• Journal of Chest Surgery
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• v.30 no.1
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• pp.108-111
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• 1997

Congenital unilateral agenesis of pulmonary artery is a rare anomaly and it usually occurs in association with other cardiac anomaly such as tetralogy of Fallot. Since most patients affected by this defect without associated congenital cardiac anomaly or pulmonary Infection are asymptomatic, the clinical diagnosis of this anomal is first recognized by a characteristic pattern in chest roentgenogram taken as a routine checking; the findings on chest film consists of cardiac and mediastinal displacement, absence of the pulmonary arterial shadow, smaller hemithorax, and elevationof the hemidiaphragm, all on the affected side. We experienced rlght pulmonary artery agenesis in a 48 year-old male, who complained of massive hemoptysis, and it was diagnosed by digital subtraction pulmonary arteriogram and perfusin scan, and treated by right middle and lower lobe bi-lobectomy, and we report this case with the review of relevant literatures.

• Journal of Chest Surgery
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• v.13 no.4
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• pp.364-374
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• 1980

Twenty-two patients were selected for evaluation of pre-and postoperative pulmonary function. These patients were performed open cardiac surgery with the extracorporeal circulation from March 1979 to July 1980 at the Department of Thoracic and Cardiovascular Surgery, Kyungbook National University Hospital. Patients were classified with ventricular septal defect 5 cases, atrial septal defect 5 cases, tetralogy of Fallot 5 cases, mitral stenosis 4 cases, rupture of aneurysm of sinus Valsalva 1 case, left atrial myxoma I case, and aortic insufficiency 1 case. The pulmonary function tests were performed and listed: [1] respiratory rate, tidal volume [TV], and minute volume[MV], [2] forced vital capacity [FVC] and forced expiratory volume[FEV 0.5 & FEV 1.0], [3] forced expiratory flow [FEF 200-1200 ml & FEF 25-75%]. [4] Maximal voluntary ventilation [MVV], [5] residual volume [RV] and functional residual capacity[FRC], measured by a helium dilution technique. Respiratory rate increased during the early postoperative days and tidal volume decreased significantly. These values returned to the preoperative levels after postoperative 5-6 days. Minute volume decreased slightly, but essentially unchanged. Preoperative mean values of the forced vital capacity, functional residual capacity and total lung capacity decreased [63.2%, 87.2% & 77.3% predicted, respectively], and early postoperatively these values decreased further [19.6%, 76.0% & 38.0% predicted], but later progressively increased to the preoperative levels. In residual volume, there was no decline in the preoperative mean values [100.9% predicted] and postoperatively the value rather increased [106.3-161.7% predicted]. Forced expiratory volume [FEV 0.5 & FEV 1.0] and forced expiratory flow [FEF 200-1200 ml & FEF 25-75%] also revealed significant declines in the early postoperative period. There was no significant difference in values of the spirometric pulmonary function tests, such as FEF 1.O and FEF 25-75% between successful weaning group [17 cases] extubated within 24 hrs post-operatively and unsuccessful weaning group [5 cases] extubated beyond 24 hrs. Static compliance and airway resistance measured for the two cases during assisted ventilation, however, any information was not obtained. Long term follow-up pulmonary function studies were carried out for 8 cases in 9 months post-operatively. All of the results returned to the pre-operative or to normal predicted levels except FVC, FEV 1.0, and FEF 25-75% those showed minimal declines compared to the pre-operative figures.

