Coronary sinus ostial atresia (CSOA) with persistent left superior vena cava (LSVC) in the absence of an unroofed coronary sinus is a benign and rare anomaly that may be taken lightly in most instances. However, if overlooked in patients undergoing univentricular heart repair such as bidirectional Glenn or Fontan-type surgery, fatal surgical outcomes may occur due to coronary venous drainage failure. We report a case of CSOA with a persistent LSVC that was managed through coronary sinus rerouting during a total cavopulmonary connection, and provide a review of the literature regarding this rare anomaly.
Coronary Sinus* ; Drainage ; Fontan Procedure ; Heart ; Heart Bypass, Right ; Humans ; Vena Cava, Superior

BACKGROUND: We evaluated the early and mid-term results for repair of Ebstein's anomaly. MATERIAL AND METHOD: Between January 1989 and June 2004, 29 patients underwent repair of Ebstein's anomaly. The median age was 11.4 years (4 days-50 years). Tricuspid insufficiency equal to or greater than grade 3 was present in 21 (72.4%) patients. Surgical techniques included tricuspid valve repair with vertical plication of the atrialized ventricle (n=14), Carpentier's technique (n=7), tricuspid valve replacement (n=4), systemic-to-pulmonary arterial shunt (n=2), tricuspid valve repair (n=1), and Fontan operation (n=1). Bi-directional cavopulmonary shunt (BCPS) was required in 5 patients. Among the 2 neonates, one patient underwent successful biventricular repair, and the other patient underwent systemic-to-pulmonary arterial shunt. Follow-up was possible in 21 patients (75%), and the average follow-up was 37.6 months (3 months~11.3 years). RESULT: There were 1(3.4%) early and 1 late deaths. Reoperation was required in 4 patients. Two patients underwent tricuspid valve re-replacement, and the other 2 tricuspid valve repair. At recent follow-up, only 2 patients showed tricuspid insufficiency equal to or greater than grade 3, and most patients showed clinical improvement. Excluding the patients who underwent tricuspid valve replacement, the actuarial rate of freedom from reoperation at 1 and 5 years were 94.7% and 79.0%, respectively. CONCLUSION: Tricuspid valve repair was possible in most patients with good mid-term outcome. Most patients showed clinical and hemodynamic improvement. Indications for the BCPS should be clarified.
Ebstein Anomaly* ; Follow-Up Studies ; Fontan Procedure ; Freedom ; Heart Bypass, Right ; Hemodynamics ; Humans ; Infant, Newborn ; Reoperation ; Tricuspid Valve

BACKGROUND: Pulmonary atresia with intact ventricular septum(PA/IVS) is an anatomically heterogenous anomaly with a variety of surgical strategies possible. The purpose of the study is to evaluate the influence of right ventricular size on the early and midterm results of surgical repair of PA/IVS. MATERIAL AND METHOD: Medical records of 20 consecutive patients with PA/IVS operated on between January 1993 and August 1999 were retrospectively reviewed. There were 12 boys and 8 girls whose ages ranged from 2 days to 14.5 months (median 6 days). Their body weight ranged from 2.52kg to 9.35 kg(median 3.18kg). The preoperative Z-value of the diameter of the tricuspid valve(T-valve) was less than or -4 in 5 patients, between -4 and -2 in 1, between -2 and 0 in 7, between 0 and 2 in 6, and greater than or 2 in 1. All patients who had z-value of tricuspid valve greater than -2.05 were attempted biventricular repair(n=15) and all patients who had it smaller than -4.4 underwent systemic-pulmonary shunt operation only(n=3) or bidirectinal cavopulmonary shunt with right ventricular reconstruction(n=2). RESULT: Two early deaths(2/20, 10%) occurred. Both were infants who underwent transannular patch with shunt. One of these two had huge right ventricle(Z-value of tricuspid valve = 5). There were 2 late non-cardiac deaths 3 and 7 months after operations respectively. Follow-up was completed in all children at a mean of 35.3 months(range, 5 to 54 months). 10 of 11 survivors who underwent transannular patch or valvotomy with or without shunt procedure were in NYHA functional class I even though some of them had small interatrial communication or patent shunt. All three patients who had shunt procedure only at initial palliation completed Fontan procedures with no death. Two patients who underwent right ventricular outflow reconstruction with bidirectional cavopulmonary shunt were also in good condition. CONCLUSIONS: The transanular RVOT patch or valvotomy with or without systemic-pumonary shunt as an initial palliative procedure to achieve biventricular repair for the patients who had neither too small nor too large right ventricle(-2.05< or =Z-value of T-value of T-valve< or =2) could be performed at low operative risk(1/14 7.1%). Systemic-pulmonary shunt procedure and bidirectional cavopulmonary shunt procedure for the patients who had small right ventricle(Z-value of T-valve< or =4.4) could be also performed with low risk. But a patient with huge right atrium and ventricle(Z-value of t-valve=5) had poor operative result.
Body Weight ; Child ; Female ; Follow-Up Studies ; Fontan Procedure ; Heart Atria ; Heart Bypass, Right ; Humans ; Infant ; Medical Records ; Pulmonary Atresia* ; Retrospective Studies ; Survivors ; Tricuspid Valve ; Ventricular Septum*

