Long-term results of the fenestrated fontan operation

The fenestrated Fontan operation was introduced as a modification of the “completed” Fontan operation for patients with high risk factors, and low operative mortality has frequently been reported. However, use of the umbrella device is now restricted, and this procedure should be performed without subsequent closure. In this paper, we review our clinical experience with this procedure and discuss ongoing problems. Sixteen patients (4 tricuspid atresia and 12 other cardiac anomalies including 5 cases of univentricular heart) underwent the fenestrated Fontan operation (7 atriopulmonary and 9 total cavopulmonary connection). All of them have some risk factors for a completed Fontan operation. There were three early deaths of the 16. Two experienced an anticipated thromboembolic accident, one of which involved the pulmonary aspect while the other involved the arterial aspect. Patients who survived the operation have progressed well and have a clinical status of New York Heart Association class I, with the exception of one late death due to congestive heart failure. There have been no thromboembolic accidents in this group during the late follow-up period. Spontaneous closures of the fenestrations were noted in two patients. The late mean Qp/Qs value in patients with patent fenestrations was 0.80 ± 0.1, SaO₂ was 88.8 ± 5.6%, and right atrial pressure was 9.7 ± 3.8 mmHg. No major problems have been encountered in patients with a patent fenestration over extended periods. A modified Fontan operation to fit a permanently open fenestration may be considered as a final surgical option for certain high-risk patients.     

Evolution of the Fontan procedure in a single center

Background. Surgical approaches to single ventricle variants include staged, fenestrated, and completed Fontan operations. This study compares outcomes with these modifications of the Fontan operation at a single center.

Methods. Preoperative risk factors and operative results were analyzed by multivariate techniques in 129 patients undergoing modified Fontan operations since March 1988.

Results. Overall early and late mortality was 5.4% and 0.8%, respectively. Before 1993, completed Fontan operation using right atrial to pulmonary artery anastomosis without fenestration was performed in the majority of patients (44 of 58; 76%). During this period, 10 of 17 patients at high risk had completed Fontan with three takedowns. In 1994, the staged hemi-Fontan and modified Fontan with a lateral tunnel anastomosis and with or without small fenestration (2.5 to 4 mm) were introduced. The majority of patients at high risk during this period underwent hemi-Fontan followed by fenestrated Fontan with no takedowns. Late atrial dysrhythmias occurred in 6 patients (4.7%), generally with larger fenestrations or right atrial to pulmonary anastomoses. Three patients (2.3%) had a stroke, 2 with large (≥ 4 mm) fenestrations. Of 38 fenestrations, 32 (84%) closed spontaneously by 1 year. No protein-losing enteropathy occurred. Most patients (118 of 121) were in New York Heart Association class I/II 4.5 years postoperatively. By multivariate analysis, only Down’s syndrome (p < 0.001) predicted early mortality, whereas both Down’s syndrome and a systemic right ventricle decreased late survival (p < 0.006).

Conclusions. Proper selection of patients for modifications of the Fontan procedure resulted in excellent early and late survival with a low incidence of atrial dysrhythmia and stroke. Midterm functional outcomes were excellent.     

Clipped tube fenestration after extracardiac Fontan allows for simple transcatheter coil occlusion

BACKGROUND: Expensive devices are increasingly used to close a patent fenestration after a modified Fontan operation. We report our 5-year institutional experience of clipped tube fenestration after extracardiac Fontan operation, which allows for simple transcatheter coil occlusion. METHODS: We retrospectively reviewed 30 children, median age of 4.0 years (range, 2.4 to 8.8 years) who underwent extracardiac Fontan operation between May 1996 and May 2001, and were fenestrated using a 4- to 8-mm diameter clipped tube graft. RESULTS: Ten children had a patent fenestration occluded by transcatheter placement of 15 detachable coils (5- to 8-mm diameter). Aortic oxygen saturations increased on average by 5.5% (2% to 14%) and mean pressures in the Fontan circuit by 2.5 mm Hg (0 to 3 mm Hg). Four had immediate complete occlusion angiographically and 6 had trivial residual shunt, but complete occlusion by echocardiography at follow-up. There have been no immediate complications, late coil embolizations, thromboembolic events, or documented hemolysis within a follow-up after coil implantation of 1.7 years (0.4 to 4.5 years). Spontaneous fenestration closure was documented in 8 patients at cardiac catheterization and 9 patients by echocardiography with consistent improvement in resting transcutaneous oxygen saturation. Two children with a patent fenestration have been considered inappropriate for closure, and there was one early surgical death. There have been no complications related to the tube fenestration modification within a follow-up postoperation of 2.6 years (0.1 to 5.5 years). CONCLUSIONS: Clipped tube fenestration after extracardiac Fontan operation is a useful surgical modification that allows for simple transcatheter coil occlusion.     

