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.

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.

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 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).

Five-year experience with mini-volume priming in infants ≤5 kg: safety of significantly smaller transfusion volumes.

Reducing the cardiopulmonary bypass (CPB) priming volume in congenital cardiac surgery is important because it is associated with fewer transfusions. This retrospective study was designed to compare safety and transfusion volumes between the mini-volume priming (MP) and conventional priming (CP) methods. Between 2007 and 2012, congenital heart surgery using CPB was performed on 480 infants (≤5 kg): the MP method was used in 331 infants (MP group, 69.0%), and the CP method was used in 149 infants (CP group, 31.0%). In the MP group, narrow-caliber (3/16″) tubing was used, and the pump heads were vertically aligned to shorten the tubing lengths. The smallest possible oxygenators and hemofilters were used, and vacuum drainage was applied. Ultrafiltration was vigorously applied during CPB to avoid excessive hemodilution. The mean age and body weight of the patients were 48 ± 41 (0-306) days and 3.8 ± 0.8 (1.3-5.0) kg, respectively. The total priming and transfusion volumes during CPB were lower in the MP group than in the CP group (141 ± 24 mL vs. 292 ± 50 mL, P < 0.001, and 82 ± 40 mL vs. 162 ± 82 mL, P < 0.001, respectively). In the MP group, the smallest priming volume was 110 mL. However, there was no significant difference in the lowest hematocrit level during CPB between the two groups (22 ± 3% vs. 22 ± 3%, P = 0.724). The incidence of postoperative neurological complications was not significantly different between the MP and CP groups (1.8% vs. 2.7%, P = 0.509). After adjustment for the Risk Adjustment for Congenital Heart Surgery category, body surface area, and age, MP was not an independent risk factor of postoperative neurological complications or early mortality (P = 0.213 and P = 0.467, respectively). The MP method reduced the priming volume to approximately 140 mL without increasing the risk of morbidity or mortality in infants ≤5 kg. The total transfusion volume during CPB was reduced by 50% without compromising hematocrit levels. We recommend the use of mini-volume priming, which is a safe and effective method for reducing transfusion volumes.

Aortopexy with preoperative computed tomography and intraoperative bronchoscopy for patients with central airway obstruction after surgery for congenital heart disease: postoperative computed tomography results and clinical outcomes.

Bronchoscopy-guided aortopexy is a surgical management option for patients with central airway obstruction after congenital heart surgery. This study aimed to evaluate the usefulness of bronchoscopy-guided aortopexy based on midterm follow-up evaluation with computed tomography (CT) and clinical outcome. From January 2004 to August 2011, bronchoscopy-guided aortopexy was performed for 16 patients (median age 0.5 years, M:F = 10:6) who had central airway obstruction caused by extrinsic compression (13 in the left main bronchus, 2 in the trachea, 1 in the diffuse trachea and bronchus) after congenital heart surgery. The surgical site for aortopexy was determined by the anatomic relationship between the aorta and the compressed bronchus according to preoperative CT and intraoperative bronchoscopy. The median follow-up period was 2.3 years. The ratios of the diameter and area of stenosis at the narrowed point were estimated using pre- and postoperative CT. Almost all the patients (15/16) showed relief of their preoperative symptoms. The median extubation time was 18 h. The stenosis diameter and area ratios significantly improved, as shown by with the immediate postoperative CT (7.7-48.5%, p = 0.003; 54.8-80.5%, p = 0.006). Airway stenosis of more than 75% (p = 0.013), immediate diameter ratio improvement of <50% (p = 0.015), preoperative severe respiratory insufficiency (p = 0.038), and male sex (p = 0.024) were associated with recurrent minor respiratory susceptibility. Bronchoscopy-guided aortopexy is a safe and reliable surgical management choice for central airway obstruction after congenital heart surgery. Furthermore, airway improvement after aortopexy was maintained during the midterm follow-up evaluation, according to CT measurements.

What factors predict long-term survival and valve durability in patients with atrioventricular valve regurgitation in single-ventricle physiology?

