• Journal of Chest Surgery
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• v.12 no.2
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• pp.105-109
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• 1979

Since 1973, 40 patients with Patent Ductus Arteriosus were operated in The Department of Thoracic and Cardiovascular Surgery, Korea University Medical College. Clinical analysis of these cases: 1. Age ranged from 8 months old to 28 years old Sex ratio was 22 females to 18 males. 2. Pulse pressure widening above 50 mmHg was appeared in 27 patients. In 31 patients, continuous machinery murmur was heard on left second and third intercostal space, but 9 patients has holosystolic murmur on left sternal border. 3. Retrograde Aortography was performed in 11 patients and right heart catheterization, 17 patients, In 8 patients, pulmonary hypertension [above 30 mmHg in` systole] was noted. 3 out of 8 patients was combined with Ventricular Septal Defect. Severe pulmonary hypertension [above 80mmHg in systole] was presented in 3 patients. 4. In 38 patients, operative method was performed with multiple suture ligation of PDA, and in 2 patients, suture closure through pulmonary arteriotomy under cardiopulmonary bypass. 5. One operative death occurred in a patient in this group. Cause of death was right heart failure after multiple suture ligation of PDA and pulmonary artery bandings.

• Journal of Chest Surgery
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• v.13 no.3
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• pp.243-249
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• 1980

Truncus ateriosus is one of the cyanotic congenital heart disease. The incidence is relatively uncommon, as 0.4% of totoal congenital heart disease. Embryologically the defect is due to a lack of partitioning of the embryonic truncus and conus during the first few weeks of fetal life. The ventricular septal defect is invariable present. A single arterial vessel arises from the heart and supplies blood to the aorta, the lung, and the coronary arteries. In 1949, collett and Edwards classified this defect according to anatomic variation to four major types, such as type I, II, III, and IV. Type IV is defined that pulmonary arteries are absent, and the pulmonary arterial supply arises from the descending thoracic aorta. This patients often have a continuous murmur head particularly well in the interscapular area. No effective surgical treatment is available. We have experienced one case of truncus arteriosus, type IV of Collett and Edwards in the Department of Thoracic and Cardiovascular Surgery, Kyungbook National University Hospital. This patient was 10 year-old girl. The chief complaints were cyanosis and dyspnea on exertion since birth. She was admitted at this hospital on April 16, 1980. The continous machinery murmur was heard loudest at the interscapular area. The chest X-ray films revealed cardiomegaly with an increase in pulmonaryvascular markings. The pulmonary secotr was significantly concave. No filling of pulmonary arteries noticed by the right ventriculogram. There was possible biventricular hypertrophy in EKG. The echocardiogram showed that the demension of the aortic root was larger than normal and minimal increase of the left ventricular internal dimension. The cardiac catheterization data was obtained by use of the great saphenus vein approach. The systolic pressure of the right ventricular outflow tract was 80 mmHg and was similar to that of the aorta. The oxygen saturation data revealed the evidence of the left to right shunt at the level of ventricular septum. The patient was operated and the diagnosis was confirmed as trucus arteriosus, type IV. No effective surgical interventins were performed.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.263-269
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• 1997

From April 1987 to May 1996, 13 infants underwent a Norwood operation for complex congenital heart diseases including hypoplastic left heart syndrome (n : 7), mitral stenosis with small VSD and subaortic stenosis (n : 1), mitral atresia with ventricular septal defect, coarctation of aorta, and subaortic stenosis (n = 1), interrupted aortic arch with ventricular septal defect and subaortic stenosis (n : 1), tricuspid atresia with transposition of the great arteries (n = 1), and complex double-inlet left ventricle (n : 2). All patients without hypoplastic left heart syndrome were associated wit hypoplasia of ascending aorta and arch. Age at operation ranged from 3 days to 8.7 months (mean 60.6 $\pm$ 71.6 days, median 39 days). The operative mortality( < 30 days) was 46% (6 patients). Late mortality was 15% (2 patients). All operative deaths occured during the Erst 24 hours after the operation as a result of cardiopulmonary bypass weaning failure (5 patients) and sudden hemodynamic instability postoperatively (1 patient). Late death was due to aspiration pneumonia in two cases. There are 5 long-term survivals (39%). Three of them have undergone a two-stage repair with a modified Fontan operation in two and total cavopulmonary shunt in one at 12, 17, 4.5 months after Norwood procedure with no mortality. Two patients have entered a three-stage repair strategy by undergoing a bidirectional cavopulmonary shunt at 3 and 5.5 months after initial operation with 1 operative death. The actuarial survival rate for all patients at the first-stage operation, including hospital deaths and ate death was 30.8% at 1 year. In conclusion, the operative mortality of Norwood operation was relatively high compared to other operation for major cardiac anomalies, continuing experience will lead to an improvement in result.