Intermediate procedures after first-stage Norwood operation facilitate subsequent repair

Actuarial analysis of survival after first-stage palliative reconstructive operation for hypoplastic left heart syndrome has revealed a high out-of-hospital attrition rate over the first 18 months to 2 years postoperatively. Some of this mortality is related to development of anatomical problems such as restrictive atrial septal defect, neoaortic arch obstruction, and pulmonary artery distortion. The bidirectional Glenn shunt has proved to be an ideal adjunctive procedure for high-risk patients at the time of operation to correct such intermediate-term problems. The fenestrated Fontan procedure, which involves fenestration of the interatrial baffle placed as part of our current standard Fontan procedure, is applied for patients considered to be at moderate risk for a Fontan procedure. The decision regarding closure of the fenestration is made by hemodynamic study including temporary balloon occlusion of the fenestration. The fenestration is closed with the double-clamshell device, which is placed percutaneously in the catheterization laboratory and which is currently used for secundum atrial septal defect closure. Appropriate selection of patients for the bidirectional Glenn shunt or fenestrated Fontan procedure with or without fenestration closure has resulted in a dramatic decrease in mortality and morbidity for patients with all forms of single ventricle and for patients with hypoplastic left heart syndrome.     

The Fontan-operation: from intra- to extracardiac procedure

PURPOSE: For treatment of univentricular heart, the Fontan operation has been established as the definitive palliation. The current controversy is mainly based on the high incidence of arrhythmias after an intra-atrial lateral tunnel Fontan operation. METHODS: From January 1995 until April 2002, 46 children underwent a Fontan-type operation with or without a small fenestration. In 33 patients (group I) an intracardiac tunnel and in 13 patients (group II) an extracardiac conduit procedure was performed. PRINCIPAL FINDINGS: There was no perioperative mortality. All patients showed postoperative a significant increase of arterial oxygen saturation, from 76 to 86% after surgery with fenestration, or to 90.5% without fenestration respectively. In patients with fenestration procedure, the saturation rose to 90% after closure of fenestrations 9 to 12 months after operation. CONCLUSIONS: Modified Fontan operations can be performed in normothermia on the beating heart with acceptable mortality. The extracardiac conduit Fontan procedure has the benefits of less surgical injury and a higher intraoperative flexibility.     

Clinical outcome of patients 20 years after Fontan operation — effect of fenestration on late morbidity

Objective: The Fontan operation has been proposed as definitive palliation for an increasing variety of hearts with complex univentricular anatomy, but late morbidity after Fontan operation is still a matter of concern. This retrospective study evaluates the late outcome in patients with Fontan circulation. Methods: We included 121 consecutive patients that underwent Fontan operation between 1984 and 2004. Modifications of the Fontan operation included atriopulmonary anastomosis (APA; n = 28), total cavopulmonary connection (TCPC; n = 63), and fenestrated TCPC (f-TCPC; n = 30). Mean age was 5.8 ± 5.5 years. Post operative mortality, morbidity, hemodynamics, and somatic development were analyzed. Results: Actuarial survival was 87% at 20 years after Fontan operation. There were 10 early deaths, 5 late deaths, and 2 takedowns followed by successful conversion and heart transplantation. Among 108 early-survivors with Fontan circulation, 19 underwent reoperation, including 3 conversions of APA to TCPC. Freedom from reoperation was 76% at 20 years. Freedom from intervention was 34% at 20 years. Freedom from tachyarrhythmia or pacemaker implantation was 23% and 77%, respectively at 20 years. Heterotaxy and atrioventricular valve anomaly were risk factors for late failure and tachyarrhythmias. Patients with fenestrated TCPC had reduced incidence of late tachyarrhythmias, and patients with APA who developed collaterals showed low incidence of late tachyarrythmia. Postoperative sinus node dysfunction or tachyarrhythmias was associated with significantly lower cardiac index. Somatic development was gradually compensated after Fontan operation. Weight normalized completely 15 years postoperatively. Conclusions: Long-term survival after Fontan procedure is encouraging, but late morbidity remains suboptimal. During follow-up, emerging complications should be managed by surgical and interventional procedures. Fenestration in Fontan circulation provided better cardiac output and lower incidence of late tachyarrhythmias, suggesting a benefit of fenestration for late outcome.     

