• Clinical and Experimental Pediatrics
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• v.56 no.3
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• pp.125-129
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• 2013

Purpose: The use of implantable cardioverter defibrillators (ICDs) to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease. Methods: This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011. Results: Fifteen patients underwent ICD implantation. Their mean age at the time of implantation was $14.5{\pm}5.4$ years (range, 2 to 22 years). The follow-up duration was $28.9{\pm}20.4$ months. The cause of ICD implantation was cardiac arrest in 7, sustained ventricular tachycardia in 6, and syncope in 2 patients. The underlying disorders were as follows: ionic channelopathy in 6 patients (long QT type 3 in 4, catecholaminergic polymorphic ventricular tachycardia [CPVT] in 1, and J wave syndrome in 1), cardiomyopathy in 5 patients, and postoperative congenital heart disease in 4 patients. ICD coils were implanted in the pericardial space in 2 children (ages 2 and 6 years). Five patients received appropriate ICD shock therapy, and 2 patients received inappropriate shocks due to supraventricular tachycardia. During follow-up, 2 patients required lead dysfunction-related revision. One patient with CPVT suffered from an ICD storm that was resolved using sympathetic denervation surgery. Conclusion: The overall ICD outcome was acceptable in most pediatric patients. Early diagnosis and timely ICD implantation are recommended for preventing sudden death in high-risk children and patients with congenital heart disease.

• Journal of the Korean Burn Society
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• v.24 no.2
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• pp.68-73
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• 2021

Purpose: A large defect by fourth-degree burns in the upper limb requires flap reconstruction. Since severe vascular damage and decrease in blood circulation after vascular anastomosis can occur in defects caused by fourth-degree burns. Because of the disadvantages, it is difficult to apply free flap surgery to fourth-degree burns. We reconstructed a upper extremity using the pedicled Latissimus Dorsi (LD) flap in two stages. The purpose of our study is to review our experience and suggest two-staged pedicled Latissimus Dorsi (LD) flap in fourth-degree burns of upper extremities. Methods: A retrospective review was performed from 2016 to 2019, on a total of 12 fourth-degree burn patients undergone two-staged pedicled LD flap surgery as reconstruction of upper extremities in our hospital. We reviewed the location of the injury, etiology, TBSA (%), size of burns requiring flap surgery, period from 1st surgery to secondary division surgery, complications. Results: Using two-staged LD flap as a primary reconstruction, the outcome is satisfactory. This flap preserves the elbow joint and maintains the length of the forearm. We obtain low donor-site morbidity, simplicity and a small incision in the donor site. Conclusion: Using two-staged LD flap in fourth-degree burns of upper extremity is effective, such as preserving elbow joint and maintaining the length of the forearm. Successful reconstruction was achieved with excellent cosmetic results with reducing a postoperative scar, donor-site morbidity. Due to these advantages, two-staged pedicled LD flap can be an optimal option for reconstruction of fourth-degree burns in the upper limb.

• Journal of Chest Surgery
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• v.33 no.5
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• pp.419-421
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• 2000

Noonan syndrome is characterized by typical facies, congenital heart defect, and some clinical features similar to Turner syndrome, but with normal chromosomes. The most commonly associated cardiac defects are pulmonary valvular stenosis and strial septal defect. We experienced a case of Nonan syndrome associated with pulmonay valve stenosis with double-chambered right ventricle and atrial septal defect and cryptorchidism. Pulmonary valvotomy was done through transannular incision. Hypertrophied muscle bundles were excised. Atrial septal defect was closed directly. RVOT was reconstructed with pericardial transannular patch. Orchiopexy was performed simultaneously without any problem.

