• Journal of Chest Surgery
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• v.18 no.4
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• pp.635-642
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• 1985

Double outlet left ventricle [DOLV] is a rare cardiac anomaly in which both great arteries arise entirely, or predominantly above the morphologically left ventricle. About 100 cases of DOLV have been reported in the literatures by 1984. We have experienced eight cases of DOLV at Seoul National University Hospital during the period from October 1981 to July 1905. Ages of the patients were ranged from 12 months to 24 years old, and chief complaints on admission were frequent URI and DOE in 5 cases and cyanosis in other 3 cases. In all eight patients, Cardiac catheterization and cineangiography were performed but pre-operative diagnoses were incorrect except one case [VSD in 2 cases, DORV in 2cases, c-TGA in 2 cases and TOF in one case] We have performed total corrective surgery in seven patients. In case I, patch closure of VSD aligning aorta and pulmonary artery with LV, ligation of proximal pulmonary artery and the use of external valved conduit from RV to PA have been employed. In other 6 cases, intraventricular repair using boomerang shaped Dacron patch with correction of associated anomalies were employed. In remaining one patient who had coexistent PDA and coarctation of aorta, we have performed coarctoplasty and PDA ligation initially and the patient is waiting for subsequent total corrective procedure. In seven patients whom we have performed total corrective surgery, there is one hospital mortality due to right heart failure and one complication of complete heart block necessitating permanent pacemaker implantation. All survivors are doing well in follow up period of 9 months to 4 years. To our knowledge, this is the first report of surgical experiences for DOLV in the Korean literature.

• Journal of Chest Surgery
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• v.21 no.4
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• pp.727-733
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• 1988

Two cases of the discrete membranous subaortic stenosis were experienced at the Department of Thoracic & Cardiovascular Surgery, National Medical Center, Seoul, Korea. Case I was 31 years old male with a history of aortic valve replacement[Ionescu-Shiley, 19mm] at the other hospital in 1980. Heart failure was noticed 6 years later. On cardiac catheterization, pressure gradient between left ventricle 4 aorta was 104 mmHg, but subvalvular aortic stenosis was not detected by preoperative 2-D echo <% left ventriculogram. Above case revealed stenoinsufficiency of the prosthetic valve. Under the diagnosis of the prosthetic valve failure, re-do aortic valve replacement[Bjork-Shiley, 23 mm] was done. During operation, the discrete membranous subaortic stenosis was incidentally found, and it was completely resected. So we thought that above discrete membranous subaortic stenosis was not detected at first operation, and it was progressed during 6 years, and accelerated the degeneration of the prosthetic valve. Case II was 20 years old female. Her complaints were exertional dyspnea, angina, syncope, which were aggravated since 5 years ago. 2-D echo <% left ventriculogram revealed the discrete membranous subaortic stenosis. Pressure gradient was 20 mmHg, but her symptoms were serious. Associated cardiac anomaly was the persistent left superior vena cava without connection with right superior vena cava. Complete excision of the membranous tissue was done. Post-operative pressure gradient between left ventricle & aorta was absent, and her complaints were nearly subsided. Both cases were type I according to the Newfeld classification of the discrete subvalvular aortic stenosis, and complete excision of the membranous tissue was done without myotomy or mymectomy. And short-term follow-up results[Case I:2 years, Case II: 1 ~ years] were good except soft systolic murmur[grade II/VI] at the aortic area in both cases.

• Journal of Chest Surgery
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• v.9 no.2
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• pp.271-275
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• 1976

Truncus arteriosus is a rare and highly lethal cardiac anomaly characterized by a single arterial trunk emerging from the heart and supplying the coronary, systemic, and pulmonary circulations, The first successful correction of truncus arteriosus was reported by McGoon et al. in 1968 and was based on experimental work reported by Rastelli et al. in 1967 in which a conduit consisting of a homograft of the ascending aorta and aortic valve was used to establish continuity between the right ventricle and the pulmonary arteries, Modification of this procedure using a Dacron tube valved with porcine xenograft instead of a homograft have resulted in the current definite treatment for truncus arteriosus. This report describes an 3 years and 4 months old boy with heart failure from type I truncus arteriosus who was diagnosed as the V. S. D. with pulmonary hypertension preoperatively and underwent corrective surgery employing the Rastelli procedure using a Dacron conduit valved with canine xenograft, but died due to massive bleeding from the anastomosis sites in operating room.

