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A bilateral bidirectional cavopulmonary shunt was performed in a cyanotic 14-month-old girl who had tricuspid and pulmonary valve atresia, with right pulmonary artery (RPA) hypoplasia (3 mm), bilateral superior vena cavae and a ductus arteriosus-dependent pulmonary blood flow. Because of 62% postoperative arterial oxygen saturation and a right superior vena cava (RSVC) pressure of 30 mmHg, a 5 mm Gore-Tex tube was interposed to connect the two superior venae cavae. The creation of a 'new-innominate' vein allowed decompression of the right superior vena cava and an increase in arterial oxygen saturation to 86%.

Atypical right-to-left shunts following Fontan surgery were closed by transcatheter techniques using various devices in 4 patients. Three had variants of hypoplastic left heart syndrome, while the other had tricuspid atresia. Large Fontan baffle leaks were present in 2, a residual fistula from the inferior vena cava to the coronary sinus was present in 1, and another patient had surgical tube fenestration of an extracardiac conduit. When careful catheter and transesophageal echocardiographic exploration is performed, the selection of appropriate devices makes closure of these defects feasible.

Two patients operated on by one of the authors (MK) developed hemodynamic instability after otherwise uneventful completion of total cavopulmonary anastomosis with an extracardiac tube. In both, a stenosis of the right pulmonary veins was demonstrated during routine intraoperative transesophageal echocardiography. The transesophageal probe was found to be the underlying problem. Apparently, the pulmonary veins became compressed between the probe and the extracardiac conduit.

Objective: This study was undertaken to characterize morphologic substrate of tricuspid atresia with ventriculoarterial concordance and discriminate management strategies that lead to successful definitive repair., Methods: From 1999 to 2004, a total of 150 babies with type I tricuspid atresia were enrolled from first diagnosis at 26 institutions. Antegrade pulmonary blood flow was absent in 19%, restricted in 54%, and unrestricted in 28%. Competing-risk methodology determined the time-related prevalence and risk factors for death versus cavopulmonary anastomosis and subsequent death versus Fontan completion., Results: Overall 5-year survival was 86%. Initial palliation included systemic-pulmonary arterial shunt in 64%, pulmonary artery banding in 11%, and cavopulmonary anastomosis in 24%. Median age at cavopulmonary anastomosis was 6 months, with 83% undergoing bidirectional Glenn shunt and 17% undergoing hemi-Fontan procedure. By the age of 2 years, 89% had cavopulmonary anastomosis, 6% were dead, and 4% remained alive without cavopulmonary anastomosis. Risk factors for death without cavopulmonary anastomosis included presence of mitral regurgitation (P = .03) and palliation with systemic-pulmonary arterial shunts not originating from the innominate artery (P = .04). Factors associated with decreased transition rate to cavopulmonary connection included patient variables (younger admission age to a participating institution, noncardiac anomalies) and procedural variables (larger systemic-pulmonary arterial shunt diameter, previous palliation). Of patients undergoing cavopulmonary anastomosis, 75% had undergone a Fontan operation within 3 years., Conclusion: Smaller shunt size and decreased pulmonary blood flow decrease mortality after initial palliation and increase the rate of successful transition to cavopulmonary anastomosis. Outcomes can be improved by placing smaller shunts from the innominate artery, especially in patients with any mitral regurgitation.

A variety of techniques have been utilized to close Fontan fenestrations. Among 20 patients who underwent a cardiac catheterization for fenestration closure, 3 patients had residual native atrial septum, forming an additional intermediate chamber on the pulmonary venous side of the fenestration. Three different methods were used to close these fenestrations.

Sinus node dysfunction is relatively common in patients with Fontan palliation for single ventricle congenital heart disease, and such patients often are in junctional rhythm or may have pacemaker systems for bradycardia. Because the physiologic determinants of left atrial pressure play a major role in determining pulmonary blood flow and therefore cardiac output in Fontan patients, the loss of atrioventricular (AV) synchrony in junctional rhythm or demand ventricular pacing in these patients might be expected to influence cardiac output. We report two cases of Fontan patients with the absence of AV synchrony that resulted in reversal of flow through the Fontan fenestration during ventricular systole. In both cases, restoration of AV synchrony by atrial pacing resulted in the elimination of retrograde fenestration flow, increased cardiac output, and improved clinical status.

Plastic bronchitis is a rare, potentially life-threatening condition in which protein casts form within and occlude the bronchus, resulting in pulmonary failure, and has been identified as a complication after the Fontan procedure. We present a case of a 5-year-old girl who had undergone an extracardiac fenestrated Fontan repair as a component of staged palliation for tricuspid atresia. Six weeks following surgery, the patient presented with airway obstruction, coughing a bronchial cast. Medical therapies to optimize heart function and attempt to control cast formation were implemented, with little clinical impact. Following cardiac catheterization to stent open the fenestration, the symptoms of plastic bronchitis resolved. Cast expectoration recurred following spontaneous closure of the stented fenestration and again resolved with recreation of the baffle defect. Fenestration of the Fontan circuit alters hemodynamics, thereby providing an additional therapeutic option for this devastating disorder.

We report a case of successful staged repair of tricuspid atresia with dysplasia of the right ventricular myocardium and absence of the pulmonary valve. The patient underwent the modified Blalock-Taussig shunt operation at 1 month the bidirectional Glenn procedure at 11 months, and the total cavopulmonary connection procedure using extracardiac graft at 2 years. It is important to prevent the left ventricular outflow tract obstruction (LVOTO) caused by the non-functioning right ventricle when the Fontan completion. We were safely able to disconnect the pulmonary trunk from the right ventricle using the transesophageal echocardiography to evaluate the LVOTO during operation.

