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

Although compressed gas (CO2) blowers have been used safely to aid accurate grafting during off-pump coronary bypass surgery, hemodynamic collapse due to gas embolism into the right coronary artery may occur. Supportive measures to facilitate gas clearance by increasing the coronary perfusion pressure have been reported to be successful in restoring hemodynamic stability. However, right ventricular dysfunction and atrioventricular nodal ischemia may hinder effective systemic delivery of the vasoactive medications, even when performing resuscitative measures such as direct cardiac massage. We herein report a case of cardiac arrest that was caused by a right coronary gas embolism and that could not be restored by cardiac resuscitation. When supportive measures fail, direct aortic injection of epinephrine to increase the coronary perfusion pressure can be attempted before initiating cardiopulmonary bypass, and this approach may be life-saving in situations that limit systemic drug delivery from the venous side despite the performance of direct cardiac massage.
Aorta ; Cardiopulmonary Bypass ; Coronary Artery Bypass, Off-Pump ; Coronary Vessels ; Embolism* ; Embolism, Air ; Epinephrine* ; Heart Arrest* ; Heart Massage ; Hemodynamics ; Ischemia ; Perfusion ; Resuscitation* ; Transplants ; Ventricular Dysfunction, Right

Congenital tracheal stenosis (CTS), though rare, is important because the mortality and morbidity rates are high in infants. Especially, associated congenital heart disease (CHD) in these infants may compound the effects of airway pathology. A 3-week-old patient with long-segmental tracheal stenosis below an anomalous right-upper lobe (RUL) bronchus had undergone a total correction of double outlet right ventricle. On third postoperative day, hypercarbia developed, and severe airway obstruction and atelectasis were detected. An emergency slide tracheoplasty was performed under cardiopulmonary bypass (CPB). The patient recovered well after the surgery. Thus, special attention needs to be paid during the postoperative intensive care of patients with congenital tracheal anomalies. Early detection and prompt diagnosis of airway obstruction can help reduce the morbidity and mortality rates. Further, it is important to select the suitable treatment of CTS associated with CHD.
Airway Obstruction ; Bronchi ; Cardiopulmonary Bypass ; Constriction, Pathologic ; Double Outlet Right Ventricle ; Emergencies ; Heart ; Heart Diseases ; Humans ; Infant ; Critical Care ; Pulmonary Atelectasis ; Trachea ; Tracheal Stenosis

BACKGROUND: During coronary anastomosis in off-pump coronary artery bypass surgery (OPCAB), hemodynamic alternations can be induced by impaired diastolic function of the right ventricle. This study was designed to examine the effect of milrinone on right ventricular function and early outcomes in patients undergoing OPCAB. METHODS: Forty patients undergoing OPCAB were randomly assigned in a double-blind manner to receive either milrinone (milrinone group, n = 20) or normal saline (control group, n = 20). Hemodynamic variables were measured after pericardiotomy (T1), 5 min after stabilizer application for anastomosis of the left anterior descending coronary artery (LAD, T2), the obtuse marginalis branch (OM, T3), the right coronary artery (RCA, T4), 5 min after sternal closure (T5), and after ICU arrival. The right ventricular ejection fraction (RVEF) and right ventricular volumetric parameters were also measured using the thermodilution technique. For evaluation of early outcomes, the 30-day operative mortality and morbidity risk models were used. RESULTS: There was no significant difference in hemodynamic variables, including mean arterial pressure, between the 2 groups, except for the cardiac index and RVEF. The cardiac index and RVEF were significantly greater at T3 in the milrinone group than in the control group. CONCLUSIONS: Continuous infusion of milrinone demonstrated a beneficial effect on cardiac output and right ventricular function in patients undergoing OPCAB, especially during anastomosis of the graft to the OM artery, and it had no adverse effect on early outcomes.
Arterial Pressure ; Arteries ; Cardiac Output ; Coronary Artery Bypass, Off-Pump ; Coronary Vessels ; Heart Ventricles ; Hemodynamics ; Humans ; Milrinone ; Pericardiectomy ; Stroke Volume ; Thermodilution ; Transplants ; Ventricular Function, Right

OBJECTIVETo review the experience of staged total cavopulmonary connection (TCPC) in complex congenital heart diseases.

