• Journal of Chest Surgery
• /
• v.28 no.6
• /
• pp.565-570
• /
• 1995

Seventy-three patients with isolated total anomalous pulmonary venous connection the patients associated with other major cardiac anomalies such as single ventricle, DORV[Double Outlet Right Ventricle and large VSD[Ventricular Septal Defect were excluded were underwent surgical repair from January 1980 through October 1993. There were 45 boys and 28 girls. The mean age at operation was 19.9 months[range 6 days to 24 years and mean body weight was 7.1kg[range 2.6kg to 45kg . The anomalous locations of connection were supracardiac in 38, cardiac in 21, infracardiac in 5, and mixed in 9. In 38 patients[52% , the venous drainage was obstructed. The obstruction ratios according to the connection type were as follows: 53%[28/38 in supracardiac, 52%[11/21 in cardiac, 100%[5/5 in infracardiac, 22%[2/9 in mixed type. The associated cardiac anomalies were persistent left SVC[2 , tricuspid valve regurgitation[3 , cor triatriatum[1 , and mitral cleft[1 . And associated noncardiac anomalies were imperforate anus[1 and Neil Weightman syndrome[1 . The operative mortality was 23%. The causes of death were pulmonary hypertensive crisis, perioperative myocardial failure, pneumonia with sepsis, arrhythmia and etc. The statistically significant factors in postoperative mortality were the pulmonary venous obstruction and age [p<0.01 . The operative mortality was high in groups of age under 1 month and pulmonary venous obstruction. The mean follow-up was 27.1 months. There were two late deaths. The first patient was three months old boy with supracardiac type and severe obstructive symptoms. The postoperative echocardiography was showed anastomotic stenosis and reoperations were performed twice but the patients expired due to pneumonia and sepsis. The second patient was three month old boy with supracardiac type and total correction was done and was doing well postoperatively. Eight years later, he expired suddenly due to arrhythmia. But all the other patients were in NYHA Fc I and received no medications. The 5-year survival rate excluding early expired patients is 97.1 $\pm$ 0.03 %. In conclusion, although the operative mortality of total anomalous pulmonary venous connection was relatively high compared to other major cardiac anomalies, we could expect excellent long-term results by early surgical correction.

• Journal of Chest Surgery
• /
• v.12 no.3
• /
• pp.281-288
• /
• 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
• /
• v.12 no.3
• /
• pp.289-296
• /
• 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 Yeungnam Medical Science
• /
• v.13 no.2
• /
• pp.225-233
• /
• 1996

From August 1992 to July 1996, 63 consecutive patients underwent coronary artery bypass surgery. The mean age of these patient was 57 years(range form 30 to 71years). There were 44 men and 19 women. Preoperative 12 patients had stable angina pectoris and 23 patients were unstable angina pectoris. 8 patients had previous myocardial infarctation history and emergency or urgent myocardial revascularization were performed in 9 cases. In the risk factors of coronary atherosclerosis, 25 patients(40%) were hypercholesterolemia, 38 patients(60%) have smoking history and 19 patients(30%) have hypertension history. In the patterns of disease, 9 patients were single vessel disease, 18 patients were two vessele disease and 33 patients were three vessel disease. We performed total 284 distal anastomosis(mean 3.5 anastomosis per patient) and performed one case of ascending aorta graft interposition, two cases of mitral valve replacement, one case of aortic valve replacement, one case of ventricular septal defect repair and one case of atrial septal defect repair and the mean aortic cross clamp time was 115.3 minutes. The common complications were arrhythmia(7cases), wound infection(5cases), perioperative myocardial infarction(4cases), reoperation for bleeding control(4cases) and stroke(4cases). There were six hospital deaths due to low cardiac output syndrome, ventricular arrhythmia and respiratory failure. In the evaluation of operative risk factors, preoperative intravenous nitroglycerin requirement and prolonged aortic cross clamp tirne(>2hours) were found to be predective factor of morbidity and old age(>65years) was found to be predective factor of mortality.