Background: There is evidence that "inappropriate hypertrophy" of the single left ventricle, which occurs as a result of acute preload reduction, leads to adverse consequences on ventricular function. However, a systematic study of the capability of tissue Doppler imaging (TDI) to assess systolic and diastolic ventricular functions after the Fontan procedure is still missing., Methods: Twenty-four postoperative patients aged 12-33 years were prospectively evaluated with two-dimensional echocardiography equipped with TDI capabilities. Nineteen age-matched normal subjects were selected as controls. Good-quality echoes for the measurement of ejection fractions were available in 21 patients. Ten patients (group 1) had systolic dysfunction (ejection fraction < 50%), and 11 patients (group 2) had normal systolic function. Peak systolic and diastolic wall velocities were acquired from the two-chamber view in the myocardia and mitral annulus., Results: Compared with controls, the Fontan patients had a significantly reduced peak systolic velocity at wall and annulus sites. A linear correlation existed between ejection fraction and systolic myocardial velocity from the annular sites. Group 1 patients had lower wall velocities and lower annulus velocities both in systole and diastole. Group 2 patients had preserved systolic velocities but decreased regional and annular early diastolic velocities, suggesting impaired filling. Multiple correlation analysis showed a relation between peak early diastolic mitral velocity and ventricular ejection fraction, mean mitral annular motion at systole, mass/volume ratio, and the number of years post Fontan revision., Conclusions: Myocardial velocities recorded after the Fontan operation give insight into systolic and diastolic ventricular functions. The peak systolic mitral annular velocity correlated well with the ventricular ejection fraction. The peak early diastolic velocity and the ratio between the early and late diastolic mitral annular velocity are reduced and reflect diastolic dysfunction even in the presence of normal systolic ejection fraction.

Introduction: A direct or tubular communication between the systemic venous system and the systemic atrium, generally called fenestration, is surgically created to improve the postoperative period of patients undergoing total cavopulmonary connection. However, a fenestration prompts a potentially deleterious right to left shunt, and is generally closed after the postoperative period. Direct fenestrations can be closed using coils, or devices designed for atrial septal defect closure. However, no devices have been designed for closure of extracardiac fenestrations. We report our experience concerning the closure of extracardiac Fontan fenestration by the Amplatzer duct occluder (ADO)., Methods: From January 2001 to December 2002, we closed extracardiac Fontan fenestrations using the ADO device in 10 consecutive patients. Indications to fenestration closure were: low velocity shunt through the fenestration, mild desaturation, and absence of effusions., Results: All patients had a successful closure of the fenestration. The procedure was performed through the femoral vein in 7 cases and through the right jugular vein in 3. Mean central venous pressure increased not significantly from 12 to 13-mmHg. Mean oxygen saturation increased significantly from 90 to 97% (p<0.001). Immediate shut abolition was obtained in 9 cases. No complications were observed. At a median follow-up of 12 months (range 6-18 months), all patients are free of symptoms and have a normal oxygen saturation at rest as well as at exertion., Conclusion: The ADO device allowed closing the extracardiac Fontan fenestration in all patients with no mortality, no morbidity and a rate of 100% of complete closure at mid-term follow-up.

Streptococcus pneumoniae is now a rare cause of endocarditis in humans. We report a patient with a double outlet right ventricle and mitral atresia, who underwent the Fontan procedure without prosthetic materials after treatment for penicillin-resistant Streptococcus pneumoniae endocarditis. Postoperative infectious endocarditis was not found. In patients with a history of infectious endocarditis, direct anastomosis of the main pulmonary artery and inferior vena cava would reduce the risk of recurrent infectious endocarditis.

Posterior fossa extradural hematoma (PFEDH) is an infrequent condition. Though numerous neuropathological sequelae of cardiopulmonary bypass have been described, to the best of the authors' knowledge, PFEDH after cardiac surgery has not been reported in the English literature. We report a 4-year-old boy with tricuspid atresia, who underwent cardiac surgery under cardiopulmonary bypass. On the 2nd postoperative day, his neurological condition deteriorated, and a CT scan of the head revealed a large PFEDH with mass effect and hydrocephalus. He underwent a midline suboccipital craniectomy and evacuation of PFEDH. There was diffuse bleeding from the dura; however, no specific site of bleeding was identified. The child started following commands on the 1st postoperative day, had an uneventful recovery and was discharged on the 10th postoperative day. On follow-up, 1 year after the surgery, he was doing well., (Copyright 2005 S. Karger AG, Basel.)

Study Objectives: To examine the results of right heart derivations and clinical outcomes according to preoperative characteristics and operative strategy implemented., Methods: Fontan operations were performed in 65 patients (mean age = 10.3 years, 41 males). The majority of cardiopathies were single ventricles (SV) with (49% of patients) or without (26%) tricuspid atresia. A palliative bidirectional cavo-pulmonary (BCP) anastomosis was performed prior to Fontan in 15 patients. Intra-atrial Fontan tunnelling was performed in 43 patients, Kreutzer-type operations in 10, and extracardiac tubes were used in 8 patients. The mean duration of follow-up was 6.1 +/- 0.3 years., Result: The 30-day mortality was 13.8%. Early mortality was higher among patients with SV with than without tricuspid atresia (P < 0.01), and among patients < 4 years old. Early reoperations were required in 5 patients, including dismounting in 1, BCP anastomosis after Kreutzer procedure in 1, and tube thrombosis in 1 patient. A single death occurred past 30 days, and late adverse events included protein-losing enteropathy in 1 patient, complete atrioventricular block in 1, and tube thrombosis treated with heparin in 2 patients. At the end of follow-up, 75% were in New York Heart Association functional class I., Conclusion: Our intermediate-term results of Fontan-type operations were satisfactory, and steadily improving. The prognosis was better in patients operated at age 4 or older. A prior BCP anastomosis improved the results. A higher morbidity was observed with intra- than with extra-atrial Fontan procedures. The merit of fenestration procedures with respect to morbidity remains the be evaluated.