• Clinical and Experimental Pediatrics
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• v.59 no.1
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• pp.16-23
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• 2016

Purpose: The 1p36 deletion syndrome is a microdeletion syndrome characterized by developmental delays/intellectual disability, craniofacial dysmorphism, and other congenital anomalies. To date, many cases of this syndrome have been reported worldwide. However, cases with this syndrome have not been reported in Korean populations anywhere. This study was performed to report the clinical and molecular characteristics of five Korean patients with the 1p36 deletion syndrome. Methods: The clinical characteristics of the 5 patients were reviewed. Karyotyping and multiplex ligation-dependent probe amplification (MLPA) analyses were performed for genetic diagnoses. Results: All 5 patients had typical dysmorphic features including frontal bossing, flat right parietal bone, low-set ears, straight eyebrows, down-slanting palpebral fissure, hypotelorism, flat nasal roots, midface hypoplasia, pointed chins, small lips, and variable degrees of developmental delay. Each patient had multiple and variable anomalies such as a congenital heart defect including ventricular septal defect, atrial septal defect, and patent duct arteriosus, ventriculomegaly, cryptorchism, or hearing loss. Karyotyping revealed the 1p36 deletion in only 1 patient, although it was confirmed in all 5 patients by MLPA analyses. Conclusion: All the patients had the typical features of 1p36 deletion. These hallmarks can be used to identify other patients with this condition in their early years in order to provide more appropriate care.

• Clinical and Experimental Pediatrics
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• v.56 no.3
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• pp.101-106
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• 2013

Despite developments in surgical techniques and other interventions, right ventricular (RV) failure remains an important clinical problem in several congenital heart diseases (CHD). RV function is one of the most important predictors of mortality and morbidity in patients with CHD. RV failure is a progressive disorder that begins with myocardial injury or stress, neurohormonal activation, cytokine activation, altered gene expression, and ventricular remodeling. Pressure-overload RV failure caused by RV outflow tract obstruction after total correction of tetralogy of Fallot, pulmonary stenosis, atrial switch operation for transposition of the great arteries, congenitally corrected transposition of the great arteries, and systemic RV failure after the Fontan operation. Volume-overload RV failure may be caused by atrial septal defect, pulmonary regurgitation, or tricuspid regurgitation. Although the measurement of RV function is difficult because of many reasons, the right ventricle can be evaluated using both imaging and functional modalities. In clinical practice, echocardiography is the primary mode for the evaluation of RV structure and function. Cardiac magnetic resonance imaging is increasingly used for evaluating RV structure and function. A comprehensive evaluation of RV function may lead to early and optimal management of RV failure in patients with CHD.

• Journal of Chest Surgery
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• v.30 no.7
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• pp.641-646
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• 1997

Understanding of the surgical anatomy of patients with double outlet right ventricle (DORV) is important in the planning of biventricular repair From May 1995 to September 1996, 7 patients underwent biventricular repair for DORV with remote ventricular septal defect. There were 5 males and 2 (tamales. Age at operation varied from 2 to 9 years(mean 3.4$\pm$ 2.7years). Preoperative diagnostic assessment was made by two-dimensional echocardiography and cardiac catheterization. Ventricular septal defect was perimembranous inlet type in all patients. Associated cardiac anomalies were pulmonary atresia in two, pulmonary stenosis in five and tricuspid chordal attachment to zonal septum in five. The operations were performed intraventricular repair and pulmonary enlargement in two, REV operation in two, and Rastelli operation in three. There was no early postoperative deaths and complications. The follow-up period war from 1 month to 18mon1hs, averaging 10: 6.1 months. In the past, we considered the Fontal operation indicative as primary choice when DORV was associated with abnormal tricuspid chordal attachment to the zonal septum, but now we believe that biventricular repair is feasible for those cases by making conal flap or reattachment method. Biventricular repair has theoretic advantages because it estabilishes normal anatomy and physiology, and it was concluded that the precise preoperative evaluation using both echocardiography and cardiac catheterization was essential to the successful surgery.

