• 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.26 no.9
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• pp.701-704
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• 1993

Four to seven percent of infants born with aortic atresia have a normal-sized left ventricle in association with a ventricular septal defect and a normal mitral valve. In contrast to the more common group of infants with aortic atresia whose left ventricle is hypoplastic, this important subgroup has potential for complete operative correction involving both the right and left ventricle. Our approach was to perform complete repair at one stage. The patient who underwent single-stage repair was discharged from the hospital in good condition. Achieving a physiologically normal circulation simplifies the postoperative management of this condition.

• 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.12 no.2
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• pp.135-139
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• 1979

Interruption of the aortic arch may be defined as discontinuity of the aortic arch in which either an aortic branch vessel or a patent ductus arteriosus supplies the descending aorta. This uncommon lesion was described first by Raphe Steidele in 1778 and was later classified into 3 types by Celoria and Patton. This anomaly rarely occurs as an isolated anomaly. Most commonly, a ventricular septal defect, patent ductus arteriosus, and abnormal arrangement of the brachiocephalic arteries occurs together with arch anomaly. Rarely, more complex anomaly, such as transposition of the great vessel, or single ventricle, is coexistent. We present the case of an 6 year-old boy with D-transposition of great vessel single ventricle, patent ductus arteriosus and patent foramen ovale with interruption of the aortic arch (Type A).

• Journal of Chest Surgery
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• v.51 no.3
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• pp.167-171
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• 2018

Background: Heart transplantation (HTx) can be a life-saving procedure for patients in whom single ventricle palliation or one-and-a-half ($1\text\tiny{1/2}$) ventricle repair has failed. However, the presence of a previous bidirectional cavopulmonary shunt (BCS) necessitates extensive pulmonary artery angioplasty, which may lead to worse outcomes. We sought to assess the post-HTx outcomes in patients with a previous BCS, and to assess the technical feasibility of leaving the BCS in place during HTx. Methods: From 1992 to 2017, 11 HTx were performed in patients failing from Fontan (n=7), BCS (n=3), or $1\text\tiny{1/2}$ ventricle (n=1) physiology at Asan Medical Center. The median age at HTx was 12.0 years (range, 3-24 years). Three patients (27.3%) underwent HTx without taking down the previous BCS. Results: No early mortality was observed. One patient died of acute rejection 3.5 years after HTx. The overall survival rate was 91% at 2 years. In the 3 patients without BCS take-down, the median anastomosis time was 65 minutes (range, 54-68 minutes), which was shorter than in the patients with BCS take-down (93 minutes; range, 62-128 minutes), while the postoperative central venous pressure (CVP) was comparable to the preoperative CVP. Conclusion: Transplantation can be successfully performed in patients with end-stage congenital heart disease after single ventricle palliation or $1\text\tiny{1/2}$ ventricle repair. Leaving the BCS in place during HTx may simplify the operative procedure without causing significant adverse outcomes.

• Journal of the Korean Society of Radiology
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• v.5 no.5
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• pp.289-294
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• 2011

The aim of this study is to evaluate the differences of ejection fraction of left ventricle through the quantitative analysis of diastolic and systolic volumes according to slices selected using cardiac MR imaging. A total of 12 volunteers (7 normal, 1 myocardium bridge, and 4 arrhythmia) underwent cardiac MRI on a MR scanner(Magnetom Trio, Siemens, Germany). Ejection fractions for quantitative analysis were calculated at single slice of center of left ventricle, 3, 5, and 6-7 slices extending from the center of left ventricle. Average values were analyzed for evaluating differences of ejection fraction according to the number of slices selected. Mean value of normal person of ejection fraction were 67.14% at single slice of center of left ventricle, 66.24% at 3 slices, 65.63% at 5 slices, and 65.29% at 6-7 slices. While ejection fraction obtained from a patient with 61.74% at single slice of center of left ventricle, 60.92% at 3 slices, 60.89% at 5 slices, and 61.89% at 6-7 slices. There was no significant differences by the number of slices selected. This study demonstrates that ejection fraction obtained from single slice of center of left ventricle may represent a optimum parameter for cardiac function, instead of the value calculated on the variable slices selected.

