• Journal of Chest Surgery
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• v.18 no.3
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• pp.391-397
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• 1985

Ebstein`s anomaly is a rare congenital cardiac malformation and characterized by downward displacement of an abnormal tricuspid valve cusps. But until now, the ideal surgical correction is still controversial. We present a case of Ebstein`s anomaly experienced lately. The patient was 13 year old girl with the complaints of dyspnea on exertion, fatigability and cyanosis. Echocardiography revealed downward displacement of septal leaflet of tricuspid valve and atrialized right ventricle. Cardiac catheterization and cineangiography revealed ASD and large right atrium. During operation, the atrialized right ventricle was plicated and tricuspid annuloplasty was done. And the two secundum type ASD`s were closed by direct suture closure. The patient`s postoperative course was uneventful and discharged on the 24th postoperative days.

• Journal of Chest Surgery
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• v.15 no.1
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• pp.112-117
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• 1982

Communications of coronary arteries with the cardiac cavities have first time been described by Krause in 1865 in a case of an accessory artery draining into the pulmonary artery and later Cayla in a case of a right coronary artery entering the right ventricle. The initial cases have been found accidentally at autopsies, however In recent years after the Introduction of angiography and coronary arteriography, the malformation Is diagnosed during life and is corrected surgically. These conditions are unusual entitles since the advent of angiography they are being diagnosed with increasing frequency. Three patients who had surgical correction of coronary-cardiac chamber fistula at our hospital are presented. In the first case and second case, coronary arteriovenous fistula was corrected horizontal mattress suture ligation with pladget under the cardiopulmonary bypass and third case was corrected double ligation with cardiopulmonary bypass standby. The postoperative courses were uneventful. They discharged without any fistula related complica-tions.

• Journal of Chest Surgery
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• v.48 no.5
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• pp.355-358
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• 2015

Isolated congenital left ventricular diverticulum is a rare cardiac malformation. Here, we report the case of a 33-year-old woman who had suffered from recurrent transient ischemic attacks for 6 years. Preoperative cardiac magnetic resonance imaging and computed tomography angiography revealed a diverticulum near the apex. The diverticulum was successfully obliterated by cardiopulmonary bypass. We suggest that isolated congenital left ventricular diverticulum can be easily corrected with a low surgical risk by patch repair and plication techniques.

• Korean Journal of Radiology
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• v.24 no.10
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• pp.960-973
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• 2023

The bicuspid aortic valve (BAV) is the most common congenital cardiovascular malformation. Patients with BAV are at higher risk of other congenital cardiovascular malformations and valvular dysfunction, including aortic stenosis/regurgitation and infective endocarditis. BAV may also be related to aortic wall abnormalities such as aortic dilatation, aneurysm, and dissection. The morphology of the BAV varies with the presence and position of the raphe and is associated with the type of valvular dysfunction and aortopathy. Therefore, accurate diagnosis and effective treatment at an early stage are essential to prevent complications in patients with BAV. This pictorial essay highlights the characteristics of BAV and its related congenital cardiovascular malformations, valvular dysfunction, aortopathy, and other rare cardiac complications using multimodal imaging.

• Journal of Chest Surgery
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• v.30 no.12
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• pp.1242-1246
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• 1997

Criss-cross heart which is a cardiac malformation caused by abnormal rotation of the ventricles early in embryonic development, is rare but a double outlet of right ventricle in priss-cross heart is very rare. We experienced a case of criss-cross heart which is situs solidus, concordant atrioventricular connection and double outlet of rig t ventricle with remote ventricular septal defect of perimembranous inlet type. A 4-years old female was diagnosed as a double outlet of right ventricle in criss-cross heart after echocardiography, cardiac catheterization and cardiac angiography. The surgical correction was a intraventricular reconstruction of left ventricular outflow with 314 circle of 20 mm Hemashield vascular graft from the ventricular septal defect to the aorta. The patient had a temporary atrioventricular block but was recovered uneventfully, and a postoperative echocardiogram showed no left ventricular outflow obstruction, no intracardiac shunt.

• Journal of Chest Surgery
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• v.19 no.1
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• pp.116-121
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• 1986

Patients over 15 years of age who have undergone a surgical correction of a congenital cardiac malformation during period of January 1958 through January 1986 have been reviewed. During this period there were 3957 congenital cardiac lesions consisting of 2712 acyanotic and 1245 cyanotic cases. Among them, a total of 725 adults [18.3%] with a variety of congenital heart lesions, 548 acyanotic group and 177 cyanotic group were operated on. 372 patients were male and 252 patients were female. There were 280 patients under 20 years of age, 206 between 20-24 years, 102 between 25-29 years, 48 between 30-34 and 89 over 35 years. The most common defects were atrial septal defect which accounted for 207 cases [28.6%] and other common anomalies were VSD [140 cases, 19.3%], TOF [136 cases, 18.6%], PDA [120 cases, 16.6%] and PS [33 cases, 4.6%] in order of incidence. Overall operative mortality for this series was 3.6% [1.8% of a cyanotic group and 9.0% of cyanotic group] compared with 2.8% of total cases of congenital heart disease [acyanotic group 1.1% and cyanotic group 6.5%]. This reviewed series reveals the incidence of operable congenital heart defects appearing in any adult life and demonstrates that surgical repair can be accomplished with a satisfactory low mortality rate.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.618-626
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• 1986