Underlying mechanisms in the development of atrial flutter or intra-atrial re-entry tachycardia in patients with structural cardiac abnormalities remain poorly defined. The right atrial myocardium from two patients with congenital heart disease was evaluated, of whom one presented with severe right atrial dilation and arrhythmia and the other with a normal right atrium, to assess whether increased right atrial pressure and volume overload give rise to sarcolemmal alteration. N-terminus dystrophin staining in the atrium from the patient who had undergone a Fontan procedure showed a normally distributed but significantly reduced staining signal compared with the second patient. This is the first report that patients with severe right atrial dilation and atrial flutter have marked reduction in atrial dystrophin expression.

Objective: We set out to examine whether the extracardiac Fontan operation (ECFO) in young children is beneficial for the early postoperative course and whether it has a negative impact on the mid-term hemodynamics and growth of the children due to absent growth potential of the prosthetic conduit. Therefore we compared our medium-term experience with ECFO in children under 4 years of age to that in older children regarding the incidence of postoperative complications, somatomotoric development and conduit status., Methods and Results: Between 11/95 and 12/02 ECFO was performed in 30 children under 4 years of age and 21 older children aged 4-13 years. There were no deaths in children under 4 years of age and two older children died. No prolonged support (>72 h) of suprarenin was required in small children compared to 4 older children. In twenty-seven children, who underwent postoperative heart catheterization no pulmonary artery or systemic vein distortion occurred. One re-operation and one transcatheter intervention were performed because of the partial conduit stenosis. During the median follow-up of 4.8 years a manifestly accelerated postoperative weight gain was observed in children operated on under 4 years of age, compared to that in older children (up to the 50 vs. 10th percentile, P<0.05)., Conclusions: The ECFO could be performed in young children with low morbidity and mortality rates. In the majority of patients, implanted prosthetic grafts maintain stable form without the development of stenosis. Accelerated somatic development, especially in small children, is to be observed after completion of the Fontan circulation.

Objective: The left ventricular performance in patients with pulmonary atresia with intact ventricular septum who were Fontan candidates before and after the bidirectional Glenn procedure and a staged total cavopulmonary connection was compared with that in patients with tricuspid atresia., Methods: Contractility (end-systolic elastance), afterload (effective arterial elastance), and ventricular efficiency (ventriculoarterial coupling, arterial elastance/end-systolic elastance ratio), and the ratio of stroke work and pressure-volume area were approximated on the basis of cardiac catheterization data before the bidirectional Glenn procedure, before and after staged total cavopulmonary connection, and approximately 1 year after the completion of total cavopulmonary connection in 20 patients with pulmonary atresia with intact ventricular septum and 21 patients with tricuspid atresia., Results: The end-systolic elastance of the pulmonary atresia with intact ventricular septum group was significantly inferior to that of the tricuspid atresia group after bidirectional Glenn procedure and total cavopulmonary connection (1 year after total cavopulmonary connection 1.85 +/- 0.51 mm Hg . m 2 . mL -1 vs 2.84 +/- 0.96 mm Hg . m 2 . mL -1 , P < .01). The arterial elastance was not different between groups throughout the assessment period and tended to increase in a stepwise fashion after bidirectional Glenn procedure and total cavopulmonary connection. The arterial elastance/end-systolic elastance ratio and ratio of stroke work and pressure-volume area of the pulmonary atresia with intact ventricular septum group tended to worsen, whereas those of the tricuspid atresia group tended to improve. The difference reached statistical significance 1 year after total cavopulmonary connection (1.15 +/- 0.35 vs 0.82 +/- 0.23 and 64.2% +/- 6.7% vs 71.3% +/- 5.7%, respectively, P < .05 and P < .05, respectively)., Conclusion: The contractility and ventricular efficiency of patients with pulmonary atresia with intact ventricular septum are inferior to those of patients with tricuspid atresia after bidirectional Glenn procedure and total cavopulmonary connection. A high-pressure residual right ventricle may impair the left ventricular performance of patients with pulmonary atresia with intact ventricular septum after bidirectional Glenn procedure and total cavopulmonary connection.

It is rare to find multiple fistulas arising from all three coronary arteries and draining into the left ventricle. Coronary angiography revealed this anomaly in a one-year-old girl with tricuspid atresia after conversion to the Fontan circulation. To the best of our knowledge, this is the first report of such multiple fistulas in the setting of tricuspid atresia, and also the first report in childhood.

Background: Patients with double inlet left ventricle/l-transposition and similar morphologies have their systemic outflow traverse a bulboventricular foramen (BVF), which has a propensity to narrow over time. A Norwood procedure may be performed as the initial palliation. We prefer aortic arch repair and pulmonary artery banding, delaying Damus-Kaye-Stansel (DKS) or BVF resection until the second palliation. The aims of this study were to compare our results with those reported for Norwood strategy and examine the development of systemic outflow obstruction., Methods: Retrospective study of patients with double inlet left ventricle, L-TGA or similar morphology presenting between 1990 and 2000. Follow-up with clinical assessment, echocardiography and catheter studies., Results: Twenty-five patients had initial palliation with pulmonary artery banding with repair of any associated arch obstruction. Twelve patients had DKS performed as part of their second stage procedure, and 3 had DKS performed later for recurrent stenosis after prior enlargement of BVF. Six patients had BVF resection without later restenosis and 4 patients did not develop BVF stenosis. There was one early death (4%) and two late (8%). Fontan completion was achieved in 20 of the 22 survivors. There were no cases of DKS obstruction, no pulmonary valve had more than mild regurgitation., Conclusions: Our approach achieves low operative mortality and morbidity and compares favorably with reported results for Norwood palliation. The significant rate of systemic outflow obstruction in those who did not undergo DKS at the second stage confirms the utility of early DKS in children with this morphology.

