Single ventricle palliation: greater risk of complications with the Fontan procedure than with the bidirectional Glenn procedure alone

BACKGROUND: This study was performed to evaluate and compare the early, intermediate, and long-term outcomes of the bidirectional Glenn procedure and Fontan procedure in patients who live at moderately high altitude. METHODS: The outcome of each method of palliation for patients with a functionally single ventricle was retrospectively evaluated from a review of medical records. RESULTS: The bidirectional Glenn procedure was performed in 177 patients from October 1984 to June 2004. The Fontan procedure was performed in 149 patients from June 1978 to June 2004. Cardiovascular death or heart transplantation occurred in 8% of patients after the bidirectional Glenn procedure and 17% of patients after the Fontan procedure. Complications of systemic thromboembolic events, bleeding associated with anticoagulation therapy, protein losing enteropathy, and arrhythmias requiring implantation of a pacemaker, cardioversion, or radiofrequency ablation occurred in 7% of patients after the bidirectional Glenn procedure and 47% of patients after the Fontan procedure. Cardiovascular deaths and heart transplantation occurred less frequently when the Fontan procedure was performed in patients with a previous bidirectional Glenn procedure. However, the actuarial transplant-free survival and freedom from complications was not superior for a subgroup of patients who had a Fontan procedure after a bidirectional Glenn procedure in comparison to a subgroup of patients who had a bidirectional Glenn procedure alone. CONCLUSIONS: The bidirectional Glenn procedure can be used for long-term palliation of patients with a functionally single ventricle. Additional palliation with a Fontan procedure may increase the risk of stroke, protein losing enteropathy and arrhythmias without improving survival.     

Outcome of single ventricle and total anomalous pulmonary venous connection

Management of patients with single-ventricle physiology is significantly affected by anomalies of pulmonary venous return at all stages, whether primary palliation, bidirectional Glenn shunt, or completion of Fontan circulation. We treated 25 patients with pulmonary venous anomalies and single ventricle by staged palliation, from June 1996 to May 2005. Visceral heterotaxy with atrial isomerism was present in 19 of them. Primary palliation with a systemic-to-pulmonary artery shunt was undertaken in 15 patients. There were 5 early deaths, of which 4 were due to obstruction of pulmonary venous return. A bidirectional Glenn shunt was constructed in 17 patients including 10 who had it as a primary palliative procedure. There were 7 early deaths after the bidirectional Glenn procedure; only one was due to pulmonary venous obstruction. Five patients attained completion of the Fontan procedure. There was one early death after the Fontan operation. Anomalous pulmonary venous return can significantly complicate the management of patients with single ventricle, with an impact on survival in early infancy. Palliation with the aim of instituting extracardiac conduit Fontan circulation allows greater latitude and more streamlined management.     

Risk factors in the Glenn bidirectional shunt as an intermediate procedure before Fontan correction

OBJECTIVE: The bidirectional Glenn shunt is the most common palliation before the Fontan repair, especially in high-risk patients. We studied the influence of certain risk factors in bidirectional Glenn results, with and without an additional source of pulmonary blood flow. METHODS: Between 1993 and 1998 twenty patients (6-53 months of age) underwent a bidirectional Glenn shunt as the intermediate repair for the Fontan procedure. Diagnoses were: 7 cases of double inlet single ventricle, 4 of tricuspid atresia, 3 of unbalanced AV septal defect, 4 of mitral atresia, 1 hypoplastic left heart syndrome and 1 TGA with hypoplasia of the right ventricle. 17 patients had undergone previous operations. Mean preoperative arterial oxygen saturation was 78.5%. In 6 patients an auxiliary source of pulmonary blood flow was added. RESULTS: Hospital mortality was 15%. In 4 patients the bidirectional Glenn failed. By univariate analysis low weight, preoperative functional status and high pulmonary pressure were factors associated with early death. In bidirectional Glenn failure only the duration of ventilatory support was significant. By multivariate analysis, preoperative functional status and pulmonary pressure were significant. Mean postoperative arterial oxygen saturation at a mean follow-up of 10 months was 84%. CONCLUSIONS: Bidirectional Glenn shunt is an effective and low-risk palliation for patients with univentricular hearts. Only low weight and high pulmonary pressure were significant in hospital mortality, and we advise the association of an additional source of pulmonary blood flow in these patients at the start. Early extubation provides correct performance of the shunt.     

