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We present a 19-year-old college student completely asymptomatic who underwent successful Bjork surgery (right atrium-to-right ventricle connection) in infancy with excellent results. Surgical approach for tricuspid atresia is done in three stages. Blalock Tuassig shunt soon after birth or pulmonary artery band depending on the size of the ventricular septal defect (VSD) and the main pulmonary artery, Glenn surgery (bidirectional cavopulmonary shunt) at 3-6 months of age, and Fontan surgery at 2-3 years age. There are many short- and long-term complications after Fontan surgery and patients require multiple medications throughout life. For tricuspid atresia with normally related great arteries, large VSD, and normal pulmonary valve, Bjork surgery offers the advantages of right ventricular growth, biventricular function, and good outcome without any medications.

This report describes the uses of a large Eustachian valve as a cardiovascular patch. In one patient, the large Eustachian valve was used to close an atrial septal defect and in another patient it was used to create a tunnel during the lateral tunnel Fontan operation.

Purpose of Review: This article reviews three common abnormalities requiring surgical treatment in adults with congenital heart disease (CHD): Ebstein's anomaly, the failing Fontan, and pulmonary valve disease., Recent Findings: As of 2000, more adults than children are alive with CHD. Each year, more of these adults with CHD undergo surgery. Three common operations performed on adults with CHD are surgery for Ebstein's anomaly, Fontan revision, and pulmonary valve replacement (PVR). Recent advances have been made in each of these three operations., Summary: Surgery for Ebstein's anomaly can now be offered to older patients at low risk and with good late outcome. The operation includes tricuspid valve repair or replacement and frequent concomitant procedures such as atrial septal defect closure, arrhythmia surgery (the maze procedure), and coronary artery bypass grafting. Fontan conversion involves: conversion of the previously created atriopulmonary connection to a total cavopulmonary artery, extracardiac Fontan; arrhythmia surgery, typically with a modified biatrial maze procedure along with placement of an antitachycardia, dual-chamber pacemaker with steroid-eluting epicardial leads in all patients; and concomitant procedures to treat associated lesions. The need for PVR is increasing for many adults with CHD. In the past, residual defects such as chronic pulmonary regurgitation following repair of tetralogy of Fallot were considered benign. Recent evidence suggests that pulmonary regurgitation causes significant morbidity, producing right ventricular dilatation and dysfunction, exercise intolerance, arrhythmias, and sudden death. Multiple options exist for PVR including several recent developments such as PVR with a man-made polytetrafluoroethylene bicuspid valve and percutaneous PVR.

Patients undergoing bi-directional Glenn shunt for various congenital anomalies of the heart will have their superior vena cava (SVC) clamped during the procedure. The duration of the procedure is variable, ranging from five to 30 minutes. This can affect the cerebral perfusion due to raised venous pressure [Cerebral blood flow = Mean arterial pressure-(Intracranial pressure + Central venous pressure)]. Shunting away the SVC blood is a well known technique to counter this problem, but we describe two cases where a novel technique was successfully used to decompress the SVC.

Improved surgical and medical management has led to an increase in survival after staged univentricular palliative procedures. Subsequently, this improved survival has led to an increase in the number of patients who will present for noncardiac surgical interventions with Fontan physiology. A comprehensive understanding of normal Fontan physiology and the perturbations that the proposed surgical procedure will likely have is necessary to care for and design a comprehensive anesthetic plan that takes into account the effects of anesthetic agents, ventilation strategies, cardiovascular drugs, and various other perioperative factors. Applying the knowledge presented in this article should enable the anesthesiologist with the necessary principles to care for the patient with Fontan physiology.

An infant of a diabetic mother with a prenatal diagnosis of tetralogy of Fallot and tricuspid atresia is presented. To the best of the authors' knowledge, this is the first report of such an association.

The modified Fontan procedure may be complicated by prolonged pleural drainage. Predisposing factors are not fully understood. This study examines perioperative variables associated with prolonged effusions. We examined the Fontan procedure in 41 patients. Mean age was 45 months (range, 9 to 113 months). Mean weight was 14.7 kg (range, 6.9 to 30.4 kg). Diagnoses included tricuspid atresia in 29 per cent, pulmonary atresia in 12 per cent, and double-outlet right ventricle in 12 per cent. Fontan revisions were excluded. The extracardiac Fontan was performed in 22 patients (54%) and the lateral tunnel (LT) in 18 (44%). Fenestration was performed in 56 per cent. Ten patients (24%) had prolonged pleural effusions. Mean chest tube output (24.2 vs 14.1 mL/kg/d, P < 0.01), days with chest tubes (10.2 vs 5.8 days, P < 0.01), and length of stay (20.8 vs 8.13 days, P < 0.01) were significantly greater in those with effusions. Preoperative mean pulmonary artery pressure was higher in patients with prolonged effusions (12.7 vs 9.90 mmHg, P = 0.001). No other factors were identified as risk factors. Prolonged pleural effusions (greater than 14 days) are common after the modified Fontan procedure. A higher preoperative mean pulmonary artery pressure may be predictive of these effusions. Fenestration, type of Fontan reconstruction, and size of extracardiac conduit did not predispose to postoperative effusions.

