Second harvest of Congenital Heart Surgery Database in Türkiye: Current outcomes.

Background: This second harvest of the Congenital Heart Surgery Database intended to compare current results with international databases. Methods: This retrospective study examined a total of 4007 congenital heart surgery procedures from 15 centers in the Congenital Heart Surgery Database between January 2018 and January 2023. International diagnostic and procedural codes were used for data entry. STAT (Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery) mortality scores and categories were used for comparison of the data. Surgical priority status was modified from American Society of Anesthesiologist guidelines. Centers that sent more than 5 cases to the database were included to the study. Results: Cardiopulmonary bypass and cardioplegic arrest were performed in 2,983 (74.4%) procedures. General risk factors were present in 22.6% of the patients, such as genetic anomaly, syndrome, or prematurity. Overall, 18.9% of the patients had preoperative risk factors (e.g., mechanical ventilation, renal failure, and sepsis). Of the procedures, 610 (15.2%) were performed on neonates, 1,450 (36.2%) on infants, 1,803 (45%) on children, and 144 (3.6%) on adults. The operative timing was elective in 56.5% of the patients, 34.4% were urgent, 8% were emergent, and 1.1% were rescue procedures. Extracorporeal membrane oxygenation support was used in 163 (4%) patients, with a 34.3% survival rate. Overall mortality in this series was 6.7% (n=271). Risk for mortality was higher in patients with general risk factors, such as prematurity, low birth weight neonates, and heterotaxy syndrome. Mortality for patients with preoperative mechanical ventilation was 17.5%. Pulmonary hypertension and preoperative circulatory shock had 11.6% and 10% mortality rates, respectively. Mortality for patients who had no preoperative risk factor was 3.9%. Neonates had the highest mortality rate (20.5%). Intensive care unit and hospital stay time for neonates (median of 17.8 days and 24.8 days, respectively) were also higher than the other age groups. Infants had 6.2% mortality. Hospital mortality was 2.8% for children and 3.5% for adults. Mortality rate was 2.8% for elective cases. Observed mortality rates were higher than expected in the fourth and fifth categories of the STAT system (observed, 14.8% and 51.9%; expected, 9.9% and 23.1%; respectively). Conclusion: For the first time, outcomes of congenital heart surgery in Türkiye could be compared to the current world experience with this multicenter database study. Increased mortality rate of neonatal and complex heart operations could be delineated as areas that need improvement. The Congenital Heart Surgery Database has great potential for quality improvement of congenital heart surgery in Türkiye. In the long term, participation of more centers in the database may allow more accurate risk adjustment.

Clinical effects of major aortopulmonary collateral arteries in term neonates diagnosed with transposition of the great arteries.

BACKGROUND: Transposition of the great arteries is a severe CHD that affects term neonates. The presence of major aortopulmonary collateral arteries in neonatal transposition of the great arteries patients is rare. This study investigated the clinical and haemodynamic implications of the presence of major aortopulmonary collateral arteries in neonatal transposition of the great arteries patients who underwent an arterial switch operation. MATERIALS AND METHODS: The study was a retrospective analysis conducted on neonates diagnosed with transposition of the great arteries who underwent arterial switch operation within the period from 1 May 2020 to 1 January 2023 at two high-patient-volume paediatric cardiac surgery centres in Turkey. The patients' demographic characteristics, echocardiographic features, and clinical data were analysed. Additionally, the possible clinical effects of the presence of major aortopulmonary collateral arteries were statistically evaluated. RESULTS: Two hundred cases of neonatal transposition of the great arteries were included in this study, with 55% of the cases male. All the patients underwent arterial switch operation. The median age at the time of arterial switch operation was 5 days (interquartile range 3-7), with a median weight of 3,100 g (interquartile range 2,900-3,400). The median pre-operative saturation level was 76% (interquartile range 70-82%). Prior to arterial switch operation, 32 patients underwent balloon atrial septostomy.In all the patients, the interatrial septum was checked to determine if the atrial septum was intact. A patent foramen ovale (≤ 3 mm) was found in 112 patients, and a non-restrictive atrial septal defect (> 3 mm) was found in 88. Forty-eight patients had ventricular septal defects, and 72 had coronary anomalies. Major aortopulmonary collateral arteries were found in 4 patients pre-operatively and in 12 patients after arterial switch operation (echocardiography, n = 8; angiography, n = 4). Of the patients with post-operative detection of cumulative number of major aortopulmonary collateral arteries were on post-operative day 1 in 2 patients, on post-operative day 3 in 5 patients, on post-operative day 7 in 6 patients, and on post-operative day 14 in 11 patients.Transcatheter closure was performed in 3 cases due to recurrent extubation failure. Major aortopulmonary collateral artery shrinkage was observed in one case under medical treatment. The length of paediatric cardiac intensive care unit stay (10 days versus 8 days; p < 0.005), mechanical ventilator time (4 days versus 2 days; p = 0.02), and inotrope use time (5 days versus 3 days; p = 0.04) were higher in the major aortopulmonary collateral artery cases than patients without major aortopulmonary collateral artery. CONCLUSION: Major aortopulmonary collateral arteries are frequent in transposition of the great arteries patients and may have clinical effects. The presence of major aortopulmonary collateral arteries should be investigated in patients who do not have a favourable post-operative course after arterial switch operation.