Postoperative and long‐term outcomes in children with Trisomy 21 and single ventricle palliation

Objective: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long‐term outcomes in patients with T21 and SV palliation.
Design: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anom‐ aly following Fontan palliation for similar diagnoses. Kaplan‐Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) through 2014 for patients with adequate identifiers.
Results: We identified 118 children with T21 who underwent initial surgical SV pallia‐ tion. Among 90 (75.6%) patients surviving their first surgery, 66 (73.3%) underwent Glenn anastomosis and 25 (27.8%) completed Fontan palliation with in‐hospital sur‐ vival of 80.3% and 76.0%, respectively. Fifty‐three patients had sufficient identifiers for PCCC‐NDI‐OPTN linkage. Ten‐year survival, conditioned on discharge alive after the Fontan procedure, was 66.7% compared to 92.2% for 51 controls without genetic anomaly (P = .001). Median age at death for T21 patients following initial surgical SV palliation was 2.69 years (IQR 1.34‐7.12) with most deaths (89.2%) attributed to the underlying congenital heart disease (CHD).
Conclusions: Children with T21 and SV are at high risk for procedural and long‐term mortality related to their genetic condition and underlying CHD. Nevertheless, a se‐ lect group of patients can successfully complete Glenn or Fontan palliation, reaching satisfactory long‐term survival.     

Somatic Growth after Fontan and Mustard Palliation

Objective. Patients with complex congenital heart disease frequently develop early growth failure; however, the long‐term outcome for growth after surgery for single ventricle or anatomic right ventricle as systemic ventricle is not clear. This study was designed to determine long‐term growth in patients following the Fontan and Mustard operations. Method. We retrospectively reviewed the growth parameters of children who had previously undergone the Fontan (n = 80) or Mustard (n = 66) palliation at the Riley Hospital for Children, Indiana. Results. Both the Fontan and Mustard groups had normal height and weight at birth. At the time of their Fontan or Mustard palliation, there was a significant retardation in weight (Z‐score: ?0.98 and ?1.79, respectively) and height (Z‐score: ?0.96 and ?1.03, respectively). Both cohorts postoperatively demonstrated significant catch‐up in their weights. Although the Mustard group normalized their heights, the Fontan patients continued to demonstrate short statures in long‐term follow‐up. Conclusion. Children with single ventricles and those with palliated d‐loop transposition of the great arteries suffer somatic growth delay prior to definitive surgery, despite being of normal size at birth. Catch‐up growth in weight occurs after the Fontan and Mustard operations. In the Mustard population, height also normalizes, whereas in patients with univentricular circulation, height remains abnormally low.     

Somatic growth failure after the Fontan operation

Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was -1.5 +/- 1.2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (-0.91 +/- 0.99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were -0.67 +/- 1.1 and -0.89 +/- 1.2 respectively. At most recent follow-up, with a mean age of 6.1 +/- 1.3 years, and a mean time from the Fontan operation of 4.4 +/- 1.4 years, the mean Z score for height was -1.15 +/- 1.2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings.     

De Vega tricuspid annuloplasty for systemic tricuspid regurgitation in children with univentricular physiology

BACKGROUND AND AIM OF THE STUDY: Significant tricuspid valve regurgitation (TR) is problematic in children with univentricular physiology and a systemic tricuspid valve occasionally requiring tricuspid (systemic atrioventricular) valve replacement. Since 1998, the De Vega tricuspid annuloplasty technique has been applied for TR in these children. METHODS: Twelve children (median age 2.2 years; range: 6 months to 17 years) with moderate or severe systemic TR underwent a De Vega tricuspid annuloplasty during a bidirectional Glenn anastomosis (n = 3), Fontan procedure (n = 8) or aortic valve replacement late after a Fontan procedure (n = 1). Nine patients (75%) had prior Norwood palliation for hypoplastic left heart syndrome. Four patients had simultaneous repair of an abnormal tricuspid valve in addition to the De Vega procedure. RESULTS: There were no deaths during a mean follow up of 2.0 +/- 1.4 years (range: 6 months to 5.1 years). One child required pacemaker implantation early after operation, and one child with a Glenn anastomosis underwent cardiac transplantation 21 months postoperatively. In the remaining 11 patients, the most recent echocardiogram showed mild or no TR in eight children, mild-to-moderate TR in one child, and moderate TR in two children. No child had symptomatic TR (including the two with moderate TR), significant tricuspid stenosis, or late pacemaker implantation. CONCLUSION: The De Vega tricuspid annuloplasty safely provides excellent relief of systemic TR in children with univentricular physiology, with a majority of patients (73%) having mild or less residual TR at follow up examination. This simple technique is preferred to tricuspid (systemic) valve replacement in these children.     

