• Journal of Chest Surgery
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• 제26권11호
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• pp.886-889
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• 1993

The DiGeorge syndrome is a rare congenital anomaly of absent or hypoplastic thymus and parathyroid glands. Authors experienced a case of DiGeorge syndrome with complex cardiac anomaly. The complex cardiac anomaly was tetralogy of Fallot with origin of the right pulmonaly artery from the posterolateral ascending aorta.His face showed hypertelorism,short philtrum,"fish-like"mouth and micrognathia. This patient underwent total correction of tetralogy of Fallot and end-to-side anastomosis between right pulmonaly artery and side of main pulmonaly artery. He expired on postoperative second day due to right heart failure and hypoxia.d hypoxia.

• Korean Journal of Radiology
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• 제22권9호
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• pp.1441-1450
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• 2021

Anomalous origin of the coronary artery from the pulmonary artery is a rare and potentially fatal congenital heart defect. Up to 90% of infants with an anomaly involving the left coronary artery die within the first year of life if left untreated. Patients who survive beyond infancy are at risk of sudden cardiac death. Cardiac CT and MRI are increasingly being used for the accurate diagnosis of this anomaly for prompt surgical restoration of the dual coronary artery system. Moreover, life-long imaging surveillance after surgery is necessary for these patients. In this pictorial review, multimodal cardiac imaging findings of this rare and potentially fatal coronary artery anomaly are comprehensively discussed, and representative images are provided to facilitate the understanding of this anomaly.

• Journal of Chest Surgery
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• 제18권3호
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• pp.398-406
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• 1985

103 cases of open heart surgery were performed in the Department of Thoracic and Cardiovascular Surgery, Kyungpook National University Hospital in 1984. There were 90 congenital cardiac anomaly and 13 acquired heart diseases. Out of 90 congenital cardiac anomaly, 63 cases of acyanotic group and 27 cases of cyanotic group were noted. In 63 cases of acyanotic group, 11 ASD, 45 VSD and 7 other acyanotic anomaly were included. In 27 cases of cyanotic group, 4 Trilogy of Fallot, 15 TOF, 3 Pentalogy of Gasul and 5 other cyanotic anomaly were found. Among 13 cases of acquired heart diseases, 12 valvular lesions and 1 atrial myxoma were noted. Two open mitral commissurotomy and ten valve replacements were performed for 12 valve lesions. The frequent complications were acute respiratory insufficiency and low cardiac output syndrome occurred in 5 cases. The perioperative mortality was 4.8% in acyanotic congenital cardiac anomaly, 7.4% in cyanotic congenital cardiac anomaly, and 0% in acquired heart diseases. Overall mortality for 103 cases of open heart surgery was 4.9%.

• Journal of Chest Surgery
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• 제27권1호
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• pp.57-59
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• 1994

Ebstein`s anomaly, a rare congenital cardiac anomaly, is characterized by downward displacement of abnormal tricuspid valve. Indication for surgical repair and the optimal surgical approach are still controversy. Recently, we experience a case of Ebstein anomaly, which was treated by atrilized right ventricular plication and annuloplasty. The patient was discharged with good result on 17th post-operative day.

• Journal of Chest Surgery
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• 제32권6호
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• pp.584-587
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• 1999

DiGeorge증후군은 드문 선천성 기형으로 3번째와 4번째 인후낭의 발생과정 이상에 의한 흉선과 부갑상선의 형성부전과 관련이 있다. 이 증후군은 흔히 선천성 심장기형과 안면기형을 동반한다. 저자들은 DiGeorge증후군을 동반한 선천성 심장기형을 1례 수\ulcorner 치험하였다. 환아는 생후 1개월된 남아로 몸무게는 3.5kg이었다. 동반된 선천성 심장기형은 심실중격결손증, 심방중격결손증, 대동맥축착증 및 동맥관 개존증이었다. 수술은 좌측방 개흉 및 흉골 정중절개를 통하여 일차에 거쳐 시행하였고 환아는 수술 후 별다른 문제없이 회복하였다. 현재 6개월 째 외래 추적관찰 중에 있으며 양호한 상태로 성장하고 있다.

• Journal of Chest Surgery
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• 제52권4호
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• pp.236-238
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• 2019

Aortopulmonary window (APW) is a rare cardiac anomaly that was reported to occur in only 43 cases over 33 years at a large-volume cardiac center. It can present as an isolated anomaly or in combination with another cardiac anomaly. The surgical technique for APW has evolved from simple ligation to separation of the 2 great arteries. However, because of the rarity of APW, there is no standard surgical treatment for this disease entity. Herein, we present successful aortic reconstruction using a main pulmonary artery flap after separation of the 2 great arteries in a neonate with isolated APW.

