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Background: We aimed to evaluate the effect of age at operation on postoperative outcomes in children undergoing a Kawashima operation., Methods: The Society of Thoracic Surgeons Congenital Heart Surgery Database was queried for Kawashima procedures from January 1, 2014, to June 30, 2020. Patients were stratified by age at operation in months: 0 to <4, 4 to <8, 8 to <12, and >12. Subsequently, outcomes for those in whom the Kawashima was not the index operation and for those undergoing hepatic vein incorporation (Fontan completion or hepatic vein-to-azygos vein connection) were evaluated., Results: We identified 253 patients who underwent a Kawashima operation (median age, 8.6 months; median weight, 7.4 kg): 12 (4.7%), 0 to <4 months; 96 (37.9%), 4 to <8 months; 81 (32.0%), 8 to <12 months; and 64 (25.3%), >12 months. Operative mortality was 0.8% (n = 2), with major morbidity or mortality in 17.4% (n = 44), neither different across age groups. Patients <4 months had a longer postoperative length of stay (12.5 vs 9.3 days; P = .03). The Kawashima was not the index operation of the hospital admission in 15 (5.9%); these patients were younger (6.0 vs 8.4 months; P = .05) and had more preoperative risk factors (13/15 [92.9%] vs 126/238 [52.9%]; P < .01). We identified 173 patients undergoing subsequent hepatic vein incorporation (median age, 3.9 years; median weight, 15.0 kg) with operative mortality in 6 (3.5%) and major morbidity or mortality in 30 (17.3%)., Conclusions: The Kawashima is typically performed between 4 and 12 months with low mortality. Morbidity and mortality were not affected by age. Hepatic vein incorporations may be higher risk than in traditional Fontan procedures, and ways to mitigate this should be sought., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Tricuspid atresia with normally related great vessels (TA) is considered the optimal substrate for the Fontan pathway. The factors associated with death or transplantation after cavopulmonary shunt (CPS) are underappreciated. We aimed to determine factors associated with CPS-Fontan interstage death/transplantation versus transition to Fontan in TA., Methods: A total of 417 infants younger than 3 months of age with TA were enrolled (January 1999 to February 2020) from 40 institutions into the Congenital Heart Surgeons' Society TA cohort. Parametric competing risk methodology was used to determine factors associated with the competing end points of death/transplantation without Fontan completion, and transition to Fontan., Results: CPS was performed in 382 patients with TA; of those, 5% died or underwent transplantation without transition to Fontan and 91% transitioned to Fontan by 5 years after CPS. Prenatal diagnosis (hazard ratio [HR], 0.74; P < .001) and pulmonary artery band (PAB) at CPS (HR, 0.50; P < .001) were negatively associated with Fontan completion. Preoperative moderate or greater mitral valve regurgitation (HR, 3.0; P < .001), concomitant mitral valve repair (HR, 11.0; P < .001), PAB at CPS (HR, 3.0; P < .001), postoperative superior vena cava interventions (HR, 9.0; P < .001), and CPS takedown (HR, 40.0; P < .001) were associated with death/transplantation., Conclusions: The mortality rate after CPS in patients with TA is notable. Those with preoperative mitral valve regurgitation remain a high-risk group. PAB at the time of CPS being associated with both increased risk of death and decreased Fontan completion may represent a deleterious effect of antegrade pulmonary blood flow in the CPS circulation., (Copyright © 2021 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Fontan failure often requires surgical therapy in the form of