• Journal of Chest Surgery
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• v.38 no.12 s.257
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• pp.815-820
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• 2005

Background: Pulmonary atresia with intact ventricular septum is a rare congenital cardiac disease. There have been various surgical options, but there are still controversies. Material and Method: Fourteen neonates who were operated on between 1999 and 2000 were enrolled in this study. We measured Z-value of tricuspid valve by echocardiography. We performed right ventricular outflow tract transannular patch in 9 patients. Modified Blalock-Taussig shunt was placed according to the condition of patients. Mean follow up period was 46.0 months. Result: There were 2 operative mortality and 1 late mortality. Biventricular repair was possible in 7 patients. One and a half ventricular repair were done in 3 (tricuspid valve Z-value was -2, -2.5 and -3) and single ventricular repair in 2 patients (tricuspid z-value was -4.6, -4.5) The tricuspid valve Z-value for the patients who had biventricular repair and one and a half ventricular repair were -0.8$\pm$1.50 (-3.2$\∼$1.2) and -2.5$\∼$0.5 (-3$\∼$ -2) respectively. All patients who survived had fair to good right ventricular function, good left ventricular function and good clinical states. Conclusion: Neonatal surgical management of pulmonary atresia with intact ventricular septum has a good surgical outcome, z-value of tricuspid may be helpful for the determination of surgical options.

• Journal of Chest Surgery
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• v.21 no.5
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• pp.816-827
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• 1988

Since 1978, We have experienced 87 cases of Fontan operations and the candidates of that increased in numbers recently with the improvement of the diagnostic and operative technique. We studied the prerequisite factors and hemodynamics of 22 cases of Fontan operations, done during the last one year period, which were 3 tricuspid atresia, 16 functional single ventricle and 3 anatomic single ventricle. The mean age was 68 months and the mortality rate 24%, and 9 patients of under 4 years of age were operated with 22.2% mortality rate, but the youngest, 16 months of age, patient survived well without problems. The preoperative pulmonary artery pressure[PAP], pulmonary vascular resistance[PVR] and postoperative right atrial pressure[RAP], left atrial pressure[LAP] value influenced the mortality, but age, preoperative Hb, preoperative PaO2 and pulmonary artery index[PAI] did not. There were favorable survival tendency in under 15mmHg of preop. PAP, 2a of preop. PVR and under 25cmHyO of postop. RAP, under 15cmHyO of postop LAP. The younger, the more pleural effusion and the longer postoperative admission days. The higher preop. Hb related to the higher postop. transpulmonary pressure gradient and the lower preop. PaO2 and PAI. The higher preop. PaO2, the less pleural effusion and postop. admission days. Preop. PAP closely related to preop. PVR and postop. LAP and high PVR increased the pleural effusion and postop. admission days. The larger PAI, the larger CI. We concluded that there were so many factors influencing the postoperative condition, but preop. PAP, PVR, Hb, postop. RAP and LAP were the most ones.

• Journal of Chest Surgery
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• v.22 no.4
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• pp.594-600
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• 1989

Primary intracardiac repair of tetralogy of Fallot with low mortality and early good results, has been accomplished in recent years. But palliative procedures have been reserved for those hypoplastic pulmonary arteries, a hypoplastic left ventricle or anomalies of the coronary artery would make total correction difficult. And the Blalock-Taussig shunt operation is recognized as a standard and popular palliative procedure. I undertook a retrospective determination of the effect of the Blalock-Taussig shunt operation on the development of the main pulmonary artery and the right and left pulmonary arteries. Between January, 1980, and April, 1987, at the Severance Hospital, 16 patients were studied by cardiac catheterization and angiocardiography, before undergoing Blalock-Taussig shunting procedures for the palliation of severe symptoms of tetralogy of Fallot, and some time later, usually prior to a second procedure. The mean interval between catheterizations was 22.25 months. Patients with tetralogy of Fallot and pulmonary atresia or with an occluded shunt were not included. The primary and secondary angiograms of each patient were reviewed, and measurements of the diameter of the main pulmonary artery, the right and left pulmonary arteries, and the descending thoracic aorta were taken. The results are as follows; 1. The hematocrit decreased from 56.39% to 50.34%[p< 0.05], and the arterial oxygen saturation increased from 62.00 % to 81.31 %[p< 0.001] following shunt procedures 2. The ratio of the diameter of the right pulmonary artery plus the left pulmonary artery to the diameter of the descending thoracic aorta increased 1.30 k 0.28 times [p< 0.01]; but the ratio of the diameter of the main pulmonary artery to the diameter of the descending thoracic aorta increased 1.10 * 0.33 times, which was not. significant[p< 0.05]. 3. The interval between shunting and second catheterization was not related to the magnitude of change in the pulmonary arteries[r=0.141, p >0.05]. 4. The changes in the ratio of the diameter of the right pulmonary artery plus the diameter of the left pulmonary artery to the diameter of the descending thoracic aorta was inversely related to the initial ratio[r=0.757, p >0.001], but the change in the ratio of the diameter of the main pulmonary artery to the descending thoracic aorta was not related[r=0.059, p >0.05]. 5. There were no differences in enlargement of the pulmonary artery on the side of the shunt [ipsilateral] versus enlargement on the opposite side [p >0.05], nor according to the size of the shunt[p >0.05]. In conclusion, this study suggests that the Blalock-Taussig shunt is effective for the development of the right and left pulmonary arteries but not effective for the main pulmonary artery.