• Journal of Chest Surgery
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• v.36 no.10
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• pp.759-765
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• 2003

Truncus arteriosus with interrupted aortic arch is a very rare congenital cardiac anomaly that has an unfavorable natural course. We report a successful one-stage repair of truncus arteriosus with interrupted aortic arch through median sternotomy in a 25-day-old neonate weighing 3.1 kg. We reconstructed the aortic arch with direct side-to-end anastomosis between ascending and descending aortas. The right ventricular outflow reconstruction was performed with untreated autologous pericardial conduit without valve following Lecompte maneuver. The patient has been grown-up in good condition (25 ∼ 50 percentile of body weight) and shows the right ventricular outflow tract wide 1 year after the operation.

• Journal of Chest Surgery
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• v.51 no.5
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• pp.356-359
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• 2018

We report a case of successful repair of truncus arteriosus (TA) associated with complete atrioventricular septal defect (c-AVSD) using a staged approach. TA associated with c-AVSD is a very rare congenital cardiac anomaly. No report of successful staged repair in South Korea has yet been published. We performed bilateral pulmonary artery banding when the patient was 33 days old, and total correction using an extracardiac conduit was performed at the age of 18 months. The patient recovered uneventfully and is doing well.

• Journal of Chest Surgery
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• v.18 no.2
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• pp.273-278
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• 1985

The case of a patient with abnormal position of the atrial septum resulting in a left atrium with two atrioventricular valves and a disconnected right atrium is presented with review of related articles. Anatomic details showed atrial situs solitus and a left sided cardiac apex. The right atrium received both venae cavae and a coronary sinus. No AV valve was found in the right atrium, and the floor of this chamber was placed above the posterior wall of right ventricular chamber. The atrial septum with secundum ASD was displaced to right anteriorly at its lower portion and inserted to right of tricuspid annulus. The tricuspid and mitral valve configuration was that of so-called partial ECD, i.e. mitral cleft with large anterior mitral leaflets. The ventricular septum was intact and both ventricular chambers were equally well developed with normal relationships. Surgical repair of this anomaly was performed by resecting the abnormally positioned lower part of the atrial septum, repairing the cleft of the anterior mitral leaflet, and septating the atrium for diverting the systemic and pulmonary venous blood to RV and LV, respectively.

• Journal of Chest Surgery
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• v.24 no.2
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• pp.115-122
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• 1991

From April 1986 to December 1989, 25 infants under the age of 12 months with tetralogy of Fallot were operated on. Age ranged from 3 to 12 months[mean 8.9$\pm$4.9 months] and mean body weight was 7.8$\pm$ 2.6kg. All the patients were deeply cyanotic, 12 of them experienced anoxic spell. Transannular patch was laid down in 19 patients, in 7 of them monocuspid patch was utilized. Postrepair P RV/LV was measured at operation room in 17 patients[mean 0.48$\pm$0. 14]. Hospital mortality was 20Yo. Causes of deaths include right ventricular failure and low cardiac output. The mortality was closely related with patient`s age and body surface area at operation. Also higher mortality was noticed in patients having major associated anomaly or previous palliative operation, preoperative management with propranolol and transannular repair. 18 patients were followed up for 12 to 50 months with a mean follow-up time of 24 months after operation. There were no late deaths and late ventricular arrhythmia or congestive heart failure was not detected as yet. Redo operation was performed in one case because of residual pulmonic stenosis. Considering several advantages of early primary repair, primary repair of symptomatic infants with tetralogy of Fallot should be encouraged despite somewhat high mortality rate as yet and better results could be anticipated along with improvement of myocardial protection method and postoperative care.

