• Journal of Chest Surgery
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• v.23 no.4
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• pp.654-658
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• 1990

Between January of 1980 and December of 1989, we are encountered 121 cases of Down syndrome here at Yonsei University Medical Center. of these being endocardial cushion defect, ventricular septal defect, tetralogy of Fallot, atrial septal defect, patent ductus arteriosus and complicated anomalies. The mean age was 1 month 2 years with the sexual division at 31 males and 29 females. Among these 60 patients, 10 of them were treated trough surgical management, 8 of them being open heart surgeries, the 8 open heart surgeries are broken down as follow: 4 total correction of ECD, 2 patch repair of VSD, 1 total correction of TOF, 1 patch repair of ASD secundum. Another 2 operative management are ligation of PDA and modified Blalock - Taussig shunt of TOF. Postoperatively all patients were weaned and extubated on an artificial ventilator without any respiratory complications, and were discharged without incident.

• Journal of Chest Surgery
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• v.12 no.3
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• pp.247-258
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• 1979

Two hundred and six cases of open heart surgery were done with over all mortality of 8.3% during 1978 in this Department. There were 130 congenital and 76 acquired cases. In congenital anomaly 58 cases were acyanotic and 72 cyanotic, which showed 3.4% and 12.5% of operative mortality respectively. Thirty-six cases of ventricular septal defect and 10 atrial septal defect were repaired without mortality but in 60 cases of tetralogy of Fallot it was 10%. In 76 cases of acquired lesions 74 were valvular cases. There were 75 cases of valve replacement with over all operative mortality of 8%. In 75-valve replacement one case of Ebstein anomaly was found. There were 62 cases of single valve and 13 double valve replacement. In every case tissue valve was utilized and coumadin and Persantin 75 were administered as anticoagulant treatment for one year.

• Journal of Chest Surgery
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• v.26 no.12
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• pp.904-908
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• 1993

Twelve patients had undergone repair of atrioventricular septal defects. Age at operation ranged from 2.4 years to 17 years[mean, 8.25 years]. Five patients were male and seven were female. Three patients had complete atrioventricular septal defect[Rastelli type A] associated with Down`s syndrome. One of the three patient with complete atrioventricular septal defect had tetralogy of Fallot. Three patients had the intermediate form and seven patients had the partial form. The primum atrial septal defect was closed with pericardial patch. The atrioventricular valve septal commissure[mitral cleft] was closed with pledgeted sutures. Three complete atrioventricular septal defect were undergone by two-patch technique. A crescent-shaped Dacron patch was used to occlude the ventricular septal defect. One patient of partial form was sudden death 5 days postoperatively. There were no another complications after surgery. One patient underwent reoperation for opened mitral cleft 2.5 years postoperatively. New York Heart Association functional class of patients was improved postoperatively.

• Journal of Chest Surgery
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• v.13 no.1
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• pp.34-40
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• 1980

Total 193 patients over 16 years of age who have underwent a surgical correction of congenital heart diseases during the period 1964 to September of 1979 were reviewed. 106 patients were male and 87 patients were female. 85 patients were in the ages of 16 years through 20 years. The oldest patient was 54 years old male who had atrial septal defect. The commonest defects were atrial septal defect that accounted for 66 cases [34.2%]. Ventricular septal defect was next one that accounted for 66 cases [34.2%]. Patients with tetralogy of Fallot defects were 34 cases [17.6%]. 25 cases had patent ductus arteriosus [13.0%]. Patients with pulmonary stenosis were 17 cases [8.8%] and transposition of the great arteries cases were 2 cases [1%]. There were 14 cases of operative death in this series. So operative mortality rate was 7.3%. The commonest cause of death was low output syndrome and next was renal failure. This reviewed series reveals the incidence of operable congenital heart defects appearing in adult cardiac surgical patients and an aggressive surgical approach can be justified with low operative mortality like as pediatric age group.

• Tuberculosis and Respiratory Diseases
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• v.46 no.1
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• pp.116-121
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• 1999

Unilateral hypoplasia of the pulmonary artery is an uncommon anomaly, which commonly develops in combination with congenital cardiovascular defects such as tetralogy of Fallot, patent ductus arteriosus and septal defect of atrium or ventricle, but may also present as an isolated lesion. We have recently experienced a case of the left pulmonary artery hypoplasia in adult by chance of during the general health screen, which diagnosed by chest X-ray, chest spiral CT, lung perfusion and ventilation scan, digital substraction angiogram and bronchoscopy, then presented hereby with the review of relevant literature.

• Journal of Chest Surgery
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• v.19 no.1
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• pp.122-133
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• 1986

Thirteen patients with cyanotic cardiac malformations having more complex intracardiac defects, hemodynamics and operative procedures than ones in Tetralogy of Fallot undertaken total surgical corrections from July 1981 to August 1985. The cases of corrective surgery for complex cardiac malformations were 3.9% of all congenital cardiac malformations and 12.6% of cyanotic cardiac malformations. Six patients died within 30 days after surgery. So operative mortality was 46%; Transposition of the great arteries, two of 4 patients, due to low cardiac output syndrome and tracheal bleeding ; Univentricular heart, one of 3 patients, due to bleeding; Corrected transposition of the great arteries, one of 2 patients, due to acute heart failure; Tricuspid atresia, one of 2 patients, due to low cardiac output syndrome; Double outlet right ventricle, one of single patient, due to respiratory failure. The cases of surgical correction for complex cardiac malformations are progressively increasing in numbers. The more accurate evaluation of anatomical condition and hemodynamics in preoperative diagnosis, studies on applicable surgical procedure and perioperative care of patients are necessary in the improvement of clinical and surgical results.