• Journal of Chest Surgery
• /
• v.35 no.12
• /
• pp.854-861
• /
• 2002

In this study, we reviewed our early and long-term surgical results of complete atrioventricular septal defect during the last 16 years at our hospital. Materials and Methods: Between April 1986 and March 2002, 73 patients with complete atrioventricular septal defect underwent total correction without preceding palliation. Age at repair ranged from 2 to 85 (median age, 7) months, and weight ranged from 3 to 22 (median weight, 5.9)kg. Follow-up was complete with a mean duration of 69$\pm$51 months. Results: Overall operative mortality was 16.4%(12) with 3 late deaths. One, 5, and 10 year actuarial survival rates were 96.3 %, 94.2 % and 94.2 % respectively Sixteen of 61 (22.2%) operative survivors have undergone reoperation for postoperative mitral regurgitation or left ventricular outflow tract obstruction (LVOTO). Freedom from mitral reoperation at 1, 5, and 10 years were 87.8 %, 72.4 %, and 57.8 % and freedom from LVOTO at 1, 5, and 10 years were 98.2 %, 86.3 %, and 83.2 % respectively. Summary: In this study, we found that our early surgical results improved with quite an acceptable long-term outcome. Close observation of remaining mitral regurgitation was necessary, A precise evaluation of the atrioventricular valve morphology, a meticulous surgical technique, and the adequate postoperative management are mandatory for the excellent results.

• Clinical and Experimental Pediatrics
• /
• v.46 no.7
• /
• pp.661-667
• /
• 2003

Purpose : This study was performed to find the chief clinical problems associated with the ages of adult patients of tetralogy of Fallot(TOF) who had undergone total correction. Methods : Of the 30 patients who were registered at the Grown-Up Congenital Heart Disease (GUCH) Clinic of Samsung Medical Center for TOF, a retrospective investigation was carried out on 28 patients who underwent total correction. Results : Mean age at retrospective study was 30.8(range : 16-53) years old. Age at total correction was 15.8(range : 2-49) years old. Problems after corrective surgery were assessed. They were arrhythmia, pulmonary valve regurgitation, left pulmonary artery stenosis, residual ventricular septal defect, mitral valve regurgitation, tricuspid valve regurgitation, right ventricle outflow tract obstruction, aortic valve regurgitation, infective endocarditis and protein losing enteropathy. After repair of TOF, such arrhythmias as atrial arrhythmia and AV conduction disturbances were observed in some patients. Cardiomegaly was found significantly in the subjects with arrhythmia(P<0.05), and arrhythmia was less observed in patients who underwent surgery at a young age. Eight patients required a reoperation; the main indications were residual ventricular septal defect, right ventricle outflow tract obstruction and peripheral pulmonary artery stenosis. Conclusion : The majority of the patients seemed to live normal lives after Tetralogy of Fallot repair. However, as residual anatomic and functional abnormalities exist postoperatively, continued careful follow-up is needed to detect and correct structural and functional abnormalities.

• Journal of Chest Surgery
• /
• v.32 no.7
• /
• pp.665-667
• /
• 1999

We report a case of ruptured sinus of Valsalva aneurysm in 48-year-old male, presenting the symptom of congestive heart failure. Echocardiography showing a hairpin-like sac ex tended from the left coronary sinus to the left ventricle adjacent to the anterior mitral valve leaflet, suggested ruptured sinus of Valsalva aneurysm or aorto-left ventricular tunnel. Operative findings revealed that left sinus of Valsalva aneurysm had multiple openings faced left ventricle, 7 mm in diameter, 20 mm in length. The proximal opening was closed with bovine pericardium and distal openings were closed with spaghetti pledgeted reinforced sutures. The patient was discharged on the 14th postoperative day, and follow up for 8 months uneventfully. This case was reported for its rarity and to describe the techniques of surgical repair.

• Journal of Chest Surgery
• /
• v.42 no.5
• /
• pp.630-634
• /
• 2009

According to the 2007 International Society for Heart and Lung Transplantation (ISHLT) report, a congenital diagnosis, infantile transplantation and being on extracorporeal membrane oxygenation (ECMO) at the time of transplant are risk factors for mortality for the patients who undergo a heart transplant, and a large body weight ratio also increases the risk of mortality. The patient of this case underwent a Ross operation and mitral valve repair due to left ventricle outflow track obstruction and mitral regurgitation. But the baby was treated with ECMO due to heart failure after the operation. When he was 3-months-old and had been. on 30 days of ECMO, he underwent a heart transplant with a heart that had a high donor-recipient weight ratio (4.42). We present this case from a technical standpoint and we include a review of the relevant literature.