Objectives: Symptoms from low cardiac output or refractory atrial arrhythmias are complicating atriopulmonary (classical) Fontan connections. We present our experience of converting such patients to total cavopulmonary connections with and without arrhythmia surgery., Methods: Between 1997 and 2002, 15 patients (mean age, 19.7 +/- 7.0 years) underwent conversion operations 12.7 +/- 3.5 years after atriopulmonary Fontan operations. Preoperative New York Heart Association functional class was I in 2 patients, II in 2 patients, III in 6 patients, and IV in 5 patients. Four patients underwent intracardiac lateral tunnel conversion alone, and 11 received extracardiac total cavopulmonary connection, right atrial reduction, and cryoablation., Results: No mortality occurred. One patient had conduit obstruction in the immediate postoperative period requiring replacement, and another required a redo operation for endocarditis. Average hospitalization was 17.9 +/- 9.38 days; chest drains were removed on median day 4 (range, 1-29; mean, 7.4 +/- 7.58 days). At follow-up (mean, 42.6 +/- 22.1 months), late atrial arrhythmias had recurred in 3 of 4 patients with intracardiac total cavopulmonary connections (without ablation) and 1 of 11 patients with extracardiac total cavopulmonary connections with ablation. All patients are in New York Heart Association class I or II. Exercise ability (Bruce protocol) improved 69% from a mean of 6.18 +/- 4.01 minutes to 10.45 +/- 2.11 minutes (P <.05). Need for antiarrhythmic agents decreased postoperatively (patients receiving < or =1 antiarrhythmic: 9 preoperatively vs 15 at long-term follow-up, P <.05). No patient has required transplantation. Protein-losing enteropathy, which was present in 1 patient, improved transiently with conversion. There was 1 late death from gastrointestinal hemorrhage., Conclusions: Fontan conversion can be achieved with low mortality and improvement in New York Heart Association class and exercise ability. Concomitant arrhythmia surgery reduces the incidence of late arrhythmias.

A 31-year-old woman with tricuspid atresia and a palliative Waterston shunt presented with intractable poorly tolerated supraventricular tachycardia. Electrophysiologic evaluation was consistent with AV nodal reentrant tachycardia. The fast pathway region was mapped electroanatomically during tachycardia and with constant rate ventricular pacing. Successful radiofrequency ablation was achieved by placement of lesions in an area adjacent and inferior to the His-bundle electrogram on the left side of the septum.

Background: The growing population with Fontan operation surviving into adulthood has significant morbidity and mortality rates from recurrent atrial tachyarrhythmias. We hypothesized that the structural characteristics and electrical behavior of atria may differ in these patients compared with those without arrhythmias., Methods and Results: We studied 33 consecutive patients (age, 25.4+/-9.5 years) with Fontan circulation, of whom 19 had a history of documented sustained atrial tachyarrhythmias. We analyzed their clinical and investigational data, including echocardiographic assessment of atrial dimensions and surface 12-lead ECG measurement of the P-wave duration and its dispersion between leads. Twenty age- and sex-matched healthy control subjects were also studied. First, patients who had the Fontan procedure overall had longer P-wave duration (144+/-33 versus 100+/-7 ms, P<0.001) and greater P-wave dispersion (74+/-33 versus 34+/-9 ms, P<0.001) than control subjects. Among the patients who had the Fontan procedure, those with atrial tachyarrhythmias had longer P-wave duration (159+/-28 versus 123+/-28 ms, P<0.001) and greater P-wave dispersion (91+/-30 versus 50+/-19 ms, P<0.001) than those without. Second, the patients with atrial tachyarrhythmias who had the Fontan procedure had larger right atrial dimension than those without arrhythmias (6.4+/-1.4 versus 5.0+/-1.0 cm, P=0.01). Third, both P-wave duration and dispersion were significantly correlated to right atrial dimension within the Fontan group (r=0.55, P=0.002, and r=0.56, P=0.002, respectively)., Conclusions: Patients with atrial tachyarrhythmias late after Fontan operation have longer P-wave duration and P-wave dispersion and larger right atrial dimension than those without the arrhythmias; these abnormalities are interrelated. This observation represents an atrial mechano-electrical remodeling phenomenon in parallel to an increase in arrhythmia propensity in this vulnerable population and warrants further investigation.

To elucidate the relation between exercise capacity and peripheral hemodynamics in patients after the Fontan operation, near-infrared spectroscopy was performed on the vastus lateralis muscle, and flow-mediated vasodilation of the brachial and posterior tibial arteries was done with high-resolution ultrasonography. In Fontan patients, diminished exercise capacity was related to a reduced blood flow supply and an attenuated post-exercise oxygen resaturation of the working skeletal muscle, which also was related to impaired endothelium-dependent vasodilation.

Background: Whereas indications expand and results improve with increasing refinements to the Fontan procedure the overall impact on outcomes related to tricuspid atresia remains suboptimally defined., Methods: We reviewed 225 consecutive patients presenting between 1971 and 1999. All patients had classic tricuspid atresia with absent right atrioventricular connection and with D-transposition of the great arteries in 21%, pulmonary outflow obstruction in 75%, and aortic outflow obstruction in 11%., Results: Ten patients died before any intervention and 3 patients were lost to follow-up. Palliative procedures (includes 151 with systemic shunt, 27 pulmonary artery banding, 60 venous shunt) were performed in 203 patients, with 44 deaths, 8 patients awaiting Fontan, 12 patients Fontan contraindicated, and 11 patients lost-to-follow-up. A total of 137 patients had the Fontan procedure (9 patients without previous procedures) with 7 early deaths, 11 late deaths, and 3 patients progressing to heart transplantation. Total survival for the cohort was 90% at the age of 1 month, 81% at 1 year, 70% at 10 years, and 60% at 20 years with no significant change over the time period. Independent factors associated with ineligibility or death without Fontan (n = 68, 30%) included earlier birth date, lower birth weight, presence of aortic arch anomaly and greater right ventricular hypoplasia, and no palliative procedure. There were no significant changes in mortality with Fontan over the study time period with survival of 95% at 1 month, 93% at 1year, and 82% at 10 years., Conclusions: Improvements in outcomes with tricuspid atresia will require attention to management and risk factors before Fontan.