• Journal of Chest Surgery
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• 제25권12호
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• pp.1455-1460
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• 1992

Anomalous drainage of the right superior vena cava into the left atrium is a very rare congenital cardiac anomaly. Recently a patient with this venous anomaly was surgically corrected and forms the basis of this report. Patient findings were as follows: The patient has no other symptom but cyanosis which prompted cardiac evaluation Chest PA and electrocariogram were usual. Cross-sectional echocardiogram showed normally connected heart without intracardiac defect, Inferior vena cava drained normally into right atrium and coronary sinus was not dilated. Contrast, given into the right atrium, appeared in the left atrium This rare venous anomaly was confirmed by surgery. Surgical correction consisted of division of superior vena cava above the junction of left atrium and reanastomosis into right atrial appendage. Postoperative digital subtracion angiography confirmed the successful repair. She has doing well for 6months since operation. Systemic venous anomalies without intracardiac defect are very rare. However this anomalies should be considered in the differential diagnosis of cyanosis. The successfully corrected case is reported and literature is reviewed.

• Journal of Chest Surgery
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• 제20권2호
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• pp.432-437
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• 1987

Pulmonary atresia with intact ventricular septum is a extremely rare congenital cardiac anomaly. With the history of cyanosis and failure to thrive, this anomaly should not be excluded and emergency management is necessary. Our patient was 69 day-old male with pulmonary atresia which was confirmed by cardiac angiography. Prostaglandin E1 was used for maintenance of pulmonary blood flow preoperatively. Right ventricular outflow reconstruction with pericardial patch and concomitant pulmonary valvotomy were done on beating heart for palliation. With this method, growing of right ventricle and tricuspid annulus are highly expected.

• Journal of Chest Surgery
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• 제29권11호
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• pp.1202-1206
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• 1996

Ebstein기형은 삼첨판, 우심실 및 우심방의 특징적인 변형를 보이는 희귀한 선천성 심질환으로, 삼첨판의 후판막첨과 중격판막첨이 우심실로 나선형의 변형을 보이는 것이 특징적이다. 본 교실에서는 1988년 1월 1일부터 1995년 12월 31일까지 7년간 11명의 환자를 경험하였으며 평균연령은 29.6$\pm$11.1세, 성비는 남자 5명 여자 6명이었고, 주증상으로는 운동시 호흡곤란이었다. 모든례에서 삼첨판의 전형적인 변형을 보였으며 동반된 심기형으로는 개방성 난원창, 심방중격결손, 심실중격결손이 있었다. 9명은 Danielson씨 삼첨판성형술과 주름성형술을 시행하고 2명은 삼첨판막치환술과 주름성형술을 시행하였으며 동반된 심기형을 교정하였다. 수술후 5명의 환자에서 저심박출증, 부정맥, 창상감염 등의 합병증이 발생하였으며 수술사망례는 없었다. 술후 평균 33.3개월의 추적관찰 결과 모든 환자에서 술전보다 양호한 심기능을 보였다.

• Korean Circulation Journal
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• 제54권2호
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• pp.78-90
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• 2024

Background and Objective: We aimed to investigate long-term clinical and echocardiographic outcomes, including tricuspid valve durability, annular growth, and left ventricular reverse remodeling, after modified cone reconstruction in patients with Ebstein's anomaly. Methods: This was a retrospective analysis of all pediatric patients who underwent modified cone reconstruction for Ebstein's anomaly at a single tertiary center between January 2005 and June 2021. Results: A total of 14 pediatric patients underwent modified cone reconstruction for Ebstein's anomaly; the median age was 5.8 years (range, 0.01-16.6). There were three patients (21.4%) with Carpentier type B, ten patients with Carpentier type C (71.4%), and one patient with Carpentier type D (7.1%). There was no early or late mortality, arrhythmia, or readmission for heart failure at a 10-year follow-up. There were no cases of more than mild tricuspid stenosis or more than moderate tricuspid regurgitation during the study period, except for one patient with severe tricuspid regurgitation who underwent reoperation. The z value for tricuspid valve annular size significantly decreased immediately after the operation (2.46 vs. -1.15, p<0.001). However, from 1 year to 7 years after surgery, the z values were maintained between -1 and +1. Left ventricular end-systolic volume, end-diastolic volume, and stroke volume increased after surgery and remained elevated until seven years postoperatively. Conclusions: Ebstein's anomaly in children can be repaired by modified cone reconstruction with low mortality and morbidity, good tricuspid valve durability, and annular growth relative to somatic growth.