Parameters associated with perioperative baffle fenestration closure in the Fontan operation

OBJECTIVE: To identify clinical parameters indicating perioperative fenestration closure in children who underwent the fenestrated Fontan operation. DESIGN: Retrospective. SETTING: Single children's hospital. PARTICIPANTS: Patients who underwent a fenestrated Fontan operation in 1996 through 1997 (n = 101). INTERVENTION: A fenestrated Fontan operation was performed in children with single-ventricle physiology. MEASUREMENTS AND MAIN RESULTS: Early perioperative closure of the fenestration occurred in 14 patients (group 1), whereas the fenestration remained patent in 87 patients (group 2). The groups were compared by the following parameters: demographics, cardiac catheterization and ultrasound data, and use of aspirin or warfarin preoperatively and intraoperatively by assessing the composition of the cardiopulmonary bypass solution, use of ultrafiltration and antifibrinolytics, protamine dose, last hematocrit on cardiopulmonary bypass, and requirement of blood products. Immediately postoperatively in the intensive care unit (ICU), cardiac filling pressures (central venous and left atrial pressure), coagulation profile, cardiac rhythm, chest tube drainage, length of stay in the ICU, and use of atrial pacing were reviewed. Significant indicators of early fenestration closure in this study as determined by multivariate stepwise logistic regression were a high transpulmonary pressure gradient (p = 0.015) and a higher oxygen saturation (p = 0.001) 1 hour after arrival in the ICU, a low fibrinogen level (p < 0.0001), and the need for temporary atrial pacing (p = 0.029). The fenestration was reopened in 13 patients in group 1. In 101 patients, there was no early mortality, and all patients survived to discharge. CONCLUSION: Factors that correlated with postoperative fenestration closure in the fenestrated Fontan operation in this study were a high transpulmonary pressure gradient and a high oxygen saturation 1 hour after arrival in the ICU, a low fibrinogen level, and the need for temporary atrial pacing.     

Factors influencing early and late outcome following the Fontan procedure in the current era. The 'Two Commandments'?

OBJECTIVE: This study was undertaken to identify the factors affecting early and late outcome following the Fontan procedure in the current era. We have examined whether conventional selection criteria, the 'Ten Commandments', are still applicable in the current era. MATERIALS AND METHODS: Between January 1988 and July 2004, 406 patients underwent a modified Fontan procedure at a median age of 4.7 years (IQR, 3.8-7.1 years). The single functional ventricle was of left (n=241, 59%) or right ventricular morphology (n=163, 40%). The modified Fontan procedure was performed using an atriopulmonary connection (n=162, 40%) or total cavopulmonary connection (TCPC) involving a lateral atrial tunnel (n=50, 12%) or extracardiac conduit (n=194, 48%). They were fenestrated in 216 patients (53%). RESULTS: The early mortality was 4.4% (n=18) and four other patients required takedown of the Fontan circulation. On multivariable analysis, early outcome was adversely influenced by two factors (p<0.05): preoperative impaired ventricular function and elevated pulmonary artery pressures. Two risk models were constructed for early outcome based on preoperative and predictable operative variables (Model 1) and all preoperative and operative data (Model 2). Both models were calibrated across all deciles (p=0.83, p=0.25) and discriminated well. The area under the ROC curve was 0.85 and 0.89, respectively. There were 21 late deaths, 1 patient required late takedown of the Fontan circulation and 3 required orthotopic cardiac transplantation. Actuarial survival was 90+/-2%, 86+/-2% and 82+/-3% at 5, 10 and 15 years, respectively. Multivariable analysis identified that outcome was influenced by preoperatively impaired ventricular function, elevated preoperative pulmonary artery pressures and an earlier year of operation. The freedom from reintervention was 83+/-4%, 76+/-4% and 74+/-8% at 5, 10 and 15 years, respectively. Additional risk factors for reintervention were right atrial isomerism and preoperative small pulmonary artery size. CONCLUSIONS: Late outcome of the Fontan circulation is encouraging. Ventricular morphology, surgical technique and fenestration do not appear to influence early or late outcome. Preoperatively impaired ventricular function and elevated pulmonary artery pressures have an adverse influence on both early and late outcome. Reintervention is common, with small preoperative pulmonary artery size being an additional risk factor.     