Fontan revision or heart transplantation. We sought to characterize national trends in the surgical burden of Fontan failure and identify risk factors for adverse outcomes., Methods: Fontan patients undergoing Fontan revision or transplantation from January 2010 to June 2018 were included. We evaluated baseline characteristics and outcomes and used multivariable logistic regression to identify risk factors for operative mortality and composite mortality and major morbidity., Results: A total of 1135 patients underwent Fontan revision (n = 598) or transplantation (n = 537) at 100 centers. Transplantations increased from 34 in 2010 to 76 in 2017, largely owing to an increase in patients with hypoplastic left heart syndrome (HLHS) (18 in 2010 to 49 in 2017), while Fontan revision decreased (75 in 2010 to 49 in 2017). Transplantation patients were younger (median 14 years of age vs 18 years of age; P < .001), more often had preoperative risk factors (66% vs 40%; P < .001), and more often had HLHS (51% vs 15%; P < .001). Operative mortality and composite major morbidity and mortality were 7.6% and 35% for transplantation and 7.1% and 22% for Fontan revision, respectively. Multivariable risk factors for mortality included older age (odds ratio [OR], 1.08/y; P = .007), presence of preoperative risk factors (OR, 3.33; P = .002), and concomitant pulmonary artery reconstruction (OR, 2.7; P = .029) for Fontan revision but only older age (OR, 1.06/y; P = .020) for transplantation., Conclusions: Both transplantation and Fontan revision are associated with high morbidity and mortality. There has been evolution of practices in surgical therapy for Fontan failure, perhaps related to rising prevalence of HLHS staged palliation., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Fontan survivors have depressed cardiac index that worsens over time. Serum biomarker measurement is minimally invasive, rapid, widely available, and may be useful for serial monitoring. The purpose of this study was to identify biomarkers that correlate with lower cardiac index in Fontan patients. Methods and results This study was a multi-centre case series assessing the correlations between biomarkers and cardiac magnetic resonance-derived cardiac index in Fontan patients ⩾6 years of age with biochemical and haematopoietic biomarkers obtained ±12 months from cardiac magnetic resonance. Medical history and biomarker values were obtained by chart review. Spearman's Rank correlation assessed associations between biomarker z-scores and cardiac index. Biomarkers with significant correlations had receiver operating characteristic curves and area under the curve estimated. In total, 97 cardiac magnetic resonances in 87 patients met inclusion criteria: median age at cardiac magnetic resonance was 15 (6-33) years. Significant correlations were found between cardiac index and total alkaline phosphatase (-0.26, p=0.04), estimated creatinine clearance (0.26, p=0.02), and mean corpuscular volume (-0.32, p<0.01). Area under the curve for the three individual biomarkers was 0.63-0.69. Area under the curve for the three-biomarker panel was 0.75. Comparison of cardiac index above and below the receiver operating characteristic curve-identified cut-off points revealed significant differences for each biomarker (p<0.01) and for the composite panel [median cardiac index for higher-risk group=2.17 L/minute/m2 versus lower-risk group=2.96 L/minute/m2, (p<0.01)]., Conclusions: Higher total alkaline phosphatase and mean corpuscular volume as well as lower estimated creatinine clearance identify Fontan patients with lower cardiac index. Using biomarkers to monitor haemodynamics and organ-specific effects warrants prospective investigation.