Situs inversus with dextrocardia and transposition of great arteries.

Transposition of the great arteries with left ventricle outflow tract obstruction in combination with situs inversus totalis and dextrocardia is a very rare anomaly and carries high morbidity and mortality. Only few cases have been reported with this anomaly. We describe a 21-day of infant girl with transposition of the great arteries and mirror image dextrocardia and pulmonary stenosis who underwent successful neonatal arterial switch operation and left ventricle outflow tract obstruction resection following PDA stent implantation.

Predictors of prolonged pleural effusion after Fontan operation.

Prolonged pleural effusion is a fairly common condition which has considerable impact on complicated and longer hospital stays after Fontan surgery. Identifying the patient population prone to have pleural effusions is still seeking for an answer. This study is to determine the variables that may predict prolonged pleural effusion according to the data of 69 patients who underwent Fontan operation between June 2018 and December 2020 and survived to date. Prolonged pleural effusion was defined as the need for a chest tube for more than 7 days. Two patient groups, with and without prolonged effusion, were compared in terms of pre-, peri-, and post-operative variables. The patients were subdivided into "high-risk" and "low-risk" groups based on the pre-operative catheterisation data. The most frequent main diagnosis was tricuspid atresia (n: 13, 19%). Among 69 patients, 28 (40%) had prolonged pleural effusion whereas 11 (16%) had effusions that lasted longer than 14 days. Ten patients among prolonged effusion group (35%) had pulmonary atresia coexistent with the main diagnosis. Fontan operation was performed in 6 patients (8.7%) over the age of 10, and 4 of these patients (67%) had prolonged pleural effusion. Among numerous variables, statistical significance between the two groups was achieved in pre-operative mean pulmonary artery pressure, post-operative albumin, C-reactive protein levels, length of hospital stay, duration of chest tube drainage, and amount of effusion per day. Early recognition and treatment strategies with routine medical protocol use remain to be the cornerstone for the management of post-operative prolonged pleural effusions after Fontan surgery.

Biventricular repair in an infant with transposition of the great arteries and straddling of the tricuspid valve.

Biventricular repair is challenging in patients with transposition of the great arteries and straddling of the atrioventricular valves. Biventricular repair is the preferred option because of its anatomical and physiological advantages. However, in cases where biventricular repair carries operative risks that are too high or cases with unsuitable intracardiac anatomy, univentricular heart repair may have to be chosen. We report a five-month-old male patient with transposition of the great arteries, an inlet ventricular septal defect and anomalous coronary anatomy who had previously undergone a pulmonary banding operation and balloon atrial septostomy. Successful biventricular repair was performed while the patient had straddling of the tricuspid valve.

Comparison of the effects of conventional method and primary sutureless techniques on early postoperative rhythm problems in patients with total abnormal pulmonary venous return anomaly.