Fenestrated Fontan operation with delayed transcatheter closure of atrial septal defect. Improved results in high-risk patients.

Ten patients, each with two or more risk factors for morbidity and death, underwent a fenestrated Fontan procedure in which a 4 to 6 mm circular fenestration was left between the systemic and pulmonary venous chambers. None died; a similar group of high-risk patients without fenestration had a mortality rate of 2 of 8. Patients with fenestration had significantly less drainage from the chest tube, less need for inotropic support, and shorter intensive care and hospital stays than did patients without fenestration. Comparison with a group of low-risk patients undergoing the Fontan operation showed no statistical difference in these postoperative parameters. Fenestrations were closed in all 10 patients at from 9 days to 6 months after operation by means of the transcatheter clamshell occluder device. Two patients had left pulmonary artery balloon angioplasty and three patients had other atrial communications closed with additional clamshell devices. During short-term follow-up periods averaging 18 months, all patients were clinically well; however, one patient with mitral atresia required reoperation for obstruction between the left atrium and the tricuspid valve, not related to the clamshell device. These data indicate that fenestration may be one method of achieving lower morbidity and mortality rates among high-risk patients undergoing the Fontan procedure.     

Modified technique creating fenestration in the extracardiac Fontan completion; kissing anastomosis

Acute or subacute occlusion of a fenestration between the Fontan chamber and the atrium causes low cardiac output status, sustained pleural effusion, and protein losing enteropathy in critical cases. To achieve reliable and long patency of the fenestration in extracardiac Fontan operations, we modified our technique for creating a fenestration, namely "kissing" anastomosis, in which the atrial incision is directly sutured to the surface of the extracardiac conduit leaving a few millimeters outside from the edge of the punched-out hole (fenestration). We applied this modification in 2 cases. The diameter of the hole is 4 mm (case 1) and 3 mm (case 2). The patients were anticoagulated by warfarin potassium after the surgery. The fenestrations were patent at 3- (case 1) and 1-month (case 2) after the surgery. Peripheral oxygen saturations at discharge were 86 (case 1) and 88% (case 2). There was no death or major complication. The described procedure is an effective alternative to maintain patency and function of the fenestration in extracardiac Fontan operations.     

As originally published in 1996. Usefulness of pulsatile bidirectional cavopulmonary shunt in high-risk Fontan patients. Updated in 2002

BACKGROUND: A bidirectional cavopulmonary shunt has been performed for the high-risk Fontan patient. It is well known that in the presence of the bidirectional cavopulmonary shunt alone to secure pulmonary blood flow, the central pulmonary artery size decreases over time. We have performed pulsatile bidirectional cavopulmonary shunt (PBCPS), keeping pulmonary blood flow from the ventricle through the stenotic pulmonary valve, or a Blalock-Taussig shunt in patients who do not meet the criteria for the Fontan operation. METHODS: Eleven patients who underwent PBCPS between 1989 and 1993 were reviewed. We compared the results of cardiac catheterization immediately before PBCPS and during the postoperative observation period (310 +/- 257 days). RESULTS: Pulmonary blood flow and arterial oxygen saturation increased significantly after PBCPS (p = 0.01). Pumonary artery area index showed a tendency to increase (p = 0.11). The mean number of risk factors for the Fontan procedure decreased significantly from 1.8 +/- 1.1 to 0.7 +/- 0.8 after PBCPS (p < 0.05). Overall, 5 of the 11 patients (45.5%) met the criteria for the Fontan procedure, and a fenestrated Fontan procedure was carried out in 4 of them. CONCLUSIONS: The PBCPS is useful for high-risk Fontan patients not only in the staged Fontan operation, but also as definitive palliation.     