Single-stage versus two-stage modified Fontan procedure

We compared surgical outcomes of the single-stage and two-stage modified Fontan procedures to clarify clinical superiority. Of 28 children undergoing a modified Fontan procedure from October 1995 to October 2005, 15 had a 1-stage and 13 had a 2-stage operation. In the 2-stage group, pulmonary artery growth was evaluated before and after the first stage. Operative mortality was 26.6% in the 1-stage group and 0% in the 2-stage group. The benefits of a previous bidirectional Glenn shunt were decreased cyanosis and volume overload, but there was no significant difference in pulmonary artery growth reflected in pulmonary artery indices before and after the bidirectional Glenn procedure. Older children underwent a 2-stage modified Fontan procedure and had better outcomes in terms of lower mortality, improved oxygen saturation, decreased volume load, and less deterioration of atrioventricular valve regurgitation.     

Somatic growth in children with single ventricle physiology impact of physiologic state.

OBJECTIVES: We sought to define somatic growth patterns for patients with single ventricle (SV) physiology and associated factors. BACKGROUND: Infants with SV physiology might have somatic growth retardation associated with volume overload and hypoxemia, which might improve after surgical palliation. METHODS: We reviewed 126 patients (35% male) who underwent the Fontan procedure from 1994 to 2004. Demographic data, hemodynamic variables, and surgical procedures were recorded. Serial weights and heights were converted to z-scores. Linear regression analysis adjusted for repeated measures was used to model growth trends. RESULTS: Median z-score for weight was -0.7 at birth, -1.6 before bidirectional cavopulmonary shunt (BCPS), -0.7 before Fontan procedure, and -0.7 after Fontan procedure. A significant decline in z-scores for weight was seen before BCPS, which was reversed after the hemi-Fontan and stabilized after Fontan procedure. The z-scores for weight before the BCPS were lower in patients with lower birth weight (p < 0.01), nutritional difficulties (p = 0.01), and higher right atrial pressures (p = 0.02). After the BCPS, impaired growth was seen in patients who had systemic venous collaterals (p < 0.01). Patients who had collaterals embolized had the same growth trends as patients with no collaterals (p = 0.29). CONCLUSIONS: Infants with SV physiology show impaired somatic growth before BCPS. Although catch-up growth occurs after BCPS, effective interventions such as more intensive nutritional strategies before BCPS might be targeted at this high-risk population. The presence of systemic venous collaterals might impede growth secondary to hemodynamic impairment. Embolization of collaterals might allow for maximum growth potential.     

