Results of Mitral Valve Repair in Patients with Congenital Mitral Disease / 대한흉부외과학회지

BACKGROUND: Mitral valve abnormalities in the pediatric population are rare. Mitral valve replacement for pediatric mitral lesions can cause problems such as a lack of growth potential. There are only limited experiences with mitral valve repair at any institution, so the purpose of this study is to evaluate the outcomes of mitral valve repair in pediatric patients. MATERIAL AND METHOD: Sixty-four consecutive children (28 males and 36 females) with a mean age of 5.5+/-4.7 years underwent mitral valve repair for treating their congenital mitral valve disease between January 1996 and December 2005. The patients were divided into two groups: group 1 (34 patients (53.1%)) had isolated disease (mitral anomaly with or without atrial septal defect or patent ductus arteriosus) and group 2 (30 patients (46.9%)) had complex disease (mitral anomaly with concurrent intracardiac disease, except atrioventricular septal defect). RESULT: The overall in-hospital mortality was 6.3%; group 1 had 5.9% mortality and group 2 had 10.0% mortality. The postoperative morbidity was 18.8%; group 1 and 2 had 14.7% and 23.3% postoperative morbidity, respectively, and there was no significant difference among the groups. The median follow-up was 4.6 years (range: 0.5~12.2 years). The 10-year survival rate was 95.3%. The 10-year freedom from re-operation rate was 76.1% with 10 re-operations. The majority of the functional classifications were annular dilatation and leaflet prolapse. A mean of 2.1+/-1.1 procedures per patient were performed. The echocardiography that was done at the immediate postoperative period showed a significant improvement in the mitral valve function. The follow-up echocardiographic results were significantly improved. However, mitral stenosis newly developed over time, and there were significant differences according to the repair strategies. CONCLUSION: The patients who underwent mitral valve repair for congenital mitral anomalies showed good results. The follow-up echocardiography revealed satisfactory short-term and long-term results. Close follow-up is necessary to detect the development of postoperative mitral stenosis or regurgitation.

Long Term Results of Rastelli Operation with a Mechanical Valve / 대한흉부외과학회지

BACKGROUND: Homografts and bioprostheses are most commonly used for Rastelli operation in congenital heart disease, but the limited durability is responsible for multiple reoperations associated with increased morbidity. This study evaluated long-term results after Rastelli operation with a mechanical valved conduit. MATERIAL AND METHOD: A total of 20 patients underwent Rastelli operation with mechanical valved conduit from January 1990 to July 1992. Operative mortality was 1 of 20 patients, and a retrospective review of 19 patients (10 males, 9 females) was done. Initial diagnosis was congenitally corrected transposition of great arteries (cc-TGA, n=4), complete TGA (n=2), ventricular septal defect with pulmonary atresia (VSD with PA, n=9), truncus arteriosus (n=2), double outlet right ventricle with pulmonary stenosis (DORV with PS, n=2). The mean age at Rastelli operation was 4.6+/-3.4 years, and mean follow-up period was 12.8+/-2.7 years. Patients underwent Rastelli opearation using 16 CarboMedics mechanical valve, and 3 Bjork-Shiley mechanical valve (17+/-2 mm). RESULT: There were 15 reoperations for failed mechanical valved conduit. The freedom from reoperation at 5 and 10 years was 53% and 37%. Most patients were received oral anticoagulation with warfarin, and maintained the international normalized ratio (INR) of 1.5 to 2.0. There was no anticoagulation or thromboembolism related complication. There was a significant difference in the causes of a conduit failure between early (within 3 years) and late (after 3 years) failure groups. The six patients reported early prosthetic valve failure, mainly due to valvular dysfunction by thrombosis or pannus formation. The other nine patients reported late prosthetic valve failure, mainly due to dacron conduit stenosis at anastomosis sites, whereas their valvar motion was normal except 1 patient. CONCLUSION: To avoid early prosthetic valve failure, strict anticoagulation therapy would be helpful. About the late development of obstructive intimal fibrocalcific peels within the Dacron conduit, an improvement of conduit material is necessary to reduce late prosthetic valve failure. In selected patients, the long term results were satisfactory.