Background: Although the Fontan procedure is now being applied to younger patients, the influence of patients' age at the time of surgery on long-term results remains unclear. We investigated whether age at Fontan completion affects subsequent hemodynamics and exercise capacity in patients with a dominant left ventricle followed up for more than 5 years., Methods: Between January 1990 and December 2001, 68 patients with a dominant left ventricle underwent total cavopulmonary connection (TCPC) at our institute. The survivors routinely underwent postoperative exercise tests and catheterization at 1 year and then every 5 years after TCPC, and were divided into group A (< 3 years; n = 32) and group B (> or = 3 years; n = 33), according to age at time of the Fontan procedure and retrospectively reviewed. We also examined the correlation between age at Fontan and data from postoperative catheterization or exercise tests., Results: None of the patients died during a follow-up period of 91.2 +/- 46.7 months. The postoperative exercise capacity (peak oxygen consumption; % of normal [61.3 +/- 11.5 versus 51.9 +/- 9.1, p = 0.0001]), cardiac index at 5 years (3.31 +/- 0.62 versus 2.86 +/- 0.69, p = 0.0133) and at 10 years after TCPC (3.24 +/- 0.46 versus 2.61 +/- 0.65, p = 0.0194), and ventricular ejection fraction (%) at 10 years after TCPC (62.1 +/- 7.6 versus 53.0 +/- 8.0, p = 0.0131) were significantly higher in group A. Age at Fontan completion inversely correlated with postoperative peak oxygen consumption (R = -0.374, p = 0.0006) and cardiac index at 5 years (R = -0.452, p = 0.0003) and 10 years after TCPC (R = -0.491, p = 0.0072)., Conclusions: Earlier TCPC is beneficial in the long term from the viewpoint of exercise capacity and hemodynamics among patients with a dominant left ventricle.

Objective: To assess the surgical results of the Norwood procedure and subsequent clinical outcome in the setting of transposition of the great arteries (TGA) with a dominant morphologic left ventricle., Methods: Among 486 patients who underwent the Norwood procedure from 1988 to 2007 at our institution, there were 37 patients with TGA and left ventricular dependant circulation with the following associated lesions: double inlet left ventricle (DILV) (n=24), tricuspid atresia (n=9), ventricular septal defect (VSD) with hypoplastic right ventricle (RV) (n=4). Outcomes for all three-staged procedure were compared with the overall Norwood group., Results: Early mortality was 21.6% (8/37) compared to 26.7% (120/449) in the overall Norwood group (p=ns). There was only one subsequent death giving a 5- and 10-year actuarial survival of 72.8+/-7.4% compared to 55.3+/-2.6% and 52+/-2.9% at 5 and 10 years for the overall series (p=0.06). Median follow-up was 4.7 (0.7-10.2) years. Eighteen patients underwent stage III completion at 3.9+/-1.5 years from the second stage with no mortality. Preoperative mean pulmonary artery (PA) pressure and transpulmonary gradient were respectively 11.6+/-3.4 and 5.2+/-3.3 mmHg. All patients had good left ventricle (LV) function at time of stage III. All patients except one are currently in NYHA I. One patient (with DILV) had congenital heart block and required a pacemaker. There was no postoperative heart block. The systemic outflow was unobstructed in all patients and no patient required any additional intracardiac procedure., Conclusions: The Norwood procedure provides good palliation in this subgroup of patients and avoids the need for subsequent intracardiac operations, maintaining unobstructed systemic outflow tract and avoiding the risk of postoperative heart block.

Most commonly encountered cyanotic cardiac lesions in children, namely, tetralogy of Fallot, transposition of the great arteries and tricuspid atresia are reviewed. Pathology, pathophysiology, clinical features, non-invasive and invasive laboratory studies and management are discussed. The clinical and non-invasive laboratory features are sufficiently characteristic for making the diagnosis and invasive cardiac catheterization and angiographic studies are not routinely required and are needed either to define features, not clearly defined by non-invasive studies or as a part of catheter-based intervention. Surgical correction or effective palliation can be undertaken with relatively low risk. However, residual defects, some requiring repeat catheter or surgical intervention, are present in a significant percentage of patients and therefore, continued follow-up after surgery is mandatory.