BACKGROUND: Total abnormal pulmonary venous return anomaly is a CHD characterised by abnormal pulmonary venous flow directed to the right atrium. In this study, we aimed to compare the effects of these techniques on early rhythm problems in total abnormal pulmonary venous return anomaly cases operated with conventional or primary sutureless techniques. METHOD: Seventy consecutive cases (median age 1 month, median weight 4 kg) who underwent total abnormal pulmonary venous return anomaly repair with conventional or primary sutureless technique between May 1 2020 and May 1 2022 were evaluated. The rate, diagnosis, and possible risk factors of postoperative arrhythmias were investigated. The results were evaluated statistically. RESULTS: When the total abnormal pulmonary venous return anomaly subgroup of 70 cases was evaluated, 40 cases were supracardiac, 18 cases were infracardiac, 7 cases were cardiac, and 5 cases were mixed type. Twenty-eight (40%) cases had a pulmonary venous obstruction. Primary sutureless technique (57%, supracardiac n = 24, mixed = 3, infracardiac = 13) was used in 40 patients. Median cardiopulmonary bypass time (110 versus 95 minutes) and median aortic clamp time (70 versus 60 minutes), median peak lactate (4.7 versus 4.8 mmol/l) in the first 72 hours, and median peak vasoactive inotropic score in the first 72 hours of the primary sutureless and conventional technique used cases value (8 versus 10) were similar. The total incidence of arrhythmias in the conventional group was significantly higher than in the primary sutureless group (46.7% versus 22.5%, p = 0.04). Supraventricular early beat was observed in 3 (7.5%), sinus tachycardia was seen in 6 (15%), junctional ectopic tachycardia was seen in 1 (2.5%), intra-atrial reentry tachycardia was seen in 1 (2.5%), usual supraventricular tachyarrhythmia was seen in 2 cases (5%) in the primary sutureless group. In the conventional group, supraventricular early beat was observed in six of the cases (20%), sinus tachycardia in five (16.7%), junctional ectopic tachycardia in four (13.3%), intra-atrial reentry tachycardia (10%) in three, and supraventricular tachyarrhythmia in seven cases (23.3%). In the first 30 days, there was a similar mortality rate (10% versus 10%), with four patients in the primary sutureless group and three in the conventional group. The median follow-up period of the cases was 8 months (interquartile range (IQR) 6-10 months). In the follow-up, arrhythmias were detected in two cases (one supraventricular tachyarrhythmia and one intra-atrial reentry tachycardia) in the primary sutureless group and three cases (two supraventricular tachyarrhythmia, one intra-atrial reentry tachycardia) in the conventional technique. All cases were converted to normal sinus rhythm with cardioversion and combined antiarrhythmic therapy. CONCLUSION: Different arrhythmias can be observed in the early period in patients with operated total abnormal pulmonary venous return anomaly. Although a higher rate of rhythm problems was observed in the early period in the conventional method compared to the primary sutureless technique, no significant effect was found on mortality and morbidity between the groups.

Clinical Characteristics and Mid-term Follow-up in Children with Isolated Complete Atrioventricular Block.

BACKGROUND: Isolated complete atrioventricular block is a rare disease often associated with maternal autoantibodies. This study aimed to present the midterm data of patients at our clinic diagnosed with isolated complete atrioventricular block. METHODS: We evaluated 108 patients diagnosed with isolated complete atrioventricular block. Demographic data of the patients, electrocardiography, echocardiography, 24-hour Holter monitoring data, and follow-up and complications of the patients who underwent pacemaker implantation were evaluated retrospectively. RESULTS: The mean age of the patients at diagnosis was 5.51 ± 5.05 years. At the time of diagnosis, 74.8% of the patients had no symptoms associated with complete atrioventricular block. The most common symptom was fatigue. Pacemaker implantation was needed in 88 (81.4%) patients during follow-up. Significant bradycardia was the most common pacemaker implantation indication. The mean battery life was 5.41 ± 2.65 years. The battery replacement-free period of 68 patients who underwent pacemaker implantation and continued their follow-up was 4.18 ± 2.89 (0.1-10) years. Pacemaker-related complications developed in 8 patients during follow-up. Left ventricular dysfunction developed (dyssynchrony induced) in 3 patients at follow-up, and all were paced from the right ventricular anterior wall. Those patients underwent cardiac resynchronization therapy and their left ventricular dysfunction improved. CONCLUSION: Isolated complete atrioventricular block is a rare disease requiring careful clinical follow-up. Patients are often asymptomatic and significant bradycardia is the most common indication for pacemaker implantation. Left ventricular dysfunction is an important cause of morbidity, especially in patients with right ventricular anterior wall pacing. Physicians should be aware of left ventricular dysfunction during follow-up. Cardiac resynchronization therapy should be considered as a treatment option for left ventricular dysfunction.

Isolated coarctation repair through a left thoracotomy in children.