Objective: Postoperative management after the Norwood procedure is aimed at optimizing systemic oxygen delivery and mixed venous oxygen saturation. High levels of fraction of inspired oxygen and hyperventilation may increase pulmonary blood flow at the expense of systemic flow. This study determines the effects of these interventions on mixed venous saturation and systemic oxygen delivery in postoperative neonates., Methods: We prospectively studied the effects of 100% fraction of inspired oxygen and hyperventilation in 14 neonates (median age 8 days) 1 to 3 days after the Norwood procedure, while they were sedated, paralyzed, and mechanically ventilated. After establishment of baseline conditions (fraction of inspired oxygen = 29% +/- 2%, normal ventilation), patients were exposed to each of the 2 interventions in random order. Mixed venous saturation was measured through a transthoracic line in the superior vena cava. Oxygen excess factor (Omega = systemic oxygen delivery/oxygen consumption) was used as an indicator of systemic oxygen delivery., Results: High levels of fraction of inspired oxygen produced significant increases from baseline in systemic saturation (90% +/- 1% vs 80% +/- 1%, P <.01), mixed venous saturation (54% +/- 3% vs 44% +/- 2%, P <.01), and oxygen excess factor (2.6% +/- 0.2% vs 2.3 +/- 0.2%, P <.01), but there was no change in arteriovenous saturation difference or blood pressure. Hyperventilation resulted in no changes in systemic or mixed venous saturation, arteriovenous saturation difference, oxygen excess factor, or blood pressure., Conclusions: High levels of fraction of inspired oxygen can improve mixed venous oxygen saturation and systemic oxygen delivery after the Norwood procedure. Hyperventilation does not change either mixed venous saturation or oxygen delivery. Management protocols aimed at minimizing the fraction of inspired oxygen and carefully controlling ventilation may not be warranted.

Objectives: We sought to determine the long-term outcomes and risk factors for mortality in patients with double-inlet left ventricle (DILV) or tricuspid atresia with transposed great arteries (TA-TGA)., Background: Patients with DILV or TA-TGA are at risk of systemic outflow obstruction and a poor outcome. The impact of various management strategies on the long-term outcomes of these patients remains unknown., Methods: We reviewed the outcomes of 164 consecutive pediatric patients with DILV or TA-TGA who underwent surgical palliation between 1983 and 2002. Patients with a Holmes heart or heterotaxy syndrome or who were lost to follow-up (n = 24) were excluded. Risk factors for mortality or the need for orthotopic heart transplantation (OHT) were assessed by multivariate analysis., Results: There were 105 patients with DILV and 35 patients with TA-TGA. The overall mortality rate, including OHT, was 29%. Patients with DILV had a lower mortality rate than patients with TA-TGA (23% vs. 49%, p = 0.007). Multivariate analysis showed the presence of arrhythmia and pacemaker requirement as independent risk factors for mortality, whereas pulmonary atresia or stenosis and pulmonary artery banding were associated with decreased mortality. Gender, era of birth, aortic arch anomaly, and systemic outflow obstruction were not risk factors. The perioperative and overall mortality were similar between patients who underwent the Damus-Kaye-Stansel procedure beyond the neonatal period and those had subaortic resection., Conclusions: The mortality of patients with DILV or TA-TGA remains high. The outcomes of these patients are influenced by restriction of pulmonary blood flow, arrhythmia, and pacemaker requirement. Surgical palliation to relieve systemic outflow obstruction is not associated with a poor outcome.

We describe a 5-year-old boy with Fontan physiology and a ventricular pacemaker who developed severe plastic bronchitis. Evaluation by cardiac catheterization revealed profoundly altered hemodynamics, which improved with atrial pacing. Following implantation of an atrial pacemaker, which restored atrioventricular (A-V) synchrony, the patient's hemodynamics greatly improved and his plastic bronchitis resolved.

The status of pulmonary circulation is of utmost importance to the success of the modified Fontan operation. Pulmonary artery distortion, hypoplasia of the total pulmonary vascular bed, and elevated pulmonary vascular resistance are all risk factors for adverse outcome after modified Fontan operations. In cases of irreparable acquired atresia of one or the other branch pulmonary artery, clinicians are forced to contemplate the possibility of total cavopulmonary connection to one lung. The combined experience of the authors with 12 cases suggests that the likelihood of operative survival following Fontan's operation to one lung is predicted based on the usual hemodynamic parameters: pulmonary artery pressure and flow, ventricular end diastolic pressure, transpulmonary gradient, and pulmonary vascular resistance. In this series, there were no operative mortalities among patients undergoing Fontan's operation to one lung, all of whom meet the usual criteria for hemodynamic acceptability. There may, however, be a higher incidence of protein-losing enteropathy than in Fontan patients with normal pulmonary vascular beds. All possible means of resuscitating the lost elements of the pulmonary vascular bed and re-establishing pulmonary artery continuity should be attempted to minimize pulmonary vascular capacitance of patients undergoing Fontan's operation. It is clear, however, that the presence of only one pulmonary artery does not in and of itself preclude satisfactory outcome.

