• Journal of Chest Surgery
• /
• v.33 no.4
• /
• pp.290-300
• /
• 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
• /
• v.9 no.2
• /
• pp.198-206
• /
• 1976

A Total of Forty eight patients underwent open-heart surgery for correction of tetralogy of Fallot at the Seoul National University Hospital from January 1974 to October 1976, with an overall survival rate of 77 per cent. Operative mortality varied according to severity of the lesion, age of the patient, nature of previous surgical treatment and presence or absence of an outflow tract patch across the pulmonary valve ring. Eleven patients died in the early postoperative period and thirty seven patients were discharged from the hospital alive. A patch of the right ventricular outflow tract and pulmonary annulus was required to relieve pulmonic stenosis in 24 patients. There were 10 deaths in this group (42%) as compared to 1 death in the group of 24 patients who were corrected without a patch. Operative mortality was especially higher when an inlay patch was placed across the pulmonary valve ring. This may be related to the possibly greater anatomic severity of these cases and to the longer operating time when a patch was used. The electrocardiogram showed right ventricular hypertrophy in 35 cyanotic patients. Intraventricular conduction was normal in 34 patients before operation. It was normal postoperatively in only 5 of 34 patients in this group who survived surgery. Complete right bundle branch block appeared at operation in 21 patients, and 8 patients developed incomplete right bundle branch block. Major causes of death were progressive cardiac failure (4), Complete atrioventricular dissociation (3), bleeding (2), cardiac tamponade (1), and sudden cardiac arrest (1)

• Journal of Chest Surgery
• /
• v.28 no.11
• /
• pp.1038-1044
• /
• 1995

We have been used cryopreserved homograft valves for right ventricular outflow tract[RVOT reconstruction since November 1993. The homograft valves were harvested from the hearts of brain dead patients or hearts of transplant recipients. There were 12 male and 10 female patients. Their ages ranged from 5 months to 13 years[mean age,39.2 $\pm$ 37.4 months and the weight ranged from 5 to 48kg [mean weight, 13.7$\pm$ 9. l kg . The diagnoses included pulmonary atresia with ventricular septal defect [n=14 , tetralogy of Fallot[n=4 , truncus arteriosus[n=3 , and double outlet right ventricle with pulmonic stenosis[n=l .Monocuspid homograft patches were used for RVOT widening or REV[reparation l`etage ventriculaire operations in 4 patients. We also used homograft as valved conduits for RVOT reconstruction in 17 patients and left ventricular outflow tract reconstruction in anatomically corrected transposition in 1 patient. Among them size-reducing technique [converting a tricuspid valved conduit into a bicuspid valved conduit were applied to six patients for the correction of size mismatching. The mean follow-up period was 10.6 $\pm$ 5.4 months. There was one operative death[4.5% due to bleeding and one reoperation for removal of vegetation on the homograft leaflet. Postoperative echocardiography documented no significant homograft insufficiency and RVOT obstructions.In short-term, the homograft valves provide excellent hemodynamic characteristics, even though further studies are necessary to evaluate the long-term results.

• Journal of Chest Surgery
• /
• v.18 no.2
• /
• pp.174-181
• /
• 1985

From Jun. 1980 to Mar. 1984, 33 cases of atrial septal defect, secundum type, operated at the department of Thoracic and Cardiovascular Surgery, School of Medicine, Keimyung University, were analyzed retrospectively. They were 24.3% of all congenital heart diseases operated in the same period. Among the 33 cases, 17 cases were males and 16 cases were females. Their ages were ranged from 4 to 42 years and cases of over 15 years of age were 60.6%. Main symptoms at admission were exertional dyspnea [69.7%], palpitation [63.6%] and frequent upper respiratory infections [51.5%], but 2 cases [6.1%] were asymptomatic. All 33 cases were operated under the direct vision with cardiopulmonary bypass. All cases were secundum type defect of atrial septal defect and single defect were in 29 cases [90.6%], and oval type defect were in 31 cases [96.9%]. In 7 cases [21.9%], other lesions of cardiovascular system were associated, and the most common lesion was pulmonic valvular stenosis [4 cases; 12.5%]. The defects were closed directly in 27 cases and in 6 cases with Dacron patch. Postoperative complications were occurred in 8 cases [24.2%], and they were pleural effusion, congestive heart failure, and alopecia mainly. One case died due to air embolism postoperatively and operative mortality was 3.1%.

