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

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

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

We performed this study to evaluate hepatic venous drainage in atrial isomerism by MR and the clinical significance of anomalous hepatic venous return in total cavopulmonary shunt operation. Numbers and locations of hepatic veins in twenty-two patients with isomerism(thirteen with right isomerism and nine with left isomerism) were evaluated by MR. Operative procedure of hepatic veins and postoperative arterial oxygen saturation were compared with hepatic vein connection in six patients after total cavopulmonary shunt operation. Among nine patients with left isomerism, hepatic venous return was totally anomalous via a single opening in eight, and via two separate openings in one. Among thirteen patients with right isomerism, partial anomalous hepatic venous connection directly to the atrium was seen in four. One showed total anomalous hepatic venous connection to atrium through one opening. Total cavopulmonary shunt operation was performed in 6 patients. Hepatic veins were connected to pulmonary arteries in four patients who had one atrial opening of hepatic vein and/or IVC, or two ipsilateral atrial opening of hepatic veins and IVC. In conclusion, hepatic vein drainage to atrium is variable in atrial isomerism. MR is useful for evaluation of hepatic vein drainage in atrial isomerism and surgical planning.
Drainage ; Heart Bypass, Right ; Heart Defects, Congenital ; Hepatic Veins ; Humans ; Isomerism* ; Magnetic Resonance Imaging ; Oxygen ; Pulmonary Artery ; Surgical Procedures, Operative

Over the past several years, a number of centers have reported favorable results of anatomical repair for the congenitally corrected transpositions. However, there have been subsequent problems related mainly to the results of atrial switch procedures in patients who had small atriums because of venoatrial obstructions or supraventricular arrhythmias, especially in patients with apicocaval juxtaposition. Cavopulmonary shunt may be a useful addition to the double switch operation in certain circumstances as a means of avoiding potential atrial complications. Herein, we describe the successful anatomical repair of congenitally corrected transposition of the great artery with pulmonary atresia, ventricular septal defect, and cavo apical juxtaposition with this modification.
Anastomosis, Surgical ; Arrhythmias, Cardiac ; Arteries ; Heart Bypass, Right ; Heart Septal Defects, Ventricular ; Humans ; Pulmonary Atresia ; Transposition of Great Vessels

Sixteen-day-old baby with severe Ebstein anomaly underwent emergency operation to relieve progressive hypoxia and congestive heart failure. Operative findings showed huge right atrium and atrialized right ventricle (aRV) with very small functional RV by distal displacement of tricuspid valve mechanism. We elected to perform modified Starnes operation because biventricular repair was deemed unattainable. After pulmonary and tricuspid valves were primarily closed, aRV was obliterated with multiple sutures from RV apex to the base. Then a PTFE (Gore-Tex, USA) vascular graft was interposed between innominate artery and main pulmonary artery for systemic to pulmonary shunt. The patient was discharged uneventfully, and received bi-directional cavopulmonary shunt 6 months later.
Anoxia ; Brachiocephalic Trunk ; Ebstein Anomaly* ; Emergencies ; Heart Atria ; Heart Bypass, Right ; Heart Defects, Congenital ; Heart Failure ; Heart Ventricles ; Humans ; Polytetrafluoroethylene ; Pulmonary Artery ; Sutures ; Transplants ; Tricuspid Valve

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

OBJECTIVETo evaluate surgical methods and results of extracardiac conduit total cavopulmonary anastomosis (EC-TCPA) without cardiopulmonary bypass (CPB).

METHODSFrom May 2000 to April 2003, 11 patients with functional univentricle underwent off-pump EC-TCPA (no-CPB group). Their postoperative outcome was retrospectively compared with a 17-patient group who underwent EC-TCPA with cardiopulmonary bypass (CPB group) over a concurrent time period.

RESULTSThere was 1 operative death in no-CPB group and 2 in CPB group; early postoperative hemodynamics appeared to significantly improve in no-CPB group. Blood and platelet transfusions decreased and blood plasma transfusion significantly lowered in no-CPB group compared with CPB group (P = 0.036). Postoperative courses of patients in no-CPB group were smooth and event free, and extubation time was substantially short Intensive cares unit stay (P = 0.04) and hospital stay (P = 0.02) postoperation were significantly shorter, hospital costs were significantly reduced (P = 0.004) in no-CPB group compared with CPB group.

CONCLUSIONSEC-TCPA without use of CPB is not a difficult procedure; the procedure results in improvement in postoperative hemodynamics, and decreased use of blood and blood products. It is a more efficient operation with more short recovery time and reduced hospital stay.

Adolescent ; Blood Pressure ; Cardiopulmonary Bypass ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Heart Bypass, Right ; methods ; Heart Defects, Congenital ; physiopathology ; surgery ; Humans ; Male ; Retrospective Studies

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