Background: The aim of this study was to compare the outcomes of the lateral tunnel (LT) and extracardiac conduit (ECC) Fontan procedures at a single institution over the same time period., Methods: From November 1995 through October 2002, 70 Fontan procedures were performed: 37 LT and 33 ECC. All were fenestrated; 96% were staged with a prior superior cavopulmonary connection. Compared with the ECC patients, the LT patients were younger (2.7 +/- 1.1 vs 3.9 +/- 2.5 years; p = 0.01), had a higher incidence of hypoplastic left heart syndrome (57% vs 21%; p < 0.01), and a longer aortic cross-clamp time (55 +/- 13 vs 26 +/- 15 min; p < 0.01). Weight, gender, preoperative cardiac catheterization values, and cardiopulmonary bypass time did not differ between the two groups., Results: Operative mortality was 2.8%, 1 patient in each group (p = 1.0). Over the first 24 hours following operation the mean Fontan pressure, transpulmonary gradient, and common atrial pressure did not differ between LT and ECC patients. The median duration of mechanical ventilation (LT 12 vs ECC 18 hours), intensive care unit stay (LT 2 vs ECC 3 days), chest tube drainage (LT 10 vs ECC 8 days), and hospital stay (LT 11 vs ECC 12 days) did not differ. The ECC patients had a higher incidence of sinus node dysfunction both in the postoperative period (27% vs LT 8%; p = 0.09), and persisting at hospital discharge (10% vs LT 0%; p = 0.02). Mean follow-up was 3.6 +/- 1.6 years in LT, and 3.0 +/- 2.2 years in ECC patients (p = 0.2). There was one late death. Actuarial survival at 5 years is 97% for LT, and 91% for ECC patients (p = 0.4); 96% of patients are in NYHA class I, and 4% in class II, with no difference between groups. Sinus node dysfunction was seen during follow-up in 15% LT vs 28% ECC patients (p = 0.2)., Conclusions: The LT and ECC approaches had comparable early and mid-term outcomes, including operative morbidity and mortality, postoperative hemodynamics, resource use, and mid-term survival and functional status. ECC patients had a higher incidence of sinus node dysfunction early after operation.

A 13-year-old boy weighing 35 Kg underwent surgery to correct pulmonary valve atresia by total caval-pulmonary anastomosis (Fontan procedure). During surgery and recovery, no adverse events were observed. On the third day after surgery, the patient developed signs of low cardiac output, renal failure, and right ventricular insufficiency. In spite of an antithrombotic treatment protocol with sodium heparin (10 IU/Kg/h), venous return was found to be impeded by 2 thrombi in the inferior vena cava. Thrombolysis with urokinase was ineffective, and surgical thrombectomy was only partially successful. Finally, a high dose of intravenous heparin was prescribed (25 IU/Kg/h) and the thrombi were dissolved. The importance of prophylactic antithromboembolic therapy following corrective surgery for congenital heart disease must be emphasized. Ultrasound monitoring of these patients, even in the absence of clinical signs, has proven effective for early detection of the thrombotic events described in this report.

Ventricular dysfunction in patients after Fontan-like operations (FLOs) is a serious complication that might contribute to poor long-term results. Ischemic heart disease will have debilitating consequences on a Fontan heart. Ten patients (15.8 +/- 5.01 years) after FLO had transesophageal echocardiography and cardiac catheterization 9.3 +/- 4.2 years after surgery. Myocardial perfusion was assessed by NH3-positron emission tomography (rest/adenosine) and compared with that of 10 healthy adults (26.1 +/- 6.3 years). Ventricular function was normal in 4 and reduced in 6 patients; end systolic and end diastolic meridional wall stress was significantly elevated in the FLO group. Coronary angiography revealed no stenosis of the coronaries. Compared to normals, myocardial blood flow (MBF) at rest was higher in the FLO group (0.99 +/- 0.25 vs 0.77 +/- 0.17 ml/g/min, p <0.05), whereas MBF after vasodilatation (2.12 +/- 0.78 vs 3.10 +/- 0.85 ml/g/min, p <0.05) and coronary flow reserve (CFR) was reduced (2.5 +/- 0.88 vs 4.1 +/- 1.01, p <0.05), especially in those with impaired ventricular function. Coronary vascular resistance after vasodilatation was elevated in the FLO group (38.2 +/- 17.4 vs 24.5 +/- 8.3 mmHg/ml/g/min, p <0.05). Altered MBF, increased meridional wall stress, and impaired CFR are common findings in FLO. Attenuated CFR and reduced ventricular function are significantly correlated and may be risk factors for the long-term outcome.

Whereas the medical advances were reviewed in the previous paper, electrophysiological, transcatheter and surgical advances will be the focus in this review. Greater understanding of the arrhythmias, development of non-pharmacological treatment, namely catheter ablation and internal cardioverter-defibrillator (ICD) and miniaturization pacemakers and IDs have occurred in the last decade so that the methods could be applied to smaller and more complex patient population. Surgery has been the traditional treatment option for palliation and correction of congenital and acquired heart defects in infants and children. During the last one to one and one-half decades, a remarkable number of transcatheter methods were developed and refined. These developments during the last decade were reviewed and include long-term results of balloon dilatation procedures, transcatheter closure of atrial septal defects, patent ductus arteriosus, and ventricular septal defect, percutaneous valve replacement, intravascular stents to manage vascular obstructive lesions that can not be satisfactorily balloon-dilated, catheter completion of Fontan procedure, myocardial reduction in hypertrophic cardiomyopathy and other miscellaneous procedures. Recent advances in the transcatheter modes of therapy have added a new dimension to the management of neonates, infants and children with heart disease. They should now be added to the armamentarium available to the Pediatrician and Pediatric Cardiologist in the management of cardiac problems in the pediatric patient. Surgical methods and concepts have been greatly refined such that surgery can be undertaken even in the sickest and most complex patient. The majority of congenital heart defects can be corrected by open heart surgery; some require prior palliation and others can be operated without prior palliation. Recent advances in various defects were reviewed.

