• Journal of Chest Surgery
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• v.37 no.4
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• pp.313-321
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• 2004

Excellent clinical results of the arterial switch operation and the limited availablity of the intraventricular rerouting has recently made an arterial switch operation to become the therapeutic method of choice for the repair of double-outlet right ventricle (DORV) with subpulmonary ventricular septal defect (VSD). The early and midterm outcomes of arterial switch operation for this anomaly were evaluated. Material and Method: Between August 1994 and July 2002, 13 patients underwent an arterial switch operation for the correction of double-outlet right ventricle with subpulmonary VSD at Dong-A university hospital.. The 50% rule was used to define DORV. Median age and mean body weight were 27 days (range, 3-120 days) and 3.8$\pm$0.7kg (range, 2.92-5.3kg) respectively. Aortic arch anomalies were associated in 6 cases (46.2%), which were all repaired through one-stage operation. The relationship of the great arteries were side-by-side in 8 cases (61.5%) and anteroposterior in 5 (38.5%). Coronary artery patterns were 1 LCx-2R in 6 cases, retropulmonary left coronary artery (LCA) in 6, and intramural LCA in 1 respectively. The enlargement of VSD was required in 1 patient and the patch enlargement of right ventricular outflow tract was performed in another one patient. The Lecompte maneuver was used in all but 3 patients with a side by side relationship of the great arteries. Result: Overall postoperative hospital mortality was 23.1 % (3/13). All operative deaths were occurred in the patients with aortic arch anomalies. There was one late death related to the postoperative complication of the central nerve system during the mean follow-up of 41.3$\pm$30.7 months. Pulmonary valvar stenosis (＞30mmHg of pressure gradient) developed in 1 patient (10%) and left pulmonary artery stenosis in 2 (20%), among them, one required reoperation 52 months after repair. There was an asymptomatic patient with moderate aortic regurgitation. 5-year survival rate including operative deaths was 68.3%. Conclusion: Although the operative mortality is high in the patients with aortic arch anomaly, the arterial switch operation for DORV with supbpulmonary VSD can be performed with low operative mortality and low reoperation rate in the patients Without arch anomaly. The arterial switch operation can be considered a good option for this complex anomaly.

• Journal of Chest Surgery
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• v.32 no.9
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• pp.781-789
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• 1999

Background: Pulmonary artery banding(PAB) accompanies some risks in the aspect of band complications and mortality in the second-stage operation. To assess these risks of the second-stage operation after PAB, we reviewed the surgical results of the second-stage operation in the pediatric patients who had undergone PAB in infancy. Material and Method: From May 1988 to June 1997, a total of 29 patients with preliminary PAB underwent open heart surgery. Ages ranged from 2 to 45 months(mean 20.6$\pm$9.0 months). Preoperative congestive heart failure conditions were improved after PAB(elective operation group) in 27 patients, but early second-stage procedures were required in the remaining 2 patients due to sustaining congestive heart failure(early operation group). Preoperative surgical indications included 2 double outlet right ventricles(DORV group) and 27 ventricular septal defects as the main cardiac anomaly(VSD group). Result: The mean time interval from PAB to the second-stage operation was 15.5$\pm$8.7 months(range 5 days to 45 months). One patient in the DORV group underwent intraventricular tunnel repair and modified Glenn procedure in the other. In the VSD group, the VSD was closed with a Dacron patch in all patients. Concomitant procedures included a right ventricular infundibulectomy in 4 patients and a valvectomy of the dysplastic pulmonary valve in 1 patient. At the second-stage operations, pulmonary angioplasty was required due to the stenotic banding sites in 18 patients. One patient underwent complete ligation of the main pulmonary artery with the modified Glenn procedure. The mortality at the second-stage operation was 17.2%(5 patients). Causes of death were 4 low cardiac output, and 1 autoimmune hemolytic anemia. Diagnosis with DORV and the early operative group were the risk factors for operative death in this series. There was 1 late death. Conclusion: This study revealed the second-stage operation for pulmonary artery debanding and closure of VSD in children was complicated by the correction of the acquired lesions with a significantly high incidence of morbidity and early postoperative deaths. Primary repair is recommended for isolated VSD, if possible.

• Journal of Chest Surgery
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• v.36 no.9
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• pp.687-690
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• 2003

Although surgical options for double outlet right ventricle (DORV) with non-committed ventricular septal defect (VSD) are vary in accordance to the morphological characteristics, it is very difficult to use biventricular repair technique when there is tricuspid chordae originating from conal septum or when the distance between the tricuspid valve and the pulmonic valve is too short. We report our clinical experience of biventricular repair of DORV with non-committed VSD by VSD rerouting to the pulmonary artery and arterial switch in case of a presence of conal tricuspid chordae and short distance between the tricuspid valve and the pulmonic valve.