Although significant atrioventricular valve regurgitation (AVVR) is well known for its association with increased morbidity and mortality in patients with single-ventricle physiology, there is a lack of consensus in management of AVVR. The purpose of this study was to analyze the clinical outcomes in patients receiving AVV repair or replacement. From 2001 to 2010, a total of 33 patients (25 male and 8 female) with more than moderate-degree AVVR among 160 patients who underwent staged single-ventricle palliation were included. The median follow-up duration was 6.0 years (range 0.1-14.1). Valve repair (n = 27) or valve replacement (n = 6) was performed at the initial surgery. There were six late mortalities (18.18 %): five in the repair group and one in the replacement group and seven morbidities. Among patients with valve repair, 11 were required to undergo redo-valve operations (valve repair n = 6, valve replacement n = 5) due to deteriorated valve function. Initial shunt procedure (p = 0.04) and arrhythmia (p = 0.01) were risk factors for survival. Freedom from reoperation in the valve replacement group was higher than that in the valve repair group (67.0 ± 9.7 and 44.6 ± 11.2 % at 5 and 6 years, respectively, p = 0.03). Need for early repair (p = 0.02), presence of mitral- or tricuspid-dominant AVV (p = 0.005), and male sex (p = 0.04) were risk factors for valve durability. Early valve regurgitation affects valve durability. Thus, successful repair in the early stage may improve later outcomes. Therefore, aggressive valve surgery was required and AVV replacement might be one of the options for selected patients.

Clinical significance of thrombosis in an intracardiac blind pouch after a Fontan operation.

The univentricular heart after the Fontan operation may have a blind pouch formed by the pulmonary stump or rudimentary ventricle according to the anatomy before surgery. Thrombosis in an intracardiac blind pouch of patients with a univentricular heart is a hazardous complication. Because only a few reports have described this complication, the authors evaluated the clinical significance of thrombosis in an intracardiac blind pouch of a univentricular heart. They performed a retrospective review of medical records from August 1986 to December 2007. Four patients were confirmed as having thrombosis in a pulmonary artery stump and one patient as having thrombosis in a rudimentary ventricle shown by cardiac computed tomography (CT). This represents 1.85% (5/271) of patients with ongoing regular follow-up evaluation after the Fontan operation. The median age at diagnosis was 14.2 years. Two of the five patients were taking aspirin and one patient was taking warfarin when they were identified for the development of thrombosis. None of the patients demonstrated thrombosis in the Fontan tract or venous side of the circulation. Brain magnetic resonance imaging (MRI) showed that three patients had cerebral infarction and one patient had suggestive old ischemia. Three patients with thrombus in the pulmonary stump underwent pulmonary artery stump thrombectomy and pulmonary valve obliteration. One patient with thrombus in the rudimentary ventricle underwent ventricular septal defect (VSD) closure with thrombectomy. Thrombus in a blind pouch could cause systemic thromboembolism despite little blood communication. Therefore, surgical modification of the pulmonary stump and VSD closure of the rudimentary ventricle are required to reduce the risk of later thrombus formation. Clinicians should not overlook the possibility of thrombus in a ligated pulmonary artery stump or a rudimentary ventricle after the Fontan operation, which may increase the risk of embolic stroke for patients with single-ventricle physiology.

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.

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.

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.

Symptomatic young infants with tetralogy of fallot: one-stage versus staged repair.