• Journal of Chest Surgery
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• v.9 no.1
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• pp.1-9
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• 1976

The present. study reports four cases of congenital heart diseases, who received open heart surgery by the Sarn's Heart-Lung-Machine in the department of Thoracic Surgery, Hanyang University Hospital during the period between July 1975 and May 1976. The Heart-Lung-Machine consisted of the Sarn's five head roller pump motor system (model 5000), heat exchanger, bubble trap, the Rygg-Kyvsgaard oxygenator, and monitors. The priming of pump oxygenator was carried out by the hemodilution method using Hartman's solution and whole blood. Of the four cases of the heart diseases, three whose body weight were below 30kg, received the partial hemodilution priming and the remaining one whose body weight was 52kg received the total hemodilution priming with Hartman's solution alone. The rate of hemodilution was in the average of 60.5ml/kg. Extracorporeal circulation was performed at the perfusion flow rate of the average 94.0ml/kg/min, and at the moderate hypothermia between 35'5"C and 30'5"C of the rectal temperature. In the total cardiopulmonary bypass, arterial blood pressure was anged between 30 mmHg and 85 mmHg, generally maintaining over 60 mmHg and venous pressure was measured between 4 and $23cmH_2O$, generally maintaining below $10cmH_2O$. The first case: The patient, a nine year old girl having the symptoms and physical signs typical to cardiac anomaly was definitely diagnosed as isolated pulmonary stenosis through the cardiac catheterization. There was, however, no cyanosis, no pathological finding by X-ray and E.C.G. tracings. The valvulotomy was performed through the arteriotomy of pulmouary artery under the total cardiopulmonary bypass. Postoperative course of the patient was uneventful, and murmur and the clinical symptoms disappeared. The second case: A 12 year old boy with congenital heart anomaly was positively identified as having ventricular septal defect through the cardiac catheterization. As in the case with the first case, the patient exhibited the symptoms and physical signs typical to cardiac anomaly, but no pathological abnormality by X-ray and E.C.G. tracings. The septal defect was localized on atrioventricular canal and was 2 by 10 mm in size. The septal defect was closed by direct simple sutures under the cardiopulmonary bypass. Postoperative hemodynamic study revealed that the pressure of the right ventricle and pulmonary artery were decreased satisfactory. Postoperative course of the patient was uneventful, and murmur and the clinical symptoms disappeared. The third case: The patient, a 19 year old girl had been experienced the clinical symptoms typical to cardiac anomaly for 16 years. The pink tetralogy of Fallot was definitey diagnosed through the cardiac catheterization. The patient was placed on an ablolute bed rest prior to the operation because of severe exertional dyspnea, fatigability, and frequent syncopal attacks. However, she exhibited very slight cyanosis. Positive findings were noted on E.C.G. tracings and blood picture, but no evidence of pathological abnormality on X-ray was observed. All of the four surgical approaches such as Teflon patch closure (3 by 4cm in size) of ventricular septal defect, myocardial resection of right ventricular outflow tract, valvulotomy of pulmonary valvular stenosis, and pericardial patch closing of ventriculotomy wound were performed in 95 minutes under the cardiopulmonary bypass. Postoperative hemodynamic study revealed that the pressure of the right ventricle was decreased and pulmonary artery was increased satisfactorily. Postoperative course of the patient was uneventful, and murmur and the clinical symptoms disappeared. The fourth case: The patient, a 7 1/4 year old girl had the symptoms of cardiac anomaly for only three years prior to the operation. She was positively identified as having acyanotic tetralogy of Fallot by open heart surgery. The patient showed positive findings by X-ray and E.C.G. tracings, but exhibited no cyanosis and normal blood picture. All of the three surgical approaches, such a myocardial resection of hypertrophic sight ventricular outflow tract, direct suture closing of ventricular septal defect and pericardial patch closing of ventriculotomy wound were carried out in 110 minutes under the cardiopulmonary bypass. Postoperative hemodynamic study revealed that the pressure of the right ventricle was decreased and pulmonary artery was increased satisfactorily. Postoperative course of the patient was uneventful, and the symptoms disappeared.

• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.271-276
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• 2005

We analysed differences in operative methods and postoperative outcome according to the severity of preoperative cyanosis in adult ToF (Tetralogy of Fallot) patients. Material and Method: From August 1989 to June 2001, thirty three adult patients, 18 females and 15 males, underwent total correction for ToF. Their age ranged from 15 years to 54 years (median: 34). Patients were divided into 2 groups by preoperative $SaO_2$ (arterial oxygen saturation): group I$(n=cyanotic,\;SaO_2\;\geq94\%)$ and group II $(acyanotic,\; SaO_2\geq95%)$. Preoperative median hemoglobin level was higher in group I compared to group II (17.5 g/dl vs 15 g/dl). Postoperative follow-up duration ranged from 1 to 94 months (670 patient-month, median: 14 months), and 63 two-dimensional echocardiographic examinations were done during this period. Result: There were no early or late mortality. With regard to RVOT (right ventricular outflow tract) reconstruction, trans-annular patch and RV-PA extracardiac conduit were used in 7 and 3 patients respectively, and all of them belonged to group I. In group I, cardiopulmonary bypass time, aortic cross-clamping time, ICU day, hospital day were significantly longer than in group II, and postoperative inotropic support was significantly greater than in group II. There was no ventricular arrhythmia in both groups, and one patient in group I suffered from atrial arrhythmia, which was resolved spontaneously after tricuspid and pulmonary valve replacement. During follow-up periods, functional class, residual RVOT stenosis and pulmonary regurgitation, tricuspid regurgitation, occurrence of ventricular and atrial arrhythmias were comparable between two groups. Conclusion: In adult ToF patients with severe preoperative cyanosis, more aggressive RVOT reconstruction and careful postoperative care are mandatory. However intermediate-term outcome of this group of patients is comparable to the patients with minimal or no preoperative cyanosis.

• Clinical and Experimental Pediatrics
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• v.49 no.6
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• pp.635-642
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• 2006

Purpose : The purpose of this study is to determine whether the new pulmonary valve reconstruction technique prevents short-term postoperative pulmonary regurgitation and improves early and mid-term clinical outcome. Methods : We reviewed postoperative echocardiographic variables and chest X-ray films from 31 patients who had undergone valve reconstruction(pulmonary valve reconstruction group : PVR) for the repair of TOF between April 2000 and August 2004. We compared the clinical data of these patients with those from 47 patients who had right ventricular outflow tract reconstruction with a monocusp valve(monocusp ventricular outflow patch group : MVOP) and 22 patients who had a transannular patch repair without a monocusp valve(transannular patch group : TAP). Results : In the PVR group, 25 patients(81 percent) had trivial or mild pulmonary regurgitation in their early post operative echocardiogram. Only 12 patients(26 percent) in the MVOP group had mild pulmonary regurgitation; and no patient in the TAP group had it. Pulmonary valve function was good in 96 percent of the PVR group, 36 percent of the MVOP group, and none in the TAP group in early post-operative echocardiogram. Follow-up echocardiogram(1, 2, 3, 4 years later) of the MVOP and TAP groups showed moderate pulmonary regurgitation and severely decreased valve function in almost all cases. However, in the PVR group 54 percent(16/28), 50 percent(14/28), 37 percent(9/24), and 31 percent(5/16) of the patients had trivial or mild pulmonary regurgitation 1, 2, 3 and 4 years after operation, respectively. The valve function remained good in 80 percent(24/30), 64 percent(18/28), 57 percent(12/21), and 31 percent(5/16) of the patients 1, 2, 3 and 4 years after operation respectively. Conclusion : Pulmonary valve reconstruction is effective in reducing pulmonary regurgitation and right ventricular dilatation in the repair of TOF, even though regurgitation increases with time. Further study is needed to determine long-term results.