Analysis of the Causes of and Risk Factors for Mortality in the Surgical Repair of Interrupted Aortic Arch / 대한흉부외과학회지

BACKGROUND: Interrupted aortic arch is a rare congenital heart anomaly which still shows high surgical mortality. In this study, we investigated the causes of and the risk factors for mortality to improve the surgical outcomes for this difficult disease entity. MATERIAL AND METHOD: From 1984 to 2004, 42 patients diagnosed as IAA were reviewed retrospectively. Age, body weight at operation, preoperative diagnosis, preoperative PGE 1 requirement, type of interrupted aortic arch, degree of left ventricular outflow stenosis, CPB time, and ACC time were the possible risk factors for mortality. RESULT: There were 14 hospital deaths. Preoperative use of PGE1, need for circulartory assist and aortic cross clamp time proved to be positive risk factors for mortality on univariate analysis. Preoperative left ventricular outflow stenosis was considered a risk factor for mortality but it did not show statistical significance (p-value=0.61). Causes of death included hypoxia due to pulmonary banding, left ventricular outtract stenosis, infection, mitral valve regurgitation, long cardiopulmonary bypass time and failure of coronary transfer failure in TGA patients. CONCLUSION: In this study, we demonstrated that surgical mortality is still high due to the risk factors including preoperative status and long operative time. However preoperative subaortic dimension was not related statistically to operative death statistically. Adequate preoperative management and short operation time are mandatory for better survival outcome.

Aprotinin Attenuates the Elevation of Pulmonary Vascular Resistance After Cardiopulmonary Bypass

Pulmonary vascular resistance (PVR) is generally believed to be elevated after cardiopulmonary bypass (CPB) due to whole body inflammation. Aprotinin has an antiinflammatory action, and it was hypothesized that aprotinin would attenuate the PVR increase induced by CPB. Ten mongrel dogs were placed under moderately hypothermic CPB for 2 hr. The experimental animals were divided into a control group (n=5, group I) and an aprotinin group (n=5, group II). In group II, aprotinin was administered during pre-bypass (50,000 KIU/kg) and post-bypass (10,000 KIU/kg) periods. Additional aprotinin (50,000 KIU/kg) was mixed in CPB priming solution. PVRs at pre-bypass and post-bypass 0, 1, 2, 3 hr were calculated, and lung tissue was obtained after the experiment. Post-bypass PVRs were significantly higher than prebypass levels in all animals (n=10, p<0.001). PVR elevation in group II was less than in group I at 3 hr post-bypass (p=0.0047). Water content of the lung was lower in group II (74+/-9.4%) compared to that of group I (83+/-9.5%), but the difference did not reach significance (p=0.076). Pathological examination showed a near normal lung structure in group II, whereas various inflammatory reactions were observed in group I. We concluded that aprotinin may attenuate CPB-induced PVR elevation through its anti-inflammatory effect.

Mid-term Results of Neonatal Surgical Management of Pulmonary Atresia with Intact Ventricular Septum / 대한흉부외과학회지

BACKGROUND: Pulmonary atresia with intact ventricular septum is a rare congenital cardiac disease. There have been various surgical options, but there are still controversies. MATERIAL AND METHOD: Fourteen neonates who were operated on between 1999 and 2000 were enrolled in this study. We measured Z-value of tricuspid valve by echocardiography. We performed right ventricular outflow tract transannular patch in 9 patients. Modified Blalock-Taussig shunt was placed according to the condition of patients. Mean follow up period was 46.0 months. RESULT: There were 2 operative mortality and 1 late mortality. Biventricular repair was possible in 7 patients. One and a half ventricular repair were done in 3 (tricuspid valve Z-value was -2, -2.5 and -3) and single ventricular repair in 2 patients (tricuspid z-value was -4.6, -4.5) The tricuspid valve Z-value for the patients who had biventricular repair and one and a half ventricular repair were -0.8+/-1.50 (-3.2~1.2) and -2.5+/-0.5 (-3~-2) respectively. All patients who survived had fair to good right ventricular function, good left ventricular function and good clinical states. CONCLUSION: Neonatal surgical management of pulmonary atresia with intact ventricular septum has a good surgical outcome, z-value of tricuspid may be helpful for the determination of surgical options.