• Journal of Chest Surgery
• /
• v.18 no.2
• /
• pp.151-156
• /
• 1985

A clinical analysis was done on 50 cases of ventricular septal defect, operated from April 1981 to March 1984 at the department of Thoracic and Cardiovascular Surgery, School of Medicine, Keimyung University. Among 50 cases, 34 cases were males and 16 cases were females. Their age ranged from 1 to 26 years and the mean age was 9.7 years. The main symptoms at admission were frequent upper respiratory infection [50%], exertional dyspnea [42%] and palpitation [34%]. In anatomical classification by Kirklin, type I constituted 20%, type II 76%, type IV 4%. Associated congenital cardiac lesions were pulmonic stenosis [6 cases], patent foramen ovale [5 cases], aortic insufficiency [3 cases] and persistent left superior vena cava [1 case]. When a normal electrocardiogram pattern was present, Qp/Qs, Rp/Rs and pulmonary artery systolic pressure and Pp/Ps were relatively low. Among cases of above 1 cm2/M2 BSA in size of defect, Pp/Ps and pulmonary artery systolic pressure were increased than the cases of below 1 cm2/M2 BSA [P=0.01]. The postoperative right bundle branch block was occurred in 21 cases [75%] among 28 cases of right ventriculotomy approach. The operative mortality was 2% [1 case] among 50 cases and complication rate was 14% [7 cases].

• Journal of Chest Surgery
• /
• v.24 no.2
• /
• pp.115-122
• /
• 1991

From April 1986 to December 1989, 25 infants under the age of 12 months with tetralogy of Fallot were operated on. Age ranged from 3 to 12 months[mean 8.9$\pm$4.9 months] and mean body weight was 7.8$\pm$ 2.6kg. All the patients were deeply cyanotic, 12 of them experienced anoxic spell. Transannular patch was laid down in 19 patients, in 7 of them monocuspid patch was utilized. Postrepair P RV/LV was measured at operation room in 17 patients[mean 0.48$\pm$0. 14]. Hospital mortality was 20Yo. Causes of deaths include right ventricular failure and low cardiac output. The mortality was closely related with patient`s age and body surface area at operation. Also higher mortality was noticed in patients having major associated anomaly or previous palliative operation, preoperative management with propranolol and transannular repair. 18 patients were followed up for 12 to 50 months with a mean follow-up time of 24 months after operation. There were no late deaths and late ventricular arrhythmia or congestive heart failure was not detected as yet. Redo operation was performed in one case because of residual pulmonic stenosis. Considering several advantages of early primary repair, primary repair of symptomatic infants with tetralogy of Fallot should be encouraged despite somewhat high mortality rate as yet and better results could be anticipated along with improvement of myocardial protection method and postoperative care.

• Journal of Chest Surgery
• /
• v.25 no.1
• /
• pp.23-31
• /
• 1992

The ideal approach in the staged management of patients with pulmonary atresia has been a challenging problem and the result has not been always satisfactory. We reviewed our early result of initial palliative surgeries in fifteen cases of pulmonary atresia with interventricular communication Included are eight cases of simple pulmonary atresia with ventricular septal defect and seven cases of pulmonary atresia associated with other complex cardiac anomalies. The ages of the patients were less than one year except one. The morphology of pulmonary vasculature was highly variable and showed unfavorable conditions in most cases. Pulmonary artery was nonconfluent in two. Two-thirds of all cases showed significant problems such as juxtaductal stenosis or diffuse hypoplasia. The ductus arteriosus usually narrowed at its pulmonic end. Initial palliation was done by modified Blalock-Taussig shunt in six, central shunt with or without pulmonary angioplasty in five, right ventricular outflow tract [RVOT] reconstruction in three and direct connection of nonconfluent pulmonary arteries with bilateral cav-opulmonary shunt in one patient. There were 3 hospital deaths. Two of them underwent simultaneous repair of the associated anomaly of TAPVR. Among the six patients who received modified Blalock-Taussig shunt, three needed early second palliative procedure by central shunt, RVOT patch reconstruction and pulmonary angioplasty in each case, All patients who received central shunt showed marked clinical improvement. Among the twelve patients who survived the palliative procedures, two patients underwent total correction 13 months and 18 months after initial palliation respectively. We think that the choice of palliative procedure must be individualized according to the morphology of the pulmonary arteries. More experience and long term follow-up data are necessary to meet this challenging problem.