Improved early results with cavopulmonary connections

BACKGROUND: We describe the recent results in a large cohort of patients with functionally single ventricle who underwent various modifications of cavopulmonary connections. METHODS: Using the database at our institution, we identified all children who underwent cavopulmonary connection operations between June 1995 and June 1997. Demographic data, surgical history, and perioperative course were reviewed. RESULTS: We performed 130 consecutive operations in 113 patients. The procedures included superior cavopulmonary connections in the form of the HemiFontan procedure in 45 instances, and bidirectional Glenn procedures in 11, and bilateral superior cavopulmonary connections in 7. The median age of these patients was 7.0 months. We completed Fontan operations using a fenestrated lateral tunnel on 47 occasions, and using an extracardiac conduit 9 times, 5 of which were fenestrated. A lateral tunnel without fenestration was constructed in one patient. The median age for these procedures was 19.5 months. In the remaining 10 instances, we revised Fontan procedures at a median age of 8 years. Diagnoses included hypoplastic left heart syndrome in 43 patients, double outlet right ventricle in 22, heterotaxy in 13, tricuspid atresia in 13, and a miscellaneous group accounting for the other 22. One death (0.7%) occurred within 30 days of surgery. Clinical seizures occurred in 7 children (5.3%), 6 had no residual neurologic deficits. Atrial pacing was needed in 14 children (10.7%) because of transient junctional rhythm, and 2 received treatment for supraventricular tachycardia. Pleural effusions were diagnosed radiographically after 31 of 130 (24%) procedures. Diuretic therapy resolved the effusion in 21 of these, with only 6 children requiring thoracostomy catheter drainage, and 4 undergoing thoracentesis alone. The median length of stay on the intensive care unit was 2 days, with a range from 1 to 30 days, and median stay in hospital was 6 days, with a range from 3 to 58 days. CONCLUSION: Mortality and perioperative morbidity after cavopulmonary connections have decreased dramatically in the current era. The long-term results of staged reconstruction for functionally single ventricle, nonetheless, await ongoing study.     

Discontinuous Pulmonary Arteries Do Not Preclude Good Fontan Outcomes

Objective. Discontinuous pulmonary arteries are believed to portend poor outcomes for a single ventricle palliation leading to Fontan's operation. This is a single institutional review of patients with single ventricle and discontinuous pulmonary arteries who underwent pulmonary artery centralization as part of staged surgical palliation. Design. The study is a retrospective case series. Patients. From November 1997 to December 2005, 12 centralization procedures were performed on 12 single ventricle patients with discontinuous pulmonary arteries. The diagnoses at surgery were as follows: heterotaxy 67%, pulmonary atresia 75%, a single morphologic right ventricle 58%, a single morphologic left ventricle 33%, and functional single ventricle with atrial situs inversus 8%. Outcome Measures. The outcome was assessed by hospital survival, actuarial survival, and New York Heart Association (NYHA) classification at follow-up. Results. The overall actuarial survival following centralization is 100% (95% confidence interval = 0.698 to 1). Seventy-five percent of the patients have undergone a Fontan procedure. Median McGoon ratio pre-Fontan = 1.65 (range: 1–2.1). Median follow-up after Fontan = 4.4 years (range: 1.2–9 years). Overall actuarial survival following Fontan is 100% (95% confidence interval = 0.428–0.911). Following the Fontan, there have been no thromboembolic complications, protein-losing enteropathy, nor Fontan takedowns. One hundred percent of the Fontan patients are NYHA class I. Conclusions. This experience indicates that a resuscitative strategy for discontinuous pulmonary arteries can result in good outcomes after the Fontan procedure. The presence of discontinuous pulmonary arteries in patients with single ventricle physiology should not preclude a management strategy with the goal of Fontan candidacy.     

Outcome of Glenn anastomosis for heterotaxy syndrome with single ventricle

A retrospective study was undertaken of 157 patients who underwent a Glenn anastomosis between January 1996 and May 2001. Of these, 33 had heterotaxy syndrome: 20 males and 13 females, with a mean age of 1.26 +/- 2.8 years. Twenty-five had right atrial isomerism and 5 had left isomerism. A common atrioventricular valve was found in 24 patients, 18 had bilateral superior venae cavae, and 18 had anomalous pulmonary venous return. Repair was carried out in 8 patients with anomalous pulmonary venous return, and pulmonary artery augmentation was performed in 11. Compared to the 124 patients who had a Glenn operation for single ventricle without heterotaxy, there were significantly longer durations of mechanical ventilation, intensive care unit stay, and inotropic support, as well as higher mortality in the heterotaxy group. Heterotaxy syndrome with single ventricle still has a high rate of morbidity and mortality. Patients with severe atrioventricular valve regurgitation are at risk of early death. Complete Fontan circulation may not be possible in all patients, and Glenn anastomosis may be their final palliation.     

