• Journal of Chest Surgery
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• 제21권3호
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• pp.574-582
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• 1988

Endocardial cushion defects is a rare congenital heart disease. We experienced two complete endocardial cushion defects[ECD] and three partial ones, which were successfully repaired between 1986 and 1987. In a patient of complete ECD, associated with secundum ASD, Pulmonary stenosis and Down`s syndrome, the atrial and ventricular septal defects were closed separately with bovine pericardium and Dacron patches respectively, and then pulmonary stenosis was relieved by transannular patch widening in addition to valvotomy and infundibulectomy. In another patient with complete ECD, small interventricular communication was closed with simple suture with pledget and primum ASD was closed with pericardial patch. In first patient of partial ECD, primum atrial septal defect was closed with pericardial patch. In second patient of partial ECD, associated with secundum ASD, direct closure of secundum ASD and patch closure of primum ASD were performed. In third patient of partial ECD, associated with patent foramen ovale[PFO], primum ASD was closed with bovine pericardial patch and PFO was closed directly. In all patient except third patient of partial ECD, mitral clefts were closed with three or four 5-0 prolene interrupted sutures. Transient A-V dissociation developed postoperatively in two patients and transient nodal rhythm developed postoperatively in other two patients. Heart failure in complete ECD with Down`s syndrome was overcome with medical treatment.

• Journal of Chest Surgery
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• 제53권2호
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• pp.41-48
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• 2020

Background: Chylothorax after congenital heart surgery is not an uncommon complication, and it is associated with significant morbidity. However, consensus treatment guidelines are lacking. To improve the treatment outcomes of patients with postoperative chylothorax, we implemented a standardized management protocol at Severance Hospital in September 2014. Methods: A retrospective review of patients treated at a single center was done. All corrective and palliative operations for congenital heart disease performed at our institution between January 2008 and April 2018 were reviewed. The incidence and treatment outcomes of postoperative chylothorax were analyzed. Results: The incidence of chylothorax was 1.9%. Sixty-one percent of the patients could be managed with a low-fat diet, while 28% of the patients required complete restriction of enteral feeding. Thoracic duct embolization was performed in 2 patients and chest tube drainage decreased immediately after the procedure. No patient required thoracic duct ligation or pleurodesis. After implementation of the institutional management protocol, the number of chest tube drainage days decreased (median, 24 vs. 14 days; p=0.45). Conclusion: Implementing a strategy to reduce postoperative chylothorax resulted in an acceptable incidence of postoperative chylothorax. Instituting a clinical practice protocol helped to curtail the treatment duration and to decrease the requirement for surgical treatment. Image-guided embolization of the thoracic duct is an effective treatment for postoperative chylothorax.

• Clinical and Experimental Pediatrics
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• 제53권6호
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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.

• Journal of Chest Surgery
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• 제38권5호
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• pp.335-348
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• 2005

배경: 조기 영아기에 변형 Blalock-Taussig 단락술을 시행받은 복합 심기형 환자들을 대상으로 술 전상태, 술 후 경과, 사망률과 사망원인을 고찰하였다. 대상 및 방법: 2000년 1월부터 2003년 11월까지 조기 영아기에 변형 Blalock-Taussig 단락술을 시행받은 복합 심기형 환자 58명을 연구 대상으로 하였다. 수술 당시 환자들의 평균 연령은 $23.1\pm16.2$일($5\~1$일)이었고, 평균 체중은 $3.4\pm0.7\;kg\;(2.1\~4.3kg)$이었다. 진단은 심실중격결손을 동반한 폐동맥 폐쇄가 12예($20.7\%$), 심실중격이 온전한 폐동맥 폐쇄가 17예($29.3\%$), 단심실이 18예($31.0\%$), 좌측심장형성부전이 11예($19.0\%$)였다. 4예의 총폐정맥연결이상증이 단심실에 동반되었다. 결과: 조기 사망은 11예($19.0\%$)로, 사인은 저심박출증 9예, 부정맥 1예, 다장기부전이 1예였다. 만기 사망은 5예($10.9\%$)로, 사인은 폐렴 2예, 저산소증 1예, 패혈증이 1예였다. 단락술 후 사망에 관여하는 유의한 인자는 술 전 폐동맥 고혈압, 술 전 대사성산증, 술 중 심폐우회술의 이용, 좌측심장형성부전과 총폐정맥연결이상증이었다. 술 후 48시간 동안 24명($41.4\%$)의 환자에서 혈역학적인 불안정성을 보였다. 단락폐쇄에 의한 재수술이 6예, 폐혈류과다에 의한 단락분리술이 1예에서 시행되었다. 걸론: 조기 영아기에 변형 Blalock-Taussig 단락술을 시행받은 복합 심기형 환자들은 고위험군을 제외하면 생존률 및 술 후 경과 측면에서 만족할 만한 결과를 보였다. 수술 직후 많은 환자들에서 혈역학적인 불안정성 시기가 있으므로, 지속적인 집중 관찰 및 치료가 필요하다. 고위험군에 대해서는 단락술 후의 혈역학적 변화에 대한 이해를 바탕으로 한 적극적인 술 후 관리가 필요하다.

