• 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.30 no.7
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• pp.641-646
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• 1997

Understanding of the surgical anatomy of patients with double outlet right ventricle (DORV) is important in the planning of biventricular repair From May 1995 to September 1996, 7 patients underwent biventricular repair for DORV with remote ventricular septal defect. There were 5 males and 2 (tamales. Age at operation varied from 2 to 9 years(mean 3.4$\pm$ 2.7years). Preoperative diagnostic assessment was made by two-dimensional echocardiography and cardiac catheterization. Ventricular septal defect was perimembranous inlet type in all patients. Associated cardiac anomalies were pulmonary atresia in two, pulmonary stenosis in five and tricuspid chordal attachment to zonal septum in five. The operations were performed intraventricular repair and pulmonary enlargement in two, REV operation in two, and Rastelli operation in three. There was no early postoperative deaths and complications. The follow-up period war from 1 month to 18mon1hs, averaging 10: 6.1 months. In the past, we considered the Fontal operation indicative as primary choice when DORV was associated with abnormal tricuspid chordal attachment to the zonal septum, but now we believe that biventricular repair is feasible for those cases by making conal flap or reattachment method. Biventricular repair has theoretic advantages because it estabilishes normal anatomy and physiology, and it was concluded that the precise preoperative evaluation using both echocardiography and cardiac catheterization was essential to the successful surgery.

• Journal of Chest Surgery
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• v.12 no.3
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• pp.281-288
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• 1979

For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.

• Journal of Chest Surgery
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• v.12 no.3
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• pp.289-296
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• 1979

For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.

• Journal of Chest Surgery
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• v.18 no.4
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• pp.635-642
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• 1985

Double outlet left ventricle [DOLV] is a rare cardiac anomaly in which both great arteries arise entirely, or predominantly above the morphologically left ventricle. About 100 cases of DOLV have been reported in the literatures by 1984. We have experienced eight cases of DOLV at Seoul National University Hospital during the period from October 1981 to July 1905. Ages of the patients were ranged from 12 months to 24 years old, and chief complaints on admission were frequent URI and DOE in 5 cases and cyanosis in other 3 cases. In all eight patients, Cardiac catheterization and cineangiography were performed but pre-operative diagnoses were incorrect except one case [VSD in 2 cases, DORV in 2cases, c-TGA in 2 cases and TOF in one case] We have performed total corrective surgery in seven patients. In case I, patch closure of VSD aligning aorta and pulmonary artery with LV, ligation of proximal pulmonary artery and the use of external valved conduit from RV to PA have been employed. In other 6 cases, intraventricular repair using boomerang shaped Dacron patch with correction of associated anomalies were employed. In remaining one patient who had coexistent PDA and coarctation of aorta, we have performed coarctoplasty and PDA ligation initially and the patient is waiting for subsequent total corrective procedure. In seven patients whom we have performed total corrective surgery, there is one hospital mortality due to right heart failure and one complication of complete heart block necessitating permanent pacemaker implantation. All survivors are doing well in follow up period of 9 months to 4 years. To our knowledge, this is the first report of surgical experiences for DOLV in the Korean literature.

• Journal of Chest Surgery
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• v.24 no.3
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• pp.310-321
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• 1991

Between April 1986 and September 1990, 34 patients with a single or dominant right ventricle underwent modified Fontan procedure for definite palliation in Seoul National University Children`s Hospital. Their age at operation ranged from 8 months to 14 years [Mean 5.5 years]. The ventricular chamber was solitary and of indeterminate trabecular pattern in 6 patients. 28 patients had posteriorly located rudimentary chamber, all of which were trabecular pouches having no communication with outlet septum. The patterns of atrioventricular connection were common inlet[9], double inlet [11], left atrioventricular valve atresia [12] and right atrioventricular valve atresia with L-loop [2]. Pulmonary outflow tracts were atretic in 7 patients and stenotic in 26 patients. Major associated anomalies included anomalous systemic venous drainage [15], dextrocardia [12] and total anomalous pulmonary venous connection[3]. Shunt operations were previously performed in 13 patients and pulmonary artery banding and atrial septectomy in 1 patients. Surgery included intraatrial baffling in 26 patients, bidirectional cavopulmonary shunt in 13 patients, atrioventricular valve obliteration in 3 patients and atrioventricular valve replacement in 3 patients. Central venous pressure measured postoperatively at intensive care unit ranged from 18cm H2O to 28cm H2O [mean 23.2cm H2O]. Hospital mortality was 35.3% [12/34], all died out of low output syndrome. Suspected causes of low output syndrome include ventricular dysfunction [8], hypoplastic or tortuous pulmonary artery [2] and elevated pulmonary vascular resistance [2]. 19 patients had 31 major complications including low output syndrome [18], arrhythmia [4], acute renal failure [3] and respiratory failure [3]. Mortality rate was significantly higher in the groups receiving intraatrial baffling and AV valve replacement respectively [p<0.05]. 20 patients were followed up postoperatively with the mean follow-up period 15.0$\pm$11.6 months. There were no late death and follow-up catheterization was performed in 10 patients. Mean right atrial pressure was 15.4$\pm$6.8mmHg and ventricular contraction was reasonable in all but one case. Thus, Fontan principle can be applied successfully to all the patients with complex cardiac anomaly of single ventricle variety and better results can be anticipated with judicious selection of patient and improvement of postoperative care.