An Unusual Cause of Irritability in a Single Ventricle Patient after Bidirectional Glenn Shunt

The differential diagnosis for irritability in children is broad. In patients with congenital heart disease, one must strongly consider cardiac etiologies such as low cardiac output or elevated central venous pressure (CVP). In patients with single-ventricle physiology, the second stage of palliation includes bidirectional Glenn, which involves anastomosis of the superior vena cava to the pulmonary artery resulting in volume offloading of the single systemic ventricle. Typically, early in the post-operative period, patients may experience a headache due to the acute increase in CVP, and symptoms improve over time. Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a rare neurologic disorder in children, characterized by raised intracranial pressure (ICP) in the absence of brain parenchymal lesions or cerebrospinal fluid (CSF) abnormalities. While the pathogenesis of IIH is unknown, early recognition and treatment of IIH are important to prevent permanent vision loss. There are only rare reports of IIH in patients with Fontan circulation. To our knowledge, we report the first case of IIH in a 2-year-old female after bidirectional Glenn.     

Fontan procedure--indication and clinical results

Twenty-six patients with tricuspid atresia (15), univentricular heart (7), and single ventricle (4) underwent 27 Fontan or modified Fontan procedures between 1975 and 1981. The age of the patients varied between 4 and 26 years. Twenty patients had had a total of 33 palliative operations prior to correction. The original Fontan procedure was performed in 10 patients from 1975 to 1977. According to the various anatomical findings modifications of the Fontan procedure, such as direct anastomosis or implantation of a valveless conduit, were introduced in 1977. Early mortality among all the patients was 22% (6 patients died). Three deaths occurred in the initial period 1975 to 1977. Among the last 20 patients (1978 to 1981) there were 3 early deaths. Three patients with single ventricle survived, one died due to pulmonary failure. There were 2 late deaths (sepsis, sudden cardiac death). Postoperative cardiac catheterization performed in 17 patients revealed excellent results in 13 patients; the remaining 4 displayed diminished arterial oxygen saturation, three of them had Glenn palliation prior to corrective surgery. Postoperative right atrial mean pressure varied from 10 to 23 mmHg. The left ventricular parameters were within the normal range.     

Amplatzer occlusion of accessory ventriculopulmonary connections

Background: Children with complex congenital heart disease often require staged palliation to regulate systemic and pulmonary blood flow. Accessory sources of pulmonary blood flow including aortopulmonary collaterals, aortopulmonary shunts, and ventriculopulmonary connections following Glenn or Fontan palliation can be associated with elevated central venous pressures and persistent pleural drainage. Occlusion of accessory ventriculopulmonary connections in this setting has traditionally been accomplished surgically. Objective: To review the efficacy of Amplatzer devices in transcatheter occlusion of accessory ventriculopulmonary connections in children with complex congenital heart disease. Methods: Patients were identified and their records retrospectively reviewed for indication, procedural details, and clinical efficacy and outcome. Results: Between December 2004 and March 2008, seven patients underwent occlusion of accessory ventriculopulmonary connections using an Amplatzer Septal Occluder (3), an Amplatzer Duct Occluder (3), or an Amplatzer Vascular Plug (1). Underlying single ventricle physiology was present in six of these patients. The site of occlusion was the right ventricle to pulmonary artery (Sano) conduit in two patients, the native main pulmonary artery in three patients, the pulmonary valve in one patient, and a left ventricle to pulmonary artery homograft in one patient with biventricular physiology. There were no complications associated with these procedures. Conclusions: Amplatzer occlusion devices provide a safe and effective means of eliminating accessory ventriculopulmonary connections in children who have undergone surgical palliation of congenital heart disease. © 2008 Wiley‐Liss, Inc.     

The bidirectional Glenn operation in 100 cases with complex congenital heat diseases: factors influencing surgical results]

