• Journal of Chest Surgery
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• v.18 no.1
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• pp.75-78
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• 1985

From April, 1984 to January, 1985, 57 cases of cardiovascular surgeries were performed at Yeung Nam University Hospital consisting 31 cases of open heart surgery and 26 cases of ligating patent ductus arteriosus. Among 31 cases of open heart surgery, all cases are congenital acyanotic heart disease except a case of rheumatic aortic valvular heart diseases taken aortic valve replacement with Bjrk-Shiley 25mm. Only 2 cases of transient hemoglobinuria were complicated. A case of surgical mortality was present died of acute respiratory failure and intractable arrhythmia, which resulting 3.2% of mortality rate.

• Journal of Chest Surgery
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• v.13 no.1
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• pp.41-47
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• 1980

Fifty one consecutive patients undergoing open heart surgery, twenty eight congenital and twenty three acquired heart disease, were studied between May and August 1979 in Dept. of Thoracic and Cardiovascular Surgery SNUH. During the same time 10 patients of PDA were included in this study as control group. Four out of fifty one OHS patients, two ASD and two pulmonic stenosis patients, were operated without aortic cross-clamp. In all patients, serial determination of total level of creatine phosphokinase [CPK], lactic dehydrogenase [LDH], glutamic oxaloacetic transaminase [SGOT] were made preoperatively, operative day [immediate post-op], and post-operative days up to 7th day. Electrocardiograms were also evaluated serially. Open heart surgery patients were divided into two groups; Group A was aorta clamp time beyond SO minutes, and Group B was below 50 minutes. The peak level of each enzyme was compared, and electrocardiographic changes were also compared between groups. Although the electrocardiographic changes were more frequent in Group A [50%] than Group B [24%], the peak levels of each enzymes were almost same in Group A and Group B.

• Journal of Chest Surgery
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• v.29 no.12
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• pp.1316-1322
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• 1996

From May 1977 to December 1994, 1.060 cases of open heart surge y for congenital heart disease were performed under cardiopulmonary bypass in Chonnam University Hospital They were divided into two groups; group A(677 cases, from May 1977 lo December 1989), group B(38B cases, from January 1990 to December 1994), to be compared and analized. Of the 1, 060 cases, there were 889 acyanotic (83.9%) and 171 cyanotic(16.1%). The operative mortality was 7.8% in group A and 5.7% in group B (total : 6.6%). The operative mortality for group A was 25% in cyanotic disease and 3.7% in acyanotic disease, respectively in group B, it was 23.8%, 3.2%. There were 19 patients (3.8%) and 78 patients(20.4%) with body weight under 10kg in group A and group B respectively. The operative mortality for these patients were 31.6% in A group and 16.1% in B group. In result, increasing trend in congenital heart disease in patients under 10 kg of body weight has been noted, whereas mortality has decreased. The operative mortality of tetralogy of Fallot has decreased in group B t an group A. These results suggest that the operative result have improved in recent years.

• Journal of Chest Surgery
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• v.28 no.9
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• pp.811-816
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• 1995

Repairs of atrial septal defect utilizing cardiopulmonary bypass were performed in 50 adults, ranging age from 16 to 53 years, since April 1986 up to October 1994. They occupied 38.8% of all adult congenital heart disease operated in the same period. Preoperatively, 16 patients were functional class II, 12 patients class III and 4 patients class IV[New York Heart Association Classification , respectively. Five patients combined with atrial fibrillation and the remainders revealed regular sinus rhythm. Cardiac catheterizations were performed in 43 out of 50 patients, and revealed a systolic pulmonary arterial pressure in excess of 51 mmHg in 4 patients but none had reversed shunt. 40 patients[80% were repaired with patch closure and remainings were repaired with direct closure. 49 patients were followed up for 2 months up to 102 months[average 55 months . A comparison of the preoperative and postoperative functional class demonstrated a mean decrease of one NYHA functional level[2.5$\pm$0.63 to 1.4$\pm$0.56 . There was no operative mortality. One patient died during the follow-up period and the death was unrelated to heart disease. Operative treatment is indicated for repair of atrial septal defect with left to right shunt in the adult patient and a considerable clinical improvement can be anticipitated with low mortality.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1041-1048
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• 2007

Not a few patients in children and adolescents are suffering from right ventricular (RV) dysfunction resulting from various conditions such as chronic lung disease, left ventricular dysfunction, pulmonary hypertension, or congenital heart defect. The RV is different from the left ventricle in terms of ventricular morphology, myocardial contractile pattern and special vulnerability to the pressure overload. Right ventricular failure (RVF) can be evaluated in terms of decreased RV contractility, RV volume overload, and/or RV pressure overload. The management for RVF starts from clear understanding of the pathophysiology of RVF. In addition to correction of the underlying disease, management of RVF per se is very important. Meticulous control of volume status, inotropic agents, vasopressors, and pulmonary selective vasodilators are the main tools in the management of RVF. The relative importance of each tool depends on the individual clinical status. Medical assist device and surgery can be considered selectively in case of refractory RVF to optimal medical treatment.