BACKGROUND: The hemodynamic superiority of total cavopulmonary anastomosis (TCPA) over atriopulmonary connection as a modification of Fontan-type operation, began to be acknowledged with more applications to clinical cases. The need of adequate resolutions for the investigation and improvement of residual hemodynamic derangements including abnormal distribution of the pulmonary blood flow, is emerging. METHODS: We studied 20 patients (M:F=12:8, age:67.9+/-41.5 months) who have had the TCPA and were followed-up by cardiac catheterization, angiography and lung perfusion scan 24.5+/-15.7 months after the operation. Pulmonary arterial growth and hemodynamic influences including the pulmonary blood flow distribution were investigated to verify the appropriateness of the conventional TCPA method and to aid in the determination of the consequences and prognosis of the operation. RESULTS: The mean pulmonary arterial pressure before and after the operation was 15.1+/-3.2 mmHg and 13.9+/-4.8 mmHg respectively without significant difference and there was no significant postoperative changes in the cross-sectional area index of pulmonary artery in regard to the variability of body surface area. The pulmonary blood flow was distributed with a greater amount in the ipsilateral side of IVC flow entrance (IVCipsi) than the contralateral side (IVCcontra), with an ipsilateral to contralateral perfusion ratio (i/cPR) of 1.24+/-0.42. Comparing the subgroups by the type of superior vena caval inflow, unilateral superior cavopulmonary anastomosis (SCPA) group showed significantly higher i/cPR (1.47+/-0.33) than the bilateral SCPA group (1.07+/-0.21). Comparing the subgroups by the type of IVC inflow, the i/cPR of the intraatrial tunnel group was higher than the hemiazygous continuation group, but there was no statistical significance. CONCLUSION: TCPA does not influence the growth of pulmonary artery, and the type of cavopulmonary anastomosis and the bilaterality of superior vena cava may have major influences on the distribution of the pulmonary blood flow. The details of surgical methods should be evaluated case by case in respect to the associated anomalies in order to achieve adequate postoperative pulmonary blood flow distribution.
Angiography ; Arterial Pressure ; Body Surface Area ; Cardiac Catheterization ; Cardiac Catheters ; Fontan Procedure ; Heart Bypass, Right* ; Hemodynamics ; Humans ; Lung ; Perfusion ; Prognosis ; Pulmonary Artery ; Pulmonary Circulation* ; Vena Cava, Superior

Creation of an axillary arteriovenous fistula (AVF) was performed in two patients with high risks of Fontan operation after a cavopulmonary shunt. The patients complained of progressive cyanosis and decrease in exercise tolerance, and they showed pulmonary arteriovenous fistula on the pulmonary angiography. They were uneventfully transferred to the general ward on the first postoperative day and discharged 3 and 4 days after the operation respectively. There was no evidence of fistula-related volume loading and the other postoperative complications. An 8-month follow-up revealed improved symptoms such as, cyanosis and exercise intolerance, but not sufficient regression of pulmonary arteriovenous fistula on a lung perfusion scan and contrast echocardiography, which should be carefully checked hereafter.
Angiography ; Arteriovenous Fistula* ; Axilla ; Cyanosis ; Echocardiography ; Exercise Tolerance ; Follow-Up Studies ; Fontan Procedure* ; Heart Bypass, Right ; Humans ; Lung ; Patients' Rooms ; Perfusion ; Postoperative Complications