• Journal of Chest Surgery
• /
• v.38 no.7 s.252
• /
• pp.483-488
• /
• 2005

Aortic valve-sparing procedures could reduce valve-related morbidity, but may increase operative risks; therefore, these procedures could not be performed routinely. We attempted to find out the early results while focusing on the operative risks associated with these procedures in our hospital. Material and Method: From May 1996 to July 2003, 26 patients underwent these procedures including 15 patients with Marfan syndrome and 1 patient with Behcet disease. There were 17 men and 9 women with mean age of $37.9\pm19.2$ years (range: 6 months-74 years). Ten patients had ascending aortic dissection, 18 patients had more than moderate degree of aortic valve insufficiency (AI). Two types of valve-sparing procedures were performed: valve reimplantation in 14 and root remodeling in 12 patients. Associated procedures were performed as follows: aortic valve plasty in 6, mitral valve plasty in 5, hemi-arch replacement in 4, total arch replacement in 2, coronary artery bypass surgery in 1 and Maze procedure in 1 patient(s). Result: In four patients, valve-sparing procedures were converted to Bentall procedures during operation. Including these patients, there was no operative deaths, 3 patients underwent re-operation due to bleeding, 1 patient had permanent pacemaker. The median duration of ICU stay was 45.5 hours, the median duration of hospital stay was 10.5 days. In 22 patients excluding 4 converted patients, intraoperative transesophageal echocardiogram (TEE) showed less than mild degree of AI in all except one who had not received intra-operative TEE in the beginning and showed moderate degree of AI at discharge. The mean duration of follow-up was $21.2\pm27.4$ months. All patients were alive except one who died during other departmental surgery. In 3 patients, more than moderate degree of AI was recurred, but there were no reoperation. Conclusion: Aortic valve-sparing procedures could be performed relatively safely in selected patients who had annuloaortic ectasia.

• Journal of Chest Surgery
• /
• v.39 no.1 s.258
• /
• pp.18-27
• /
• 2006

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly, but is one of the most common causes of myocardial ischemia which would result in high mortality within the first year of life. This is our early result of the surgical management for these patients. Material and Method: From June 1989 to July 2003, 6 patients with ALCAPA and one patient with ARCAPA (Anomalous origin of the Right coronary artery from the pulmonary artery) underwent surgical repair. We have reviewed the all medical records, electrocardiogram, chest X-ray and echocardiography retrospectively. Result: Three of the patients were boys and four were girls. The median age at the operation was 5.4 months (Range: 3$\∼$33 months). The average body weight of at the operation was 6.7 kg (Range: 3.7$\∼$11.3 kg). A mean follow up period was 18 months. Only 3 patients were initially diagnosed as ALCAPA. And 3 patients had moderate mitral regurgitation. Immediate coronary artery reimplantation on diagnosis with the aim of restoring a two-coronary system circulation was done. The average bypass time was 114$\pm$37 minutes, and the average aortic cross clamping time was 55$\pm$22 minutes. The average stay of intensive care unit was 5$\pm$3 days, the mean mechanical ventilator time was 38$\pm$45 hours and the hospital stay after operation was 12$\pm$5 days. There were significant improvements in electrocardiogram and chest X-ray of the all patients except one late death patient. The ventricular function showed almost normal recovery after operation; the EF (Ejection Fraction) increased from 41.2$\pm$ 10.3$\%$ to 60.5$\pm$ 15.8$\%$ within 1 month and to 59.8$\pm$13.9$\%$ within 1 year after operation, the SF (Shortening Fraction) increased from 23.6$\pm$4.7$\%$ to 38.6$\pm$8.4$\%$ within 1 month and to 37.4$\pm$7.9$\%$ within 1 year after operation, LVEDDI (Left Ventricular End-diastolic Dimension Index) decreased from 100.8$\pm$25.6 mm/$m^{2}$ to 90.3$\pm$ 19.2 mm/$m^{2}$ within f month and to 79.3$\pm$ 15.8 mm/$m^{2}$ within 1 year after operation. Concomitant mitral repair was done in two patients with anterior mitral leaflet prolapse. In every patient, mitral valve showed less than mild regurgitation during follow up. One late death occurred in which patient Dor procedure was applied 10 months after initial operation due to the dilated cardiomyopathy Conclusion: In the management of this rare and could be fatal Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), early suspicion and correct diagnosis is of most important. But, after diagnosis, immediate restoration of 2 coronary systems could result in good outcome.