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.
Child ; Dilatation ; Echocardiography ; Follow-Up Studies ; Freedom ; Heart Septal Defects, Atrial ; Hospital Mortality ; Humans ; Male ; Mitral Valve ; Mitral Valve Stenosis ; Postoperative Period ; Prolapse ; Survival Rate

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.
Animals ; Aprotinin/*pharmacology ; *Cardiopulmonary Bypass ; Comparative Study ; Dogs ; Hemostatics/pharmacology ; Lung/*blood supply/metabolism/pathology ; Male ; Models, Animal ; Research Support, Non-U.S. Gov't ; Vascular Resistance/*drug effects ; Water/metabolism

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.
Allografts ; Bioprosthesis ; Constriction, Pathologic ; Diagnosis ; Double Outlet Right Ventricle ; Follow-Up Studies ; Freedom ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular ; Heart Valve Prosthesis ; Humans ; International Normalized Ratio ; Male ; Mortality ; Polyethylene Terephthalates ; Pulmonary Atresia ; Pulmonary Valve ; Pulmonary Valve Stenosis ; Reoperation ; Retrospective Studies ; Thromboembolism ; Thrombosis ; Transposition of Great Vessels ; Truncus Arteriosus ; Warfarin

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.
Alprostadil ; Anoxia ; Aorta, Thoracic* ; Body Weight ; Cardiopulmonary Bypass ; Cause of Death ; Constriction, Pathologic ; Diagnosis ; Heart ; Humans ; Mitral Valve Insufficiency ; Mortality* ; Operative Time ; Prostaglandins E ; Retrospective Studies ; Risk Factors*

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.
Blalock-Taussig Procedure ; Echocardiography ; Follow-Up Studies ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Infant, Newborn ; Mortality ; Pulmonary Atresia* ; Tricuspid Valve ; Ventricular Function, Left ; Ventricular Function, Right ; Ventricular Septum*

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.
Aorta, Thoracic ; Aortic Coarctation ; Arteries ; Body Weight ; Cardiopulmonary Bypass ; Constriction, Pathologic ; Diagnosis ; Double Outlet Right Ventricle ; Follow-Up Studies ; Fontan Procedure ; Heart Defects, Congenital ; Humans ; Male ; Mortality ; Multivariate Analysis ; Pulmonary Artery* ; Retrospective Studies ; Risk Factors* ; Survival Rate

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.
Aorta ; Bland White Garland Syndrome ; Coronary Vessels* ; Echocardiography ; Follow-Up Studies ; Heart Defects, Congenital ; Heart Ventricles ; Heart-Assist Devices ; Humans ; Mitral Valve ; Mitral Valve Insufficiency* ; Pulmonary Artery* ; Reoperation ; Saphenous Vein ; Survivors ; Transplants ; Ventricular Function* ; Ventricular Outflow Obstruction

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.
Child* ; Heart Defects, Congenital ; Humans ; Longevity ; Pacemaker, Artificial ; Reoperation

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.
Adult ; Child ; Ductus Arteriosus, Patent/complications ; Eisenmenger Complex/etiology/*surgery ; Female ; Heart Defects, Congenital/complications/*surgery ; Heart Septal Defects, Ventricular/complications/surgery ; *Heart-Lung Transplantation ; Humans ; Korea ; Male ; Pulmonary Atresia/complications/surgery

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.
Aortic Valve ; Aortic Valve Insufficiency ; Aortic Valve Prolapse ; Arterial Pressure ; Heart Septal Defects ; Heart Septal Defects, Ventricular* ; Humans ; Incidence ; Pathology ; Pulmonary Artery ; Risk Factors