INTRODUCTION: Isolated aortic coarctation performed through a left thoracotomy resection and end-to-end anastomosis results in low mortality and morbidity rates. Recoarctation and late hypertension are among the most important complications after such repairs. In this study, we reviewed the results of children who underwent left-side thoracotomy to correct an isolated aortic coarctation. METHOD: A consecutive sample of 90 patients who underwent resection and extended end-to-end anastomosis through a left-side thoracotomy in our centre between 2011 and 2021 was retrospectively analysed. The patients' preoperative characteristics, operative data, and post-operative early and long-term results were examined. RESULTS: All patients underwent resection and extended end-to-end anastomosis. A pulmonary artery band was applied simultaneously to three (3.3%) patients, and an aberrant right subclavian artery division was applied to one (1.1%) patient. The mean cross-clamp time was 29.13 ± 6.97 minutes. Two (2.2%) patients required reoperation in the early period. Mortality was observed in one (1.1%) patient in the early period. Eight (8.8%) patients developed recoarctation, of whom four (4.4%) underwent reoperation and four (4.4%) underwent balloon angioplasty. Twenty-two (26.8%) patients received follow-up antihypertensive treatment. The mean follow-up period was 41.3 ± 22.8 months. No mortality was observed in the late period. CONCLUSION: Isolated coarctation is successfully treated with left-side thoracotomy resection and an extended end-to-end anastomosis technique with low mortality, morbidity, and low long-term recoarctation rates. Long-term follow-up is required due to the risks of early and late post-operative recoarctation, which requires reintervention.

Effect of aortic arch surgery in newborns' cerebral and gastrointestinal hemodynamics: evaluation by Doppler ultrasonography.

AIM: In this study, we investigated changes in newborns' cerebral and intestinal blood flow who had undergone aortic arch surgery. METHOD: This study was carried out prospectively as a preliminary study in patients younger than 30 days at the time of aortic arch reconstruction between 1 August and 1 December, 2019. Cerebral and gastrointestinal hemodynamics were evaluated with Doppler USG before and 7 days after the operation. The middle cerebral artery (MCA) and celiac artery (CA) were used as measurement sites. Patients' peak systolic velocity (PSV), mean systolic velocity (MV), end diastolic velocity (EDV), resistive index (RI) and pulsatility index (PI) were evaluated. RESULTS: A total of 16 patients enrolled in the study. The patients' median weight was 3.2 kg (2.7-4.5 kg), and age was 21 days (7-30 days). Six of them were female. Seven of the patients who underwent arcus reconstruction had an additional ventricular septal defect. The preoperative Doppler USG values of patients' were as follows: for MCA, the mean PSV was 37 ± 12 cm/s, EDV 12 ± 5 cm/s, MV 22 ± 19 cm/s, RI 0.70 ± 0.03, PI 1.24 ± 0.23, and for CA mean PSV was 67 ± 32 cm/s, EDV 29 ± 14 cm/s, MV 24 ± 9 cm/s, RI 0.79 ± 0.27, and PI 1.63 ± 0.89. Doppler USG values of patients' at the postoperative seventh day were as follows: for the MCA, mean PSV 41 ± 13 cm/s, EDV 13 ± 4 cm/s, MV 25 ± 10 cm/s, RI 0.64 ± 0.05, PI 1.23 ± 0.20, and for the CA mean PSV 70.5 ± 34 cm/s, EDV 32 ± 16 cm/s, MV 26 ± 8 cm/s, RI 0.75 ± 0.1, and PI 1.60 ± 0.38. There was a significant decrease in RI of both MCA and CA on the postoperative 7th day compared to the preoperative period (p < 0.05). CONCLUSION: In newborns, there are significant changes in cerebral and intestinal blood flows after aortic arch surgery. RI decreased significantly, especially in the CA and MCA.

A Quite Rare Association: Levo-Malposition of the Great Arteries with Left Juxtaposition of the Atrial Appendages in "Double Outlet Right Ventricle"

Abstract Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. This case report underlines the rarity of this anatomical combination and its clinical significance along with the surgical management in an infant.

Comparison of echocardiography and 320-row multidetector computed tomography for the diagnosis of congenital heart disease in children.