Changes of Ventricular Function and Mitral Regurgitation after Repair of Anomalous Origin of Coronary Artery from the Pulmonary Artery / 대한흉부외과학회지

BACKGROUND: Investigation of the change of ventricular function and mitral regurgitation after surgical repair of patient with anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is key issue for the better surgical outcome. MATERIAL AND METHOD: From April 1986 to July 2002, 12 patients presented with ALCAPA. The median age at repair was 4 months. Surgical methods included left coronary artery transfer to the aorta (10), Takeuchi procedure (1), saphenous vein free graft bypass (1). Mitral valve was repaired in 1. RESULT: There were 2 hospital death (16.7%). The mean follow-up period was 7.1+/-4.1 years (range, 7 months to 13 years). Four patients required postoperative circulatory assist for 2.2+/-1.1 days and one needed left ventricular assist device (LVAD) for 1day. Postoperative echocardiography demonstrated significant improvements in mean fractional shortening (33.4+/-9.1% vs 17.7+/-9.6%, n=10, p<0.05); left ventricular end diastolic dimension (33.1+/-7.3 mm vs 41.8+/-7.0 mm, n=10, p<0.05) and systolic dimension (22.2+/-7.5 mm vs 33.4+/-7.9 mm, n=10, p<0.05). Severities of mitral regurgitation decreased in all survivors at 1st and 4th year follow-up echocardiography. There were 2 reoperation due to residual MR and right ventricular outflow obstruction (Takeuchi case). CONCLUSION: Anatomic repair of anomalous left coronary artery from the pulmonary artery offered an excellent surgical results, especially in terms of the recovery of left ventricle function and mitral regurgitation. However, preoperative indications for mitral procedure is to be evaluated.

Risk Factor Analysis and Surgical Indications for Pulmonary Artery Banding / 대한흉부외과학회지

BACKGROUND: Pulmonary artery banding (PAB) is an initial palliative procedure for a diverse group of patients with congenital cardiac anomalies and unrestricted pulmonary blood flow. We proved the usefulness of PAB through retrospective investigation of the surgical indication and risk analysis retrospectively. MATERIAL AND METHOD: One hundred and fifty four consecutive patients (99 males and 55 females) who underwent PAB between January 1986 and December 2003 were included. We analysed the risk factors for early mortality and actuarial survival rate. Mean age was 2.5+/-12.8 (0.2~92.7) months and mean weight was 4.5+/-2.7 (0.9~18.0) kg. Preoperative diagnosis included functional single ventricle (88, 57.1%), double outlet right ventricle (22, 14.2%), transposition of the great arteries (26, 16.8%), and atrioventricular septal defect (11, 7.1%). Coarctation of the aorta or interrupted aortic arch (32, 20.7%), subaortic stenosis (13, 8.4%) and total anomalous pulmonary venous connection (13, 8.4%) were associated. RESULT: The overall early mortality was 22.1% (34 of 154). The recent series from 1996 include patients with lower age (3.8+/-15.9 vs. 1.5+/-12.7, p=0.01) and lower body weight (4.8+/-3.1 vs. 4.0+/-2.7, p=0.02). The early mortality was lower in the recent group (17.5%; 16/75) than the earlier group (28.5%; 18/45). Aortic arch anomaly (p=0.004), subaortic stenosis (p=0.004), operation for subaortic stenosis (p=0.007), and cardiopulmonary bypass (p=0.007) were proven to be risk factors for early death in univariate analysis, while time of surgery (<1996) (p=0.026) was the only significant risk factor in multivariate analysis. The mean time interval from PAB to the second-stage operation was 12.8+/-10.9 months. Among 96 patients who survived PAB, 40 patients completed Fontan operation, 21 patients underwent bidirectional cavopulmonary shunt, and 35 patients underwent biventricular repair including 25 arterial switch operations. Median follow-up was 40.1+/-48.9 months. Overall survival rates at 1 year, 5 years and 10 years were 81.2% 65.0%, and 63.5% respectively. CONCLUSION: Although it improved in recent series, early mortality was still high despite the advances in perioperative management. As for conventional indications, early primary repair may be more beneficial. However, PA banding still has a role in the initial palliative step in selective groups.

Norwood Procedure on Beating Heart / 대한흉부외과학회지

Modified Norwood procedure with maintaining cardiac beat was done in a 30-day-old neonate. Procedure was done with regional perfusion of innominate and coronary artery. Postoperative course was uneventful. Second-stage operation (bi-directional cavopulmonary shunt) was done 4 months later. The diameter of ascending aorta was more than 5 mm, Norwood procedure can be done in beating hearts.