• Journal of Chest Surgery
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• v.35 no.4
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• pp.303-306
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• 2002

Patients with severe Ebstein's anomaly showing in the neonatal period, represent progressive cardiac enlargement with pulmonary hypoplasia and functional pulmonary atresia with patent ductus alteriosus-dependent pulmonary circulation. Biventricular repair in these patients had been mostly unsuccessful except for Starnes' procedure that converts the anatomy to single ventricle physiology for Fontan procedure. A 4-days old male was admitted with the diagnosis of severe Ebstein's anomaly with anatomic pulmonary atresia and severe cardiac enlargement. He successfully underwent biventricular repair with vertical plication method of atrialized right ventricle, tricupid annuloplasty, transannular right ventricular outflow tract reconstrulltion, atrial septal defect patch closure with fenestration, and right atrial reduction angioplasty Postoperatively, cardiothoracic ratio was significantly reduced and mild tricuspid regurgitation was remnant in echocardiography. The patient is currently 10 months old and is fully active without restrictions.

• Journal of Chest Surgery
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• v.33 no.4
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• pp.290-300
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• 2000

Background: To evaluate the diagnostic accuracy of EBT(Electron Beam Tomography) in the diagnosis of conotruncal anomaly and to determine whether it can be used as a substitute for cardiac angiography. Material and Method: 20 patients(11M & 9F) with TOF(n=7, pulmonary atresia 2), DORV(n=7), complete TGV(n=4), & corrected TGV(n=2) were included. The age ranged from 7 days to 26 years(median 60 days). We analyzed the sequential chamber localization, the main surgical concenrn in each disease category (PA size, LVED volume and coronary artery pattern for TOF & pulmonary atresia, the LV mass, LVOT obstruction, coronary artery pattern for complete TGV, and type of VSD and TV-PV distance for DORV, etc) and other associated anomalies(e.g., VSD, arch anomalies, tracheal stenosis, etc). Those were compared with the results of echocardiography(n=19), angiography (n=9), and surgery(n=11). The interval between EBT and echocardiography/angiography was within 20/11 days, respectively except for an angiography in a patient with corrected TGV (48 days). Result: EBT correctly diagnosed the basic components of conotruncal anomalies in all subjects, compared to echocardiography, angiography or surgery. These included the presence, type and size of VSD(n=20), pulmonic/LV outflow tract stenosis(n=15/2), relation of great arteries and the pattern of the proximal epicardial coronary arteries(16 out of 20). EBT proved to be accurate in quantitation of the intrapericardial and hilar pulmonary arterial dimension and showed high correlation and no difference compared with echocardiography, angiography, or surgery(p>0.05) except for left pulmonary arterial & ascending arterial dimension by echocardiography. LVED volume in seven TOF(no difference: p>0.05 & high correlation: r=0.996 with echocardiography), and LV mass in 4 complete TGV were obtained. Additionally, EBT enabled the cdiagnosis of subjlottic tracheal stenosis and tracheal bronchus in 1 respectively. Some peripheral PA stenosis were not detected by echocardiography, while echocardiography appeared to be slightly more accurate than EBT in detecing ASD or PDA. Conclusion: EBT can be a non-invasive and accurate modality of for the evaluation of most anatomical alteration including peripheral PS or interruption in patients with conotruncal anomalies. Combined with echocardiography, EBT study provides sufficient information for the palliative or total repair of anomalies.

• Journal of Chest Surgery
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• v.14 no.1
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• pp.26-32
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• 1981

Three cases of tricuspid atresia were treated by Fontan varieties of operation in this department in 1980. The first case was 19 year old girl who underwent Glenn operation at the age of 6 years. Her second operation was done with ASD closure and 16mm Ionescu-Shiley valved conduit insertion between right atrium and main pulmonary artery. The second case was a 5 year old boy who underwent Kreutzer operation successfully utilizing 14mm Ionescu-Shiley va]ved conduit. The above mentioned 2 cases were Type Ib after Keith`s classification, whose immediate postoperative courses were complicated by pleura] effusion [in 2nd case chylothorax] hepatomegaly, and ascites. Those complications were relieved completely by medical treatment and closed thoractomy; Postoperative follow-up up to 11 months and 1 year periods were satisfactory with disappearance of cyanosis and dyspnea. The third case was a 8 year old boy who had complete TGA with TA [Keith`s Type IIb] who underwent Kreutze`s operation utilizing 14mm Ionescu-Shiley valved conduit, he died of low cardiac output Immediately after open heart surgery.