• Journal of Chest Surgery
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• v.51 no.1
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• pp.57-60
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• 2018

Left main bronchus compression occasionally occurs in patients with cardiac disease. A 19-month-old female patient weighing 6.7 kg was admitted for recurrent pneumonia and desaturation. S he had an atrial septal defect (AS D) with a right aortic arch. Her left main bronchus had been compressed between the enlarged right pulmonary artery (RPA) and the descending thoracic aorta for 14 months. We conducted ASD closure and RPA anterior translocation via sternotomy. The left main bronchus compression was relieved despite the medium-term duration of compression.

• Journal of Chest Surgery
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• v.12 no.4
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• pp.339-345
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• 1979

Mitral valve disease is the most common disease of the acquired heart diseases, and atrial septal defect is also one of common congenital heart diseases. Coexistence of these two lesions is rare, but of great hemodynamic interest. Among 443 cases with mitral valve disease and 90 cases with atrial septal defect experienced in the Department of Thoracic Surgery, Seoul National University Hospital, there were 6 cases with atrial septal defect complicated by mitral valve disease. 1. Of the 6 patients, four were female and two were male. The age was ranged from 18 to 46. 2. Atrial septal defect was ostium secundum type in all cases, and the mitral valvular lesions were regurgitation in four and stenosis in two. Type II ventricular septal defect was also combined in one of the cases. 3. The atrial septal defect was corrected by, primary closure and the mitral valve was replaced with the prosthetic or bioprosthetic valve in all cases. The combined ventricular septal defect was closed using Teflon felt patch. 4. The operative result was good except in one who was expired of bacterial endocarditis 4 months after hospital discharge.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.3
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• pp.87-94
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• 2018

We present the case of long-term observation of a patient with chronic kidney disease (CKD) caused by advanced focal segmental glomerulosclerosis (FSGS) resulting from underlying congenital chloride diarrhea (CLD). A 20-year-old woman was admitted for prolonged proteinuria despite conservative treatment for CLD. She was diagnosed with CLD and started taking KCl salt supplementation from the time of birth. Mild proteinuria was first found at 12 years of age, which progressed to moderate proteinuria at 16 years of age. At 16 years of age, CKD stage 2 with FSGS was diagnosed based on the initial assessment of the glomerular filtration rate (GFR) and kidney histology. On admission, we re-assessed her renal function, histology and genetic analysis. GFR had deteriorated to CKD stage 4 and renal histology revealed an advanced FSGS combined with tubulointerstitial fibrosis. A homozygous mutation in the SLC26A3 gene (c.2063-1G>T) was found by diagnostic exome sequencing and may have been inherited from both parents. CLD patients can be more vulnerable to renal injury, which may also cause progression of renal failure. Therefore, even if there is an early diagnosis and adequate salt supplementation, close monitoring of renal function and tailored treatment should be emphasized for renal protection and favorable CLD prognosis.

• 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.21 no.6
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• pp.970-978
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• 1988

There is tendency of increasing number and decreasing age of patients who are indicated for Rastelli operation for their cyanotic congenital heart disease. So there is the need to find the criterion which saves the patients from early postoperative hemodynamic disturbances. We reviewed the 26 patients who had been performed Rastelli operation at Seoul national University Hospital from January 1981 to June 1988. mean age of the patients was 7.8*3.4 years[range 2.5-15years], mean body surface area[BSA] 0.79*0.25m2[range 0.49-1.51m2] and mean hematocrit 57.95*12%[range 48-80%]. We divided these patients into survived group and died group before postoperative 72 hours, and analyzed preoperative arterial oxygen saturation[SaO2], the ratio of diameter of right pulmonary artery to ascending aorta[RPA/AA], the ratio of both right and left pulmonary artery diameter to descending thoracic aorta[RPA+LPA/DTA], pulmonary artery index[PA index], cardiopulmonary bypass time, aorta cross-clamping time, postoperative perfusion state and total amount of dopamine infused postoperatively. The results showed that RPA+LPA/DTA and PA index were statistically significant factors to influence early postoperative cardiac death rate[P< 0.05]. Especially there were good linear correlations between PA index[X] and peripheral perfusion index[Y][Y= - 1.15+0.02 X, r=0.86, P<0.01]and between PA index[X] and total amount of dopamine infused before postoperative 72 hours[mg/kg, Y][Y=61.94 - 0.15 X, r=-0.80, P < 0.01]. Also there were tendencies that the higher RPA+LPA/DTA[Y], the better peripheral perfusion [X] and the lower need of dopamine[X], but no statistical significance.[Y=0.78+1.60 X, r =0. 49, P >0.05] And the discriminate analysis showed that patients with PA index over 221 mm2/BSA could undergo correction with 25 per cent of error rate. In conclusion, early postoperative hemodynamic states could be predicted by preoperatively measured PA index, and which can be used as a criterion for Rastelli operation performed on cyanotic congenital heart disease.