• Journal of Chest Surgery
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• v.25 no.11
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• pp.1327-1336
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• 1992

Between Jan. 1986 and Aug. 1992. 72 patients underwent Rastelli procedure. There were 43 male and 29 female, aged 46 days to 16 years [mean age, 5.2 years] with 18 patients less than 2 years of age. All patients had complex defect, 27 pulmonary atresia with ventricular septal defect, 18 corrected transposition of great arteries with pulmonary atresia or punmonary stenosis, 10 truncus arteriosus, 10 double outlet right ventricle with pulmonary atresia or stenosis, 7 complete transposition of great artersia with pulmonary atresia or pulmonary stenosis. The types of extracardiac valved conduit used were prosthetic valve[n=47, 24 car-bomedics, 19 Ionescu-Shiley, 4 Bjork-shiley] and hand-made trileaflet valve using pericardium. [n=23, 20 bovine pericardium, Z autologous pericardium, 1 equine pericardium] The mean size of valved cinduit was 5.25mm larger in diameter than the size of main pulmonary artery. [normalized to the patient`s body surface area] There were 17 hospital death[24%] and 4 late deaths[5.6%]. Postoperative complication rate was 38.9%a, none of which was conduit-related. All patients were followed pos-toperatively for 1 to 73 months. [mean 25.8 months] During follow-up period, reoperation was done in 6 patients due to stenosis of valved conduit. Mean interval between intial repair and reoperation was 20.3 months. In our experience, li recently extracardaic valved conduits between right ventricle [or pulmonary ventricle] and pulmonary artery were inserted with increasing frequency in infants less than 2 year, but hospital mortality was decreased, 2] Risk of reoperation due to conduit stenosis is low, so that the effect of graft failure on overall survival is minimized. 3] Nevertheless, because any type of extracardaic valved conduit is not ideal in children, we recommended that Lecompte should be done if cardiac anatomy is permitted.

• 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.

• Clinical and Experimental Pediatrics
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• v.57 no.10
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• pp.461-463
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• 2014

Decreased exercise capacity after Fontan surgery is relatively common and the failure of the Fontan state gradually increases with age. However, there is no further treatment for patients with Fontan circulation. Pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the Fontan problem. Inhaled iloprost is a chemically stable prostacyclin analogue and a potent pulmonary vasodilator. We experienced two cases of Fontan patients treated with inhaled iloprost for 12 weeks. The first patient was an 18-year-old female with pulmonary atresia with an intact ventricular septum, and the second patient was a 22-year-old male with a double outlet right ventricle. Fifteen years have passed since both patients received Fontan surgery. While the pulmonary pressure was not decreased significantly, improved exercise capacity and cardiac output were observed without any major side effects in both patients. The iloprost inhalation therapy was well tolerated and effective for the symptomatic treatment of Fontan patients.

• Journal of Chest Surgery
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• v.25 no.9
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• pp.905-911
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• 1992

Automatic junctional tachycardia is one of common atrial arrhythmia after open heart surgery which is often refractory to antiarrhythmic agents. We have experienced refractory automatic junctional tachycardia in two patients. In the first, it occured after cryosurgery for AV nodal reentry tachycardia and simultaneous dissection of a posterior septal bypass tract. In the second, it complicated the postoperative course of a patient who received intracardiac repair for double outlet right ventricle, ventricular septal defect, and pulmonary stenosis. Conventional therapy with atrial pacing, verapamil, digoxin, and electrical cardioversion were ineffective. Therefore, amiodarone was administered intravenously and it controlled automatic junctional tachycardia. The need for accurate and rapid diagnosis of this condition along with results of treatment are discussed.