• Journal of Chest Surgery
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• v.34 no.8
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• pp.597-603
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• 2001

Background: The purpose of writing this article is to get better clinical results and further clinical improvement based on subject to 110 cases of cardiac surgery which were performed and clinically analyzed. Material and Method: Since January 1995, the patent ductus arteriosus surgery had started in our hospital. In February 1999, an open heart surgery had started and up to September 2000, total of 110 cases were performed as of double ligation of patent ductus arteriosus(10 cases) and open heart surgery(100 cases). Result: Among the patients, Korean-Chines was 74(67.3%) and Han-Chinese was 35(31.8%). Congenital heart disease was 95 cases and acquired valvular heart disease was 15 cases. 83 cases of acyanotic congenital heart disease consisted of ventricular septal defect(VSD) with associated anomaly(45 cases), atrial septal defect(ASD) with associated anomaly(20 cases), patent ductus arteriosus(PDA) with associated anomaly(11 cases), cogenital aortic stenosis(5 cases), double chamber right ventrical(1 case) and Ebstein's anomaly(1 case). Among the 12 cases of cyanotic congenital heart disease, 11 cases of tetralogy of Fallot underwent total correction. Among the 15 cases of acquired valvular heart disease, valvular replacement(7 cases), double valve replacement (3 cases), mitral valve replacement(3 cases) and aortic valve replacement(1 case) were performed. And 8 cases of valvuloplasty were performed by using of commissurotomy, chordal plasty, plasty of papillary muscle, ring type annuloplasty, repair of leaflet. Conclusion: on cogenital heart disease, short term results of surgery for acyanotic cogenital heart disease was good. Among the cyanotic congenital heart disease, tetralogy of Fallot showed a little difference of recovery according to the surgery method so that further follow up observation was needed for long term result. On acquired valvular heart disease, especially, in terms of short term result of valvuloplasty, was relatively good, but further follow up observation was also needed for long term result. There wasn's any operative mortality.

• Journal of Chest Surgery
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• v.35 no.5
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• pp.350-355
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• 2002

Background: Most of pulmonary regurgitation with or without stenosis appears to be well tolerated early after the repair of pulmonary outflow tract. However, it may result in symptomatic right ventricular dilatation, dysfunction and arrhythmias over a long period of time. We studied the early outcome of pulmonary valve replacement with tissue valves for patients with the above clinical features. Material and Method: Sixteen consecutive patients who underwent pulmonary valve replacement from September 1999 to February 2002 were reviewed(9 males and 7 females). The initial diagnoses included tetralogy of Fallot(n=11), and other congenital heart anomalies with pulmonary outflow obstruction(n=5). Carpentier-Edwards PERIMOUNT Pericardial Bioprostheses and Hancock porcine valves were used. The posterior two thirds of the bioprosthetic rim was placed on the native pulmonary valve annulus and the anterior one third was covered with a bovine pericardial patch. Preoperative pulmonary regurgitation was greater than moderate degree in 13 patients. Three patients had severe pulmonary stenosis. Tricuspid regurgitation was present in 12 patients. Result: Follow-up was complete with a mean duration of 15.8 $\pm$ 8.5months. There was no operative mortality. Cardiothoracic ratio was decreased from 66.0 $\pm$ 6.5% to 57.6 $\pm$ 4.5%(n=16, p=0.001). All patients remained in NYHA class I at the most recent follow-up (n=16, p=0.016). Pulmonary regurgitation was mild or absent in all patients. Tricuspid regurgitation was less than trivial in all patients. Conclusion: In this study we demonstrated that early pulmonary valve replacement for the residual pulmonary regurgitation with or without right ventricular dysfunction was a reasonal option. This technique led to reduce the heart size, decrease pulmonary regurgitation and tricuspid regurgitation as well as to improve the patients'functional status. However, a long term outcome should be cautiously investigated.

