population, including biliary atresia in 7.8% of all patients. Of the 25 patients who underwent Kawashima procedures, 24.0% developed pulmonary arteriovenous fistulas (PAVFs). During the median follow-up period of 11.4 years (range: 1 day to 32 years), 14 patients died. The 10-year, 20-year, and 30-year survival rates were 87%, 84%, and 76%, respectively. Preoperative dysrhythmia and uncorrected atrioventricular valve regurgitation were significantly associated with late death. There was no significant difference in the number of surgical procedures and in survival expectancy between patients in the functional single-ventricle group and in the biventricular group. However, late mortality was higher in functional single-ventricle patients after 18 years of age. CONCLUSION: Patients with LI need to be carefully followed, not only for late cardiovascular problems such as dysrhythmia, valve regurgitation, and the development of PAVFs, but also for noncardiac systemic manifestations.

Experience with Mechanical Circulatory Support for Medically Intractable Low Cardiac Output in a Pediatric Intensive Care Unit.

BACKGROUND AND OBJECTIVES: Mechanical circulatory support with extracorporeal membrane oxygenation (ECMO) and ventricular assist device has always been the optimal choice for treating the majority of medically intractable low cardiac output case. We retrospectively investigated our institution's outcomes and variables associated with a high risk of mortality. SUBJECTS AND METHODS: From 1999 to 2014, 86 patients who were of pediatric age or had grown-up congenital heart disease underwent mechanical circulatory support for medically intractable low cardiac output in our pediatric intensive care unit. Of these, 9 grown-up congenital heart disease patients were over 18 years of age, and the median age of the subject group was 5.82 years (range: 1 day to 41.6 years). A review of all demographic, clinical, and surgical data and survival analysis were performed. RESULTS: A total of 45 (52.3%) patients were successfully weaned from the mechanical assist device, and 25 (29.1%) survivors were able to be discharged. There was no significant difference in results between patients over 18 years and under 18 years of age. Risk factors for mortality were younger age (<30 days), functional single ventricle anatomy, support after cardiac operations, longer support duration, and deteriorated pre-ECMO status (severe metabolic acidosis and increased levels of lactate, creatinine, bilirubin, or liver enzyme). The survival rate has improved since 2010 (from 25% before 2010 to 35% after 2010), when we introduced an upgraded oxygenator, activated heart transplantation, and also began to apply ECMO before the end-stage of cardiac dysfunction, even though we could not reveal significant correlations between survival rate and changed strategies associated with ECMO. CONCLUSION: Mechanical circulatory support has played a critical role and has had a dramatic effect on survival in patients with medically intractable heart failure, particularly in recent years. Meticulous monitoring of acid-base status, laboratory findings, and early and liberal applications are recommended to improve outcomes without critical complication rates, particularly in neonates with single ventricle physiology.

Anticalcification effect of a combination of decellularization, organic solvents and amino acid detoxification on glutaraldehyde-fixed xenopericardial heart valves in a large-animal long-term circulatory model.

OBJECTIVES: We aimed to investigate the effect of a combination of anticalcification treatments, which were effective for preventing calcification in a small animal experiment, on glutaraldehyde-fixed xenopericardial valves using a large-animal long-term circulatory model. METHODS: Valved conduits were made of porcine pericardium as a leaflet and bovine pericardium as a conduit and were implanted into the right ventricular outflow tract of goats under cardiopulmonary bypass. The goats were divided into study (glutaraldehyde + combined anticalcification treatment, n = 6) and control (glutaraldehyde alone, n = 9) groups. Upon euthanization at 1 year, echocardiography and cardiac catheterization were performed. Explanted tissues were microscopically examined and analysed for measuring the calcium content. RESULTS: Haemodynamic data were obtained from 3 and 2 goats in the study and control groups, respectively. All valves, except 1, which was limited in motion, were functioning well on echocardiography; pressure gradients across the right ventricular outflow tract were lower in the study group on cardiac catheterization. On gross inspection, all leaflets remained mobile without calcific deposits in the study group, while most leaflets were heavily calcified in the control group. The calcium content in the leaflets remained low (≤4 µg/mg) in the study group. Among the leaflets explanted from goats that survived longer (>3 months), the calcium concentration was higher in the control group than in the study group [15.1 µg/mg (n = 5) vs 2.7 µg/mg (n = 5), respectively; P = 0.008). CONCLUSIONS: Porcine pericardial leaflets treated with our anticalcification protocol showed better function and less calcification than those treated with glutaraldehyde alone in the pulmonary position.

The Effects of Modification to Lateral Tunnel Fontan Procedure for Prophylactic Arrhythmia Surgery.