Background: There is increasing interest in reducing the duration of mechanical ventilation after pediatric cardiac operations. However, the contemporary use of an early extubation strategy and its effect on clinical outcomes is poorly understood., Methods: Data from The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010 to 2013) were analyzed to determine the association of early extubation with postoperative length of stay (PLOS). Two operations were analyzed: complete repair of tetralogy of Fallot (TOF) in patients older than 45 days and the Fontan procedure. Centers were stratified into tertiles by frequency of early extubation, defined as less than 6 hours after leaving the operating room. Patients extubated after 48 hours were excluded., Results: Among 92 centers, early extubation was performed in 31.5% (478 of 1,519) of children undergoing TOF repair and in 69.8% (1,153 of 1,653) of those undergoing the Fontan procedure. Early extubation after TOF repair was associated with heavier weight at operation (p < 0.001) and fewer preoperative risk factors (p = 0.016). After adjustment for covariates, average PLOS after TOF repair was shorter for centers in the highest tertile of early extubation rate than for the lowest tertile centers, which have low early extubation rate (p = 0.04). No association was found between center early extubation rate and PLOS for Fontan procedures (p = 0.08)., Conclusions: Early extubation is common after repair of TOF and the Fontan procedure. A high institutional rate of early extubation after TOF repair is associated with shorter PLOS. Further analysis is needed to understand what effect early extubation might have on other meaningful measures such as resource use., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Extracardiac conduit and lateral atrial tunnel total cavopulmonary connection are both widely used in the management of functionally univentricular hearts. The effect of the type of connection on early outcomes after Fontan operation remains unclear. We evaluated the effect of Fontan type on early outcome in a large clinical database., Methods: Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database undergoing the Fontan operation (2000 to 2009) were included. We used multivariable analysis to evaluate the effect of Fontan type (extracardiac conduit vs lateral tunnel) on in-hospital death, Fontan takedown/revision, Fontan failure (in-hospital death or Fontan takedown/revision), postoperative length of stay, and complications, adjusting for patient, procedural, and center factors., Results: The study included 2,747 patients (61% male) from 68 centers. A right-dominant ventricle was present in 45%. Extracardiac conduit Fontan (vs lateral atrial tunnel) was performed in 63%; in all, 65% were fenestrated. In multivariable analysis with adjustment for patient, procedural (including fenestration), and center factors (including Fontan volume), the extracardiac conduit Fontan was associated with significantly higher Fontan takedown/revision (odds ratio, 2.73; 95% confidence interval, 1.09 to 6.87) and Fontan failure (odds ratio, 2.28; 95% confidence interval, 1.13 to 4.59), and longer postoperative hospital stay (adjusted estimated difference in postoperative hospital stay: +1.4 days)., Conclusions: These multicenter data suggest that of the two prevalent forms of Fontan connection in current use, the lateral atrial tunnel Fontan may be associated with superior early outcomes., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The impact of age and weight on outcomes after the Fontan operation is unclear. Previous analyses have suggested that lower weight-for-age z-score is an important predictor of poor outcome in patients undergoing bidirectional Glenn. We evaluated variation in age, weight, and weight-for-age z-score at Fontan across institutions, and the impact of these variables on postoperative morbidity and mortality., Methods: Patients in The Society of Thoracic Surgeons Congenital Heart Surgery Database undergoing the Fontan operation (2000 to 2009) were included. Center variation in age, weight, and weight-for-age z-score were described. Multivariable analysis was performed to evaluate the impact of age, weight, and weight-for-age z-score on in-hospital mortality, Fontan failure (combined in-hospital mortality and Fontan takedown/revision), postoperative length of stay, and complications, adjusting for other patient and center factors., Results: A total of 2,747 patients (68 centers) were included: 61% male; 45% right dominant lesions (38% left dominant, 17% undifferentiated). An extracardiac conduit Fontan (versus lateral tunnel) was performed in 63%; 65% were fenestrated. Median age, median weight at Fontan operation, and proportion with weight-for-age z-score less than -2 varied across centers and ranged from 1.7 to 4.8 years, 10.5 to 16.1 kg, and 0% to 30%, respectively. In multivariable analysis, age and weight were not significantly associated with outcome. Weight-for-age z-score less than -2 was associated with increased in-hospital mortality (odds ratio 2.73, 95% confidence interval: 1.09 to 6.86), Fontan failure (odds ratio 2.59, 95% confidence interval: 1.24 to 5.40), and longer length of stay (+1.2 days, 95% confidence interval: 0.1 to 2.4)., Conclusions: Weight-for-age z-score less than -2 is associated with significant morbidity and mortality after the Fontan operation independent of other patient and center characteristics., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Results of Fontan's procedure have improved considerably, but perioperative mortality still occurs, attributed to ventricular dysfunction, stroke, arrhythmia, thromboembolism, and multi-organ dysfunction. Our protocols of operative and intensive care unit management address these potential issues, and have been associated with zero mortality, even with many high-risk candidates., Methods: From 1996 to 2006, all Fontan patients were managed as follows: operative strategy based on aortic and single atrial cannulation, cooling on full-flow bypass, and hypothermic circulatory arrest to create the Fontan pathway. No direct caval cannulation. Use of central venous lines was completely avoided. Fresh whole blood was used for pump prime and for volume restoration. Inotropic and vasodilator therapy was continued for at least 48 h. Aspirin was used exclusively as anti-thrombotic therapy. Postoperative pleural drainage was accomplished with small pigtail catheters. The usual Fontan pathway was by lateral atrial tunnel (84), with extra-cardiac conduit when dictated by anatomy (16)., Results: One hundred Fontan operations were performed with no mortality. All patients were extubated by postoperative day 1. Hospital stay was 10+/-5 days. Complications were: bleeding (1), reintubation (1), emergent fenestration closure (1), pericardial effusion (4), and seizures (1). Risk factors included Fontan connection to one lung (3), diminutive pulmonary arteries (PAs) and unifocalized major aortopulmonary collateral arteries (MAPCAs) (1), discontinuous PAs (3), right ventricle dependent coronaries (3), neonatal pulmonary venous obstruction (3), Trisomy 21 (1), preoperative pacemaker dependence (2), and heterotaxy (10). No candidate was excluded., Conclusions: While many surgeons try to avoid bypass or aortic clamping when performing Fontan operations, the strategies we have employed facilitate safe accomplishment of Fontan's operation in diverse anatomic groups with multiple risk factors, with avoidance of operative mortality in 100 consecutive cases.