• Journal of Genetic Medicine
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• v.10 no.2
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• pp.99-103
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• 2013

Purpose: This study was designed to determine the frequency and echocardiographic findings of 22q11.2 deletions in fetuses with cardiac defects on fetal ultrasound or familial backgrounds of 22q11.2 deletions. Materials and methods: We retrospectively reviewed the medical and ultrasonographic records of 170 fetuses that underwent fluorescence in situ hybridization (FISH) analysis for chromosome 22q11.2 deletions between February 2001 and April 2013. Results: Among 145 fetuses with cardiac defects, six (4.1%) had 22q11.2 deletions. Deletions of 22q11.2 were detected in 6 (5%) of the 120 fetuses with conotruncal defects: 5 (8.9%) of 56 with tetralogy of Fallot (TOF) and 1 (5.9%) of 17 with double outlet right ventricle (DORV). No deletions were found in cases of pulmonary atresia, truncus arteriosus, right aortic arch, or transposition of the great arteries. No 22q11.2 deletions were found in non-conotruncal cardiac malformations. Among 25 fetuses with familial backgrounds of 22q11.2 deletions, one (4%) had a maternally inherited 22q11.2 deletion with no cardiac findings. Conclusion: Knowledge of the frequency and echocardiographic findings of 22q11.2 deletions might be helpful for prenatal genetic counseling. It is advisable to perform FISH analysis for 22q11.2 deletions in pregnancies exhibiting conotruncal cardiac defects such as TOF or DORV.

• Tuberculosis and Respiratory Diseases
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• v.56 no.4
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• pp.420-425
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• 2004

Fistula between coronary artery and pulmonary artery is a type of coronary artery anomalies. It can cause atypical chest pain and fatigue, angina pectoris, endocarditis, finally myocardial steal can result in heart failure and myocardial infarction. But only 0.1-0.2% of coronary angiographic studies reveal the communications between coronary artery and other spaces. (heart chamber, pulmonary artery etc.) It is frequently congenital, but acquired types are increasing because chest and heart manipulations such as opertion of tetralogy of Fallot, endomyocardial biopsy, radiation therapy, or penetrating blunt trauma are increasing. There are reports about repair of fistula using thrombogenic tips, coil embolization and surgical intervention. We report a connection between coronary artery and pulmonary artery in 79 years old female. She was 30 pack-years smoker and suffered from dyspnea several years with chronic obstructive pulmonary disease. She presented with atypical chest pain and palpitation after admission. Electrocardiography showed ST-T wave abnormality. Emergency coronary angiography and chest CT scan revealed coronary-pulmonary artery fistula. Transcatheter embolization was performed and she was relieved from discomforts.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1236-1240
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• 2008

Smith-Lemli-Opitz syndrome (SLOS) is a rare, autosomal recessive disease caused by an inborn error in cholesterol synthesis. Patients with this disease suffer from multiple malformations due to reduced activity of 7-dehydrocholesterol reductase (DHCR7), which increases 7-dehydrocholesterol (7DHC) and 8-dehydrocholesterol (8DHC) concentrations and decreases cholesterol concentration in body fluids and tissue. The SLOS phenotypic spectrum ranges from a mild disorder with behavioral and learning problems to a lethal disease characterized by multiple malformations. Here, we describe a newborn male with ambiguous genitalia who was diagnosed to have type II SLOS during the neonatal period. A clinical examination revealed low levels of unconjugated estriol in the maternal serum, and a variety of fetal ultrasound anomalies, including prenatal growth retardation. After birth, the infant was diagnosed to have congenital heart disease (Tetralogy of Fallot with severe pulmonary artery stenosis), cleft lip and palate, micrognathia, postaxial polydactyly, ambiguous genitalia, and cataracts. Clinical investigation revealed extremely low plasma cholesterol levels and the presence of mutation (homozygote of p.Arg352Gln) in the DHCR7 gene. The patient underwent palliative heart surgery (to widen the pulmonary artery) and received intravenous lipid supplementation. Cholesterol levels increased slightly, but not to normal values. The patient died from cardiopulmonary failure and sepsis 72 days after birth. This report provides the first description of a Korean patient with SLOS confirmed by verification of DHCR7 gene mutation and illustrates the need for early recognition and appropriate diagnosis of this disease.