Fontan procedure for asplenia syndrome

BACKGROUND: Historically the Fontan operation in patients with single ventricle asplenia syndrome has been associated with high mortality. We studied whether recent modifications of the surgical technique have improved outcome. METHODS: A retrospective review of 11 patients with asplenia syndrome who underwent a Fontan operation between 1996 and 2002 was performed. RESULTS: Anomalies of pulmonary venous return included 5 patients (46%) and 4 patients with an isolated hepatic venous return from inferior vena cava (36%). The type of Fontan procedure included 6 patients with an extracardiac tube-graft, 3 with a lateral tunnel modification, 1 with an intra-atrial tube graft, and 1 with an intra-extra atrial tube graft. A fenestration was placed in 3 patients (27%). There were no early deaths and 2 late hospital deaths (18%) due to tachyarrythmia and failed Fontan circulation. There were no late deaths and no complications during follow-up periods (mean 3 years). Of 21 patients diagnosed with asplenia syndrome between 1990 and 2000, overall survival was 57% and 12 patients underwent a Fontan-type operation. CONCLUSIONS: The Fontan operation can now be performed in patients with asplenia syndrome with good survival. Fontan staging, appropriate choice of Fontan modification, aggressive treatment of concomitant malformations, and use of a fenestration contribute to improved outcome.     

Extracardiac Fontan operation without cardiopulmonary bypass

AIM: The avoidance of cardiopulmonary bypass and aortic cross-clamping in patients possessing single ventricular physiology has potential advantages including preservation of ventricular and pulmonary functions; early extubation, decreased incidence of pleural effusions and decreased requirement of inotropic agents and blood products. In this study, we assessed the postoperative outcome of patients who have undergone extracardiac Fontan operation performed without cardiopulmonary bypass. METHODS: Between March 1999 and August 2002, 10 consecutive patients (6 males and 4 females) underwent extracardiac Fontan operation without cardiopulmonary bypass. The age of patients ranged between 1.5 to 12 (5.2+/-3.1) years. All the patients requiring any intracardiac intervention were excluded from the study. Previous operations of the patients were modified Blalock-Taussig shunt procedure in 3 patients, bidirectional cavopulmonary shunt operation in 2 patients and pulmonary arterial banding in 1 patient. All operations were performed without cardiopulmonary bypass. Bidirectional cavopulmonary anastomosis was performed by using a transient external shunt constructed between the superior vena cava and right atrium. An appropriate sized tube graft was anastomosed to the inferior surface of right pulmonary artery. Finally, inferior vena cava to tube grafts anastomosis was performed with the aid of another external shunt constructed between inferior vena cava and right atrium. During the procedure central venous pressure, blood pressure and arterial oxygen saturation levels were continuously monitored and recorded. RESULTS: The mean intraoperative Fontan pressure was 16.1+/-2.75 mmHg. Intraoperative fenestration was required in 4 patients with a Fontan pressure above 18 mmHg. There were no intra and postoperative deaths. Three patients required mild doses of inotropic support during the postoperative period. All patients were weaned off mechanical ventilation within 24 h. The mean arterial oxygen saturation raised from 74.5+/-4.2% to 93.5+/-2%. Arterial oxygen saturation was 95+/-0.6% in 6 patients without fenestration and 91.2+/-0.5% in 4 patients with fenestration (P=0.001). All patients were in sinus rhythm postoperatively. Only 2 patients required blood transfusion. Two patients suffered from prolonged pleural effusion (more than 7 days). The mean intensive care unit and hospital stay periods were 3.3+/-1.5 and 15.4+/-5.3 days, respectively. CONCLUSIONS: The extracardiac Fontan operation performed without cardiopulmonary bypass provides good results in short and midterm follow-up periods with improved postoperative hemodynamics.     