The occurrence of a restriction of the bulbo-ventricular foramen (BVF) in older patient with double inlet left ventricle (DILV) or tricuspid atresia (TA) with ventriculo-arterial discordance is a well-known condition. Today, the surgical management is to perform a Damus-type operation at the time of the bi-directional Glenn or at the Fontan completion. The ventricular septal defect (VSD) enlargement, associated with muscular resection and a patch enlargement of the subaortic accessory ventricular chamber, is rarely performed but remains indicated in cases with pulmonary valve atresia or regurgitation. This condition is essentially prevented by doing an early Norwood-type operation in the presence of DILV/TA with transposition of the great arteries associated with an aortic arch obstruction. The palliative switch operation is an option that was abandoned because of poor control of the pulmonary blood flow. It is only in cases of large unobstructed BVF that pulmonary artery banding could be undertaken in neonates, followed by close echocardiographic follow-up. The occurrence of a restriction or a closure of the VSD in complex DORV following a Fontan operation is a dramatic event and is quite "new business." It has been recently recognized that the VSD becomes restricted in a number of patients with DORV-nc-VSD treated with a Fontan palliation. This new condition is not surprising knowing that 75% of the VSDs must be enlarged preventively in DORV-nc-VSD repair. In the setting of a Fontan circulation, the supra-systemic left ventricle has severe consequences the right ventricle performance. Attempts at surgical VSD enlargement or catheter-based procedures have resulted in almost constant recurrence. This recently reported complication is in favor of also performing a VSD enlargement at the time of the Fontan completion in complex DORV. It justifies the biventricular repair in complex DORV with two viable ventricles.

Single ventricle physiology offers limited options with significant morbidity and mortality. The Glenn shunt is a mid-stage procedure for the Fontan circulation. With persistent left Superior Vena Cava (SVC) and Azygos continuation of the Inferior Vena Cava (IVC) to the SVC, needs the development of bilateral bidirectional Glenn shunts. Fifteen patients underwent Bidirectional Glen shunt procedure over a period of ten years. Of these two were found to have left SVC along with interrupted IVC and its Azygos continuation to the SVC. These two patients underwent the Kawashima operation. A seven years old girl was admitted via emergency with severe cyanosis. She had tricuspid atresia, azygos continuation of IVC with single ventricle physiology. She had a complicated postoperative course with prolonged hospital stay after bilateral bidirectional Glen shunt (Kawashima operation). She successfully has completed Fontan after 5 years. The second case ofa 7 year old girl had elective surgery for DORV (double outlet right ventricle), pulmonary atresia and azygos continuation of IVC and persistent left SVC. She had an uneventful postoperative course. The Kawashima operation is an established surgical procedure to deal with bilateral SVCs. The procedure is safe; outcome is favourable and prepares the patient for total cavo-pulmonary shunt.

We present a case of tricuspid atresia and the treatment that was used in a child now aged 12. The malformation was corrected at the age of five through intracardiac Fontan circulation. Later, the child developed a right-to-left shunt due to dehiscence in the intra-atrial conduit, causing significant desaturation. This was treated by transcatheter closure with a 10 mm Amplatzer device at 8 years of age. Currently the child is asymptomatic with no residual shunts, and the position of the device is normal. The percutaneous closure of Fontan fenestrations using the Amplatzer device is a therapeutic approach that provides good results. It results in normal oxygen saturation and has low morbidity. The technique is simple, available to most cardiac catheterization laboratories, safe, and offers a reasonable cost-benefit ratio. In addition, the design of the device is suitable for this type of defect.

Background: Tricuspid Atresia is the 3rd commonest cyanotic congenital Heart disease. It is characterized by lack of communication between the right atrium and right ventricle. The treatment often requires a palliative systemic to pulmonary shunt before definite surgery. The use of a central shunt via a median sternotomy is suggested here as an alternative to other traditional shunts via a thoracotomy., Method: The management of a 3-month-old boy who presented with dyspnoea, fever, cough and cyanosis is presented here as obtained from Clinical records., Result: Following resuscitation, a central shunt (Ascending Aorta to main Pulmonary Artery) was constructed and the patient did well despite a turbulent post-operative period., Conclusion: The management of tricuspid atresia likes other cyanotic heart disease is daunting but palliative treatment is possible in our environment and definitive treatment where possible affords a fairly satisfactory prognosis.