BACKGROUND: Refractory atrial arrhythmias and hemodynamic abnormalities are responsible for significant morbidity and mortality after the Fontan operation. We evaluated the long-term feasibility, safety, and efficacy of prophylactic atrial arrhythmia surgery performed concomitantly with the lateral tunnel Fontan operation. METHODS: From 1997 August to 2003 December, 27 patients underwent a initial lateral tunnel Fontan with an interventional atrial incision and cryoablation from the atriotomy to the coronary sinus and right atrioventricular valve annulus. This novel surgical technique consists of (1) right atriotomy extending to the coronary sinus to block the slow rate conduction isthmus; (2) cryoablation between right atriotomy and right atrioventricular valve annulus; (3) baffling to avoid injury to the crista terminalis; and (4) use of a sandwich technique with closure of right atriotomy incorporating the Gore-Tex (W. L. Gore & Associates, Flagstaff, AZ) patch to reduce atrial suture line. RESULTS: There has been no early death after operation and one late death, which was not arrhythmic in etiology. At late follow-up of 15.2 ± 2.9 years (range, 5.5 to 18.0) after Fontan, spontaneous intraatrial reentrant tachycardia occurred in 1 patient, and inducible intraatrial reentrant tachycardia in 1 patient who required beta-blocker medication without ablation attempts. There was no evidence of early or late complications related to the interventional atrial incision and cryoablation. Four patients required late pacemaker implantation for sinus node dysfunction after Fontan operation. CONCLUSIONS: The prophylactic arrhythmia surgery with our novel modification of the lateral tunnel Fontan procedure to reduce the development of intraatrial reentrant tachycardia was feasible and safe. Long-term follow-up results also demonstrated that this novel modification is effective for the prophylaxis of intraatrial reentrant tachycardia.

Long-Term Results after Surgical Treatment of Ebstein's Anomaly: a 30-year Experience.

BACKGROUND AND OBJECTIVES: The aim of the study is to evaluate the long-term results after a surgical repair of Ebstein's anomaly. SUBJECTS AND METHODS: Forty-eight patients with Ebstein's anomaly who underwent open heart surgery between 1982 and 2013 were included. Median age at operation was 5.6 years (1 day-42.1 years). Forty-five patients (93.7%) demonstrated tricuspid valve (TV) regurgitation of less than moderate degree. When the patients were divided according to Carpentier's classification, types A, B, C, and D were 11, 21, 12, and 4 patients, respectively. Regarding the type of surgical treatment, bi-ventricular repair (n=38), one-and-a half ventricular repair (n=5), and single ventricle palliation (n=5) were performed. Of 38 patients who underwent a bi-ventricular repair, TV repairs were performed by Danielson's technique (n=20), Carpentier's technique (n=11), Cone repair (n=4), and TV annuloplasty (n=1). Two patients underwent TV replacement. Surgical treatment strategies were different according to Carpentier's types (p<0.001) and patient's age (p=0.022). RESULTS: There were 2 in-hospital mortalities (4.2%; 1 neonate and 1 infant) and 2 late mortalities during follow-up. Freedom from recurrent TV regurgitation rates at 5, 10, and 15 years were 88.6%, 66.3%, 52.7%, respectively. TV regurgitation recurrence did not differ according to surgical method (p=0.800). Survival rates at 5, 10, and 20 years were 95.8%, 95.8%, and 85.6%, respectively, and freedom from reoperation rates at 5, 10, and 15 years were 85.9%, 68.0%, and 55.8%, respectively. CONCLUSION: Surgical treatment strategies were decided according to Carpentier's type and patient's age. Overall survival and freedom from reoperation rates at 10 years were 95.8% and 68.0%, respectively. Approximately 25% of patients required a second operation for TV during the follow-up.

Surgical Results of Anomalous Origin of One Pulmonary Artery Branch from the Ascending Aorta.

We reviewed our surgical experience with anomalous origin of one pulmonary artery from the ascending aorta (AOPA). From 1989 to 2012, 12 children (five neonates) aged 3-734 days (mean 152 ± 222) with AOPA underwent operations. Eight patients had right AOPA, and four patients had left AOPA. The majority of the patients had elevated right ventricular pressure, with 58 % (7 of 12) demonstrating suprasystemic right ventricular pressure. Surgery was performed by direct anastomosis (group 1) in seven patients and by employing an autologous patch (group 2) in five patients. There were two postoperative mortalities caused by heart failure and pulmonary hypertensive crisis. The mean follow-up duration was 12.6 ± 8 years. Catheterization showed that the right ventricle-to-systemic pressure ratio decreased following operation (preoperative vs. postoperative; 1.13 ± 0.19 vs. 0.48 ± 0.03, p = 0.043). There was no difference in the perfusion of the affected lung as measured by the final lung perfusion scan, between the two groups (group 1 vs. group 2; 50.0 ± 10.3 vs. 42.7 ± 28.7 %, p = 0.158). Two patients required reoperations for pulmonary regurgitation and pulmonary artery stenosis. There were two catheter-based interventions. At 20 years, survival by the Kaplan-Meier was 91.7 ± 8.0 %, freedom from reoperation was 80.0 ± 17.9 %, and freedom from catheter intervention was 80.8 ± 12.2 %. Early repair of AOPA improves right ventricular pressure and overall hemodynamics with excellent survival and low risk of reintervention. The type of surgical repair did not significantly affect the long-term outcomes (measured via lung perfusion scan).

Intra-atrial reentrant tachycardia in adult patients after Fontan operation.