OBJECTIVE: The bidirectional Glenn shunt is a well established surgical technique in children with complex congenital heart disease. The present study is a retrospective analysis of patients undergoing this operation in order to assess the influence of different variables in the outcome. PATIENTS AND METHOD: From December 1990 to June 2000, 100 patients received a bidirectional Glenn shunt.Two groups were defined, based on the outcome. Group A (n = 15, unsuccessfully result) including death and need to reoperate, and Group B (n = 85, patients with good outcome). RESULTS: The mortality was 8%. Eight patients were reoperated at follow-up. The survivors were followed for a mean of 3.5 years. Mean pulmonary artery pressure 7 mmHg was a factor associated with poor clinical progress. Other variables (age less than 1 year, excessive pulmonary blood flow, double Glenn operation, significant anatomic anomalies, and arrythmias), were also associated with outcome. Significant variations were observed in the time of by-pass or the need for aortic clamp in cases with simultaneous operative repair of pulmonary branch stenosis. Actuarial survival rate, most more than 1 year was 92%, and freedom from reoperation at 3 years was 90%. CONCLUSIONS: The bidirectional Glenn shunt is an excellent palliation in patients with functionally single ventricle. Mean pulmonary artery pressure was the most important variable related with the outcome. We are encouraged to continue with tendency to perform bidirectional Glenn shunt preferably early, avoiding, whenever possible, previous palliative surgery.     

Tricuspid atresia: results of treatment in 160 children.

Experience in managing 160 patients with tricuspid atresia is reviewed. The majority of these children require operative treatment in the 1st year of life. Clinical management of this rather complex anomaly is simplified by classifying each child according to total pulmonary blood flow. An overall plan of management for patients with decreased pulmonary flow is suggested. This consists of a Potts shunt as an initial procedure, limiting its anastomotic growth so that pulmonary flow becomes inadequate at age 10 to 12 years. A Glenn anastomosis is then constructed as the second procedue. The Fontan procedure should be considered carefully as an alternative to the Glenn anastomosis in these older children. Children with increased pulmonary blood flow and transposition of the great arteries are likely to require pulmonary arterial banding in infancy. Otherwise, patients in this smaller group are managed with the same overall plan. Results of long-term palliation have been good.     

Treatment of elevated pulmonary artery pressure in a child after Glenn procedure: transcatheter closure of pulmonary artery banding with subsequent sildenafil therapy

An additional source of pulmonary blood flow in a patient with bidirectional Glenn procedure (BGD) may cause elevation of mean pulmonary artery pressure (MPAP), precluding safe completion of the Fontan operation. We present a case of single ventricle physiology after pulmonary artery banding (PAB) and Glenn procedure. At the age of six years, cardiac catheterisation revealed in the patient elevated MPAP (22 mm Hg). The PAB was closed through the right internal jugular vein with an Amplatzer Atrial Septal Occluder. After the procedure, MPAP remained at a similar level. Sildenafil oral therapy was applied for six months. Subsequent heart catheterisation confirmed complete closure of PAB and decrease of MPAP to 10 mm Hg. The abovementioned complex treatment of elevated MPAP pressure in a child after Glenn therapy allowed safe completion of the Fontan operation.     

Diagnostic assessment before Fontan operation in patients with bidirectional cavopulmonary anastomosis: are noninvasive methods sufficient?

OBJECTIVES: This study was designed to determine if a subset of patients who have undergone bidirectional cavopulmonary anastomosis could be identified in which catheterization was of little benefit before completion of the Fontan procedure. BACKGROUND: Diagnostic evaluation before Fontan procedure has typically included cardiac catheterization. However, the overall management strategy for patients with functional single ventricle has evolved to include staging bidirectional cavopulmonary anastomosis in most, and it has become uncommon to exclude patients from Fontan based on catheterization data. METHODS: Patients who underwent bidirectional cavopulmonary anastomosis and had complete echocardiograms and catheterizations within three months of each other between January 1992 and October 1997 were evaluated with a series of clinical and echocardiographic characteristics to identify a subset in whom catheterization was predicted to be of little added value ("no-cath" group). The predictive value and sensitivity of these criteria in excluding patients who required additional intervention, were excluded from Fontan, or died within 30 days of Fontan was determined. RESULTS: A total of 99 patients who underwent bidirectional cavopulmonary anastomosis at 6.7 months (range 2.9 months to 14 years) were studied; 46 met criteria for the "no-cath" group. Noninvasive criteria stratified all patients who died (n = 5) or did not proceed to Fontan (n = 1) and 9 of 11 who required additional interventions to the "cath" group. Thus, the negative predictive value of these criteria was 93%. CONCLUSIONS: Our data suggest that catheterization before Fontan could be avoided in a large percentage of patients without adversely affecting outcome; prospective evaluation of this strategy is warranted.