Purpose: We hypothesized that a propeller pump design would function optimally to provide cavopulmonary assist in a univentricular Fontan circulation., Description: The hydraulic and hemolysis performance of a rigid three-bladed propeller prototype (similar to a folding propeller design) was characterized. Pressure and flow measurements were taken for flow rates of 0.5 to 3 liters per minute (LPM) for 5,000 to 7,000 revolutions per minute (RPM) using a blood analog fluid. Hemolysis testing was performed using fresh bovine blood for 2 LPM at 6,000 RPM for a 6-hour duration., Evaluation: The prototype performed well over the design operating range by producing a pressure rise of 5 to 50 mm Hg. Plasma free hemoglobin concentration remained less than 15 mg/dL. The normalized index of hemolysis peaked during the first hour, and then remained less than 10 mg/dL thereafter., Conclusions: A propeller pump has the pressure-flow characteristics and minimal risk of hemolysis and venous pathway obstruction which make it ideal for temporary cavopulmonary assist. This type of device has the potential to provide a new therapeutic option for patients with failing univentricular Fontan physiology as a bridge-to-recovery or transplantation.

Cardiac and vascular intervention in thrombocytopenic congenitally cyanotic patients is more dangerous. Thrombocytopenia in these patients is related to immune thrombocytopenia, polycythemia, hyperviscosity, pseudothrombocytopenia, and drugs. Herein we report on a thrombocytopenic 8-year-old girl with tricuspid valve atresia and pulmonary valve stenosis admitted for catheterization. Thrombocytopenia (21,000/mm3) and shunt occlusion was noticed. Thrombocytopenia did not recover after intravenous immunoglobulin (IVIG) and phlebotomy therapies. During preparation for surgery, she suffered cardiopulmonary arrest. A Gore-tex graft was placed in the right pulmonary artery and truncus brachiocephalicus. After surgery, her platelet count spontaneously increased to within the normal range (178,000/mm3 to 250,000/mm3). After resuscitation, she had right-sided hemiplegia sequelae, though there were no hemorrhagic findings on cranial magnetic resonance imaging (MRI) or computed tomography (CT) scans. Two months after surgery, the Blalock-Taussig (BT) shunt blood flow decreased, thrombocyte count dropped, and peripheral cyanosis reappeared. A Fontan operation was performed without hemorrhagic events, and after surgery the thrombocyte count reached 330,000/mm3. We suggest that if a patient with cyanotic heart disease has thrombocytopenia and there is no apparent cause, hypoxia-related thrombocytopenia must be considered. After reoxygenation by shunt or corrective surgeries, thrombocyte count and functions will recover.

The tricuspid atresia associated with persistent truncus arteriosus is a rare anomaly. A case is presented of one male patient of three months age in which was detected a cardiac murmur. The diagnosis was made by echocardiography and confirmed by cardiac catheterization. The first step of the palliation was done with disconnection of the trunk of the pulmonary artery combined with a systemic- to- pulmonary shunt of 4 mm. The patient died 24 hours later for persistent metabolic acidosis.

Background: Outcomes of patients undergoing cavopulmonary palliation for single ventricle physiology may be impacted by living at altitude, as the passive pulmonary circulation is dependent on the resistance of the pulmonary vascular bed. The objective of this study is to identify risk factors for failure of cavopulmonary palliation at elevated altitude., Methods and Results: Between January 1995 and March 2007, 122 consecutive patients living at a mean altitude of 1600 m (range 305 to 2570) underwent a bidirectional Glenn (BDG). There was one in-hospital mortality and 7 late deaths. 52 have proceeded to the Fontan procedure. Survival after BDG was 92.4% at 5 years. Freedom from palliation failure, defined as death, transplant, BDG/Fontan takedown, or revision was 81% at 5 years. At a mean follow-up of 39.8 months, 90 patients (75%) were in New York Heart Association class I. Patients with failing cavopulmonary circulation had higher pre-BDG pulmonary artery pressure (PAP) (18.3+/-6.1 mm Hg versus 14.8+/-5.1 mm Hg, P=0.016) and higher pre-BDG transpulmonary gradient (TPG) (11.2+/-6.2 mm Hg versus 7.7+/-4.3 mm Hg, P=0.014). Post-BDG, patients with palliation failure had increased PAP (15.0+/-5.7 mm Hg versus 10.8+/-2.8 mm Hg, P=0.008) and indexed pulmonary vascular resistance (PVRI) (2.43+/-1.0 Wood U . m(2) versus 1.52+/-0.9 Wood U . m(2), P=0.007)., Conclusions: The majority of patients at moderate altitude have favorable outcomes after BDG or Fontan palliation. Risk factors for palliation failure at elevated altitude include PAP >15 mm Hg, TPG >8 mm Hg, and PVRI >2.5 Wood U . m(2).

Fontan patients are doomed to a circulatory failure and many of them will require a circulatory assistance as a bridge to transplantation. The univentricular heart with a total cavopulmonary connection presents a special challenge for the insertion of an assist device. We report a patient in multiple organ dysfunction and failure who was supported by right-sided univentricular assistance. Technically, a new chamber was created between both vena cava for implantation of the inflow cannula, and the extracardiac conduit was used to set the outflow cannula. The patient dramatically recovered and is currently in the best condition for heart transplantation.