Objective: The modified Fontan procedure, usually performed in children, is used for the treatment of anomalies with a single functional ventricle. We reviewed our experience with the modified Fontan procedure performed in the adult patient., Methods: Between October 1973 and May 2001, the modified Fontan procedure was performed on 132 adult patients (74 men, 58 women). Median age was 23 years (range, 18 to 53 years). Diagnoses included tricuspid atresia in 34 patients (26%), double-inlet left ventricle in 48 (36%), and complex lesions in 50 (38%). The majority of patients (89%) had at least one prior palliative procedure; the most common procedures were Blalock-Taussig shunt in 85 patients and Glenn anastomosis in 31., Results: Operations included an atriopulmonary connection in 74 patients, lateral tunnel in 27, intra-atrial conduit in 14, right atrium-to-right ventricle in 9, extra-cardiac conduit in 3, and other in 5. Overall early mortality was 8.3%. Mortality was 6.5% for operations performed after 1980. This is comparable to the mortality of the modified Fontan procedure performed in children during the same time interval at our institution. All 7 of the early deaths since 1980 occurred in the complex lesion group. Morbidity included prolonged pleural effusion in 36 patients, atrial arrhythmias in 25, reoperation for bleeding in 13, permanent pacemaker in 8, and stroke in 2. Mean follow-up was 9.1 years with a maximum of 21.2 years. Actuarial survival for early survivors was 89% (84,95), 75% (67,84), and 68% (58,79) at 5, 10, and 15 years, respectively. Freedom from late reoperation was 89% (83,95), 85% (78,93), and 80% (70,91) at 5, 10 and 15 years, respectively. The majority (90%) of present survivors were New York Heart Association class I or II at follow-up., Conclusions: In properly selected adult patients with functional single ventricle, the modified Fontan procedure can be performed with early mortality similar to younger patients. Early mortality is more likely with complex lesions. The majority of late survivors have a good quality of life.

Introduction: Assessment of systolic and diastolic ventricular function in children and adults with morphologically and functionally univentricular heart is difficult using the conventional echocardiographic methods. Quantitative assessment of systolic and diastolic wall motion by TDE may provide information on abnormal systolic and diastolic ventricular function. Thus, the object of this study was to analyze the patterns of anterior and posterior wall motions in children with univentricular heart after palliative Fontan operation in comparison to normal subjects., Patients and Methods: We investigated 21 patients of an average age of 10.1 years (range 4.2 to 32 years) with the primary diagnosis of univentricular heart and tricuspid atresia after a median period of 4.3 (range 1.2 to 8) years after cavo-pulmonary anastomosis (Fontan procedure) and in comparison to a normal collective of children without cardiovascular malformations. For investigation we used the novel tissue Doppler echocardiography (TDE) (EchoPack 6.3.6, Vingmed, Norway). Thereby we chose the standardized apical view and evaluated the left and right annular systolic (S(T)), early diastolic (E(T)) and atrial (A(T)) motion. We also registered the acceleration and deceleration time of each obtained curve., Results: In comparison to the posterior myocardial wall the velocities at the anterior wall of the rudimentary ventricle were significantly reduced in all patients with tricuspid atresia and univentricular heart (p<0.0001). Particularly the annular systolic and diastolic wall motions of the rudimentary ventricle as well as their deceleration and acceleration time in patients with univentricular hearts differed significantly from those in healthy persons., Conclusion: Abnormal myocardial wall motion is detectable in children and adults with univentricular heart after palliative cardiac procedures using tissue Doppler echocardiography. The hemodynamic value of the measured abnormal wall motions, however, need further comparative studies.

Objective: This study was designed to determine whether either of 2 alternative methods of extracardiac Fontan reconstruction provides superior results., Methods: We reviewed 58 consecutive Fontan procedures performed between 1995 and 2001 with a pedicled pericardial tunnel (group P, n = 21) or an extracardiac conduit of polytetrafluoroethylene or allograft aorta (group C, n = 37). Operations were performed with cardiopulmonary bypass at 32 degrees C; an aortic crossclamp was applied in only 6 patients. All group P patients and 33 (89%) group C patients received fenestrations., Results: The groups were similar in terms of age, weight, anatomy, and preoperative hemodynamics. There were 3 hospital deaths (5%; 70% confidence limit, 2%-30%), all in group C. Median durations of mechanical ventilation (group P, 1 day; group C, 1 day), intensive care unit stay (group P, 3 days; group C, 3 days), chest tube drainage (group P, 8 days; group C, 7 days), and hospitalization (group P, 10 days; group C, 9 days) were not significantly different. There were no late deaths. All patients received warfarin sodium, and there were no late strokes. Before the Fontan procedure, 1 patient in group P and 3 patients in group C required pacemaker implants. Of the 51 surviving patients in sinus rhythm before the Fontan procedure, only 1 patient in group C subsequently required a pacemaker., Conclusions: Extracardiac Fontan procedures with either a pericardial baffle or conduit are associated with low operative mortality and low risks of arrhythmia and late thromboembolic complication.

Background: The Fontan operation eliminates the systemic hypoxemia and ventricular volume overload characteristic of prior forms of palliation, however, late outcome of systemic venous and right atrial hypertension were unknown when the procedure was initially proposed., Aim: We assessed the late mortality and the present clinical status atresia and other forms of univentricular heart who had modified Fontan procedure performed between 1981 and 1998, and survived early perioperative period., Methods: We reviewed the clinical records of 43 early survivors of the modified Fontan procedure. Patients' outcome and late mortality rates were determined and present clinical status was ascertained in all patients., Results: Late overall survival was 76.8%. Reoperation and late complication rates steadily declined according to surgery modification and homografts exclusion. Late mortality was increased only in patients with important atrio-ventricular valve dysfunction and valve replacement. Ninety seven percent of surviving patients are currently in New York Heart Association class I or II., Conclusions: Our 20-year experience with modified Fontan procedure for tricuspid atresia and various forms of univentricular heart has resulted in improved outcome with most survivors now leading lives of good quality into adulthood.