METHODSFrom June 1998 to March 2008, 22 patients underwent staged TCPC for complex congenital heart diseases. Among them, 9 were univentricular and pulmonary artery valve stenosis; 3 were univentricular and pulmonary artery atresia; 1 was transposition of great arteries, crisscross heart and pulmonary artery valve stenosis; 1 was complete atrioventricular canal defects, left ventricular hypoplasia, pulmonary artery atresia and atrioventricular valvular regurgitation; 1 was complete atrioventricular canal defects, left ventricular hypoplasia, pulmonary artery valve stenosis and atrioventricular valvular regurgitation after Glenn procedure; 1 was mirror image dextrocardia, single ventricle, pulmonary artery atresia, major aortopulmonary collateral arteries (MAPCAs) and right pulmonary arteriovenous fistula after Glenn procedure; 4 were tricuspid atresia and pulmonary artery valve stenosis; 1 was tricuspid atresia and pulmonary atresia; 1 was mirror image dextrocardia, double-outlet of right ventricle, left ventricular hypoplasia, pulmonary artery valve stenosis, tricuspid incompetence, and MAPCAs. Among them, 5 patients received systemic-to-pulmonary artery shunt, bidirectional Glenn procedure and TCPC. Seventeen patients received bidirectional Glenn procedure, the mean age was (5.9+/-4.4) years old. Pulmonary artery pressure pre-Glenn procedure was 17 to 20 mm Hg (1 mm Hg=0.133 kPa). Atrioventricular valve incompetence in 3 patients. Nakata index was less than 200 mm2/m2 in 4 patients before the first stage operation. The age of TCPC procedure was (9.6+/-4.9) years old, the interval time was (3.7+/-1.2) years.

RESULTSThere was one in-hospital death, the mortality was 4.5%. The patient with univentricular and pulmonary atresia, received systemic-to-pulmonary artery shunt, bidirectional Glenn procedure and TCPC and died of pneumorrhagia. Other patients were recovered well, postoperative central venous pressure was 12 to 18 mm Hg, percutaneous oxygen saturation was 90% to 96%. The cardiac function were in NYHA class I to II.

CONCLUSIONSThe staged TCPC was a good procedure in high-risk Fontan candidates. The results were satisfactory for those patients. This staged strategy may extend the operative indications for the Fontan procedure.

Adolescent ; Anastomosis, Surgical ; methods ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Heart Bypass, Right ; methods ; Heart Defects, Congenital ; surgery ; Humans ; Male ; Pulmonary Artery ; surgery ; Retrospective Studies ; Treatment Outcome ; Venae Cavae ; surgery ; Young Adult

OBJECTIVETo compare the postoperative outcomes of patients with the diagnostic univentricular heart undergoing lateral tunnel (LT) operation with extracardiac conduit (EC) operation.

METHODSFrom June 1996 to July 2007, 114 consecutive patients with a single ventricle underwent total cavopulmonary connection (TCPC) in Children's Heart Center, University Hospital Giessen and Marburg GmbH, Germany. A LT was performed in 19 (16.7%) patients, and an EC in 95 (83.3%) patients. The mean age of EC group was 50.8 +/- 31.6 (ranging from 22 to 212) months, and that of LT group was 61.5 +/- 41.2 (ranging from 30 to 168) months. Early and midterm outcomes of two groups were analyzed.

RESULTSOne died in LT group (5.3%) and three in EC group (3.2%). The overall mortality was 3.5%. There was no significant difference in mortality between EC and LT groups (P>0.05). The postoperative pulmonary arterial pressure, oxygen saturation, and effusion time of two groups had no significant difference (all P>0.05). No significant difference in the occurrences of complications (arrhythmias, enteropathy, and thrombosis) was found between two groups after operation (P>0.05).

CONCLUSIONSThere seems no difference between LT and EC in the clinical results in the early and middle postoperative stage. Glenn anastomosis followed by an EC seems to have some advantages.

Child ; Heart Bypass, Right ; methods ; Heart Ventricles ; abnormalities ; surgery ; Humans ; Treatment Outcome

Although numerous studies have validated the efficacy of intracoronary shunt on reducing left ventricular dysfunction during off-pump coronary artery bypass surgery (OPCAB), there is lack of evidence supporting its role on right ventricular (RV) function during right coronary artery (RCA) revascularization. Therefore, we studied the effect of intracoronary shunt during grafting of dominant RCA without visible collateral supply on global RV function using thermodilution method. Forty patients scheduled for multivessel OPCAB with right dominant coronary circulation without collateral supply confirmed by angiography were randomized to RCA revascularization either with a shunt (n=20) or soft snare occlusion (n=20). RV ejection fraction (RVEF) was recorded at baseline, during RCA grafting, and 15 min after reperfusion. Corresponding RV stroke work index (RVSWI) was calculated. RVEF and RVSWI decreased significantly during RCA grafting and returned to baseline values after reperfusion in both groups without any significant differences between the groups. Intracoronary shunt did not exert any beneficial effect on global RV function during RCA grafting, even in the absence of visible collateral supply. Regarding the possibility of graft failure by intracoronary shunt-induced endothelial damage, routine use of intracoronary shunt during RCA grafting is not recommended in patients with preserved biventricular function.
Aged ; Blood Pressure ; *Collateral Circulation ; Coronary Artery Bypass, Off-Pump/*methods ; Coronary Artery Disease/physiopathology/*surgery ; *Coronary Circulation ; Female ; Heart Rate ; Humans ; Male ; Middle Aged ; Norepinephrine/administration & dosage ; Prospective Studies ; Stroke Volume ; Sympathomimetics/administration & dosage ; Thermodilution ; *Ventricular Function, Right