In patients with Bidirectional Glenn who have undergone surgery, the superior caval venous flow provides the only pulmonary blood supply. This is the effective pulmonary flow and at the same time its volume is not enough to overflow the single ventricle. The unsaturated, inferior vena cava flow is not oxygenated, since it goes across the interatrial septal communication and gets mixed in the left ventricle with the pulmonary venous blood. In this work, a bidirectional Glenn case is analyzed. The hemodynamic data before and after the operation are shown. It was evident from this case that the use of the Fick method to measure pulmonary flow in patients with bidirectional Glenn operation is not appropriate. Alternative methods, such as Doppler echocardiography and Magnetic Resonance Imaging, are recommended. A literature review on this subject was carefully done.

We described a case of a quasi obstructive thrombus in the right atrium in a patient with a Fontan palliation. Different medical imaging techniques helped us for diagnosis and management strategy. We discussed the therapeutic choice between thrombolysis or surgery.

A median sternotomy would be very difficult for Cantrell syndrome with severe ectopia cordis. For Cantrell syndrome and tricuspid atresia after left modified Blalock-Taussig shunt with severe ectopia cordis, defect in the middle and inferior portion of the sternum, and the closing of ventriculo-peritoneal shunt, we performed extracardiac total cavopulmonary connection through a right lateral thoracotomy after establishing right modified Blalock-Taussig shunt and performing coil embolization of left modified Blalock-Taussig shunt by cardiologists.

Clinical Case: This case report documents outpatient cardiac rehabilitation (CR) in a 28-year-old woman born with transposition of the great vessels, tricuspid atresia, hypoplastic right ventricle, and an atrial septal defect. Surgical procedures were performed during childhood to correct these defects. In 2006, she underwent the following procedures: Fontan revision with a graft to an extracardiac total cavopulmonary connection; ASD creation; right atrial reduction; bidirectional Glenn shunt; right atrial and modified left atrial Maze procedures; and placement of an epicardial dual-chamber anti-tachycardia pacemaker. The patient was referred to CR because of postoperative complaints of fatigue, dyspnea on exertion, and low exercise tolerance. At intake, she underwent a cardiopulmonary stress test, measurement of percentage body fat, and completed the Medical Outcomes Survey 36-Item Short-Form Health Survey, Diet Intake Survey, and Center for Epidemiologic Studies Depression Scale. After completing 36 sessions, all outcomes demonstrated improvement with the exception of percentage dietary fat intake., Discussion: Despite increasing numbers of patients with congenital heart disease (CHD) surviving into adulthood, exercise prescription in this population remains poorly delineated. In this case, possible physiologic limitations to exercise included diminished cardiac output secondary to low pressures and flow rates in the pulmonary arteries and veins because of the absence of a functioning right ventricle, limited chronotropic response, and severe deconditioning., Summary: This case presents an adult patient who underwent surgical procedures to avoid heart transplantation. Despite severe CHD with many surgical procedures, and what is effectively a 2-chambered heart, she was able to successfully complete CR, graduate to home exercise, return to independent home living, and pursue her master's degree.

Tricuspid atresia with transposition of the great arteries produces single ventricle physiology. Ultimate goals of neonatal palliative operations are to provide optimum anatomic and physiologic conditions for a Fontan procedure. A modification of the Norwood procedure is reported, with an aorto-pulmonary anastomosis, utilizing the hypoplastic right ventricle as the pulmonary outflow conduit, avoiding a left ventriculotomy and preserving its function with excellent recovery. We believe this technique has not been previously published in the English literature.

The Fontan procedure has become a generic term to define a surgical procedure that orients the systemic venous return directly to the pulmonary arteries, and has been used as a palliative operation for pulmonary atresia, tricuspid atresia, double inlet ventricle and complex single ventricle. The earliest type of Fontan procedure was a simple atriopulmonary anastomosis between the right atrium and the pulmonary artery. Atrial arrhythmias, particularly atrial flutter and sinus node dysfunction may occur in the early and late postoperative period after simple atriopulmonary anastomosis. The case presented here represents a much delayed occurrence of an initial syncope due to alternating attacks of SVT (supraventricular tachycardia) and second degree heart block on admission 21 years after simple atriopulmonary anastomosis performed for the correction of tricuspid atresia.