• Journal of Chest Surgery
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• v.20 no.4
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• pp.659-672
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• 1987

Nitrofen [2,4-dichlorophenyl-P-nitrophenyl ether] is a diphenyl ether herbicide used for pre and post-emergent control of broad leafed weeds. This chemical was known to induce a variety of congenital cardiovascular anomalies with diaphragmatic hernia and hydronephrosis in the rate fetuses. The present study was conducted to produce congenital cardiovascular anomalies in the rat fetuses by oral nitrofen administration at the indicated doses and days of gestation, and to find the characteristics of nitrofen-induced cardiovascular anomalies. All the observed fetuses were removed from the pregnant Sprague-Dawley rats sacrificed on the twenty-first day of gestation. They were preserved in 10 per cent formalin and dissection for examination were carried out under a dissecting microscope using forceps and scissors. Following results and conclusion were based on dissecting microscopic findings on 482 offsprings. 1. The eleventh day of gestation was the most sensitive day for nitrofen induction of congenital cardiovascular anomalies in the rat. This incidence was dose-related in rats exposed on the eleventh day of gestation. 2. Ventricular septal defect was the most common single anomaly that represented more than half of the total cardiovascular anomalies, followed by aortic arch anomalies and tetralogy of Fallot. 3. Cardiac anomalies derived from infundibular maldevelopment such as tetralogy of Fallot and pulmonary atresia with ventricular septal defect were only observed in the eleventh gestation day treated group. 4. Aortic arch anomalies were found in high frequency and the great majority were characteristically anomalous right subclavian artery with left aortic arch. Key words; nitrofen, congenital cardiovascular anomalies.

• Journal of Chest Surgery
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• v.28 no.5
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• pp.447-452
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• 1995

From January 1983 to December 1992, 116 patients under 10Kg of body weight were underwent open heart surgery in National Medical Center.There were 64 males and 52 females, and their mean age was 14 2.6 months, ranged from 3 days to 38 months. Their mean body weight was 7.4$\pm$1.2Kg, ranged from 2.4Kg to 10Kg which were less than 3 percentile of normal developmental pattern in 109 out of 116 cases.The composition of the patients was VSD[73 , TOF[18 , ASD[8 , ECD[5 , TGA[4 , PS[3 , TAPVC[2 , TA[1 , cardiac rhabdomyoma with ASD[1 , and Ebstein`s anomaly[1 . The overall surgical mortality was 17.2%.In acynotic group, 11 patients died among 91 patients and the mortality was 12.1%, in cyanotic group 9 patients died among 25 patients, the mortality was 36%.The surgical result of patients under 5Kg of body weight was worse than those over 5Kg of body weight. [56.2% versus 11% The surgical result in this period was improved year by year, but poor surgical results in the group of cyanotic, under 5Kg of body weight should be improved by means of more precise preoperative diagnoses, more advanced surgical techniques and more proper postoperative management.

• Clinical and Experimental Pediatrics
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• v.57 no.6
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• pp.292-296
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• 2014

The deletion of the distal long arm of chromosome 1 is associated with a characteristic facial appearance and a pattern of associated malformations. Characteristic manifestations include a round face with prominent 'cupid's bow' and downturned corners of the mouth, thin vermilion borders of lips, a long upper lip with a smooth philtrum, a short and broad nose, epicanthal folds, apparently low-set ears, micrognathia, microcephaly, abnormal hands and feet, variable cardiac or genital anomalies, moderate to severe mental retardation, and growth retardation. Using fluorescent in situ hybridization (FISH) analysis to map precisely the deletion, we present a case of chromosome 1q44 deletion with craniofacial characteristics, multiple congenital anomalies, and growth and psychomotor retardation. In comparison with other reported cases of 1q43-44 deletion, the subject does not show hydrocephalus, seizure, syn- or polydactyly of hands, and a urogenital anomaly. However, an arachnoid cyst, pinpoint dimple on the midline of the forehead, a right-sided supernumerary nipple and auricular pit, polydactyly of the right foot, adducted thumb, and flexion restriction of the proximal interphalangeal joint with a simian line in both hands were observed additionally.