Among factors contributing to morbidity and failure of the Fontan circulation is the group of events referred to as thromboembolic complications. These events have been variously attributed to low flow states, stasis in the venous pathways, right-to-left shunts, blind cul-de-sacs, prosthetic material, atrial arrhythmias, and hypercoagulable states. Numerous investigations, most retrospective, have been undertaken to characterize thromboembolic events; describe the frequency and circumstances of these occurrences; and relate the risk of these events to patient, surgical, hemodynamic, and hematologic factors. Practices vary widely with respect to strategies of prophylactic anticoagulation in the hopes of minimizing the occurrence and morbidity of thromboembolism after Fontan operations. Review of the literature suggests that the factors associated with thromboembolic events after Fontan operations likely represent a complex field of biologic factors with multiple interactions. It is unlikely that a single agent will represent the solution to this complex problem.

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.)

Enormous progress has been made in the last decade toward decreasing the risk of the Fontan operation and optimizing early and intermediate outcomes. Heterotaxy syndrome patients, with their extensive constellation of anatomic abnormalities, constitute a challenging group to manage surgically. Palliative procedures early in life are directed at creation of a satisfactory balance between pulmonary and systemic blood flow and at the same time assurance of unobstructed pulmonary venous return. For heterotaxy patients who survive beyond the newborn period, and are considered to have nonseptatable hearts, early reduction of the volume load of the functional single ventricle is extremely important. Early conversion from parallel pulmonary and systemic circulations to a superior cavopulmonary anastomosis (bidirectional Glenn or hemi-Fontan) is the preferred strategy. Traditionally, those patients with heterotaxy who survive initial surgical management have been considered a very high-risk population with respect to an eventual Fontan procedure. In heterotaxy patients it is particularly important to understand the unique and variable anatomy of the sinus node and conduction system, the potential for occult pulmonary venous obstruction, the tendency for development of atrioventricular valve regurgitation in volume loaded ventricles, and the potential for recurrent or persistent cyanosis because of intrahepatic shunting or pulmonary arteriovenous malformations., (Copyright 2002 by W.B. Saunders Company)