• Korean Journal of Radiology
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• v.22 no.3
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• pp.308-323
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• 2021

An increasing number of adult congenital heart disease (ACHD) patients continue to require life-long diagnostic imaging surveillance using cardiac CT and MRI. These patients typically exhibit a large spectrum of unique anatomical and functional changes resulting from either single- or multi-stage palliation and surgical correction. Radiologists involved in the diagnostic task of monitoring treatment effects and detecting potential complications should be familiar with common cardiac CT and MRI findings observed in patients with repaired complex ACHD. This review article highlights the contemporary role of CT and MRI in three commonly encountered repaired ACHD: repaired tetralogy of Fallot, transposition of the great arteries after arterial switch operation, and functional single ventricle after Fontan operation.

• Clinical and Experimental Pediatrics
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• v.51 no.10
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• pp.1118-1122
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• 2008

A case of a single coronary artery complicated with a coronary artery fistula (CAF) to the right ventricle is extremely rare, and its management strategy and prognosis are not clear. A 5-year-old boy was hospitalized for evaluation of a continuous heart murmur. Transthoracic echocardiography suggested a CAF to the right ventricle, with an enlarged left coronary artery. Cardiac catheterization confirmed the CAF terminating at the right ventricle and the absence of a right coronary artery. The fistula was ligated at the right ventricular side under cardiopulmonary bypass. At follow-up 18 months later, the child was clinically asymptomatic, and coronary angiogram showed no recurrence of the fistula.

• Journal of Chest Surgery
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• v.16 no.1
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• pp.18-29
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• 1983

Thirty-two patients with a cyanotic cardiac malformations having more complex intracardiac defects than ones in a tetralogy of Fallot underwent complete intracardiac repairs in a-full-year period from July 1981 to June 1982. Twenty-two patients [68.8%] died within 30 days after surgery: Transposition of the great arteries, seven of 10 patients; Double-outlet right ventricle, four of 6 patients; Tricuspid atresia, four of 6 patients; Single ventricle, all of 4 patients; Pulmonary atresia, two of 3 patients; Double-outlet left ventricle, none of 2 patients; and Truncus arteriosus, one of a single patient. All deaths occurred with a low cardiac output syndrome or a failed off-bypass, and they were almost always accompanied with other grave postoperative complications. The complex intracardiac anatomy itself was one of the risk factors by making a complete intracardiac repair of the defects difficult in a small heart. The reconstruction of the right ventricular outflow carried a difficulty in balancing an adequate relief of the obstruction with an avoidance of making too much pulmonary valvular insufficiency as well. On the other hand, the presence of an elevated pulmonary arterial pressure and a high pulmonary vascular resistance was also the factors affecting the postoperative surviv als. The importance of detailed knowledge of intracardiac anatomy and hemodynamics from the careful preoperative evaluation of the patient was discussed along with the necessity of technical refinement of the correction.

• The Korean Journal of Physiology and Pharmacology
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• v.6 no.3
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• pp.149-154
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• 2002

The blood pressure (BP) is regulated by the nervous system and humoral factors, such as renin- angiotensin system, vasopressin and others. In the present study, we examined the central effects of glutamate and GABA on the cardiovascular regulation by injection of these substances into the lateral ventricle and also investigated the relationship between these central effects and the action of angiotensin II (Ang). Male Sprague Dawley rats, $350{\sim}400$ g, were anesthetized with urethane and instrumented with an arterial catheter for direct measurement of BP and heart rate (HR), and an guide cannula in the lateral ventricle for drug injection. A glass microelectode was inserted into the rostral ventrolateral medulla (RVLM) for recording single unit spikes. Barosensitive neurons were identified by changes of single unit spikes in RVLM following intravenous injection of nitroprusside and phenylephrine. The effects of GABA and glutamate injected into the lateral ventricle were studied in single neuronal activity of the RVLM in addition to changes in BP and heart rate, and compared the results before and after treatment with intravenous losartan, nonpeptide Ang II-type 1 receptor antagonist (1 mg/100 g BW). Intracerebroventricular administration of GABA decreased systolic blood pressure (SBP) and HR, but increased the firing rates in the RVLM. However, intracerebroventricular glutamate injection produced effects opposite to GABA. After pretreatment of intravenous losartan, the central effects of GABA on BP and firing rate in the RVLM were significantly attenuated and that of glutamate showed a tendency of attenuation. These results suggested that central GABA and glutamate regulated BP and firing rates in RVLM were inversely related to BP change. The central effects of GABA or glutamate on the autonomic nervous function were modulated by humoral factor, Ang II, by maintaining BP.