• Clinical and Experimental Pediatrics
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• v.46 no.7
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• pp.661-667
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• 2003

Purpose : This study was performed to find the chief clinical problems associated with the ages of adult patients of tetralogy of Fallot(TOF) who had undergone total correction. Methods : Of the 30 patients who were registered at the Grown-Up Congenital Heart Disease (GUCH) Clinic of Samsung Medical Center for TOF, a retrospective investigation was carried out on 28 patients who underwent total correction. Results : Mean age at retrospective study was 30.8(range : 16-53) years old. Age at total correction was 15.8(range : 2-49) years old. Problems after corrective surgery were assessed. They were arrhythmia, pulmonary valve regurgitation, left pulmonary artery stenosis, residual ventricular septal defect, mitral valve regurgitation, tricuspid valve regurgitation, right ventricle outflow tract obstruction, aortic valve regurgitation, infective endocarditis and protein losing enteropathy. After repair of TOF, such arrhythmias as atrial arrhythmia and AV conduction disturbances were observed in some patients. Cardiomegaly was found significantly in the subjects with arrhythmia(P<0.05), and arrhythmia was less observed in patients who underwent surgery at a young age. Eight patients required a reoperation; the main indications were residual ventricular septal defect, right ventricle outflow tract obstruction and peripheral pulmonary artery stenosis. Conclusion : The majority of the patients seemed to live normal lives after Tetralogy of Fallot repair. However, as residual anatomic and functional abnormalities exist postoperatively, continued careful follow-up is needed to detect and correct structural and functional abnormalities.

• Journal of Chest Surgery
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• v.36 no.7
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• pp.483-488
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• 2003

There are still debates in the literature on the relative benefits of blood cardioplegia and crystalloid cardioplegia in pediatric cardiac surgery. We performed a clinical trial to compare the myocardial protective effect between HTK solution and blood cardioplegic solution in congenital heart surgery. Material and Method: 15 patients who underwent HTK solution cardioplegia (group 1) and 15 patients who underwent blood cardioplegia(group 2) were included in this study. Preoperative and postoperative serial serum cardiac enzyme levels (troponin I, CK-MB, LDH) were measured in all patients. Clinical data were analyzed and compared between the two groups. Result: There were no differences in age and body weight between the two groups. Operative diagnosis included ventricular septal defect (VSD, n＝4), atrial septal defect (ASD, n＝1), tetralogy of Fallot (TOF, n＝4), and other complex heart diseases (n＝6) in group 1, VSD (n＝7), ASD (n＝5), and TOF (n＝3) in group 2. Cardiopulmonary bypass times were 99.1$\pm$48.1 minutes in group 1, and 69.3$\pm$27.3 minutes in group 2 (p＝0,02). Aortic clamping times were 52.1$\pm$23.6 minutes in group 1, and 37.9$\pm$20.5 minutes in group 2 (p＝0.07). There was no mortality and spontaneous defibrillation was possible in all patients. No differences were observed in the serial enzyme levels between the two groups. There were no differences in the duration of inotropic support and ventilator time between the two groups. Conclusion: HTK solution provided comparable myocardial protection compared with blood cardioplegic solution. A single high dose of HTK solution may be safely and conveniently used for an extended periods as well in congenital heart surgery.

• Journal of Chest Surgery
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• v.40 no.1 s.270
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• pp.8-16
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• 2007

Background: We performed a prospective clinical study to evaluate the ultrastructural integrity of the myocardium after using Histidine-Tryptophan-Ketoglutarate (HTK) solution in comparison with blood cardioplegic solution during congenital heart surgery. Material and Method: Twenty two patients with acyanotic heart disease, who were scheduled for elective open heart surgery, were randomized into two groups. The HTK Group (n=11) received HTK cardioplegic solution; the blood group (n=11) received conventional blood cardioplegic solution during surgery. The preoperative diagnoses included ventricular septal defect (n=9) and atrial septal defect (n=2) in each group. A small biopsy specimen was taken from the right ventricle's myocardium, and this was processed for ultrastructural examination at the end of 30 minutes of reperfusion. Semiquantitative electron microscopy was carried out 'blindly' in 4 areas per specimen and in 5 test fields per area by 'random systematic sampling' and 'point and intersection counting'. The morphology of the mitochondrial membrane and cristae were then scored. The interstitial edema of the myocardium was also graded. Result: The semiquantitative score of the mitochondrial morphology was $19.65{\pm}4.75$ in the blood group and $25.25{\pm}5.85$ in the HTK group (p=0.03). 6 patients (54.5%) in the blood group and 3 patients (27.3%) in the HTK group were grade 3 or more for the interstitial edema of the myocardium. Conclusion: The ultrastructural integrity was preserved even better with HTK solution than with conventional blood cardioplegic solution.

