• Journal of Chest Surgery
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• v.41 no.5
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• pp.568-572
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• 2008

Background: Minimally invasive surgery is currently popular, but this has been applied very sparingly to cardiac surgery because of some limitations. Our study evaluated the safety and efficacy of atrial septal defect (ASD) closure through a video-assisted mini-thoracotomy. Material and Method: Fifteen patients were analyzed. Their mean age was $31{\pm}6$ years. The mean ASD size was $24{\pm}5mm$ and there were 3 cases of significant tricuspid regurgitation. The working window was made through the right 4th intercostal space via a $4{\sim}5cm$ inframammary skin incision, CPB was conducted with performing peripheral cannulation. After cardioplegic arrest, the ASDs were closed with a patch (n=11) or direct sutures (n=4), and the procedures were assisted by using a thoracoscope. There were 3 cases of tricuspid repair and 1 case of mitral valve repair. The mean CPB time and aortic occlusion time were $160{\pm}47\;and\;70{\pm}26 $minutes, respectively. Result: There was no mortality, but there were 3 minor complications (one pneumothorax, one wound dehiscence and one arrhythmia). The mean hospital stay was $5.9{\pm}1.8$ days. The mean follow-up duration was $10.7{\pm}6.4$ months. The follow-up echocardiogram noted no residual ASD or significant tricuspid regurgitation. Three patients suffered from pain or numbness. Conclusion: This study showed satisfactory clinical and cosmetic results. Although the operative time is still too long, more experience and specialized equipment would make this technique a good option for treating ASD.

• Journal of Chest Surgery
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• v.31 no.5
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• pp.466-471
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• 1998

Purpose: The safety and efficacy of minimally invasive techniques in congenital heart surgery were tested in this study. Materal and method: Between July 1997 and November 1997, a total of 46 children were underwent minimally invasive cardiac operations at Seoul National University Children's Hospital. Age and body weight of the patients averaged 34.6${\pm}$41.8 (Range: 1∼148) months and 14.5${\pm}$9.9(Range: 3.0∼40.0) kg, respectively. Twenty eight patients were male. Preoperative surgical indications included 15 atrial septal defects, 25 ventricular septal defects, 1 foreign body in aorta, 3 partial atrioventricular septal defects, 1 total anomalous pulmonary venous connection(cardiac type), and 1 tetralogy of Fallot. After creating a small lower midline skin incision starting as down as possible from the sternal notch, a vertical midline sternotomy extended from xyphoid process to the level of the second intercostal space, where one of the T-, J-, I- or inverted C-shaped lower lying mini-sternotomy was completed with a creation of unilateral right or bilateral trap door sternal opening. A conventional direct aortic and bicaval cannulation was routine. Result: A mean length of skin incision was 6.1${\pm}$1.0(range: 4.0∼9.0) cm. A mean distance between the suprasternal notch and the upper most point of the skin incision was 4.0${\pm}$1.1 (range: 2.0∼7.0) cm. Mean cardiopulmonary bypass time, aortic cross-clamp time, and the operation time were 62.9${\pm}$20.0(range: 28∼147), 29.8${\pm}$12.8(range: 11∼79), and 161.1${\pm}$34.5 (range: 100-250) minutes. A mean total amount of postoperative blood transfusion was 71.0${\pm}$68.1 (range: 0∼267) cc. All patients were extubated mean 11.3${\pm}$13.8(range: 1∼73) hours after operation. A mean total amount of analgesics used was 0.8${\pm}$1.8(range: 0∼9) mg of morphine. The mean duration of stay in intensive care unit and hospital stay were 35.0${\pm}$32.2 (range: 10∼194) hours and 6.2${\pm}$2.0(range: 3∼11) days. There were no wound complications and hospital deaths. Conclusion: This short-term experience disclosed that the minimally invasive technique can be feasibly applied in a selected group of congenital heart disease as well as is cosmetically more attractive approach.

• Journal of Chest Surgery
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• v.26 no.8
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• pp.595-603
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• 1993