Long-term Results of Modified Lecompte Procedure for the Anomalies of Ventriculoarterial Connection / 대한흉부외과학회지

Background: The Lecompte procedure for transposition of the great arteries has an advantage because it obviates the need for an extracardiac conduit for the reconstruction of the pulmonary outflow tract. We evaluated the effectiveness and the application of the Lecompte procedure. Material and Method: A retrospective review was conducted of the records of 46 patients who underwent the Lecompte procedure during the past 15 years. Mean age at operation was 29.2+/-20.3 (range: 3~83) months. The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double-outlet right ventricle, and double-outlet left ventricle. Result: Early mortality was 4.4% (2 of 46 patients) and late mortality was 6.8% (3 of 44). The mean follow-up was 11.2+/-6.9 years. Eighteen patients (43.9% of survivors, n=41) had pulmonary stenosis (pressure gradient above 30 mmHg), the main reason for which was a calcified monocusp valve (n=15, 83.3%). Seventeen of 46 patients (37.0%) underwent reoperation: 15 for pulmonary stenosis, 5 for residual ventricular septal defect, 4 for left ventricular outflow tract obstruction, 3 for pulmonary insufficiency, and 4 for other causes. The cumulative survival rates were 91.3+/-4.2%, and 87.0+/-5.8% at 10 and 15 years, respectively. The actuarial probabilities of freedom from reoperation for pulmonary stenosis were 90.6+/-4.5%, 73.9+/-7.3%, and 54.0+/-10.4% at 5, 10, and 15 years, respectively. Conclusion: The Lecompte procedure is an effective treatment modality. Repair in early age is possible with acceptable morbidity and mortality, but recurrent right ventricular outflow tract obstruction caused by degeneration of the monocusp valve is a problem that needs resolution.

Arrhythmia Surgery in Fontan Operation / 대한흉부외과학회지

BACKGROUND: Refractory atrial arrhythmias in patients late after the Fontan operation result in significant morbidity and mortality. We reviewed our experience with arrhythmia surgery in patients who had Fontan operation. MATERIAL AND METHOD: Between July 1986 and December 2003, 275 early survivors after Fontan operation were reviewed. Fourteen patients underwent arrhythmia surgery at reoperation after Fontan operation, and mean age at reoperation was 16.8+/-7.1 (range: 4.5~30.6) years. Mechanisms of arrhythmia included atrial flutter in 8 patients, and atrial fibrillation in 2. Arrhythmia surgery has evolved from isthmus cryoablation in 12 patients to right-sided maze in 2 patients. Thirty-two patients underwent prophylactic isthmus cryoablation concomitantly at initial Fontan operation. RESULT: Postoperative arrhythmias occurred in 68 patients (24.7%) among 275. There was no early and late mortality after the arrhythmia surgery. After redo Fontan operation, all patients maintained normal sinus rhythm. Atrial flutter recurred in 3 patients who had sinus conversion with medication and 7 required permanent pacemakers with a mean follow-up of 26.5+/-29.1 (range: 2~73) months. All patients have improved to NYHA class I or II. After prophylactic cryoablation at initial Fontan operation, 29 patients (90.6%) had sinus rhythm, 1 patient had junctional tachycardia, 1 patient had sinus nodal dysfunction, and 1 patient had AV block with a mean follow-up of 51.3+/-19.8 (range: 4~80) months. CONCLUSION: Redo Fontan operation, and concomitant arrhythmia surgery reduced atrial arrhythmias and improved NYHA functional classification.

Aortic Valvuloplasty in Pediatric Age / 대한흉부외과학회지

BACKGROUND: In this study, we retrospectively analyzed the outcomes of aortic valvuloplasty in pediatric age. MATERIAL AND METHOD: Between January 1993 and March 2004, 35 patients underwent aortic valvuloplasty for aortic stenosis (AS) or aortic regurgitation (AR). The mean age was 81.1+/-61.5 (1~223) months. The mean follow up was 50.8+/-30.2 (3~121) months. Nine patients had AS, 21 had AR, and 6 had AS and AR. Valve morphology was tricuspid in 24 patients, bicuspid in 9, quadricuspid in 1, and unicuspid in 1. The mean peak pressure gradients of AS were 72.0+/-33.0 mmHg, and the mean grades of AR were 3.1+/-0.9. RESULT: There was one late mortality without early mortality. After operation, AS improved with mean peak pressure gradients of 23.5+/-21.0 mmHg (p<0.05), and AR improved with mean grades of 1.9+/-0.8 (p<0.05). At mean follow up of 35.0+/-23.0 months, AS maintained with mean peak pressure gradients of 31.5+/-24.0 mmHg, but AR progressed with mean grades of 2.8+/-1.3 (p<0.05). Reoperation was required in 6 patients 38.3+/-21.8 months after the original operation. The actuarial figures for freedom from reoperation at 2, 5 and 8 years were 96.9+/-3.1%, 79.5+/-5.5%, and 56.8+/-11.4%, respectively. Age at operation, presence of AS, preoperative severity of AS or AR, and morphology of aortic valve were not significant risk factors for reoperation, and improvement of AS or AR. CONCLUSION: Aortic valvuloplasty showed good immediate postoperative valve function. Aortic valvuloplasty offers children many years with tolerable valve function and allows to postpone aortic valve replacement or Ross procedure in pediatric patients.