Univentricular heart is a rare congenital cardiac anomaly in which the atrial chambers are connected to only one ventricular chamber and it consists of a diverse group of cardiac malformation characterized by both AV valves or a common AV valve opening into the same ventricle, or the presence of only a solitary AV valve. In spite of recent development in cardiac surgery, corrective operations for univentricular heart still have high mortality and complication rate. Twenty eight patients underwent corrective operation for univentricular heart at Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital from February 1979 to July 1986. Of the 28 patients, 7 patients were operated on by ventricular septation and 21 patients by modified Fontan operation. Of the 28 patients, 19 patients were male and 9 patients female and ages ranged from 5 months to 18 years old with the average age of 7.3 years. There were 2 mortalities in 7 patients operated on by septation with the mortality rate of 28.6% and 5 complications, 3 complete AV block, 1 low cardiac output and 1 arrhythmia. All survived patients are being followed up without specific problem till now. There were 10 mortalities in 21 patients operated on by modified Fontan operation with the mortality rate of 47.6% and 10 complications, 2 low cardiac output, 2 respiratory failure necessitating tracheostomy, 2 persistent cyanosis, 2 arrhythmia, 1 missing of left AV valve in situs inversus patient due to misdiagnosis and one rupture of closed right AV valve. Incremental risk factors for operative mortality are young age less than 5 years old, anomalous pulmonary and systemic venous drainage and atrial septation procedure. In 11 survived patients, 9 patients show good follow-up results but one patient complains of persistent cyanosis and another one patient is suffered from CHF. In our series, results of corrective operation for univentricular heart shows continuing improvement but still high mortality and complication rate. So there must be continuing improvement in surgical result by selection of patient, by adequate decision making for timing and method of operation and by improving operative methods.

• Advances in pediatric surgery
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• v.15 no.2
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• pp.173-179
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• 2009

Congenital thoracic malformations such as intra- and extra-pulmonary sequestration, cystic adenomatoid malformation, congenital pulmonary airway malformation, malinosculation, bronchogenic cyst, reduplication cyst, and foregut cyst are frequently detected on routine prenatal ultrasound. There are some controversies about treatment for postnatally persistent pulmonary sequestration. Some authors recommend expectant long term follow up but most authors advocate elective surgical excision because of complication such as respiratory distress, infection, intrathoracic bleeding, haemoptysis, cardiac failure, and potential risk of malignancy. We experienced 2 cases of prenatally diagnosed extrapulmonary sequestration which were located in the subdiaphragmatic retroperitoneum. Resections were performed at 2 months and 4 months of age using intraabdominal approach. There were no complications. In conclusion, if the prenatally diagnosed extrapulmonary sequestration remained postnatally, early operation might reduce morbidity related to extrapulmonary sequestration and parental anxiety without any postoperative complication.

• Journal of Chest Surgery
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• v.22 no.1
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• pp.95-105
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• 1989

The records of 248 patients over 16 years of age who had undergone a surgical correction of a congenital cardiovascular malformation during the period of 10 years from August, 1978 to July, 1988 were reviewed. During this period, the incidence of congenital cardiovascular malformation in adult was 18.2% of 1376 total heart disease operated on and 25.5% of 986 congenital heart defects. Among them, there were 200 patients in acyanotic group and 48 patients in cyanotic group. Male versus female ratio was 1:1.28. The oldest patient was 59 years old female who had atrial septal defect. The mean age was 24.4 years old. The distribution of the lesions showed a large preponderance of atrial septal defects [37.19o] followed in frequency by ventricular septal defects [918.1%], patent ductus arteriosus [17.3%], tetralogy of Fallot [16.1%], and a variety of other complex malformations[3.2%]. In the pediatric age group, relative frequency was different from that of this adult group, showing ventricular septal defects, tetralogy of Fallot, patent ductus arteriosus and atrial septal defects in order of incidence. The hospital mortality and late mortality were 6.0% and 1.7% respectively. The causes of hospital death were low cardiac output in 10 patients, arrhythmia in 2, air embolism in 1, sepsis in 1 and respiratory failure in l. Clinical improvement upto NYHA functional class I or II postoperatively has been achieved and sustained in all patients following repair except the patients of late death and receiving reoperation. This result confirms that congenital heart defects in the adults can be corrected with a good outcome and an aggressive operative approach seems justified.

• Journal of the Korean Society of Radiology
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• v.82 no.4
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• pp.982-987
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• 2021

The coronary sinus (CS) is the venous drainage system of the heart. CS ostium atresia is a rarely seen cardiac malformation. Congenital atresia of the CS is usually found together with persistent left superior vena cava (LSVC) and other cardiac malformations. However, isolated congenital atresia of the CS is very rare. We present a rare case of isolated congenital atresia of the CS connecting the left atrium and coronary veins without persistent LSVC in a 58-year-old female.