Total cavopulmonary connections in children with a previous Norwood procedure

BACKGROUND: Outcomes of the Fontan operation in children initially palliated with the modified Norwood procedure are incompletely defined. METHODS: From August 1993 to January 2000, 45 patients (mean age 2.6 +/- 1.1 years, weight 12.7 +/- 2.8 kg) who were palliated with staged Norwood procedures (hypoplastic left heart syndrome, n = 32; nonhypoplastic left heart syndrome, n = 13) underwent a modified Fontan operation. Preoperative features included moderate/severe atrioventricular valve regurgitation (n = 5, 11%), reduced ventricular function on echocardiography in 11 patients, McGoon index 1.56 +/- 0.38, and pulmonary artery distortion in 18 patients (40%). RESULTS: A lateral tunnel (n = 16) or an extracardiac conduit (n = 29) connection with fenestration in 38 patients (84%) was used. Concomitant procedures included pulmonary artery reconstruction (n = 24, 53%), atrioventricular valve repair (n = 4, 9%) or replacement (n = 1). Before Fontan, 12 patients (27%) had an intervention to address neoaortic obstruction, and 7 patients required balloon dilation/stenting of the left (n = 5) or right pulmonary artery (n = 5). Intraoperatively, left (n = 5) or right pulmonary artery (n = 1) stenting was performed in 5 patients (11%). On follow-up, 8 patients required additional interventional procedures to address left pulmonary artery narrowing (n = 5), or venous (n = 5) or arteriopulmonary collaterals (n = 1). Perioperative mortality was 4.4% (n = 2). There were 2 late deaths at a mean follow-up of 39 +/- 20 months. CONCLUSIONS: In relatively high-risk patients, midterm results of the Fontan operation for children initially palliated with the Norwood procedure were good. Combined interventional-surgical treatment algorithms can lead to improved outcomes.     

Staged reconstruction for hypoplastic left heart syndrome. Contemporary results.

OBJECTIVE: The authors review their experience with staged reconstructive surgery for hypoplastic left heart syndrome (HLHS) and assess current outcome for this condition. SUMMARY BACKGROUND DATA: Once considered a uniformly fatal condition, the outlook for newborns with HLHS has been altered dramatically with staged reconstructive procedures. Refinements in operative technique and perioperative management have been largely responsible for this improved outlook. METHODS: The authors reviewed their experience with 158 consecutive patients undergoing stage 1 reconstruction with a Norwood procedure from January 1990 to August 1995. All patients had classic HLHS, defined as a right ventricular dependent circulation in association with atresia or severe hypoplasia of the aortic valve. RESULTS: There were 120 hospital survivors. Among the 127 patients considered at standard risk, survival was significantly higher than that for the 31 patients with important risk factors. Adverse survival was associated most strongly with significant associated noncardiac congenital conditions and severe preoperative obstruction to pulmonary venous return. Second-stage reconstruction with the hemi-Fontan procedure was performed in 106 patients, with 103 hospital survivors and one late death. Three of the late survivors were not considered candidates for the Fontan procedure. To date, the Fontan procedure has been completed in 62 patients, with 53 survivors. Deaths after the Fontan procedure occurred early in our experience and were mostly secondary to left pulmonary artery stenosis or hypoplasia. Significant or potentially significant morbid conditions were noted in 25 of the 120 hospital survivors. Neurologic conditions were found in 6% and cardiovascular conditions in 10%, including dysrhythmia, left pulmonary artery thrombosis, and chronic pleural effusions. Among the patients considered at standard risk with typical anatomy, actuarial survival was 69 +/- 8% at 5 years. Survival was 71 +/- 17% at 5 years for standard risk patients with variant anatomy and 58 +/- 9% for the entire cohort of 158 patients. The largest decrease in survival occurred in the first month of life and late deaths primarily affected those patients in the high-risk group. CONCLUSIONS: Staged reconstruction has significantly improved the intermediate-term outlook for patients with HLHS. Factors addressing improvements in early (< 1 month) first-stage survival would be expected to add significantly to an overall improved late outcome. Currently employed refinements in operative technique are associated with eliminating or reducing pulmonary artery distortion and dysrhythmia.     