Heart-Lung Transplantation in Korea

Heart-lung transplantation is an effective treatment for patients with various forms of congenital heart disease or pulmonary hypertension. Since the first heart-lung transplantation in 1997, five transplants have been performed in Korea. Three cases were performed in 1997, one in 1998, and the latest one in 2002. The preoperative diagnoses were complex congenital heart disease (CHD) in 2, and CHD with Eisenmenger's syndrome in 3. In this paper, we report five cases of heart-lung transplantation performed in Korea, and include a review of the relevant literature.

One Stage Repair of Berry Syndrome in a Neonate / 대한흉부외과학회지

Berry syndrome (interrupted aortic arch, aortopulmonary window, and aortic origin of right pulmonary artery with intact interventricular septum) is a very rare and complex cardiac malformation. We report a successful one-stage repair in a 14-day-old neonate without circulatory arrest.

One Stage Repair of Berry Syndrome in a Neonate / 대한흉부외과학회지

Berry syndrome (interrupted aortic arch, aortopulmonary window, and aortic origin of right pulmonary artery with intact interventricular septum) is a very rare and complex cardiac malformation. We report a successful one-stage repair in a 14-day-old neonate without circulatory arrest.

Aoric Valve Lesion in Type I Ventricular Septal Defect / 대한흉부외과학회지

BACKGROUND: In this study, we investigated the risk factors for the development or progression of aortic regurgitation(AR) in patients with type I ventricular septal defect (VSD) to determine the optimal surgical timing and strategy. MATERIAL AND METHOD: Three-hundred and ten patients with type I VSD with or without AR were included. The mean of age was 73.7+/-114.7 (1~37) months. One hundred and eighty six patients (60%) had no AR, 83 (27%) had mild AR, 25 (8%) had moderate AR and 16 (5%) had severe AR. Aortic valve was repaired in 5 patients and replaced in 11 patients with closure of VSD in the first operation. Four patients required redo aortic valve repair and 11 patients required redo aortic valve replacement. Age at operation, association with aortic valve prolapse, Qp/Qs, systolic pulmonary arterial pressure, VSD size and systolic pulmonary artery to aortic pressure ratio(s[PAP/AP]) were included as risk factors analysis for the development of AR. The long-term result of aortic valve repair and aortic valve replacement were compared. RESULT: Older age at operation, association with aortic valve prolapse, high Qp/Qs, and s[PAP/AP] were identified as risk factors for the development of AR (p<0.05, Table 2). The older the patient at the time of operation, the higher the severity of preoperative AR and the incidence of postoperative AR (p<0.05, Table 1, Fig. 1). For the older patients at operation, aortic valve repair had higher occurrence of AR compared to those who had aortic valve replacement (p<0.05, Fig. 2). CONCLUSION: From the result of this study, we can concluded that early primary repair is recommended to decrease the progression of AR. Aortic valve repair is not always a satisfactory option to correct the aortic valve pathology, which may suggest that aortic valve replacement should be considered when indicated.