• Journal of Chest Surgery
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• v.29 no.9
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• pp.983-988
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• 1996

Aneurysm of the sinus of Valsalva is fairly rare. Between 1987 and 1994, we operated on a total of 12 cases of aneurysm of the sinus of Valsalva at the Inje University Seoul Paik Hospital. This represents 0.7% of all cardiac operation under cardiopulmonary bypass. There were eight male and four female patients aged from 8 to 38 years(mean, 19.2 years). 8 of 12 patients had ruptured aneurysms. The origin of aneurysm of the sinus of Valsalva was the right coronary sinus in 10(83. 3%), and the noncoronary sinus in 2(16.6%). In ruptured aneurysm, the origin was the right oronary sinus in 6, and the noncoronary sinus in 2. The aneurysms originating from the right coronary sinus ruptured into the right ventricle in 5, and into both the right atrium and right ventricle in 1. The aneurysms originating from the noncoronary sinus ruptured into the right ventricle in 1, and into the right atrium in 1. Associated congenital cardiac defects included ventricular septal defect in 10(83.3%) patients 39 cases of these were associated with the aneurysms of the right coronary sinus), aortic regurgi- tation in 3 (all of these had an additional ventricular septal defect), mitral regurgitation in 1, and double chambered right ventricle in 1. No hospital deaths occurred, although one late death occurred as a result of endocarditis 15 months after the first operation. The mean follow-up period was 29 months, range from 4 to 60 months. Eleven patients except one late death were in New York Heart Association class 1. Due to the low mortality risk o( an operation for aneur sm of the sinus of Valsalva, a ruptured aneurysm of the sinus of Valsalva should be corrected surgically when the diagnosis is mane, and unruptured aneurysm of the sinus of Valsava with complication should also be operated. In most cases the aorta was opened to examine the morphology of the aneurysm and the aortic cusps, and an associated aortic valve defect should be corrected simultaneously.

• Journal of Chest Surgery
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• v.13 no.4
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• pp.422-434
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• 1980

Since 1968 up to the end of October 1980, 448 valves were replaced in 354 patients in Seoul National University Hospital. There were 238 mitral, 38 aortic, 7 tricuspid, 45 aortic with mitral, 23 tricuspid with mitral, and 3 triple valve replacement aortic mitral and tricuspid cases. Annual increase of mitral valve replacement cases and decrease of operative maortality were remarkable. Recently operative mortality of mitral valve replacement is about 5%. Sex ratio of mitral valve replacement is almost equal and there were 12 cases of pediatric patients (5%) among 238 cases, and patients under the age of 20 years were 34 (14.3%). Mitral valve replacement was done for 199 single mitral, 38 double valve and one triple valve lesions. Among 238 mitral valve replacement paients left atrial thrombus in 23(9.7%), atrial fibrillation in 132 (55.5%), and reoperation after blind mitral commissurotomy in 12(5%) cases were noted. In recent cases bioprosthetic valves, mainly lonescu-shiley valve were utilized to overcome the difficulties of postoperative late complications in anticoagnuation, especially for the rural patients and pediatric cases, in addition to the hemodynamic advantages of lonesocu valve. Among 354 patients 16 cases were congenital heart anomaly related, 5 ventricular septal defect related aortic and 4 Ebstein related tribuspid valve replacement cases. There were 2 congenital anomaly related mitral valve replacements, one for congenital mitral insufficiency of 7 years old boy and one for corrected transposition of the great vessels associated with mitral insufficiency. Among total 354 valve replacements 49 operative deaths (13.3%) were noted and in 238 mitral valve replacement 24 operative deaths occurred (10.1%). In 39 patients among 354 total valve replacements late complications were found. In 238 mitral valve replacement cases late complications were noted in 26 patients, among whom 16 cases expired. Main late complications were thrombe-embolism, subacute becteerial endocarditis, arrythmia cerebral hemorrhage due to unsatisfactory anticoagulation, and congestive heart failure in the incipient period of valve replacement were also noted. In mitral valve replacement cases long-term survival rate was 83.2% who showed marked clinical improvement. Ther were no evidences of calcification during the 2 years follow-up period for the lonescu-valve replacement cases among 19 pediatric patients. In conclusion 238 cases of mitral valve replacement were done with 24 operative deaths and 26 late complication cases among whom 16 expired. The long term survival was 83.2% of the cases. In pediatric cases in place of coumadin anticoagulation Persantin **** 75 and aspirin were administered after valve replacement. In adult cases who have difficulaties with coumadin anticoagulation and for those even with bioprosthetic heart valve replacement who needs long-term or permanent anticoagulation persantin 75 and aspirin combination regimen were administered with antisfactory results.

• 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.

• 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.