INTRODUCTION: Echocardiography (echo) is the primary non-invasive imaging modality for the assessment of congenital heart disease (CHD). Computed tomography angiography (CTA) also has potential to examine the anatomy of complex heart anomalies as well as extracardiac involvement. OBJECTIVES: The aim of this study is to determine the impact of new CTA technology in the diagnosis of CHD and to compare echo and CTA in terms of diagnostic accuracy. METHODS: Forty-five patients who underwent preoperative echo and CTA assessment in the intensive care unit were included in this study. The results were assessed for three main types of CHD (cardiac malformations, cardiac-major vessel connections and major vessels). The main groups were also divided into subgroups according to surgical features in order to assess them more objectively. Imaging methods were compared for diagnostic accuracy, sensitivity and specificity, while surgical findings were accepted as the gold standard. RESULTS: Patients' median age and weight were two months (three days-eight years) and 12 kg (2.5-60 kg), respectively. In 45 operated cases, 205 subgroup malformations were assessed. Diagnostic accuracy was significantly greater in echo (echo vs. CTA: 98.4% and 96.2% [chi-square=6.4, p=0.011]). During surgery, 84 cardiac malformations (echo vs. CTA: 97.4% and 95.1% [chi-square=4.9, p=0.03]), 47 cardiac-major vessel connections (echo vs. CTA: 98.3% and 95.4% [chi-square=7.5, p=0.03]), and 74 major vessel malformations (echo vs. CTA: 96% and 98% [chi-square=1.8, p=0.48]) were confirmed. CONCLUSION: Echocardiography and CTA are imaging methods with high diagnostic accuracy in children with CHD. The use of echocardiography together with CTA, especially for the visualization of extracardiac anatomy, provides additional information for clinicians.

A Quite Rare Association: Levo-Malposition of the Great Arteries with Left Juxtaposition of the Atrial Appendages in "Double Outlet Right Ventricle".

Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. This case report underlines the rarity of this anatomical combination and its clinical significance along with the surgical management in an infant.

Single-center experience with routine clinical use of 3D technologies in surgical planning for pediatric patients with complex congenital heart disease.

PURPOSE: This study was planned to assess the application of three-dimensional (3D) cardiac modeling in preoperative evaluation for complex congenital heart surgeries. METHODS: From July 2015 to September 2019, 18 children diagnosed with complex congenital heart diseases (CHDs) were enrolled in this study (double outlet right ventricle in nine patients, complex types of transposition of the great arteries in six patients, congenitally corrected transposition of the great arteries in two patients, and univentricular heart in one patient). The patients' age ranged from 7 months to 19 years (median age, 14 months). Before the operation, 3D patient-specific cardiac models were created based on computed tomography (CT) data. Using each patient's data, a virtual computer model (3D mesh) and stereolithographic (SLA) file that would be printed as a 3D model were generated. These 3D cardiac models were used to gather additional data about cardiac anatomy for presurgical decision-making. RESULTS: All 18 patients successfully underwent surgeries, and there were no mortalities. The 3D patient-specific cardiac models led to a change from the initial surgical plans in 6 of 18 cases (33%), and biventricular repair was considered feasible. Moreover, the models helped to modify the planned biventricular repair in five cases, for left ventricular outflow tract obstruction removal and ventricular septal defect enlargement. 3D cardiac models enable pediatric cardiologists to better understand the spatial relationships between the ventricular septal defect and great vessels, and they help surgeons identify risk structures more clearly for detailed planning of surgery. There was a strong correlation between the models of the patients and the anatomy encountered during the operation. CONCLUSION: 3D cardiac models accurately reveal the patient's anatomy in detail and are therefore beneficial for planning surgery in patients with complex intracardiac anatomy.

Surgical treatment of Ebstein anomaly in pediatric patients: A 10-year single-center study.

OBJECTIVE: We report the early and long-term results of the strategies and surgical methods used in our center to treat pediatric patients who underwent surgical intervention to correct Ebstein anomaly (EA) in our center. MATERIALS AND METHODS: In our study, a consecutive sample of 29 patients who underwent surgery for EA between February 2011 and February 2020 in our center were evaluated retrospectively. RESULTS: The 29 patients underwent a total of 40 operations. Univentricular repair was performed in 5 (17.2%), 1.5 ventricular repair in 5 (17.2%), and biventricular repair in the remaining 19 (65.5%) patients. Cone reconstruction (CR) was performed in eight (27.5%), non-Cone tricuspid valve (TV) repair technique in five (17.2%), ring annuloplasty in two (6.9%), and TV replacement in two patients (6.9%) who had undergone biventricular repair. In two patients (6.9%), only close an atrial septal defect. Two (6.9%) patients underwent a second operation for advanced tricuspid regurgitation (TR) in the early period. None of the 15 patients who underwent CR and TV plasty had moderate or advanced TR before discharge. Early mortality was seen in 1 (3.4%) patient. The mean follow-up period of the patients was 48.4±27.6 months. Three (10.7%) of the patients who were discharged after their first operation later underwent a second operation for TV regurgitation in the long term. No mortality was observed in any patient during long-term follow-up. CONCLUSION: Surgical treatment of EA is difficult, but its overall results are good. The anatomical repair rate is lower in neonatal and infant patients requiring surgery, but most of these patients underwent biventricular repair. Our long-term results demonstrated an acceptable survival rate, low mortality in the early postoperative period, and low incidence of re-intervention and morbidity.

