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Outcomes of the Warden Procedure for Anomalous Pulmonary Venous Return to the Superior Vena Cava: A 17-Year Experience

Lim SC, Kwak JG, Cho S, Min J, Lee S, Kwon HW, Kim WH

Background: Surgical repair of partial anomalous pulmonary venous return (PAPVR) to the superior vena cava (SVC) using the Warden procedure has favorable outcomes. However, there remain some concerns after the...
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Association between Physical Activity and Chronotropic Incompetence in Patients with Complex Congenital Heart Disease

Kim HJ, Yoon JK, Kim CH, Kim SH, Franklin BA, Jae SY

Purpose: Although chronotropic incompetence (CI) is common in patients with complex congenital heart disease (CHD) and is associated with adverse cardiovascular outcomes, few data are available regarding modifiable predictors of...
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Neurodevelopmental outcomes and comorbidities of children with congenital muscular torticollis: evaluation using the National Health Screening Program for Infants and Children database

Kim OH, Lee SW, Ha EK, Kim JH, Jo YH, Rhie S, Han MY, Chae KY

Background: Congenital muscular torticollis (CMT) is a common musculoskeletal disorder occurring at birth or in infancy. Purpose: This study aimed to investigate the risk of comorbidities in CMT and explore the...
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Perinatal Prognostic Factors for Congenital Diaphragmatic Hernia: A Korean Single-Center Study

Jeon S, Jeong MH, Jeong SH, Park SJ, Lee N, Bae MH, Park KH, Byun SY, Kim SH, Cho YH, Kim C, Han YM

Purpose: This study aimed to identify prognostic factors based on treatment outcomes for congenital diaphragmatic hernia (CDH) at a single-center and to identify factors that may improve these outcomes. Methods: Thirty-five...
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Thyroid Dyshormonogenesis Due to Dual Oxidase Maturation Factor 2 Mutation as Non-Transient Status of Hypothyroidism

Lee J, Kim Sg, Oh A, Han HS

Dual oxidase maturation factor 2 (DUOXA2) is necessary for the enzymatic activity of dual oxidase 2 (DUOX2) to generate hydrogen peroxide production during thyroid hormone synthesis. We describe two Korean...
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Genetics and genomics of bone marrow failure syndrome

Kim HY, Kim HJ, Kim SH

Inherited bone marrow failure syndrome (IBMFS) is a group of clinically heterogeneous disorders characterized by significant hematological cytopenias of one or more hematopoietic cell lineages and is associated with an...
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Postcardiotomy Extracorporeal Membrane Oxygenation Support in Patients with Congenital Heart Disease

Joo S, Cho S, Lee JH, Min J, Kwon HW, Kwak JG, Kim WH

Background: This study investigated mortality and morbidity in patients requiring postcardiotomy extracorporeal membrane oxygenation (ECMO) support after operations for congenital heart disease (CHD). Methods: CHD patients requiring postoperative ECMO support between...
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Isolated Anomalous Right Coronary Artery From the Pulmonary Artery in the Elderly: The Role of Functional Echocardiographic Assessment

Vu PN, Pham TTM, Nguyen LPT, Nguyen KNN, Pham VN

A 65-year-old male was admitted with atypical fatigue and mild pedal edema. He was diagnosed with anomalous origin of the right coronary artery from the pulmonary artery on transthoracic echocardiography...
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Acute Myocardial Infarction in Patients With Single Coronary Artery: A Case Report

Thi HN, Ngoc TN, Khoi VN, Thuy LLT, Van HHT, Bao NP, Dang LV

A single coronary artery (SCA) is a rare congenital anomaly. In most cases, it is an incidental finding on coronary angiography and has no clinical significance. However, it can cause...
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Global and Regional Right Ventricular Function Investigation by 2D Speckle Tracking Echocardiography in Congenital Heart Disease Patients With Pulmonary Arterial Hypertension