• Journal of Chest Surgery
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• v.49 no.1
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• pp.39-41
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• 2016

Persistent fifth aortic arch (PFAA) is a rare congenital anomaly of the aortic arch frequently associated with other cardiovascular anomalies, such as tetralogy of Fallot and aortic arch coarctation or interruption. We report the case of a neonate with PFAA with coarctation who successfully underwent surgical repair.

• Korean Journal of Radiology
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• v.22 no.11
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• pp.1894-1908
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• 2021

Double outlet right ventricle (DORV) is a relatively common congenital heart disease in which both great arteries are connected completely or predominantly to the morphologic RV. Unlike other congenital heart diseases, DORV demonstrates various anatomic and hemodynamic subtypes, mimicking ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, and functional single ventricle. Because different surgical strategies are applied to different subtypes of DORV with ventricular septal defects, a detailed assessment of intracardiac anatomy should be performed preoperatively. Due to high spatial and contrast resolutions, cardiac CT can provide an accurate characterization of various intracardiac morphologic features of DORV. In this pictorial essay, major anatomic factors affecting surgical decision-making in DORV with ventricular septal defects were comprehensively reviewed using three-dimensional cardiac CT data. In addition, the surgical procedures available for these patients and major postoperative complications are described.

• Journal of Chest Surgery
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• v.44 no.6
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• pp.392-398
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• 2011

Background: The tetralogy of Fallot (TOF) with pulmonary atresia (PA) and a ductus-dependent pulmonary circulation (no major aorto-pulmonary collateral arteries (MAPCAs)) has been treated with staged repair or primary repair depending on the preference of surgeons or institutions. We evaluated the 19-year outcome of staged repair for this anomaly to find out whether our surgical strategy should be changed. Materials and Methods: Forty-four patients with TOF/PA with patent ductus arteriosus (PDA) who underwent staged repair from June 1991 to October 2010 were included in this retrospective study. The patients with MAPCAs were excluded. The average age at the first palliative shunt surgery was $40.8{\pm}67.5$ days (range: 0~332 days). Thirty-one patients (31/44, 70%) were neonates. The average weight was $3.5{\pm}1.6$ kg (range: 1.6~8.7 kg). A modified Blalock-Taussig (BT) shunt was performed in 38 patients, classic BT shunt in 4 patients, and central shunt in 2 patients. Six patients required concomitant procedures: pulmonary artery angioplasty was performed in 4 patients, pulmonary artery reconstruction in one patient, and re-implantation of the left pulmonary artery to the main pulmonary artery in one patient. Four patients required a second shunt operation before the definitive repair was performed. Thirty-three patients underwent definitive repair at $24.2{\pm}13.3$ months (range: 7.3~68 months) after the first palliative operation. The average age at the time of definitive repair was $25.4{\pm}13.5$ months (range: 7.6~68.6 months) and their average weight was $11.0{\pm}2.1$ kg. For definitive repair, 3 types of right ventricular outflow procedures were used: extra-cardiac conduit was performed in 30 patients, trans-annular patch in 2 patients, and REV operation in 1 patient. One patient was lost to follow-up after hospital discharge. The mean follow-up duration for the rest of the patients was $72{\pm}37$ months (range: 4~160 months). Results: Ten patients (10/44, 22.7%) died before the definitive repair was performed. Four of them died during hospitalization after the shunt operation. Six deaths were thought to be shunt-related. The average time of shunt-related deaths after shunt procedures was 8.7 months (range: 2 days~25.3 months). There was no operative mortality after the definitive repair, but one patient died from dilated cardiomyopathy caused by myocarditis 8 years and 3 months after the definitive repair. Five-year and 10-year survival rates after the first palliative operation were 76.8% and 69.1%, respectively. Conclusion: There was a high overall mortality rate in staged repair for the patients with TOF/PA with PDA. Majority of deaths occurred before the definitive repair was performed. Therefore, primary repair or early second stage definitive repair should be considered to enhance the survival rate for patients with TOF/PA with PDA.

• Clinical and Experimental Pediatrics
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• v.50 no.5
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• pp.462-468
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• 2007

Purpose : The purpose of this study was to analyze the changes of the late potential of Signal- averaged electrocardiography (SAECG) and Electrocardiography (ECG) parameters during follow up of those who had taken surgical repair of Tetralogy of Fallot (TOF). Methods : Nine patients who had taken surgical repair of TOF since 1985 checked SAECG and standard 12 leads ECG twice in 1999 and 2005 in Kyungpook national university hospital. We evaluated changes of QRS duration, QT interval and JT interval, QRS dispersion, QT dispersion and JT dispersion of standard 12 leads ECG and Filtered QRS (f-QRS), High frequency low amplitude potential (HFLA), Root mean square in terminal 40 ms (RMS) and Mean voltage in terminal 40 ms (MV) of SAECG between in 1999 and 2005. Results : There were significant decrease of JT dispersion ($101.11{\pm}50.11$ vs $71.11{\pm}22.61ms$, P< 0.05) and significant increase of HFLA ($24.67{\pm}13.19$ vs $32.89{\pm}14.21ms$, P<0.05). But there were no significant changes in other parameters. Conclusion : In repaired TOF patients, we evaluated ECG and SAECG to detect possible late complications such as tricuspid regurgitation, right ventricular enlargement, ventricualar arrythmia and sudden death. And there were significant changes of ECG and SAECG in some parameters (JT dispersion, HFLA). But to see the relationships between the changes of these parameters and the long term prognosis, we need to check more patients and longer follow-up.