Background: Thromboembolism after Fontan's operation is attributed to low flow states, stasis in venous pathways, right to left shunts, blind cul-de-sacs, prosthetic materials, atrial arrhythmias, and hypercoagulable states. We assessed the efficacy of a strategy to reduce thromboembolic events including aspirin anticoagulation., Methods: From January 1996 through December 2000, 72 patients underwent Fontan procedures. Management included (1) avoidance of direct caval cannulation and central venous lines, (2) inotropic support for 48 to 72 hours to optimize cardiac output, (3) aortopulmonary anastomosis or suture closure of patent pulmonary valves, and (4) administration of aspirin (81 mg per day) beginning on postoperative day one. No other anticoagulation strategies were used. Surveillance included intraoperative and postoperative transesophageal echo, transthoracic echo at discharge, at first reevaluation, and at 6 month intervals, and catheterization 1 year after surgery., Results: There were no early or late deaths. Follow-up was completed with 2,882 patient-months and a mean of 40 months. There were no documented thromboembolic events; however, all suspicious occurrences were investigated by echo and brain imaging. There were no hemorrhagic events or aspirin-related complications., Conclusions: Low dose aspirin can be used safely in young patients with Fontan connections. At intermediate follow-up, the strategies described appear effective in preventing thromboembolic complications. Routine use of more aggressive anticoagulation regimens seems unwarranted.

We report the case of a child with hypoplastic left heart syndrome who developed pulmonary arteriovenous (AV) malformations after superior cavopulmonary anastomoses. Resolution of the pulmonary AV malformations occurred following a completion Fontan procedure. This phenomenon has been reported previously, but only in patients with heterotaxy and polysplenia.

Background: Sinus node dysfunction has been previously reported to occur in 13% to 16% of patients after the Fontan operation. Although there is concern that an intermediate cavopulmonary connection may increase the risk of sinus node dysfunction, previous studies have not reported on patients routinely staged to a Fontan operation. This study sought to determine the early and late incidences of sinus node dysfunction in patients systematically and uniformly staged to a Fontan operation after a prior hemi-Fontan., Methods and Results: To determine the early incidence of sinus node dysfunction, hospital records and perioperative ECGs were reviewed in all 287 patients having had a staged Fontan operation between January 1990 and December 1995. A cross-sectional analysis was performed on 220 of 239 surviving patients (92%) to determine the late incidence of sinus node dysfunction. Sinus node dysfunction was present in 7% of the patients before and in 15% after the hemi-Fontan. Although most patients (81%) regained normal sinus node function between the 2 stages, 23% had sinus node dysfunction in the early postoperative period after the Fontan. Of the 95 patients followed for > 4 years after the Fontan operation, 44% had sinus node dysfunction. However, at a mean follow-up of 3.5 +/- 1.7 years, only 16 patients (6.7%) had received a pacemaker and only 10 (4.1%) had documented atrial flutter., Conclusions: Perioperative sinus node dysfunction is common after both the hemi-Fontan and the Fontan procedures. Although many patients regain sinus node function between the 2 stages, late sinus node dysfunction is common and more likely to occur in patients with early sinus node dysfunction and those with longer follow-up.

Background: This study examined the results of a Fontan operation for patients with acquired atresia of one main branch pulmonary artery., Methods: The data for 7 patients identified as having a hypoplastic left pulmonary artery discontinuous from the right pulmonary artery were compared with those for 65 patients with continuous pulmonary arteries who consecutively underwent a completion Fontan procedure., Results: No significant differences were found preoperatively with respect to right atrial pressure, aortic saturation, ventricular end-diastolic pressure, pulmonary artery pressure, pulmonary blood flow, or pulmonary vascular resistance. In the first 24 postoperative hours, there were no significant differences in heart rate, urine output, systemic venous pressure, or pulmonary venous pressure. Also, data regarding hospitalization length, effusions, and mortality were similar between the two groups. Postoperative systemic arterial saturation was lower in the one-lung group. There were no early postoperative deaths in the one-lung group, and 5 of the 7 patients are long-term survivors., Conclusions: A completion Fontan procedure can be successfully performed in patients with a hypoplastic and discontinuous left pulmonary artery, although postoperative systemic arterial saturation is not as high as in patients with continuous pulmonary arteries.