Pham TT, Nguyen HTT

Objective: The aim of our study was to evaluate the global and regional free wall longitudinal strains of the right ventricle (RV) to detect RV dysfunction in congenital heart disease...
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Congenital web of the common bile duct combined with multiple intrahepatic duct stricture: a case report of successful radiological intervention

Lim H, Hwang S, Ko GY, Han H

Congenital web formations are extremely rare anomalies of the extrahepatic biliary tree. We herein report a case of common bile duct septum combined with multiple intrahepatic bile duct strictures in...
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Neonatal Duodenal Obstruction Associated with Situs Inversus Totalis: A Case Report

Seo J, Kim YJ, Ho YH

Situs inversus totalis is a mirror reversal of the normal asymmetrical arrangement of the viscera. The incidence is approximately 0.3 in 10,000 live births. Situs inversus totalis is commonly seen...
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Diazoxide-Associated Pulmonary Hyperten sion in an Infant with Beck with-Wiedemann Syndrome: A Case Report

Ryu JH, Kang D, Lee J, Ko JM, Jung YH, Kim JH, Choi CW

Diazoxide, an ATP-sensitive potassium channel opener, has been used as 1st line treatment for hyperinsulinemic hypoglycemia. As congenital hyperinsulinism is the most common cause of hyperinsulinemic hypoglycemia, diazoxide is more...
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Pregnancy and neonatal outcomes after periconceptional exposure to isotretinoin in Koreans

Cha EH, Kim N, Kwak HS, Han HJ, Joo SH, Choi JS, Chun KC, Kim YA, Koh JW, Han JY

Objective Isotretinoin should not be used during pregnancy because of the risk of birth defects. Most pregnant women exposed to isotretinoin choose voluntary pregnancy termination due to concerns about birth defects....
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Erectile Dysfunction in Men With Adult Congenital Heart Disease: A Prevalent but Neglected Issue

Background and Objectives: For adult men with congenital heart disease (ACHD), data on erectile dysfunction (ED) is limited. We aimed to assess the frequency of ED, its role in patient-physician...
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Epidemiology and Long-Term Adverse Outcomes in Korean Patients with Congenital Adrenal Hyperplasia: A Nationwide Study

Kim JH, Choi S, Lee YA, Lee J, Kim SG

Background: Previous studies on the epidemiology and complications of congenital adrenal hyperplasia (CAH) were conducted in Western countries and in children/adolescents. We aimed to explore the epidemiology of CAH, as...
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Adrenal Morphology as an Indicator of Long-Term Disease Control in Adults with Classic 21-Hydroxylase Deficiency

Kim TM, Kim JH, Jang HN, Choi MH, Cho JY, Kim SY

Background: Monitoring adults with classical 21-hydroxylase deficiency (21OHD) is challenging due to variation in clinical and laboratory settings. Moreover, guidelines for adrenal imaging in 21OHD are not yet available. We...
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Chemical Pleurodesis Using a Viscum album Extract in an Infant with Postoperative Chylothorax: A Case Report

Yun T, Kwon HW, Park S, Kim WH

Chylothorax after thoracic surgery is a rare complication, and treatment for refractory chylothorax is challenging. We report a case of chylothorax after cardiothoracic surgery in an infant after failure of...
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Cochlear Implants for Patients With Common Cavity Deformities and the Impact of Electrode Positioning

Bae SH, Choi J, Choi JY

Objectives. Common cavity deformity is a rare congenital bony labyrinth malformation associated with profound hearing loss. Cochlear implants are widely used for hearing rehabilitation for common cavity deformities; however, the...
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Coronary Cameral Fistula and Anomalous Right Coronary Artery: A Case Report

Ng PP, Chow BCK, Cheung SCW, Ng MY

Coronary artery anomalies are uncommon with variable clinical significance. We present a case of 49-year-old gentleman with atypical chest pain, who was found to have single left coronary artery, anomalous...
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