• Journal of Chest Surgery
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• 제38권7호
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• pp.501-503
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• 2005

관상동맥루는 선천성 심질환 환자 중에서 $0.27\~0.4\%$의 유병률을 보이는 드문 질환이다. $50\%$이상의 환자에서 우관상동맥이 관상동맥루의 기시부이며 모든 관상동맥루 중 $92\%$는 우측 심장으로 유출된다. 관상동맥루가 좌측 심장으로 개구하는 경우는 $8\%$이며 그 중 좌심실로 유출되는 경우는 더욱 드물다. 본원에서는 3세 된 남자 환아에서 우연히 진단된 우관상동맥과 좌심실간의 관상동맥루를 경험하였기에 이를 보고하는 바이다.

• Journal of Preventive Medicine and Public Health
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• 제34권4호
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• pp.427-436
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• 2001

Objective : To estimate the prevalence of congenital heart disease from the 1990 student heart disease screening program. Methods : The heart disease screening program for elementary students was conducted in Kyonggi-do, in 1998. The subjects of the present study comprised the 40,402 students who attended the schools in the catchment area of a collaborative university hospital and who participated in the primary examination. The congenital heart disease (CHD) patients were initially identified through a questionnaire about prior medical history, and further through diagnostic tests & medical examinations in the secondary & the tertiary examinations. Certain assumptions were used in the estimation of the number of CHD cases among non-participants of the secondary & tertiary examinations. The overall prevalence of CHD was estimated by adding the CHD detection rates of the participants and the estimated prevalence of the non-participants. Results : Among the 40,402 primary participants, 1,655 were referred further, of whom 79.1% (1,309) participated in the secondary examination. Of these, 121 were referred to the tertiary examination, with a participation rate at this last stage of 80.2%. The positive predictive value (PPV) of the screening tools was the highest when the results of both EKG and the questionnaire were positive. Because 85.9% of the detected cases had a past history of CHD, PPV was higher when the selection criteria in the questionnaire included past CHD history than when it didnt. The CHD defection rate among the participants was 1.76 cases/1,000 and the presumed number of cases among the non-participants was 31; giving an estimated final CHD prevalence of 2.52 cases/1,000 (95% CI : 2.06-3.06). Among the identified cases of CHD, VSD (52.8%) was the most common, followed by PDA (9.7%), TOF (9.7%) & PS (9.7%). Conclusion : Because the characteristics of the non-participants differed from those of the participants, the estimation of prevalence was influenced by the participation rate. Of the detected cases, 85.9% had a past history of diagnosis or operation for CMD. These findings suggested that the prevalence estimated in this study may be an underestimation of the actual condition. Therefore, a birth cohort study is required in order to more accurately estimate the prevalence and the effects of the program.

• Clinical and Experimental Pediatrics
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• 제56권3호
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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.

• 한국임상수의학회지
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• 제19권1호
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• pp.110-113
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• 2002

An atrial septal defect (ASD) is a congenital hole in the atrial septum that allows flow between the two atria. Small ASDs are usually well-tolerated defects and do not result in significant clinical abnormalities. In large ASDs or in the presence of other cardiac defects, clinically significancy is increased. Atrial septal defects in 2 Dogs with cardiac and respiratory signs were diagnosed at seoul animal medical center. In ascultation, systolic murmur and the splitting of second heart sound were heard at pulmonary or tricuspid valve region. In radiograph, right-sided cardiomegaly, pulmonary artery dilation, increased pulmonary vasculature makings, and pleural effusion or pulmonary edema signs were observed. In echocardiography, the region, location and size of septal defect was identified. Also, the direction and degree of shunt was measured. These dogs were treated with medicine for cardiac failure. One dog is well-tolerated, the other dog died.

• Journal of Chest Surgery
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• 제17권2호
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• pp.184-188
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• 1984

As OHS is prevalent on whole world, LV-RA shunts once thought as quite rare congenital heart disease are reported frequently. Two cases of LV-RA shunts were operated at N.M.C. in 1983: One of them combined VSD, the other membranous ventricular septal aneurysm protruding into right atrial chamber. In case 1, which was diagnosed correctly, right atriotomy was enough to close the defect under ECC, but in case 2, which was misdiagnosed as ASD preoperatively, atriotomy was added to ventriculotomy. The LV-RA defects were closed by U-shaped direct suture with Teflon felt pledget. Postoperative course was uneventful in both of them.

• Journal of Chest Surgery
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• 제27권3호
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• pp.196-201
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• 1994

Endocardial cushion defect is a rare congenital heart disease. From September 1985, we experienced 20 cases of endocardial cushion defects and postoperative follow up was taken.We reviewed preoperative clinical data, echocardiography, cardiac cath data, operative method & time. After operation, we performed echocardiography and examined mitral valve function & integrity of patch closure sites. Postoperative follow up was taken regularly & follow up period was from 2 month to 91 months. Several postoperative complications were overcome with adequate treatment. There were 3 cases of early death and mortality rate was 15% [3/20].