• Journal of Chest Surgery
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• v.26 no.11
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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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• v.22 no.9
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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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• v.18 no.3
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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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• v.27 no.1
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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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• v.32 no.6
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• pp.584-587
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• 1999

The DiGeorge syndrome is a rare congenital abnormality consisting of aplasia or hypoplasia of the thymus and parathyroid glands resulting from malformation of the third and fourth pharyngeal pouches. This syndrome usually includes congenital cardiac anomalies and abnormal facial features. We experienced a case of congenital cardiac anomaly associated with DiGeorge syndrome. The patient was 1 month old boy weighing 3.5 kg. The congenital cardiac anomalies included ventricular septal defect, atrial septal defect, coactation of aorta, and patent ductus arteriosus. We performed one-stage operation with two separate incisions for these cardiac anomalies. Postoperative course was uneventful and the patient at 6 months of follow up is doing well.

• Journal of Chest Surgery
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• v.52 no.4
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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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• v.25 no.12
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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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• v.20 no.2
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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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• v.29 no.11
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• pp.1202-1206
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• 1996

Ebstein's anomaly is an unusual and a rare congenital cardiac malformation but characteristic abnormality of the development of the tricuspid valve, right atrium and ventricle. The characteristic findings are a displaced tricuspid valve with a septal leaflet spiralling into the right ventricle. The surgical correction is variable and are left unsolved problem in surgical treatment of anomaly. Between January 1988 ＆ December 1995, we experienced 11 cases of Ebstein's anomaly and associated cardiac anomalies are ASD, PFO, ASD with VSD. The typically displaced tricuspid valve leaflet was found in all cases. In the NYHA functional classification, three were in class II, seven were in class III, and one was in class IV. Nine patients were operated by tricuspid annuloplasty ＆ plication with Danielson's method and two patients were done by tricuspid valve replacement with plication, and in all cases associated anomaly was corrected. Postoperatively, five patients suffered from complication - low cardiac output syndrome, arrhythmia and wound infection. There was no operative mortality and the postoperative courses were relatively good conditions with more improvement of symptoms.

• Korean Circulation Journal
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• v.54 no.2
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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.