We describe two patients who required fenestration of an autologous pericardial extracardiac Fontan (APEF), short-medium term angiographic follow-up, and subsequent successful transcatheter closure of the fenestration.

In hearts with a functional single ventricle, cavity volume and myocardial muscle mass increase as a consequence of the excessive volume load associated with parallel pulmonary and systemic circulations. The hemi-Fontan operation was conceived as a means of accomplishing early reduction of the volume work of the single ventricle, in anticipation of eventual completion of a modified Fontan procedure. The hemi-Fontan procedure includes association of the superior vena(e) cava(e) (SVC) with the branch pulmonary arteries, augmentation of the central pulmonary arteries, occlusion of the inflow of the SVC into the right atrium, and elimination of other sources of pulmonary blood flow. Because it is preparatory to an eventual completion Fontan procedure, concomitant procedures are conveniently performed in conjunction with the hemi-Fontan. These may include atrial septectomy, relief of aortic arch obstruction, proximal main pulmonary artery to ascending aortic anastomosis, repair or revision of anomalous pulmonary venous connection, atrioventricular valvuloplasty, or other procedures., (Copyright 2003 Elsevier, Inc. All rights reserved.)

We present a case a 25-year-old pregnant female who had Fontan surgery performed 20 years ago due to a common ventricle of the left ventricular morphology. She had a history of three miscarriages and one successful pregnancy. This was a fifth pregnancy and she delivered normal healthy child. This is a second patient after Fontan procedure who had two successful deliveries. Clinical aspects of pregnancy in patients after Fontan procedure are described.

Background: The extracardiac Fontan procedure (ECF) usually requires cardiopulmonary bypass (CPB). In this report, the results and techniques of this procedure without CPB at a single institution are presented., Methods: Between August 1992 and December 2001, ECF without CPB was achieved in 24 of 44 patients undergoing an ECF. Mean age at surgery was 5.9 +/- 2.9 years, and mean weight was 20.7 +/- 12.6 kg. Diagnoses were tricuspid atresia in 9 patients, single-ventricle with pulmonary outflow tract obstruction in 7, pulmonary atresia/intact septum in 5, and other complex single-ventricle physiology in 3. Initial palliation was by arterial to pulmonary artery shunt in 21 and pulmonary artery banding in 1. A bidirectional cavopulmonary connection was created in 23 patients. A temporary inferior vena caval-to-atrial shunt was used to complete the procedure without CPB. Median graft size was 16 mm (range 14 to 20 mm)., Results: There was no early mortality, and 68% of patients were discharged without complications. Complications included persistent cyanosis in 4 patients, persistent pleural effusions in 2 (one chylous), and phrenic nerve injury in 1. Median postoperative hospital stay was 16 days (range 10 to 50) days. At a mean follow-up of 44 +/- 28 months, there was no conduit obstruction. One patient died 11 months postoperatively, and 1 patient received a heart transplant 26 months post-ECF., Conclusions: At intermediate term follow-up, the ECF without CPB appears to be safe and technically reproducible in selected cases. Ongoing follow-up of these patients is necessary to document the theoretical advantages of avoiding CPB.

Objective: Evaluation of the long-term clinical results of the Fontan operation in patients with tricuspid atresia., Methods: A retrospective analysis was made at the Instituto de Cardiologia do Rio Grande do Sul (Institute of Cardiology of Rio Grande do Sul), from August 1980 through January 2000, of 25 patients with a long-term follow-up, out of a series of 36 patients who underwent the Fontan operation or one of its variants due to tricuspid atresia. Their mean age at surgery was 5.4+/-3.1 years, and their mean weight was 15.8+/-6.1 kg, the majority of them (63.9%) being males. Four patients underwent the classical Fontan operation, 12 the Kreutzer variant, 6 the Björk variant, 9 total cavopulmonary shunt with a fenestrated tube, and 5 total cavopulmonary shunt with a nonfenestrated tube., Results: The patients were followed-up on an outpatient basis, with a mean long-term survival time of 5.5+/-4.2 years (50 days to 17.8 years) and a late mortality rate of 8%. Arterial saturation increased from 77.2+/-18.8% in the preoperative period to 91+/-6.7% upon the last outpatient visit (p>0.05). At the final check, most (67%) patients were asymptomatic and 87% could tolerate exercise. Ten (40%) patients experienced some kind of complication during the long-term follow-up, such as cardiac arrhythmia, cyanosis, protein-losing enteropathy, neurological events, right heart failure, intolerance to exercise and reoperation., Conclusion: The results indicate that, once the immediate postoperative period is over, during which the adaptations to the new circulatory physiology occur, the evolution of patients with tricuspid atresia who underwent the Fontan operation is satisfactory, in spite of a low, yet significant, morbidity.

The purpose of this study was to clarify desired stent sizes for stenotic lesions in the post-Fontan circulation. Using angiograms from 22 patients before and at late follow-up (> or = 15 years) after the Fontan operation, we measured the maximum diameters of the proximal pulmonary arteries (PA) and the descending aorta. The diameters of the PA ipsilateral to the inferior vena cava, contralateral to the inferior vena cava, and descending aorta after the Fontan were 10.6-22.6 (15.8 +/- 3.3), 8.0-19.1 (12.9 +/- 3.1), and 12.1-18.9 (15.8 +/- 2.0) mm, respectively, while the percent of normal predicted diameters (% N) were 55%-104% (70% +/- 14%), 38%-99% (66% +/- 17%), and 46%-74% (60% +/- 7%), respectively. Despite somatic growth, the % N of all vessel diameters decreased significantly after the Fontan operation. In conclusion, smaller-sized stents should be acceptable for both the pulmonary artery and descending aorta in the Fontan circulation., (Copyright 2002 Wiley-Liss, Inc.)