Thirteen-year Experience of Permanent Epicardial Pacing in Children / 대한흉부외과학회지

BACKGROUND: We investigated the longevity, thresholds of epicardial pacemaker and causes of reoperation in the pediatric patients who underwent epicardial pacemaker implantation performed during the last 13 years MATERIAL AND METHOD: 121 operations were performed in 83 patients from January 1989 to July 2002. We analyzed the stimulation threshold, resistance, R-wave and P-wave, and sensitivity of pacemaker lead at initial implantation. Longevity and causes of reoperations were investigated. RESULT: At implantation, epicardial ventricular mean stimulation threshold was 1.2+/-0.1 (0.1~5) mV, mean resistance was 519.1+/-18.1 (319~778) Ohm, and mean R-wave sensitivity was 8.9+/-0.7 (4~20) mV, and mean P wave sensivity was 2.5+/-0.7 (0.4~12) mV. The mean longevity of pacemaker generator was 64.7+/-3.7 (2~196) months. The reoperation free rate was 94.6% for 1 year, 93.6% for 2 years, 80.8% for 5years, 63.7% for 7 years, and 45.5% for 10 years. The causes of reoperation were battery waste in 26 cases and lead malfunction in 9 cases. There was no postoperative death related to pacemaker malfunction. CONCLUSION: In the childrens, average longevity of epicardial pacemaker was within acceptable range. 19.1% of the patients required pacemaker related reoperation. However, recent developments, including steroid eluting lead, 6.7% of the patients required pacemaker related reoperation, look promising in expansion of pacemaker life span.

Reoperations after Fontan Procedures / 대한흉부외과학회지

BACKGROUND: Surgical results of the Fontan procedures in patients with a single ventricle have improved. As the perioperative mortality continues to decline and late outcome is forthcoming, attention is now being directed toward late complications of the Fontan procedures. We retrospectively analyzed our experience with reoperations after Fontan procedures. MATERIAL AND METHOD: Between January 1988 and December 2002, 24 patients underwent reoperations after Fontan procedures. The median age at Fontan procedures and reoperation was 3.3 years and 9.2 years, respectively. Types of initial Fontan procedures were atriopulmonary connection (n=11), lateral tunnel Fontan (n=11), and extracardiac conduit Fontan (n=2). Indications for reoperation included atrioventricular valve regurgitation (n=7), atrial arrhythmia (n=8), Fontan pathway stenosis (n=7), residual right-to-left shunt (n=5), etc. RESULT: Procedures performed at reoperation included atrioventricular valve replacement (n=6), conversion to lateral tunnel Fontan (n=5), conversion to extracardiac conduit Fontan (n=3), cryoablation of arrhythmia circuit (n=7), etc. There was no operative mortality. There were 2 late deaths. Mean follow-up duration was 2.7+/-2.1 years. All patients except two were in NYHA class I at the latest follow-up. Among 8 patients with preoperative atrial arrhythmia, postoperative conversion to normal sinus rhythm was achieved in 7 patients. CONCLUSION: Reoperations after Fontan procedures could be achieved with low mortality and morbidity. Reoperation may lead to clinical improvement in patients with specific target conditions such as atrioventricular valve regurgitation, refractory atrial arrhythmia, or Fontan pathway stenosis, especially in patients with previous atriopulmonary connection.

Surgical Treatment of Complications after Fontan Operation / 대한흉부외과학회지

BACKGROUND: The Fontan operation has undergone a number of major modifications and clinical results have been improving over time. Nevertheless, during the follow-up period, life-threatening complications develop and affect the long-term outcomes. Surgical interventions for these complications are needed and are increasing. MATERIAL AND METHOD: From April 1988 to January 2000, 16 patients underwent reoperations for complications after Fontan operation. The mean age at reoperation was 8.8+/-5.5 years. Initial Fontan operations were atriopulmonary connections in 8 and total cavopulmonary connections in 8. Total cavopulmonary connections were accomplished with intracardiac lateral tunnel in 5 and extracardiac epicardial lateral tunnel in 3. Five patients had variable sized fenestrations. The reasons for reoperations included residual shunt in 6, pulmonary venous obstruction in 3, atrial flutter in 3, atrioventricular valve regurgitation in 2, Fontan pathway stenosis in 1, and protein-losing enteropathy in 1. RESULT: There were 3 early and late deaths respectively. Patients who had residual shunts underwent primary closure of shunt site (n=2), atrial reseptation for separation between systemic and pulmonary vein (n=2), conversion to lateral tunnel (n=1), and conversion to one and a half ventricular repair (n=1). Four patients who had stenotic lesion of pulmonary vein or Fontan pathway underwent widening of the lesion (n=3) and left pneumonectomy (n=1). In cases of atrial flutter, conversion to lateral tunnel after revision of atriopulmonary connections was performed (n=3). For the atrioventricular valve regurgitation (n=2), we performed a replacement with mechanical valve. In one patient who had developed protein-losing enteropathy, aorto-pulmonary collateral arteries were obliterated via thoracotomy. Cryoablation was performed concomitantly in 4 patients as an additional treatment modality of atrial arrhythmia. CONCLUSION: Complications after Fontan operation are difficult to manage and have a considerable morbidity and mortality. However,more accurate understanding of Fontan physiology and technical advancement increased the possibility of treatment for such complications as well as Fontan operation itself. Appropriate surgical treatment for these patients relieved the symptoms and improved the functional class,Although the results were not satisfactory enough in all patients.