First Harvest of Pediatric and Congenital Heart Surgery Multicenter Database in Turkey: Novel Application of Real-Time Online Reporting.

OBJECTIVES: A new congenital heart surgery database (CKCV) with real-time online reporting function was recently developed in Turkey. All standard international parameters were used, but Aristotle Comprehensive Complexity score was modified. In this study, the first analysis of the CKCV Database is reported. METHODS: The CKCV Database included 2307 procedures from 12 centers between January 2018 and March 2020. All parameters, including 10 real-time online reports, which represent the number of centers, number and mortality rates of all procedures, number of extracorporeal membrane oxygenation (ECMO) and results, details of postoperative complications, age-group statistics, analysis for priority status, mean intensive care and hospital stay durations of the procedures, results of Aristotle Basic, Modified Aristotle Comprehensive (MACC) and Society of Thoracic Surgeons-European Association (STAT) Score Categories, comparison of centers were analyzed. RESULTS: Most common 10 procedures were ventricular septal defect (VSD) repair (n = 273), tetralogy of Fallot (TOF) repair (n = 243), atrial septal defect (ASD) repair (n = 181), complete AVSD repair (n = 95), cavopulmonary anastomosis (n = 81), systemic to pulmonary shunt (n = 79), modified Fontan (n = 71), subaortic resection, (n = 66) PA banding (n = 66), and arterial switch operation (n = 66). Cardiopulmonary bypass was used in 84.6% of the procedures. Overall mortality rate was 6.0%. A total of 618 major and 570 minor complications were observed in 333 and 412 patients, respectively. According to six MACC categories, number of the patients and mortality rates were I (293; 0.3%); II (713; 1.4%); III (601; 3.3%); IV (607; 12%); V (84; 35.7%); and VI (9; 55.6%), respectively. Analysis of five STAT Categories showed 0.7, 3.8, 5.4, 14.9, and 54.7% mortality rates. CONCLUSIONS: CKCV Database has a great potential for nationwide quality improvement studies. Users could instantly analyze and compare their results to national and international aggregate data using a real-time online reporting function. This is the first multicenter congenital database study in Turkey.

Clinical and genetic characteristics and course of congenital long QT syndrome in children: A nine-year single-center experience.

OBJECTIVE: Long QT syndrome (LQTS) is an inherited primary arrhythmia syndrome associated with life-threatening ventricular arrhythmias and sudden death. This study aimed to report the clinical and genetic characteristics and outcomes of children diagnosed as having LQTS in a tertiary pediatric cardiology center in Turkey. METHODS: This was a retrospective review of pediatric patients diagnosed as having LQTS at our center from January 2011 to April 2020. RESULTS: A total of 145 patients (76 males) were included, with a mean age of 9.2±4.5 years and a mean weight of 35.7±18.5 kg; 38 (26.2%) were identified as having LQTS during family screening, whereas a significant proportion of patients were asymptomatic at presentation, 15 patients (10.3%) were diagnosed after previous cardiac arrest, and 26 patients (18%) had syncope. The mean Schwartz score was 4.5 points (range, 3-7.5 points). Furthermore, 107 patients (82%) were confirmed to have a pathogenic mutation for LQTS genes. All patients received beta-blockers. Implantable cardioverter-defibrillator insertion was performed in 34 patients (23.4%). Left or bilateral cardiac sympathetic denervation was performed in 9 patients (6.2%). Median follow-up time was 35.6±25.8 months. Five (3.4%) patients died during the follow-up. Statistical analyses of risk factors for major cardiac events revealed that the QTc was >500 ms and that T wave alternans, high Schwartz score, and Jervell and Lange-Nielsen syndrome were strong and significant predictors of cardiac events. CONCLUSION: LQTS has a variety of clinical manifestations. Patients' symptoms ranged between asymptomatic and sudden cardiac death (SCD). By raising the awareness of physicians regarding the disease, SCD might be prevented in the early period.