BACKGROUND AND AIM OF THE STUDY: Debate continues over the optimal timing for total repair of tetralogy of Fallot. Here, we report our experience with early one-stage total repair of tetralogy of Fallot. METHODS: Between January 2000 and March 2008, surgical treatment was required in 24 symptomatic patients who were diagnosed with tetralogy of Fallot at less than three months of age. Among them, total repair was performed in 13 patients (group 1) and initial palliation was done in the others (group 2). The median follow-up duration was 20 months (5 to 40 months) and 67 months (1 to 100 months) in each group. RESULTS: There was one operative death in group 2. The duration of mechanical ventilatory support, and the lengths of intensive care unit stay and hospital stay were not statistically different between both groups. The ventricular septal defects were repaired through the right atrium in 12 patients (12/13, 92.3%) of group 1 and nine patients (9/11, 81.8%) of group 2 (p = 0.576). Transannular repair was performed in six patients (6/13, 46.2%) of group 1 and 10 patients (10/11, 90.9%) of group 2 (p = 0.036). Reintervention or reoperation was required in two patients of group 1 and three of group 2 (p = 0.630). During follow-up, the pulmonary arterial growth was more prominent in group 1. CONCLUSIONS: Early one-stage total repair of tetralogy of Fallot can be performed safely without increasing the risk for reoperation or reintervention, in the short term. The pulmonary annulus is likely to be preserved when it is repaired at an early age. Early total repair may be better for the pulmonary arterial growth than staged repair.

Mid-term results of the Hancock II valve and Carpentier-Edward Perimount valve in the pulmonary portion in congenital heart disease.

BACKGROUND: As the number of cases with artificial pulmonary valve implantation increases for congenital heart disease, the number of young adults with artificial pulmonary valves has also increased. METHODS: From 2000 to 2007, 146 artificial valves, such as the Carpentier-Edward Perimount, Hancock II, Biocor, homograft and hand-made valves were implanted for pulmonary valve in 132 patients with various forms of congenital heart disease. Among them, the outcomes of the Carpentier-Edward Perimount (n=63) and the Hancock II (n=40) valves were reviewed retrospectively. The mean age at initial implantation was 12.8+/-6.6 years. The overall duration of follow up was 36.0+/-24.2 months. RESULTS: There was an early death due to right ventricular failure with intractable ventricular arrhythmia and 3 late deaths due to progressive right ventricular failure, dilated cardiomyopathy and infective endocarditis. The overall survival and re-operation free rate was 96.3% and 89.8% respectively. Eight out of 63 Carpentier-Edward patients (12.6%) underwent re-replacement at 49.2+/-25.2 months. The re-operation free rates were 97.7%, 87.7% and 50% at 1, 3 and 5 years respectively. There was no re-operation required for the 40 Hancock II patients over 18.0+/-10.8 months. There was no statistical significance in the re-operation free rates between these 2 valves (p-value=0.51). CONCLUSIONS: The overall survival rate associated with pulmonary valve bioprosthetic valve implantation was acceptable. However, the re-operation freedom rate was not satisfactory at mid-term for the Carpentier-Edward.

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.

Impact of lifetime subthreshold depression and major depression before internship on psychopathology and quality of life in Korean interns: 6 month follow-up study.

OBJECTIVE: The aim of the present study was to investigate the influences of lifetime subthreshold depression and major depression before internship on the changes in psychopathology and subjective quality of life during internship. METHOD: A total of 151 interns at Seoul National University Hospital completed questionnaires including World Health Organization Quality of Life Assessment Instrument, Brief Version (WHOQOL-BREF), Symptom Checklist-90-Revised (SCL-90-R), and Beck Depression Inventory (BDI) twice at an interval of 6 months. Their lifetime diagnosis of depression before internship was made using the Korean version of Mini International Neuropsychiatric Interview (MINI), Modified Form for Self-Administration. RESULTS: Significant changes over time were detected including total score in the BDI and the Somatization, Depression, Anxiety, and Phobic Anxiety dimension scores in the SCL-90-R as well as all four domain scores in WHOQOL-BREF. In addition, in all scores except Somatization dimension score, group difference existed. Post-hoc comparisons showed that interns without lifetime depression were significantly different from those with lifetime subthreshold depression and those with lifetime major depression. CONCLUSIONS: Even a subthreshold-level, lifetime depression before internship has an adverse effect on the psychopathology and the subjective quality of life during internship. Therefore, earlier detection, monitoring, and management of this population would be needed throughout the intern year and beyond.

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.

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.