Background: After Fontan surgery there is no subpulmonary ventricle to modify pulmonary blood flow. The influence of the cardiac cycle on pulmonary blood flow patterns in various types of Fontan patients is unknown., Methods: Blood flow patterns were investigated using phase-velocity cine magnetic resonance imaging in the pulmonary artery of 17 patients (21.1 +/- 7.3 years old, 6 females) with Fontan circulation. These patterns were compared with those of 12 healthy volunteers (26.3 +/- 6.0 years old, 10 females) obtained in the superior vena cava and the main pulmonary artery. Measurements were sampled for a period of about 3 minutes to rule out respiratory effects. Blood flow patterns were depicted by interpolating the variable number of measured phases in every patient to 100 phases and normalizing flow to mean blood flow in that vessel. Then, average flow patterns were calculated throughout the patient groups to depict a typical pattern., Results: In Fontan patients, peaks and troughs are highly variable. In averaged flow patterns for the whole Fontan group, only a slight late diastolic flow acceleration could be detected. This is in contrast to the pattern of the control subjects in whom typical systolic peaks and late diastolic troughs could be found in both the superior vena cava and in the pulmonary artery., Conclusions: There are no typical pulmonary blood flow patterns of cardiac origin in patients with Fontan circulation, except for slight late diastolic flow acceleration representing diastolic inflow restriction.

Acquired inhibitors of coagulation factors, particularly to factor V (FV) and thrombin, after topical bovine thrombin exposure may result in clinically important coagulopathies. While bovine thrombin is commonly used in pediatric patients for surgical hemostasis, the reported cases of acquired inhibitors in children are few. We report two cases of children who developed factor inhibitors after bovine thrombin exposure. One child developed a FV inhibitor at 3 months of age after exposure to bovine thrombin during cardiac surgery. The inhibitor resolved with intravenous immunoglobulin (IVIG) and steroids. The other child developed concomitant FV and bovine thrombin inhibitors after cardiac surgery at age 11 years. The presence of these inhibitors complicated post-operative anti-coagulation management, but the inhibitors were transient. In addition to these two cases, we identified all the pediatric patients with bovine thrombin-induced inhibitors who were reported in the world's literature, and reviewed their clinical characteristics. These cases underscore the fact that bovine thrombin can be antigenic in infants and children and can result in significant coagulopathies., (2007 Wiley-Liss, Inc)

Complex congenital heart disease is the cause for 1.5%-2.4% of heart transplantations in adults. Indications for heart transplantation are ventricular dysfunction, failed Fontan physiology, and cyanosis. We present a 43-year-old man, 20 years after a Fontan operation by an univentricular heart, with a tricuspid atresia, L-Transposition of the Great Arteries, and high-grade subpulmonary stenosis. Persistent bleeding after successful transplantation necessitated major blood transfusions and reoperation. We reconstructed superior vena cava and vena brachiocephalica using a pericardial patch, and because of postoperative low output syndrome, he was connected to extracorporeal circulation through cannulation of femoral artery and explanted after hemodynamic stability. Heart transplantation can be successful in patients with end-stage congenital heart diseases after Fontan procedure, because of improved surgical and medical management. Challenges include limited ability to assess hemodynamics, complex anatomy, increased risk of bleeding caused by multiple prior procedures, and unique underlying pathologic states. Careful patient selection and surgical timing are essential to increase long-term survival.

Objective: To analyze 68 pediatric cases with functional univentricle heart who underwent bidirectional Glenn procedure during from April 1998 to December 2005., Methods: There were 47 males and 21 females in this group, aged from 5 months to 14 years old and weighed from 6.7 to 30.0 kg. Among them, 39 cases were received bidirectional Glenn procedure on the right side, 13 cases on the left side and 16 cases on both sides. Three cases had the pulmonary artery banded; one case had the pulmonary artery ligated;one case had the original A-P shunt cut off; six cases had the PDA ligated; four cases had the MAPCAs cut off; one case had TAPVC corrected contemporarily; two cases of PAPVC were also corrected; four cases had the atrial-ventricular valve repaired., Results: Three cases died. The mortality was 4.4%. The mean post-operative pressure of super vena cava was (15.9 +/- 2.4) mm Hg (1 mm Hg = 0.133 kPa), higher than the pre-operative one (8.3 +/- 1.8) mm Hg (P < 0.01). The mean post operative SpO(2) was (89.3 +/- 4.2)%, higher than the pre-operative one (78.4 +/- 6.0)% (P < 0.01)., Conclusions: Bidirectional Glenn procedure is of satisfied effect on surgical treatment for functional univentricle heart. The persistent forward flow from pulmonary artery should be reserved in bidirectional Glenn procedure.

Congenital sinus of Valsalva aneurysm is a rare cardiac malformation that usually becomes symptomatic as a consequence of intracardiac or extracardiac rupture. It is difficult to suspect in association with complex cardiac defects and its rupture may be misdiagnosed as progressive aortic regurgitation. This case report refers to a patient with tricuspid atresia submitted to Fontan procedure five years previously, in whom a sinus of Valsalva aneurysm rupture into the accessory ventricular chamber caused rapidly progressive heart failure. The malformation was suspected by echocardiography and treated by percutaneous implantation of an Amplatzer duct occluder, with immediate improvement of the patient's clinical and functional status.