• Journal of Chest Surgery
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• v.29 no.3
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• pp.263-270
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• 1996

Twenty two patients underwent total anatomic correction of complete atrioventricular septal defect associated with other cardiac anomalies between July 1986 and December 1994. Age ranged from 6 months to 11 years(mean 49.6 $\pm$ 35.8 months), and they were composed of 7 males and 15 females. Combined major cardiac anomalies were tetralogy of Fallot(TOF) in 11 cases, double outlet of right ventricle (DORV) in 6 ca es, and transposition of great arteries (TGA) in 5 cases. Down's syndrome was associated in 5 patients with TOF and 1 patient with DORV. They were classified as Rastelli type A in 3 patients, B in 2 patients, and C in 17 patients. Modified Blalock-Taussig shunt was performed.in 5 patients and Waterston shunt in 1 patient as a palliative procedure. There were 7 perioperative deaths(31.8%) and the causes were pump weaning failure, low cardiac output, acute renal failure, persistant pulmonary hypertension and hypertensive crisis, and sepsis. Reoperations were performed in 4 cases to repair atrioventricular valvular regurgitation or to relieve the right ventricular outflow tract (RVOT) or pulmonary arterial stenosis. One late death was due to aspiration pneumonia. Second reoperation was necessary for progressive worsening of left atrioventricular regurgitation and RVOT stenosis in one patient. Fourteen survived patients were followed up for a mean of 66.0 $\pm$ 26.7months and all of them w re NYHA functional class I or II.

• 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.

• 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.18 no.1
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• pp.36-45
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• 1985

During the twelve-year period from March 1973 through July 1984, 23 consecutive operations for coarctation of the aorta were performed at Seoul National University Hospital. The patients included 19 male and 4 female in the range of 4 months and 16 years old. Associated cardiac anomalies were present in 19 patients [70%] and they were VSD+PDA [9 patients], VSD[2], PDA[1], VSD+ASD+PDA[1], VSD+MS+AS+PDA[1], D-TGA+VSD+PDA[1], P-ECD[1], MS[1], Al[1], and DORV+PDA[1]. The preoperative main symptoms included congestive heat failure, hypertension, subacute bacterial endocarditis and nonspecific symptoms. Congestive heart failure was the most common symptom in the group younger than 2 years and hypertension in the adult group. Operative techniques for coarctation of the aorta were resection and end to end anastomosis in 10 patients, prosthetic patch aortoplasty in 8, subclavian flap aortoplasty in 4, and LSCA-aortic anastomosis in 1. There were 4 operative deaths among the nine patients less than 2 years old[44.4% mortality]: all of these patients had associated cardiac anomalies. And only one operative death occurred in patients older then 2 years old[7.1% mortality]. No hospital death occurred in patients with isolated coarctation of the aorta. Operation of the coarctation was performed primarily in 6 patients associated with ventricular septal defect and subsequently underwent successful VSD closure except one operative death.

• Journal of Chest Surgery
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• v.20 no.1
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• pp.22-29
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• 1987

From Sep. 1978 to Aug. 1986, 44 cases of Fontan operation were performed at Seoul National University Hospital. 1] The diagnoses were TA in 13 [38.6%], UVH in 21 [47.79`], DORV in 3 [6.8%], TGA in 2 [4.5%] and C-ECD with DORY in 1[2.3%]. 2] There were 20 operative deaths [44.5%]. 3] The operative risk factors were early date of operation between 1978 and 1983, young age below 3 years old, direct atriopulmonary anastomosis without roofing, and postoperative high CVP above 25cmH,O. But the relation between operative mortality and various cardiac diseases was absent. 4] survived patients were followed from 1 to 54 months except 3 patients who were lost to follow up. 16 patients were in functional class I and 1 in class II, 2 of the above 17 patients were reoperated due to residual right to left shunt. In remained 4 patients, 3 patients persisted cyanosis after operation and 1 patients died 1 month postoperatively due to pulmonary embolism. 5] As 4 result, the Fontan procedure can be done with a good result for tricuspid atresia and other complex lesions. The operative mortality can be reduced further with a correct anatomical diagnosis preoperatively, rigid operative criteria to pulmonary vascular resistance, direct atriopulmonary anastomosis with roofing, and use of `Venous Assist Device` postoperatively in low cardiac output patients.

• Journal of Chest Surgery
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• v.22 no.6
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• pp.944-950
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• 1989

Children with congenital cardiac defects associated with high pulmonary artery pressure may die despite accurate surgery. Postoperative mortality and morbidity have been attributed to acute rises in pulmonary artery pressure and resistance. Acute pulmonary hypertensive crisis is defined as a paroxysmal event in which pulmonary arterial systolic pressure rises to or above systemic levels followed by a rapid fall in systemic pressure and a minor pulmonary hypertensive event is defined as an acute rise in pulmonary arterial pressure to more than 80 % of systemic levels but without a fall in systemic pressure. From Oct. 1988 to Jul. 1989, we experienced 23 patients who showed many pulmonary hypertensive crises after operation in the Department of Thoracic and Cardiovascular Surgery, Seoul National University Children\ulcorner Hospital. Their preoperative PAP/SAPs were 53 to 123 %[mean 93.3%] and diagnoses were VSD[7], TAPVR[5], TGA[4], AVSD[3], MS[1], DORV[1], Truncus arteriosus[1], and AP window[l]. There were 9 deaths among 23 patients and they showed many pulmonary hypertensive crisis episodes during postoperative intensive care, which was managed by sedation, hyperventilation, oxygen, and acidosis correction and which decreased after using tolazoline. In view of our experience, we recommend that pulmonary artery pressure should be monitored in congenital heart defected patient with preoperative pulmonary hypertension to confirm and to manage the pulmonary hypertensive crisis accurately and using tolazoline is helpful in the treatment of pulmonary hypertensive crisis.

• 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.