Omission of a prior Glenn anastomosis is a risk factor for prolonged pleural drainage after the fenestrated extracardiac conduit Fontan procedure

Abstract Objective: Factors related to prolonged pleural drainage after the Fontan operation have not been clearly defined. We investigated perioperative variables to establish factors predicting operative morbidity including prolonged chest tube drainage. Also, we pursued the fate of the fenestration during the follow‐up period. Methods: We retrospectively reviewed 52 patients who had undergone a fenestrated extracardiac Fontan procedure between August 1998 and June 2008. The median age at the time of surgery was 34.8 (range: 18.5 ～ 156) months and the median body weight 13.2 kg (range: 9.5 ～ 33). A multivariable logistic regression model was used to compare demographic, anatomic, and physiological variables for postoperative morbidity. Results: Operative mortality occurred in one patient (1.9%). The mean duration of respiratory support, chest tube drainage, and hospital stay was 13 hours (range: 4 to 328 hours), six days (range: 2 to 45 days), and 16 days (range: 7 to 444 days), respectively. Statistically, an operation without previous bidirectional cavopulmonary shunt (OR 30, 95% CI 3.1 to 289) was the only independent risk factor for prolonged pleural drainage. Aortic cross‐clamp time was identified as a risk factor for prolonged mechanical ventilatory support. During a median follow‐up at 62 months (range: 17 to 137 months), there was one late death (1.9%). Twenty‐two patients (43%) underwent intervention for fenestration closure. Conclusions: Previous bidirectional cavopulmonary shunt and shortened aortic cross‐clamp time may reduce postoperative morbidity including prolonged chest tube drainage and mechanical ventilator support after the fenestrated extracardiac conduit Fontan procedure. (J Card Surg 2011;26:509‐514)     

Results of an extracardiac pericardial-flap lateral tunnel Fontan operation.

Objective: Extracardiac pericardial-flap lateral tunnel Fontan operation has the theoretical advantage of growth potentiality of the extracardiac tunnels. The mid-term results of this technique and morphologic change of the lateral tunnel were studied. Methods: Clinical data were reviewed in 42 patients who underwent extracardiac pericardial-flap lateral tunnel Fontan operation between November 1993 and December 2004. The age was 2.8+/-1.5 years and the body weight was 12.3+/-3.2kg. Extracardiac tunnel was constructed using the pedicled pericardium with the base undetached. By reviewing the follow-up cardiac angiograms (2.3+/-1.4 years postoperatively), ratios of diameter and cross-sectional area of the lateral tunnel to those of inferior vena cava were obtained. Results: There were 4 surgical mortalities (10%). Postoperative morbidity included prolonged pleural effusion in 5 patients and heart block in 1 patient. Follow-up was possible in 37 patients and the follow up duration was 3.8+/-2.2 years. There were two late deaths due to ventricular dysfunction and sudden death of unknown causes. Two patients required reoperation due to subaortic stenosis and stenosis between inferior vena cava and lateral tunnel. In one patient, bradyarrhythmia was observed but there was no thromboembolic complication. Follow-up anteroposterior and lateral diameter ratio were 1.1+/-0.5 and 1.2+/-0.5. The cross-sectional area ratio was 2.6+/-2.3. In 5 patients, fusiform dilatation of the lateral tunnel was observed, but in the remaining patients, the lateral tunnel preserved tubular morphology with good hemodynamics. Conclusions: Extracardiac pericardial-flap lateral tunnel Fontan operation is relatively simple and feasible even in patients with previous median sternotomies. The mid-term results were acceptable, and the lateral tunnel demonstrated a tendency to preserve its tubular shape. However in some patients, dilatation of the pericardial-flap tunnel was observed during follow up. Longer follow-up is required to determine the morphologic changes of the lateral tunnel and the value of this technique.     

Diaphragmatic fenestration for resistant pleural effusions after univentricular repair

A 12-year-old child with chronic pleural effusions for a month and a half after a fenestrated Fontan operation underwent bilateral diaphragmatic fenestrations with complete relief. We suggest this approach as an alternative treatment for chronic pleural effusions that may ensue after total cavopulmonary connection.     

Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a risk factor?