• Journal of Chest Surgery
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• v.16 no.4
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• pp.431-443
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• 1983

A hundred and fifty-one patients with congenital heart disease less than 24 months old underwent intracardiac repairs from January 1982 to July 1983, which consists 24.2% of all the patients with congenital heart diseases operated during the same period. There were 98 patients[64.9%] with acyanotic congenital heart disease and 53 patients[35.1%] with cyanotic congenital heart disease, and 55 patients[36.4%] were less than 1 year of age. Twenty-two patients died within 30 days after surgery and 3 patients died after postoperative 30th day: Ventricular septal defect, four of 90 patients; Tetralogy of Fallot, five of 23 patients; Transposition of great arteries, nine of 17 patients; Tricuspid atresia, four of 5 patients; Pulmonary atresia, all of 2 patients; Single ventricle, one of single patients. Over all mortality was 16.6% and mortality of acyanotic congenital heart disease, cyanotic congenital heart disease and patients less then 1 year of age was 4.1%, 39.6% and 20.0% respectively. Still the mortality of cyanotic congenital heart disease is high. Careful preoperative evaluation of the detailed intracardiac anatomy and hemodynamics of the patients and proper selection of surgical treatment yield better clinical results.

• Journal of Chest Surgery
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• v.18 no.4
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• pp.605-614
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• 1985

The principal aim of surgery for congenital heart anomalies is the establishment of normal hemodynamic function. Palliative and corrective operations are selected with time to attain this end with minimal risk. In recent years, as operative mortality after primary total correction is lower than the mortality after early palliation and delayed correction, corrective operations in infants have increasingly supplanted palliative ones. Two hundred and eighteen infants below 10 kg with congenital heart anomalies underwent primary surgical intervention at Yonsei Medical Center from March 1979 to June 1985. There were 155 infants with VSD, 35 Infants with TOF, 5 infants with ECD, 4 infants with TGV, 3 infants with DORV, 3 infants with Pulmonary atresia, 3 infants with ASD and PDA, 2 infants with DOLV, and the remainders were Sinus Valsalva rupture, residual mitral regurgitation after total correction of ECD, PAPVR, Cor triatriatum, Truncus arteriosus, and Tricuspid atresia. The overall surgical mortality was 15.1%. In the acyanotic group, 13 infants died among 168 infants, and mortality was 7.7%. But in the cyanotic group, the mortality rate was very high and 20 infants died among 50 infants raising the mortality to 40.0%. These poor surgical results in the cyanotic or complicated group was due to inaccurate diagnosis, improper surgical methods and inadequate post-operative care which should be improved.

• Journal of Chest Surgery
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• v.37 no.10
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• pp.856-860
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• 2004

Interrupted aortic arch with concomitant intracardiac defects is a rare congenital anomaly that has an unfavorable natural course. We report a successful staged operation of interrupted aortic arch with apical muscular ventricular septal defect associating esophageal atresia with tracheoesophageal fistula in a 3-day-old neonate weighing 2.6 kg. We repaired esophageal atresia through the right thoracotomy and subsequently performed extended end-to-end anastomosis of the aortic arch with pulmonary artery banding through the left thoracotomy at same operation. The apical muscular VSD was repaired 87 day after first operation. The patient required multiple additional interventions before closure of the apical muscular ventricular septal defect, such as pyloromyotomy for idiopathic hypertrophic pyloric stenosis, anterior aortopexy for airway obstruction, and balloon aortoplasty for residual coarctation. She is now doing well.

• Journal of Chest Surgery
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• v.31 no.7
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• pp.660-667
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• 1998

Background: This study is to evaluate the effectiveness and application of Lecompte procedure as a treatment for various complex cardiac anomalies with pulmonary outflow tract obstruction. Methods: Between July 1988 and December 1997, 44 patients underwent Lecompte procedure in Seoul National University Children's Hospital. The male to female ratio was 24 to 20 and the mean age was 29.2 months(range, 3 to 83). Of these patients, 28(63.6%) had transposition of great arteries with ventricular septal defect and pulmonary stenosis(or pulmonary atresia), 14(31.8%) had double outlet right ventricle with pulmonary stenosis(or pulmonary atresia), and so on. The principles of the technique are 1) extension of the ventricular septal defect or conal resection, 2) construction of a intracardiac tunnel connecting the left ventricle to the aorta, and 3) direct connection, without a prosthetic conduit, of the pulmonary trunk to the right ventricle. Results: There were 3 in-hospital deaths and their causes were sustained hypoxia, myocardial failure, and sepsis, respectively. There was 1 late death due to sepsis. Reoperations were performed in 6 patients who had pulmonary outflow tract obstructions(4 cases), residual muscular ventricular septal defect(1 case), and recurrent septic vegetation(1 case). The cumulative survival rates by the Kaplan-Meier method were 92.7%, 92.7%, and 92.7% at 1, 2, and over 4 years. The reoperation free survival rates were 92.7%, 92.7%, and 70.2% at 1, 3, and over 5 years. Among the risk factors for the operative death, aortic cross clamping time had statistical significance(p<0.05) and all the risk factors for the recurrent pulmonary stenosis such as age, pulmonary artery index, and materials used for the pulmonary outflow tract reconstruction had no statistical significance(p>0.05). Conclusions: Our review suggests that Lecompte procedure is an effective treatment modality for various complex cardiac anomalies with pulmonary outflow tract obstruction. Repair in early age is possible and the rates of mortality and morbidity are also acceptable.