• Journal of Chest Surgery
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• v.31 no.10
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• pp.931-938
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• 1998

Background: Anatomic correction of transposition of the great arteries by means of the arterial switch operation is now accepted as the therapeutic method of choice. This retrospective study attempts to assess the results of the neonatal arterial switch operation for transposition of the great arteries performed by our newly established institution. Materials and methods: 33 consecutive neonates underwent the arterial switch operation between October 1991 to November 1997. There were 27 neonates with transposition and intact ventricular septum, 3 with ventricular septal defect, and 3 with Taussig-Bing anomaly. The mean age was 10.9$\pm$7.9 days and mean body weight was 3.29$\pm$0.44kg. Results: Overall postoperative hospital mortality was 30.3% (10 patients). The mortality has improved with time; 75% (6 patients) among first 8 consecutive patients before 1994, 20% (2 patients) among 10 patients in 1994 and 1995, and 13.3% (2 patients) among 15 patients since 1996. Univariated analysis of risk factors revealed that earlier date of the operations and one of preoperative events were determinants for operative death. There were two late deaths. A mean follow-up of 17.4$\pm$16.5 months was achieved in all 21 survivors. All were in New York Heart Association functional class I. One patient had mild pulmonary stenosis and two had mild aortic valve regurgitation on their echocardiography. Conclusions: We concluded that we should continue to perform arterial switch operation for neonates with transposition of the great arteries because the mortality of the operation has been improved and the operative survivors have good functional results with low incidence of late complications.

• Journal of Chest Surgery
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• v.29 no.11
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• pp.1197-1201
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• 1996

From October, 1987 to December, 1995, nine patients underwent total correction of tetralogy of Fallot in adults over 20 years of age. There were 5 male and 4 female patients aged from 22 to 42 years(mean, 29.6 years). Three patients were in New York Heart Association(NYHA) functional class II, and 6 patients in class III. The hemoglobin values ranged from 10.8 to 20.7 gm/㎗ (average, l5.6gm/㎗). The preoperative clinical features were as follows: cyanosis, 8 patients; dyspnea on exertion, 6 patients; clubbing of fingers, 5 patients; frequent upper respiratory infection, 3 patients. At the operation both infundibular and valvular stenosis were present in all patients. Reconstruction of right ventricular outflow tract(RVOT) using Goretex was required in 7 patients, and transannular patching with Goretex in 2 pateints. Left pulmonary angioplasty with pericardium was done in 2 patients. No hospital deaths occurred. Four of 9 patients(44.4%) had postoperative low cardiac output syndrome, and postoperative bleeding in 5. One patient required reoperation due to residual ventricular septal defect and tricuspid regurgitation 3 months after the first operation. The mean follow-up period was 25 months, range 11 to 77 months. All was asymptomatic and in NYHA class I. We suggest that advanced age is not contraindication to surgery in tetralogy of Fallot, and tetralogy of Fallot in adults could be operated on due to low mortality.

• Journal of Chest Surgery
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• v.35 no.6
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• pp.430-438
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• 2002

It is known that low birth weight is a risk factor for poor outcome in cardiac surgery for many cardiac defects. We reviewed our recent surgical experiences on congenital heart defect (other than patent ductus arteriosus) in low birth weight babies. Material and Method: From September 1994 to February 2001, 31 consecutive infants weighing 2500 g or less underwent cardiac surgery with (OHS group n=12) or without cardiopulmonary bypass (CHS group n=19). A retrospective study was carried out to evaluate short-and intermediate-term outcome. Mean gestational age and age at operation were 36.9 weeks(range, 32.3-42weeks) and 32.1days (range, 0-87days) respectively. Mean body weight at birth and operation were 1972g (range, 1100-2500g) and 2105g (range, 1450-2500 g) respectively. There was no difference between the two groups in age and body weight. Defects included ventricular septal defect (VSD) (n=3), VSD with arch anomaly (n=2), total anomalous pulmonary venous return (n=2), transposition of the great arteries (TGA) (n=2), truncus arteriosus (n=2), and univentricular heart with cor triatriatum (n=1) in OHS group, and coarctation of aorta (n=7), tetralogy of Fallot (TOF) (n=3), TOF with pulmonary atresia (n=3), multiple muscular VSDs (n=1), double outlet right ventricle (n=1), pulmonary atresia with intact ventricular septum (n=2), tricuspid atresia (n=1), and TGA with multiple VSD (n=1) in CHS group. 13 patients (41.9%) were intubated pre-operatively. Result: There were 4 early deaths(＜30 days); 1 (8.3%) in OHS group and 3 (15.8%) in non-OHS group. All these early deaths were related to the pulmonary artery banding(PAB). There was no operative mortality in infants undergoing complete repair and palliative operations other than PAB. Delayed sternal closure was required in 3 patients. Prolonged postoperative mechanical ventilation (>7 days) was required in 7 patients(58.3%) in OHS and 7(38.8%) in CHS group. Late mortality occurred in 3 patients, two of which were non-cardiac. A patient in OHS group was documented to have neurologic sequelae. All the survivors except two are in NYHA class 1. Conclusion: Complete repair and palliative operations other than PAB can be performed in low birth weight infants with low operative mortality and an acceptable intermediate-term result. However, about a half of the patients required long-term postoperative mechanical ventilation.