Objectives: Atrial tachyarrhythmia is a common complication late after the atriopulmonary connection Fontan procedure. The P-wave duration and dispersion have been regarded as useful markers for its prediction. The purpose of this study was to analyze change in the P-wave characteristics over time after the atriopulmonary connection Fontan procedure, with a special emphasis on the timing of the Fontan conversion., Methods: We reviewed clinical and electrocardiographic data in 18 patients with classic tricuspid atresia who underwent the atriopulmonary connection Fontan procedure. Their median age at operation was 4.1 years (range, 1.1-22 years), and the median follow-up period was 18.1 years (range, 10-25 years). We measured the P-wave duration, dispersion, and axis from consecutive 12-lead electrocardiograms in each patient., Results: There was a significant increase in the maximum P-wave duration as well as the P-wave dispersion over time. Transient ectopic atrial rhythm was observed in 16 patients during follow-up. Atrial tachyarrhythmia developed subsequent to transient ectopic atrial rhythm in 8 patients. Ten patients underwent the conversion. Atrial tachyarrhythmia recurred in 2 patients who had sustained arrhythmia (6.0 and 8.0 years) before the conversion. In contrast, the other 8 patients with transient ectopic atrial rhythm alone or nonsustained atrial tachyarrhythmia have been free from arrhythmia after the conversion., Conclusions: Patients commonly and increasingly had prolonged P-wave duration, larger P-wave dispersion, and ectopic atrial rhythm, along with an atrial tachyarrhythmia propensity, late after the atriopulmonary connection Fontan procedure. These P-wave characteristics were informative when considering the Fontan conversion before a sustained atrial tachyarrhythmia ensued.

Background: Doppler myocardial echocardiography (DME) may be an excellent additional means of assessing determinants of systolic and diastolic ventricular function in patients with tricuspid atresia (TA) after total cavopulmonary connection (TCPC)., Methods and Results: Thirty-three patients with TA and normal systolic shortening/ejection fraction (M-mode) after TCPC were studied by DME at a median age of 7.6 years (range 1.5-17 years). These results were compared to 16 age matched normal controls. Median time under a cavopulmonary shunt was 5.2 years (range 0.6-13.3 years). Isovolumic acceleration and isovolumic velocity did not differ significantly. All other systolic (S-wave acceleration-velocity and S-wave duration) and diastolic DME indices (E-deceleration-velocity, A-velocity and E/A ratio) were significantly lower in TCPC patients in comparison to normals (p<0.0001). Furthermore, isovolumic relaxation time (IRT) was significantly prolonged in the patient group (p<0.0001). Even though there was a significant correlation between the time of volume unloading and left ventricular end-diastolic diameter, exclusion of all patients with short period of volume unloading and relatively large systemic ventricles did not alter the results., Conclusions: Patients with TA after TCPC and normal shortening and ejection fraction have normal isovolumic acceleration. Load dependent DME indices of systolic and diastolic function, however, were significantly reduced. These findings suggest that the described DME indices mirror the "normal" hemodynamics after TCPC in patients with TA. It needs to be assessed, whether this is an index of adverse prognosis in long-term surveillance of these patients.

Staged single-ventricle palliation is used to treat many cyanotic congenital heart diseases. Hemoglobin sickle cell disease is associated with anemia and significant vascular sickling sequelae, which increase the risk associated with single-ventricle palliation. To our knowledge, there are no reports in the English-language medical literature of single-ventricle palliation having been performed on a patient who had either sickle cell anemia or sickle cell-hemoglobin C disease. Herein, we discuss our clinical and surgical management of an infant with tricuspid atresia type IA and hemoglobin sickle cell disease who survived single-ventricle palliative procedures through the 2nd stage of a bidirectional Glenn anastomosis.

We report a case of long-term, successful, endocardial atrioventricular pacing in a 32-year-old man who had severe heart failure and ascites after having undergone a Fontan procedure for tricuspid atresia 9 years earlier. The patient was referred to our hospital for Fontan revision. However, electroanatomic mapping of the right atrium revealed viable tissue at the interatrial septum above the os of the coronary sinus, and it appeared that the left ventricle could be paced from a coronary sinus branch. Therefore, instead of Fontan revision, an endocardial atrioventricular pacemaker was implanted transvenously. On 5-year follow-up, the patient remained in New York Heart Association functional class I and had not been readmitted to the hospital for congestive heart failure or arrhythmias. His atrial and ventricular leads continued to show excellent pacing and sensing results.