• Journal of Chest Surgery
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• v.30 no.5
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• pp.486-492
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• 1997

We r viewed a policy of primary surgical closure of large ventricular septal defects in small infants. Sixty-three infants met criteria for inclusion in the study, and were divided into two groups based on age: group 1 infants aged less than 5 months(n = 31), and group 2 infants aged 5 months or more(n = 32). Both groups had similar variation in ventricular septal defect location(paramembranous versus muscular), and showed no significant difference in left to right shunt and in ratio of systemic and pulmonary vascualr resistance. Three early deaths(9.7%) occurred in group 1, but no death(0%) in group 2. The causes of death were preoperative cardiac arrest and cerebral injury followed by postoperative respiratory insufficiency in two patients, and preoperative tracheomalacia followed by laryngeal edema and respiratory arrest in one Two patients in group 1 showed postoperative low cardiac output syndrome(6.5% in group 1 versus 0% in group 2). There was no late death during the follow-up per od in both groups. No surviving patients had postoperative patch leakage, or required a second operation. These results indicate that primary surgical closure of large ventricular septal defects, if logical perioperative care is accompanied, can be saefly performed in small infants aged less than 5 months with low postoperative mortality or morbidity rates.

• Journal of Chest Surgery
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• v.39 no.8 s.265
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• pp.633-636
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• 2006

The partial endocardial cushion defect including ostium primum atrial septal defect and anterior mitral leaflet cleft, presents less significant clinical symptoms than complete endocardial cushion defect. But, as mitral insufficiency develops, cardiomegaly, congestive heart failure, pulmonary arterial hypypertension appear. So, partial endocardial cushion defect has poor prognosis and is rarely seen in elderly patients. A 67 years old woman admitted at our hospital for operative treatment with partial endocardial cushion defect. She had increased pulmonary pressure of 45/22 mmHg, mean 32 mmHg. She had repair of ostium primum defect with patch, and the mitral valve was treated with valve replacement. Because advanced atrioventricular block developed postoperatively, she received permanent pacemaker.

• Journal of Chest Surgery
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• v.19 no.2
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• pp.265-272
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• 1986

We operated on 199 patients of VSD from 1976 to April l986. Among them, patients of VSD whose medical records were available were analyzed clinically. Operation on patients of VSD occupied 23.9% of total open heart surgery [832 cases] during those days. Of the 164 patients, 93 patients were male [56.7%]. 71 patients were female [43.3%]. Their age ranged from 6 months to 28 years and the mean age was 9.5 year and 82.2% of the patients were between 2 and 15 year of age. Of the patients, body weight below 10Kg were 19 cases. The most common complaints were frequent URI and DOE. On Kirklin`s anatomical classification, type II defect was most common [60.1%], type I [38.4%], combined type I+II, type III, combined type II+Ill and combined type II+IV in orders. Associated anomaly was found in 66 patients [42.5%>]. Pulmonary stenosis was most commonly associated cardiac anomaly [8.4%] and aortic insufficiency [7.1%], ASD, Lt. SVC and PDA in orders. There were extracardiac anomalies such as polydactyly, cleft palate, hypospadia and congenital aniridia, etc. Relationship between ventricular hypertrophy and defect size and cardiac cath. data was analyzed. The overall mortality was 7.0% [14 cases] and complication rate was 22.5% [35 cases].

• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.494-498
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• 2009

Purpose : Applicability of transcatheter closure of atrial septal defect (ASD) has been expanded by accumulation of clinical experiences and evolutions of the device. This study was performed to evaluate the safety and efficacy of transcatheter closure of ASD with Amplatzer septal occluder (ASO) in young children less than 3 years of age. Methods : From May 2003 to December 2005, 295 patients underwent transcatheter closure of ASD with ASO in the Severance Cardiovascular Hospital, Yonsei University Health System. Among them, 51 patients less than 3 years of age were enrolled in this study. We investigated procedural success rate, rate of residual shunt, frequency of complications, procedure/fluoroscopy time, and need of modified techniques for device implantation. Results : The median age was 2.1 years and median body weight was 12 kg. Implantation of device was successful in 50 patients (98%). Seven patients (15%) showed a small residual shunt 1 day after the procedure, but complete occlusion had been documented at 6 month follow-up in all patients (100%). The pulmonary to systemic flow ratio (Qp/Qs), peak systolic pulmonary artery pressure, and peak systolic right ventricular pressure had decreased significantly after closure of ASD. There were 2 complications including device embolization (1, 2%) and temporary groin hematoma (1, 2%). Conclusion : Transcatheter closure of ASD with ASO can be performed with satisfactory results and acceptable risk even in young children less than 3 years of age. We could suggest that even in very young children with ASD, there is no need to wait until they grow to a sufficient size for the transcatheter closure.