Background: Significant morbidity after Fontan operation results in either takedown, heart transplantation, or death. Initial creation of a fenestration results in less morbidity and mortality; however, the role of late creation of a fenestration in aiding patients manifesting morbidity after an initial nonfenestrated Fontan operation is unclear., Methods and Results: We reviewed our experience with late creation of a surgical fenestration in 9 patients (5.2 +/- 3.1 years old) exhibiting chronic effusions (n = 4) or protein-losing enteropathy (PLE) (n = 5) after lateral tunnel-type Fontan operation. Patients with effusions had creation via coronary punch of two or three 3-mm defects; patients with PLE had creation of a large, 5-mm defect. One child with effusions and multisystem organ failure before fenestration died 7 weeks after surgery secondary to low cardiac output; the other 3 had resolution of effusions within 4 to 6 weeks. Of the 5 with PLE, 3 had normalization of serum proteins and resolution of symptoms at 2 to 6 weeks. The 2 failures had arterial saturations > 89% after surgery. Follow-up was from 25 to 30 months. Spontaneous closure of defects occurred in all 3 with effusions. No return of symptoms was noted in 2; however, the third reaccumulated effusions and has undergone refenestration with a large defect. All 3 patients with PLE have remained asymptomatic with patency of the fenestration (4 to 5 mm on echocardiography) and arterial saturation < or = 85% for > 2 years., Conclusions: Late surgical creation of fenestration results in resolution of morbidity after Fontan operation. Improvement is related to the degree of right-to-left shunt created.

Background: A high incidence of pulmonary arteriovenous malformations (PAVMs) has been reported in patients who have polysplenia and congenital heart disease after superior cavopulmonary anastomosis. Interruption of hepatic venous return to the pulmonary circulation is believed to potentiate the development of PAVMs. Surgical inclusion of hepatic flow in the pulmonary circulation may result in their resolution., Methods: We reviewed 3 patients with congenital heart disease and polysplenia in whom PAVMs developed and who had subsequent hepatic vein inclusion in the pulmonary circulation., Results: Patients underwent superior cavopulmonary connection at a median age of 8 months. The PAVMs were diagnosed at a median duration of 8 months after operation (arterial saturation <75% in room air). Hepatic venous flow was included in the pulmonary circulation at operation. Resolution of PAVMs occurred at a median duration of 7 months after operation (arterial saturation >90% in room air)., Conclusions: Surgical inclusion of hepatic venous blood in the pulmonary circulation results in the resolution of PAVMs. Electively associating the hepatic veins with the pulmonary vasculature may prevent the development of PAVMs in patients who are at risk.

Background: In hearts with a functional single ventricle, cavity volume and myocardial muscle mass increase as a consequence of the excessive volume work 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., Methods: All patients presenting in infancy with single-ventricle physiology were managed by early hemi-Fontan operation in anticipation of a subsequent completion Fontan operation. Between May 1989 and August 1995, 400 patients less than 2 years of age underwent hemi-Fontan operations. Mean age at operation was 8.5 months (range, 2 months to 24 months). The hemi-Fontan operation included association of superior vena(e) cava(e) with the branch pulmonary arteries, augmentation of the central pulmonary arteries, occlusion of the inflow of the superior vena cava into the right atrium, and elimination of other sources of pulmonary blood flow., Results: Operative mortality ( < 30 days) was 31 of 400 patients (7.8%). For the last 200 patients, operative mortality was 8 of 200 (4.0%). Younger age at operation was not an independent risk factor for operative mortality. Urgent operation in the presence of a hemodynamic burden requiring concomitant procedures was associated with increased mortality., Conclusions: The hemi-Fontan operation can be accomplished with low operative mortality in young patients, achieving early reduction of the volume work of the single-ventricle heart.