• Journal of Chest Surgery
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• v.48 no.5
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• pp.351-354
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• 2015

A five-month-old boy who had undergone previously transcatheter balloon atrioseptostomy at 3 days of age for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis underwent pulmonary root translocation with the Lecompte maneuver. This operation has the advantages of maintaining pulmonary valve function, preserving the capacity for growth, and avoiding problems inherent to the right ventricular to pulmonary artery conduit. This patient progressed well for 9 months postoperatively and we report this case of pulmonary root translocation with the Lecompte maneuver.

• Journal of Chest Surgery
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• v.50 no.3
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• pp.207-210
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• 2017

The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arteriovenous malformation after an extracardiac conduit Fontan operation.

• Clinical and Experimental Pediatrics
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• v.53 no.6
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• pp.718-721
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• 2010

Rubinstein-Taybi syndrome (RTS) is a congenital disorder characterized by typical facial features, broad thumbs and toes, with mental retardation. Additionally, tumors, keloids and various congenital anomalies including congenital heart defects have been reported in RTS patients. In about 50% of the patients, mutations in the $CREB$ $binding$ $protein$ ($CREBBP$) have been found, which are understood to be associated with cell growth and proliferation. Here, we describe a typical RTS patient with Arnold-Chiari malformation. A mutation in the CREBBP gene, c.4944_4945insC, was identified by mutational analysis.

• Clinical and Experimental Pediatrics
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• v.59 no.1
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• pp.40-42
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• 2016

Lynch syndrome is the most common inherited colon cancer syndrome. Patients with Lynch syndrome develop a range of cancers including colorectal cancer (CRC) and carry a mutation on one of the mismatched repair (MMR) genes. Although CRC usually occurs after the fourth decade in patients with Lynch syndrome harboring a heterozygous MMR gene mutation, it can occur in children with Lynch syndrome who have a compound heterozygous or homozygous MMR gene mutation. We report a case of CRC in a 13-year-old patient with Lynch syndrome and congenital heart disease. This patient had a heterozygous mutation in MLH1 (an MMR gene), but no compound MMR gene defects, and a K-RAS somatic mutation in the cancer cells.

• Journal of Chest Surgery
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• v.48 no.6
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• pp.422-425
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• 2015

Amyloid deposits in the heart are not exceptional in systemic amyloidosis. The clinical manifestations of cardiac amyloidosis may include restrictive cardiomyopathy, characterized by progressive diastolic and eventually systolic biventricular dysfunction; arrhythmia; and conduction defects. To the best of our knowledge, no previous cases of isolated tricuspid regurgitation as the initial manifestation of cardiac amyloidosis have been reported. We describe a rare case of cardiac amyloidosis that initially presented with severe tricuspid regurgitation in a 42-year-old woman who was successfully treated with tricuspid valve replacement. Unusual surgical findings prompted additional evaluation that established a diagnosis of plasma cell myeloma.

• Journal of Chest Surgery
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• v.28 no.4
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• pp.340-345
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• 1995

Between 1983 and 1993, 250 patients over 16 years of age who had undergone a surgical correction of a congenital cardiovascular disease were reviewed. 222 patients were divided into acyanotic group and 28 patients were cyanotic group. The most common defects were atrial septal defect [96 patients and ventricular septal defect [95 patients . There were 128 patients in the third decade, 71 patients under 20 years of age, 40 patients in the fourth decade and 11 patients over 40 years of age. The male to female ratio was 1.05:1. Operative mortality was 6.8% [4.1% in the acyanotic group and 26.8% in the cyanotic group and the most common cause of death was low cardiac output syndrome.

• Journal of Genetic Medicine
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• v.5 no.2
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• pp.136-138
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• 2008

Townes-Brocks syndrome (TBS) is an autosomal dominant disorder with multiple malformations which include dysplastic ears, hearing loss, preaxial polydactyly and/or triphalangeal thumbs, imperforate anus, renal anomalies, congenital heart defects, and mental retardation. However, hypothyroidism is not a common feature of TBS. There have been only three reported cases of TBS associated with hypothyroidism. We report the first case of TBS associated with hypothyroidism in Korea.