BACKGROUND: We retrospectively evaluated the clinical results of surgically managing patients with Ebstein's anomaly. MATERIAL AND METHOD: Between Feb. 1984 and June 2006, 50 patients who underwent surgical treatment for Ebstein's anomaly at Yonsei Cardiovascular Center were retrospectively reviewed. The mean age of the patients was 26.9 years and 19 patients were male. Associated anomalies included atrial septal defect (33), patent ductus arteriosus (2), ventricular septal defect (1), and pulmonary stenosis (4), and 90% (45/50) of the patients had more than a moderate degree of tricuspid regurgitation. Carpentier type A was present in 6 patients, type B in 26, type C in 14 and type D in 4. Ten patients were associated with WPW syndrome. Conservative surgery was possible in 31 patients (tricuspid annuloplasty, plication of the atrialized RV), Fontan's operation was performed in 4 patients, tricuspid valve replacement was done in 12 and palliative surgery was done in 2 patients. Thirteen patients were associated with bi-directional cavopulmonary shunt (BCPS: one and a half ventricular repairs); 10 patients with WPW syndrome and 4 patients with atrial fibrillation underwent concomitant ablation. RESULT: The postoperative median NYHA functional class (3-->1) and the mean cardio-thoracic ratio (0.65-->0.59) were decreased significantly (p<0.001, p=0.014). The mean oxygen saturation (86.6-->94.1%), and median TR grade (4-->1) were also significantly improved (p=0.004, p<0.001). For comparison of BCPS and conservative surgery, the preoperative right ventricular pressure (33.0 vs. 41.3 mmHg), the ICU stay (2.86 vs. 1.89 days), the hospital say (10.6 vs. 16.8 days), and the left ventricular ejection fraction (64.3 vs. 72.8%) were statistically different. Postoperative mortality occurred in 3 patients (6%) due to biventricular failure in 2 patients and sepsis in the other patient. The mean follow up duration was 101.5 months, and one patient died of Fontan failure and 6 patients required reoperation (bioprosthetic degenerative change (2) and Fontan conversion (4)). The overall survival rate at 10 years was 90.2%; the freedom from reoperation rate and rate of cardiac related events were 78.9% and 49.2%, respectively. CONCLUSION: Surgical management of Ebstein's anomaly can be performed safely, and the associated BCPS may be helpful for high-risk patients. Adequate application of surgical management may increase the long-term survival with a reduced rate of reoperation.
Atrial Fibrillation ; Ductus Arteriosus, Patent ; Ebstein Anomaly* ; Follow-Up Studies ; Fontan Procedure ; Freedom ; Heart Bypass, Right ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Male ; Mortality ; Oxygen ; Palliative Care ; Pulmonary Valve Stenosis ; Reoperation ; Retrospective Studies ; Sepsis ; Stroke Volume ; Survival Rate ; Tricuspid Valve ; Tricuspid Valve Insufficiency ; Ventricular Pressure ; Wolff-Parkinson-White Syndrome

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

This investigation evaluated the effect of continuous milrinone infusion on right ventriclular (RV) function during off-pump coronary artery bypass graft (OPCAB) surgery in patients with reduced RV function. Fifty patients scheduled for OPCAB, with thermodilution RV ejection fraction (RVEF) <35% after anesthesia induction, were randomly allocated to either milrinone (0.5 microgram/kg/min) or control (saline) group. Hemodynamic variables and RV volumetric data measured by thermodilution method were collected as follows: after anesthesia induction (T1); 10 min after heart displacement for obtuse marginal artery anastomosis (T2); after pericardial closure (T3). Cardiac index and heart rate increased and systemic vascular resistance significantly decreased in milrinone group at T2. Initially lower RVEF of milrinone group was eventually comparable to control group after milrinone infusion. RVEF did not significantly change at T2 and T3 in both groups. RV end-diastolic volume in milrinone group consistently decreased from the baseline at T2 and T3. Continuous infusion of milrinone without a bolus demonstrated potentially beneficial effect on cardiac output and RV afterload in patients with reduced RV function during OPCAB. However, aggressive augmentation of intravascular volume seems to be necessary to maximize the effect of the milrinone in these patients.
Ventricular Function, Right/*drug effects ; Phosphodiesterase Inhibitors/*pharmacology ; Milrinone/*pharmacology ; Middle Aged ; Male ; Humans ; Heart Rate/drug effects ; Female ; Echocardiography, Transesophageal ; *Coronary Artery Bypass, Off-Pump ; Blood Pressure/drug effects ; Aged