• Journal of Genetic Medicine
• /
• v.16 no.1
• /
• pp.1-9
• /
• 2019

Noonan syndrome (NS) and NS-related disorders (cardio-facio-cutaneous syndrome, Costello syndrome, NS with multiple lentigines, or LEOPARD [lentigines, ECG conduction abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, retardation of growth and sensory neural deafness] syndrome) are collectively named as RASopathies. Clinical presentations are similar, featured with typical facial features, short stature, intellectual disability, ectodermal abnormalities, congenital heart diseases, chest & skeletal deformity and delayed puberty. During past decades, molecular etiologies of RASopathies have been growingly discovered. The functional perturbations of the RAS-mitogen-activated protein kinase pathway are resulted from the mutation of more than 20 genes (PTPN11, SOS1, RAF1, SHOC2, BRAF, KRAS, NRAS, HRAS, MEK1, MEK2, CBL, SOS2, RIT, RRAS, RASA2, SPRY1, LZTR1, MAP3K8, MYST4, A2ML1, RRAS2). The PTPN11 (40-50%), SOS1 (10-20%), RAF1 (3-17%), and RIT1 (5-9%) mutations are common in NS patients. In this review, the constellation of overlapping clinical features of RASopathies will be described based on genotype as well as their differential diagnostic points and management.

• Journal of Chest Surgery
• /
• v.36 no.12
• /
• pp.921-927
• /
• 2003

Arterial switch operation (ASO) has been the most effective surgical option for transposition of the great arteries. But, the inappropriate dilation of the neoaortic root has been reported and its effect on neoaortic valve function and growth of aorta has not been well documented. Material and Method: Forty-eight patients who underwent cardiac catheterization during follow up after arterial switch operation were included in this study. Arterial switch operation was performed at a median age of 18 days (range 1∼211 days). Preoperative cardiac catheterization was performed in 26 patients and postoperative catheterization was performed in all patients at 15.8$\pm$9.6 months after ASO. Postoperative ratios of the diameters of neoaortic annulus, root and aortic anastomosis against the descending aorta were compared to the size of preoperative pulmonary annular, root and sinotubular junction. Preoperative and operative parameters were analyzed for the risk factors of neoaortic insufficiency. Result: There were two clinically significant neoaortic insufficiencies (grade$\geq$II/IV) during follow up, one of which required aortic valve replacement. Another patient required reoperation due to aortic stenosis on the anastomosis site. Post-operatively, neoaortic annulus/DA ratio increased from 1.33$\pm$0.28 to 1.52$\pm$.033 (p=0.01) and neoaortic root/DA ratio increased form 2.02$\pm$0.40 to 2.56$\pm$0.38 (p<0.0001). However, the aortic anastomosis/DA ratio showed no statistically significant difference (p=0.06). There was no statistically significant correlation between the occurrence of neoaortic insufficiency and neoaortic annulus/DA ratio and neoaortic root/DA ratio. Non-neonatal repair (age>30days) (p=0.02), preopeative native pulmonaic valve stenosis (p=0.01), and bisuspid pulmonic valve (p=0.03) were the risk factors for neoaortic insufficiency in univariate risk factor analysis. Conclusion: After ASO, aortic anastomosis site showed normal growth pattern proportional to the descending aorta, but neoaortic valve annulus and root were disproportionally dilated. Significant neoaortic valve insufficiency rarely developed after ASO and neoaortic annulus and root size do not correlate with the presence of postoperative neoarotic insufficiency. ASO after neonatal period, preoperative native pulmonary valve stenosis, and bicuspid native pulmonic valve are risk factors for the development of neoaortic insufficiency.

• Journal of Chest Surgery
• /
• v.38 no.12 s.257
• /
• pp.852-855
• /
• 2005

A 85-day-old infant was successfully operated on for truncus arteriosus (type I) with interrupted aortic arch (type A) using one-stage anterior approach without circulatory arrest. Aortic arch was reconstructed by direct anastomosis of ascending aorta and descending aorta with regional perfusion and continuity of right ventricle to pulmonary artery was established with $Shelhigh^{circledR}$ pulmonic conduit. The patient experienced left bronchus compression by descending aorta immediately postoperatively, which was improved with positional change and physiotherapy. The patient had reoperation due to stenosis of valved conduit at 13 months later. The patient is currently well under follow-up of 14 months from initial repair.