We analysed 60 consecutive patients who got Senning operation for transposition of the great arteries [TGA] with or without ventricular septal defects [VSD]. There were 41 simple TGA [group I] and 19 TGA with VSD [Group II], the operative mortality was 20 % [in group I 4.9 %, group II 52.6 %]. Among the survivors [n=48], the mean follow-up period was 7 years [range, 1 year to 13.5 years] and the actuarial survival rate at 13 years were 95 % in group I and 42 % in group II. Preoperative high left ventricular pressure and high pulmonary arterial pressure affected the surviving [p<0.01]. There occurred various type of arrhythmia like junctional rhythm, first degree atrioventricular [AV] block, sick sinus syndrome and complete AV block, and we inserted 2 permanent pacemakers for these patients. The incidence of arrhythmia were 28.2 % [11/39] in group I and 55.6 % [5/9] in group II, and the actuarial freedom from arrhythmia at 13 years after operation was 66 % [71 % in group I, 44 % in group II]. Increased aortic cross clamping time had affected the development of arrhythmia [p<0.05] which meant the complexity of the operation. The total incidence of left ventricular outflow tract obstruction [LVOTO] was 31.3 % [15/48], but only 3 patients [6.25 %] showed the significant gradient requiring reoperation. The pulmonary venous pathway obstruction [PVO] were found in 3 patients, all in group I, and among them only one required the reoperation. The estimated freedom from PVO was 89 % at 13 years [87 % in group I, 100 % in group II], but we couldn`t find any significant systemic venous obstruction in our series. There occurred 27.1 % [13/48] mild degree tricuspid valve regurgitation without necessary surgical correction. We experienced 14.6 % [7/48] reoperation rate: 3 residual VSD, 3 LVOTO, 1 PVO, 3 atrial baffle leakage. For this high incidence of complication rate after Senning operation and high mortality in TGA with VSD, We do not use this kind of surgical modality any more and do the Jatene operation for all the TGA patients since several years ago.

• Journal of Chest Surgery
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• v.32 no.8
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• pp.722-725
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• 1999

Background: To secure a rapid and safe approach which is at the same time cosmetically appealing, we employed the right anterolateral thoracotomy incision for repair of atrial septal defects and valvular heart diseases in the adult. Material and method: Between October 1989 and June 1998, 44 adult patients underwent open heart surgery through right anterolateral thoracotomy at our institution. Operative time, cardiopulmonary bypass time, aortic cross clamp time, blood loss until chest tube removal, length of ICU stay, days to discharge, and survival were compared with those that received cardiac surgery via conventional sternotomy. Result: No significant differences were observed between the two groups. There was no death and no additional morbidity directly related to this approach. Cosmetically satisfying results were obtained with safety using the right anterolateral thoracotomy approach. Conclusion: Our data show that the right anterolateral thoracotomy approach is a safe alternative to conventional median sternotomy as it offers excellent exposure and aesthetically more acceptable wounds while not adding on to the operative risks.

• Journal of Genetic Medicine
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• v.11 no.2
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• pp.86-90
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• 2014

Smith-Lemli-Opitz syndrome (SLOS) is a rare autosomal recessive disorder caused by 7-dehydrocholesterol reductase deficiency. The characteristic clinical features are syndactyly of the second and third toes, facial dysmorphism, multiple malformations, and intellectual disability. Few cases of SLOS have been reported in Korea. We observed a male patient with SLOS who presented with typical facial features, undescended testes, microcephaly, bilateral syndactyly of the second and third toes, and cardiac defects, including patent ductus arteriosus and atrial septal defect. Mutation analysis of the DHCR7 gene identified compound heterozygous mutations of c.907G>A (p.Gly303Arg) and c.1055G>A (p.Arg352Gln). In a review of the literature, c.1054C>T (p.Arg352Trp) was the most common mutation reported in Far East Asian countries. This report describes the clinical features, biochemical data, molecular characteristics, and clinical outcome of a Korean patient with SLOS.

• Journal of Chest Surgery
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• v.31 no.12
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• pp.1183-1194
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• 1998

Background: From Sept. 1985 to Sept. 1997, 2,000 cases of open heart surgery(OHS) were performed in the Department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, College of Medicine, Inje University. Material and Method: Among the total of 2,000 cases of OHS, 1532 cases were congenital heart disease(CHD) and 468 cases were acquired heart disease(AHD). The age distribution was 9 days(4.0kg) to 68 years in CHD and 11 to 66 years in AHD. In 1532 cases of CHD, there were 1403 acyanotic cases and 129 cyanotic cases. Result: The CHD cases consisted of 940 ventricular septal defects(61.4%), 324 atrial septal defects(21.1%), 112 tetralogy of Fallot(7.3%), 46 pulmonary stenosis(3%), 38 endocardial cushion defects(2.5%), 15 valsalva sinus ruptures(1%), 4 transposition of great arteries (0.3%), 4 double outlet right ventricles(0.3%), and etc. Corrective operations were applied for congenital heart disease with a result of 3.1% hospital mortality. Of 468 AHD, 381 cases were valvular heart diseases, 48 ischemic heart diseases, 12 cardiac tumors, 8 annuloaortic ectasias, 16 dissecting aortic aneurysms and etc. In the 381 valvular heart diseases, there were 226 single valve replacements(36 aortic valve replacements(AVR), 188 mitral valve replacements(MVR), and 2 tricuspid valve replacements(TVR), among these were 71 cases of double valve replacements(AVR & MVR), 54 cases of MVR with tricuspid valve annuloplasty(TVA), and 18 cases of AVR, MVR with TVA. The total implanted prosthetic valves were 466. In MVR, 123 St. Jude Medical valves, 90 Carpentier-Edwards valves, 65 CarboMedics valves, 42 Sorin valves and 16 other valves were used. In AVR, 68 St. Jude Medical valves, 36 CarboMedics valves, 14 Carpentier-Edwards valves and 9 other valves were used. Coronary Artery Bypass Surgery(CABG) were performed in 48 cases. The patterns of bypass graft were 14 patients of single vessel graft, 21 patients of two vessels graft, 10 patients of three vessels graft and 3 patients of four vessels graft. Conclusion: The hospital operation mortality rate of congenital acyanotic, cyanotic and acquired heart diseases were 2.0%, 15.5%, and 5.1% respectively. The overall mortality rate was 3.6%(72/2,000).

• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.195-202
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• 2010

Purpose : Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity. Methods : We retrospectively reviewed 21 patients who underwent surgical closure of LRSL with severe PAH (${\geq}8$ Wood unit) from January 1995 to April 2009. The median age at operation was 26 years. Atrial septal defect, ventricular septal defect (VSD), VSD and patent ductus arteriosus (PDA), and PDA was present in 11, 4, 4, and 2 patients, respectively. Results : Operability was based on vasoreactivity of PAH. Of the 21 patients, 5 showed response to pulmonary vasodilator therapy and 8 showed vasoreactivity after balloon occlusion of defects. The remaining 8 patients were considered operable because of significant left-to-right shunt (Qp/Qs ${\geq}1.5$). Five patients underwent total closure of defects and 16 were left with small residual shunts. The median follow-up duration was 32 months. There was no significant postoperative mortality or morbidity. Systolic pulmonary artery pressure (PAP) decreased in all but 2 patients. All patients except 1 showed improvement of New York Heart Association functional class. Conclusion : Closure of LRSL in patients with severe PAH on the basis of pulmonary vasoreactivity seems reasonable. PAP and clinical symptoms improved in most patients. Further research is needed for the evaluation of long-term results.

• 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 The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.103-108
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• 2017

Kabuki syndrome is a rare congenital disorder that causes multiple birth defects and mental retardation. Mutation of the lysine methyltransferase 2D (KMT2D) gene is the primary cause of Kabuki syndrome. We report a 4-year-old Korean girl diagnosed with Kabuki syndrome based on distinctive facial features (eversion of the lower lateral eyelid, arched eyebrows, depressed nasal tip, prominent ears), skeletal anomalies, short stature, and molecular analysis, which revealed a novel frameshift mutation in the KMT2D gene. A 4-year-old patient had a past history of congenital cardiac malformations (coarctation of the aorta, ventricular septal defect, atrial septal defect, patent ductus arteriosus), subclinical hypothyroidism and dysmorphic features at birth including webbed neck, short fingers, high arched palate, micrognathia and horseshoe kidney. She showed unique facial features such as a long palpebral fissure, long eyelashes, arched eyebrows with sparseness of the lateral third, broad nasal root, anteverted ears, and small mouth. Her facial features suggested Kabuki syndrome, and genetic analysis discovered a novel heterozygous frameshift mutation (c.4379dup, p.Leu1461Thrfs*30) in exon 15 of the KMT2D gene. The diagnosis of our 4-year-old patient was made through thorough physical examination and history taking, and genetic testing. It is challenging to diagnose patients with Kabuki syndrome at birth, since the characteristic facial features are expressed gradually during growth. Clinical suspicion aroused by regular follow-ups may lead to earlier diagnosis and interventions.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.380-391
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• 2010

Purpose : Respiratory syncytial virus (RSV) is one of the main pathogens causing lower respiratory infections (LRI) in young children, usually of limited severity. However, in congenital heart disease (CHD) patients, one of the high-risk groups for RSV infection, RSV can cause serious illnesses and fatal results. To elucidate the effects of RSV infection in CHD patients, we observed RSV infection cases among CHD patients and non-CHD patients. Methods : On admission of 343 LRI patients over 3 years, 77 cases of RSV infection were detected by the RSV antigen rapid test of nasopharyngeal secretion. We compared RSV infection cases among groups of CHD and non-CHD patients. Results : During the winter season, RSV caused 20-0% of LRI admissions in children. In patients with completely repaired simple left to right (L-R) shunt diseases such as ventricular septal defect, atrial septal defect, and patent ductus arteriosus, RSV infections required short admission days similar to non-CHD patients. In patients with repaired CHD other than simple L-R shunt CHD, for whom some significant hemodynamic problems remained, RSV infection required long admission days with severe clinical course. In children with unrepaired CHD, RSV infection mostly occurred in early infant age, with long admission days. RSV infections within a month after cardiac surgery also required long admission days and severe clinical course. Conclusion : To avoid the tragedic outcome of severe RSV infection in the CHD patients, efforts to find the subgroups of CHD patients at high risk to RSV infection are needed, and effective preventive treatment should be applied.