PURPOSE: Despite extracardiac conduit Fontan had many advantages, long-term results related to longevity of conduit, and anticoagulation were not proven. This study was to evaluate the long-term outcome of hospital survivors with extracardiac Fontan circulation. METHODS: Between 1996 and 2006, 200 patients underwent extracardiac conduit Fontan operation. Median age at the Fontan operation was 3.4 years (range:16 months-35.7 years). All patients (89.5 %) except 21 patients of one stage Fontan operation underwent bi-directional cavopulmonary shunt. Fenestration was required in 85 patients (42.5%). RESULTS: There has been 6 hospital mortalities (3.0%), and 7 late mortalities (3.6%) at a mean follow-up of 52.4+/-32.2 months (range; 18days-120 months). Overall 10-years survival was 92.4 2.1%. Multivariate analysis identified severe infection at early postoperative periods (hazard ratio =12.439, P=0.001), and high pulmonary arterial pressure at preoperative period (hazard ratio=3.445, P=0.038) as risk factors for mortality. Reoperation was performed in 24 patients (12.0%), and freedom from reoperation was 82.4+/-4.1% at 10 years. Arrhythmia occurred in 32 patients (16.0%) after Fontan operation, and freedom from arrhythmia was 85.14.4% at 10 years. Risk factors for arrhythmia were heterotaxy syndrome (P=0.001), Follow up duration (P=0.027) and the age at Fontan operation (P=0.001). Freedom from thromboembolism was 92.91.9% at 10 years. The conduit cross- sectional area decreased by 14%, and the extent of decrease of the conduit cross-sectional remained stable irrespective of the follow-up duration. 95.2% of patients had New York Heart Association class I. CONCLUSIONS: After 10 years of follow-up, the overall survival, and the functional status of survivors of the extracardiac Fontan procedure are satisfactory. Fenestration has a beneficial effect on the results of high-risk Fontan patients. The incidence of late deaths, reoperations, obstructions of the cavopulmonary pathway, arrhythmias and thromboembolisms is low.
Arrhythmias, Cardiac ; Arterial Pressure ; Follow-Up Studies ; Fontan Procedure* ; Freedom ; Heart ; Heart Bypass, Right ; Heterotaxy Syndrome ; Hospital Mortality ; Humans ; Incidence ; Korea* ; Longevity ; Mortality ; Multivariate Analysis ; Postoperative Period ; Preoperative Period ; Reoperation ; Risk Factors ; Survivors ; Thromboembolism

From April 1987 to May 1996, 13 infants underwent a Norwood operation for complex congenital heart diseases including hypoplastic left heart syndrome(n=7), mitral stenosis with small VSD and subaortic stenosis(n=1), mitral atresia with ventricular septal defect, coarctation of aorta, and subaortic stenosis(n=1), interrupted aortic arch with ventricular septal defect and subaortic stenosis(n=1), tricuspid atresia with transposition of the great arteries(n=1), and complex double-inlet left ventricle(n=2). All patients without hypoplastic left heart syndrome were associated with hypoplasia of ascending aorta and arch. Age at operation ranged from 3 days to 8.7 months(mean 60.6+/-71.6 days, median 39 days). The operative mortality(<30 days) was 46%(6 patients). Late mortality was 15%(2 patients). All operative deaths occured during the first 24 hours after the operation as a result of cardiopulmonary bypass weaning failure(5 patients) and sudden hemodynamic instability postoperatively(1 patient). Late death was due to aspiration pneumonia in two cases. There are 5 long-term survivals(39%). Three of them have undergone a two-stage repair with a modified Fontan operation in two and total cavopulmonary shunt in one at 12, 17, 4.5 months after Norwood procedure with no mortality. Two patients have entered a three-stage repair strategy by undergoing a bidirectional cavopulmonary shunt at 3 and 5.5 months after initial operation with 1 operative death. The actuarial survival rate for all patients at the first-stage operation, including hospital deaths and late death was 30.8% at 1 year. In conclusion, the operative mortality of Norwood operation was relatively high compared to other operation for major cardiac anomalies, continuing experience will lead to an improvement in result.
Aorta ; Aorta, Thoracic ; Aortic Coarctation ; Cardiopulmonary Bypass ; Fontan Procedure ; Heart ; Heart Bypass, Right ; Heart Defects, Congenital* ; Heart Diseases ; Heart Septal Defects, Ventricular ; Hemodynamics ; Humans ; Hypoplastic Left Heart Syndrome ; Infant* ; Mitral Valve Stenosis ; Mortality ; Norwood Procedures* ; Pneumonia, Aspiration ; Survival Rate ; Tricuspid Atresia ; Weaning

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

There are various surgical techniques in repairing Ebstein anomaly, but residual tricuspid regurgitation and compromized right heart function may ensue in some cases. We report our clinical experience of Ebstein anomaly and atrial flutter in a 19-year-old male patient who underwent simple modified tricuspid annuloplasty, bi-directional cavopulmonary shunt and cryoablation of cavotricuspid isthmus.
Atrial Flutter ; Cryosurgery ; Ebstein Anomaly* ; Heart ; Heart Bypass, Right ; Humans ; Male ; Tricuspid Valve Insufficiency ; Young Adult

There are various surgical techniques in repairing Ebstein anomaly, but residual tricuspid regurgitation and compromized right heart function may ensue in some cases. We report our clinical experience of Ebstein anomaly and atrial flutter in a 19-year-old male patient who underwent simple modified tricuspid annuloplasty, bi-directional cavopulmonary shunt and cryoablation of cavotricuspid isthmus.
Atrial Flutter ; Cryosurgery ; Ebstein Anomaly* ; Heart ; Heart Bypass, Right ; Humans ; Male ; Tricuspid Valve Insufficiency ; Young Adult