BACKGROUND: Atrial tachyarrhythmia is a major late complication in adult Fontan patients. This study examined the clinical features and risk factors of late intra-atrial reentrant tachyarrhythmia (IART) in adult patients after Fontan surgery and the mid-term outcome of Fontan conversion with or without antiarrhythmic surgery in these patients. METHODS: We conducted a retrospective study on adult patients who were born before 1994 and survived at least 3 months after a Fontan operation at Seoul National University Children's Hospital. RESULTS: We followed 160 patients over 20.9 ± 4.1 years. Sustained atrial tachycardia was identified in 51 patients, and IART was found in 41, appearing a mean 13.6 years after surgery. By the 25 year follow-up, 40% had developed IART. The incidence of IART significantly increased over time. Patients with an atriopulmonary connection (APC) (n=65) had significantly longer follow-up duration and higher incidence of IART than patients with a lateral tunnel (n=86) or extracardiac conduit Fontan (n=9). On multivariate analysis, APC, sinus node dysfunction, and nonsustained atrial tachycardia were found to be significantly associated with IART. Twenty-four patients with IART underwent Fontan conversion. Over the follow-up period, IART severity scores in the 22 patients who survived after Fontan conversion decreased significantly, and New York Heart Association functional class significantly improved. On multivariate analysis, protein losing enteropathy and ventricular dysfunction were found to be significant risk factors for mortality. CONCLUSIONS: IART was common in adult Fontan patients, and Fontan conversion with or without antiarrhythmic surgery and pacemaker placement helped to control it.

Surgical indications and results of mitral valve repair in pediatric patients with ventricular septal defects accompanied by mitral valve regurgitation.

BACKGROUND: Ventricular septal defects (VSDs) can be accompanied by mitral regurgitation (MR) owing to chronic volume overload or mitral valve (MV) abnormalities. This study investigates the surgical indications, results, and natural course of pediatric VSD patients with MR. METHODS: One hundred seven patients (median age, 4.5 months; median body weight, 6.2 kg; M:F = 46:61) who had VSD with accompanying MR between 2002 and 2012 were retrospectively analyzed and classified into two groups: group I, MV repair group; group II, no MV repair group. RESULTS: Of 107 patients, 48 patients (44.9%) required MV repair. Annular dilatation (n = 46) and anterior MV prolapse (n = 35) were the representative MV diseases in group I and their incidences were higher than in group II (n = 26 and n = 6, respectively; both p < 0.001). Increased preoperative z values of MV annulus, and mean diastolic and systolic left ventricle internal diameters were observed in group I (1.9 ± 1.0, 3.4 ± 1.3, and 3.1 ± 1.3, respectively) in comparison with group II (1.0 ± 0., 2.6 ± 1.7, and 2.3 ± 1.7, respectively; p < 0.001, p = 0.02, and p = 0.024, respectively). Posteromedial commissure annuloplasty in 23 patients and posterior annuloplasty in 19 patients were the most commonly used techniques to repair the MV; no reoperations were performed owing to MR. In group I, all patients showed an MR grade of 2 or less during the follow-up period. In group II, all patients showed the same or lower MR degree. CONCLUSIONS: Mitral valve repair in pediatric VSD patients with accompanying MR is a feasible and durable procedure with growth potential.

Safety and efficacy of the off-label use of milrinone in pediatric patients with heart diseases.

BACKGROUND AND OBJECTIVES: Milrinone is often used in children to treat acute heart failure and prevent low cardiac output syndrome after cardiac surgery. Due to the lack of studies on the long-term milrinone use in children, the objective of this study was to assess the safety and efficacy of the current patterns of milrinone use for ≥3 days in infants and children with heart diseases. SUBJECTS AND METHODS: We retrospectively reviewed the medical records of patients aged <13 years who received milrinone for ≥3 days from January 2005 to December 2012. Patients' characteristics including age, sex, height, weight, and body surface area were recorded. The following parameters were analyzed to identify the clinical application of milrinone: initial infusion rate, maintenance continuous infusion rate, total duration of milrinone therapy, and concomitantly infused inotropes. The safety of milrinone was determined based on the occurrence of adverse events such as hypotension, arrhythmia, chest pain, headache, hypokalemia, and thrombocytopenia. RESULTS: We assessed 730 admissions (684 patients) during this period. Ventricular septal defects were the most common diagnosis (42.4%) in these patients. Milrinone was primarily used after cardiac surgery in 715 admissions (97.9%). The duration of milrinone treatment varied from 3 to 64.4 days (≥7 days in 149 admissions). Ejection fraction and fractional shortening of the left ventricle improved in patients receiving milrinone after cardiac surgery. Dose reduction of milrinone due to hypotension occurred in only 4 admissions (0.5%). Although diverse arrhythmias occurred in 75 admissions (10.3%), modification of milrinone infusion to manage arrhythmia occurred in only 3 admissions (0.4%). Multivariate analysis indicated that the development of arrhythmia was not influenced by the pattern of milrinone use. CONCLUSION: Milrinone was generally administered for ≥3 days in children with heart diseases. The use of milrinone for ≥3 days was effective in preventing low cardiac output after cardiac surgery when combined with other inotropes, suggesting that milrinone could be safely employed in pediatric patients with heart diseases.