A 4-year-old child with hypoplastic left heart syndrome and a 6-year-old child with tricuspid atresia had both undergone staged reconstructive operations culminating in a Fontan operation. Peripheral edema, ascites, and hypoalbuminemia refractory to dietary manipulation and steroid therapy developed in both patients. After hemodynamic assessment, each child underwent surgical creation of a 4.8-mm fenestration in the previously placed baffle that separated the systemic venous pathway from the pulmonary venous atrium. Peripheral edema and ascites promptly resolved and serum protein levels normalized within 2 weeks after operation. Systemic arterial saturation is 86% in each child, and both children remain clinically well with no evidence of protein-losing enteropathy on normal diets and without specific medical therapy.

Background: After surgical removal of a volume load, regression of myocardial mass proceeds slowly relative to diminution in ventricular cavity size, resulting in increased wall thickness and decreased cavity dimensions, which may affect the filling properties and performance of the heart. We investigated the acute changes in ventricular geometry that occur after the Fontan operation and hemi-Fontan operation for tricuspid atresia, and compared them with closure of a ventricular septal defect in a two-ventricle heart., Methods: We reviewed the results of echocardiography performed before and 8 +/- 7 days after (1) Fontan operation for tricuspid atresia (n = 9), (2) hemi-Fontan operation for tricuspid atresia (n = 10), and (3) closure of a ventricular septal defect (n = 13). Measurements were made from images of the left ventricle at end-diastole: (1) apical, septal, and posterior wall thickness; and (2) long- and short-axis cavity diameters, cross-sectional areas, and ventricular volume. Posterior wall thickness to cavity dimension ratio was calculated., Results: Wall thickness increased in all groups, with the greatest degree of increase after the Fontan operation. Cavity measures decreased most dramatically after the Fontan operation, with less dramatic and equivalent changes noted after the hemi-Fontan operation and ventricular septal defect closure. Posterior wall thickness to cavity diameter ratios were equivalent in all before operation, increased after operation, and were greatest after the Fontan operation., Conclusions: Changes in ventricular geometry identified as an increase in wall thickness and a decrease in cavity dimension are most dramatic after the Fontan operation. Changes seen after the hemi-Fontan operation are of a milder degree, which may in part explain the excellent clinical course after this operation.

The mortality rate of the Fontan operation for heart malformations with a single or dominant ventricle has been reduced by dividing the procedure into two stages. The hemi-Fontan procedure allows early reduction of the volume work of the single ventricle and remodeling of ventricular geometry before a completion Fontan operation. Despite the improvement of survival with this strategy (8% mortality for completion Fontan versus 16% mortality for primary Fontan operation), morbidity related to serous effusions remains substantial. Further technical modifications have been undertaken in an effort to reduce morbidity and further reduce mortality. From January 1990 through June 1993, 200 patients underwent completion Fontan procedures after previous hemi-Fontan operations. Mean age was 23 months, and 157 patients were less than 24 months of age. Diagnoses were hypoplastic left heart syndrome (127 patients), tricuspid atresia (19 patients), single left ventricle (17 patients), complex double-outlet right ventricle (16 patients), pulmonary atresia with intact ventricular septum (8 patients), and other (13 patients). Overall, early mortality rate was 8% (16 patients). In the last 112 patients, the procedure was modified technically by creating one or more fenestrations in the baffle used to separate systemic venous blood from pulmonary venous blood (36 patients), or by excluding one or more hepatic veins from the systemic venous pathway (76 patients). Early mortality for these 112 patients was reduced to 4.5% (5 patients). Substantial morbidity from serous effusions occurred at a rate of 45% (35 of 78 patients) among survivors who had received neither technical modification.(ABSTRACT TRUNCATED AT 250 WORDS)