We report the first successful totally percutaneous pacing and defibrillation of a single ventricle in a 52-year-old woman who had undergone a classic Fontan operation (atriopulmonary connection) for tricuspid atresia 21 years earlier. Left ventricular pacing and sensing was obtained with a bipolar lead in the lateral cardiac vein, and defibrillation was obtained with a coronary sinus coil lead and an "active can" in the retromammary position to optimize the current vector. This approach has significant potential benefits because it avoids a repeat thoracotomy, with its associated mortality and morbidity.

Pleural effusions after modified Fontan operations are unpredictable and contribute significantly to the morbidity and prolonged hospitalization. Chemical pleurodesis is widely performed in adults for malignant pleural effusions, but has found less chance of usage for benign cases or the cases with unidentified etiology. Although it is a safe and easily performed procedure, the use of bleomycin for the treatment of pleural effusions in the pediatric age population has been rarely reported in the literature. In this manuscript, we present a case with prolonged pleural effusion after extracardiac Fontan operation, which was successfully treated with bleomycin pleurodesis.

Background: Renal calcification is a known complication of Furosemide therapy., Methods: We describe 3 children who were diagnosed with renal calculi, not nephrocalcinosis, following the use of Furosemide for 4 weeks. All the infants (24, 18, and 8 months) had successful repair/palliation of congenital heart disease. The dose of Furosemide was 1-2 mg/kg/day and the duration between starting treatment and development of hematuria was 4 weeks., Results: In all 3 patients renal ultrasound confirmed the presence of renal calculi with no nephrocalcinosis. On follow-up, hematuria improved after 3-4 months., Conclusion: Renal calculi can complicate short-term Furosemide treatment in children after repair of congenital heart disease. Patients on Furosemide should be closely monitored by urine analysis and/or urine calcium/creatinine ratio for early detection of this complication.

We report a patient with tricuspid atresia after total cavopulmonary connection who developed significant mitral valve regurgitation. Doppler myocardial echocardiography tracings showed "pseudonormalization" of diastolic velocities. After successful surgical reconstruction of the mitral valve, the diastolic myocardial velocities changed to the typical Fontan physiology with predominant late diastolic ventricular filling.

For 5 patients with univentricular heart associated with apico-caval juxtaposition, an extracardiac Fontan procedure was carried out using an artificial graft bridging the vertebra to avoid graft compression by the vertebra and the ventricle. For 2 patients representing nonconfluency between the inferior caval vein and the hepatic vein, a hand-made, shoe-tree graft was used. Postoperatively all patients are doing well without a stenotic venous pathway. This extracardiac operation using an artificial graft bridging the vertebra may be advantageous for univentricular heart associated with apico-caval juxtaposition to prevent a postoperative stenotic venous pathway.

Introduction: Ablation of atrial tachycardia (AT) occurring late after cardiac surgery for congenital heart disease can be challenging due to the complexity of the arrhythmogenic substrate., Methods and Results: We performed an ablation procedure in a Fontan patient with an AT using a new approach combining 3D electroanatomical mapping with multislice computed tomography (MSCT). This technique visualizes the position of the catheter in relation to the endocardium, thereby improving delineation of scar tissue. The AT had a focal origin located between areas of scar tissue and was successfully ablated at the earliest activated site., Conclusion: Ablation of complex arrhythmias can be facilitated by fusion of electroanatomical map and MSCT.

Background: The Fontan procedure performed with an extracardiac conduit (ECC) has gained wide acceptance as an alternative to the intracardiac lateral tunnel because it avoids placement of extensive atrial suture lines and use of prosthetic material in the systemic circulation. The extracardiac lateral tunnel (ELT) is a modification of the Fontan procedure which uses pericardium and the external surface of the atrium to create a conduit from the inferior vena cava to the pulmonary artery. This surgery theoretically avoids disruption of intra-atrial conduction by eschewing endocardial suturing while maintaining conduit growth potential and the ability to easily create a fenestration to the systemic circulation., Methods: We retrospectively analyzed the short-term outcome of 96 consecutive patients who underwent an extracardiac Fontan procedure. An ELT using bovine pericardium was performed in 59 patients, whereas 37 patients received a traditional ECC., Results: The 2 groups were similar with respect to age (P = .96), body surface area (P = .54), number of preoperative Fontan risk factors (P = .43), and ventricular morphology (P = .72). There were no significant differences in the following outcome variables between the ELT and the traditional ECC: length of hospitalization (P = .73), duration of chest tube drainage (P = .48), abnormal rhythm at time of discharge (P = .27), and mortality (P = .63)., Conclusions: The ELT Fontan can be performed with a low risk of mortality and complications. The results are equivalent to the traditional ECC procedure. The theoretical advantages of the procedure suggest that it should be considered a useful modification of Fontan surgery.