Evaluation of Prognostic Factors in Corrected Transposition of the Great Arteries at Mid-term Follow-up

PURPOSE: The prognosis of patients with corrected transposition of the great arteries(C-TGA) is variably affected by associated intracardiac defects, systemic right ventricular function, tricuspid valve competence, and conduction disturbances. This study aims to evaluate the importance of those factors at mid-term follow-up. METHODS: Medical records of 94 patients(males 58, females 36; mean age at last follow-up, 12+/-9 years; mean follow-up duration, 9+/-6.4 years) diagnosed between January 1980 and May 2002 at Seoul National University Children's Hospital were studied retrospectively. RESULTS: Among 94 patients, operations were performed in 72 patients(classic operations in 55; double switch operations in 17). Among prognostic factors including associated intracardiac anomalies(at least moderately severe tricuspid insufficiency(TI), ventricular septal defect, pulmonary stenosis and pulmonary atresia), intracardiac operation and complete atrioventricular block, TI was the only significant factor for death(P=0.001), and in turn, Ebstein anomaly and high grade atrioventricular block predicted TI. 20-year survival without TI was 77%, but only 35% with TI(P=0.0002); excluding perioperative death, the 20-year survival rates with and without TI were 48% and 87% respectively(P= 0.008). There was no statistical difference in 20-year survival rate or association with TI between classic and double switch operation. CONCLUSION: TI was the major prognostic factor for C-TGA and was associated with Ebstein anomaly and high grade atrioventricular block at mid-term follow-up. Long-term follow-up is required to evaluate other factors, including double switch operations and associated intracardiac defects more exactly.

Long-term Results of Surgical Correction for Partial Atrioventricular Septal Defects: Seventeen-year Experience / 대한흉부외과학회지

BACKGROUND: In this study, we analyzed the long-term surgical outcome of partial atrioventricular septal defects during the past 17 years at Seoul National University Hospital. MATERIAL AND METHOD: A retrospective analysis on mortality, survival, and reoperation and their risk factors was done in 93 patients who underwent surgical correction of partial atrioventricular septal defects between April 1986 and December 2002. 32 patients were male and 61 were female with a median age of 68 months (3~818 months) and a mean follow-up period of 108 months (1~200 months). RESULT: There were 4 operative deaths (4.3%) and one mortality during the follow-up period. 3, 5, 10, and 15 year actuarial survival rates were 95.7%, 94.3%, 94.3%, and 94.3%, respectively. After the surgical correction, left atrioventricular valve incompetence was improved in 61patients (67.7%), remained same as the preoperative status in 14 patients (15.1%), and was aggravated in 12 patients (12.9%). Reoperation was performed in 8 patients (9.0%) after a mean interval of 38.6 months (3~136 months). Freedom from reoperation rates at 3, 5, 10, and 15 years after surgical correction were 94.0%, 91.4%, 91.4%, and 88.2%, respectively. Reasons for reoperation were 7 left atrioventricular valve incompetence, 2 left ventricular outflow tract obstruction, a residual atrial septal defect, a left atrioventricular valve stenosis, and a right ventricular failure. Left ventricular outflow tract obstruction was the only statistically significant factor. In ten patients, significant arrhythmia was developed and three of them were supraventricular arrhythmia. Complete atrioventricular block occurred in 7 patients and permanent pacemakers were implanted in six of them. CONCLUSION: Surgical corrections of partial atrioventricular septal defects were performed with low operative mortality. Sicnce left atrioventricular valve incompetence was the most common cause of reoperation and left ventricular outflow tract obstruction was the only risk factor for reoperation, a precise estimation of the left atrioventricular valve morphology and the structure of left ventricular outflow tract are needed. Although left ventricular outflow tract obstruction rarely developed, reoperation was frequently required and resection of subaortic tissue could be performed but the possibility of recurrence was high, so modified Konno operation could be performed with satisfactory results. Complete atrioventricular block developed frequently in early periods, but was overcome with a precise anatomical understanding of conduction system and experience.