OBJECTIVE: This study was undertaken to evaluate factors contributing to a decrease in early mortality and morbidity after the Fontan procedure between January 1, 1992, and December 31, 1999. METHODS: Outcomes evaluated were early survival, duration of pleural effusions, and duration of hospitalization. Potential predictors evaluated included anatomic diagnosis, presence of a common atrioventricular valve, preoperative pulmonary artery pressure, type of Fontan operation, type of intentional right-to-left shunt or baffle fenestration, and use of modified ultrafiltration. RESULTS: The modified Fontan procedure was performed in 332 patients at a median age of 22 months (range, 11-380 months) and a median weight of 11 kg (range, 5.8-120 kg). Prior stage I reconstructive surgery for classic or variant hypoplastic left heart syndrome had been performed in 205 (53%) of 332 patients, and 318 (96%) had undergone an interim superior cavopulmonary connection. A lateral-tunnel Fontan operation was performed in 281 patients, and an extracardiac conduit Fontan operation was performed in 51 patients. An intentional right-to-left shunt was created in 298 (90%) patients. Between 1992 and 1999, the outcome after the modified Fontan operation improved significantly. Overall mortality was 6.6% (22/332), with only 2 deaths since 1994. Morbidity was also reduced, with a decreased duration of pleural effusions and decreased hospital stay. In a multivariable analysis of the entire cohort, only the presence of a common atrioventricular valve (odds ratio, 7.64; 95% confidence limits, 2.07-28.14; P =.0002) and increased preoperative pulmonary artery pressure (odds ratio, 1.46/1 mm Hg increase; 95% confidence limits, 1.2-1.78; P <.001) increased the risk of early death, whereas use of a single-punch fenestration in a lateral-tunnel Fontan (odds ratio, 0.06; 95% confidence limits, 0.01-0.65; P =.02) and use of modified ultrafiltration (odds ratio, 0.14; 95% confidence limits, 0.03-0.72; P =.019) decreased the risk of death. The risk of prolonged pleural effusions (>3 days) was increased in patients with hypoplastic left heart syndrome (odds ratio, 1.73; 95% confidence limits, 1.07-2.81; P =.03) and was decreased by use of a single-punch fenestration in a lateral-tunnel Fontan operation (odds ratio, 0.17; 95% confidence limits, 0.07-0.4; P <.001), as well as by the use of modified ultrafiltration (odds ratio, 0.25; 95% confidence limits, 0.15-0.40; P <.01). CONCLUSIONS: In a contemporary series of Fontan operations performed largely in patients with hypoplastic left heart syndrome or variants, systemic ventricle morphology had no effect on mortality. Some patient characteristics, however, continue to influence outcome. The decrease in mortality and morbidity in the current era is attributed to changes in management strategies, specifically the use of modified ultrafiltration and baffle fenestration.     

Improving results of the modified Fontan operation in patients with heterotaxy syndrome

BACKGROUND: Historically the Fontan operation in patients with single ventricle heterotaxy syndrome and atrial isomerism has been associated with high mortality. We studied whether recent modifications of the surgical technique have improved outcome. METHODS: A retrospective review of 135 patients with heterotaxy syndrome who underwent a Fontan operation between 1981 and 2000 was performed. RESULTS: There were 93 patients with right isomerism and 42 with left isomerism. Anomalies of venous return included 25 patients with extracardiac pulmonary venous connection (19%) and 37 patients with an interrupted inferior vena cava (27%). Thirty-six patients (27%) had at least moderate atrioventricular valve regurgitation. The type of Fontan procedure included 17 patients with an atriopulmonary Fontan connection, 67 with a lateral tunnel modification, 19 with an intraatrial tube graft, 25 with an extracardiac tubegraft, and 7 with an intra-extra atrial tube graft. A fenestration was placed in 93 patients (78%). Early mortality was 19% before 1991, 3% since 1991, and no patient has died early since 1993. Ten-year survivals were 70% for Fontan operations before 1990 and 93% for Fontan operations after 1990. Thirty-two patients (23%) had prolonged pleural effusions. Risk factors for death included anomalous pulmonary venous connection (p = 0.02) and higher preoperative pulmonary vascular resistance (p = 0.002). Sixty-two patients (47%) had some form of early postoperative arrhythmia. At 10 years, freedom from late bradyarrhythmia and late tachyarrhythmia were 78% and 70%, respectively. Preoperative arrhythmias, older age at operation, and anatomic features were each independent predictors of late arrhythmia. CONCLUSIONS: The Fontan operation can now be performed in patients with heterotaxy syndrome with excellent survival. However, morbidity in terms of postoperative arrhythmias and prolonged pleural effusions remains significant. Fontan staging, appropriate choice of Fontan modification, aggressive treatment of concomitant malformations, and use of a baffle fenestration contribute to improved outcome.