Fontan Approach Research Articles

Outcomes of bidirectional cavopulmonary shunt in patients younger than 4 months of age.

Bidirectional cavopulmonary shunt (BCPS) has played an important role in the staged Fontan approach; however, the timing remains controversial, especially in younger patients. Therefore, we examined the outcomes of BCPS in infants younger than 3 months of age. From 2004 to 2018, 120 patients underwent BCPS at <4 months of age (younger group). For reference, we also reviewed the data from 204 patients who had undergone the BCPS procedure during the same period at more than 4 months of age (older group). The median age and body weight at the time of the BCPS were 102 days and 4.2 kg for the younger group versus 196 days and 6.3 kg for the older group, respectively. Forty-eight patients (14.8%, 48 of 324; 16 in the younger group, 32 in the older group) had primary BCPS; the remaining 276 (104 in younger group, 172 in older group) had various forms of single-ventricle palliation before the BCPS procedure. Although preoperatively, 7 patients required extracorporeal membrane oxygenation (ECMO) support due to haemodynamic instability, they were successfully weaned from ECMO through haemodynamic benefits after BCPS. The 10-year actual survival rate (Kaplan-Meier) was 89% in the younger group and 86% in the older group (P = 0.55). Atrioventricular valve regurgitation (AVVR) was identified as a factor associated with hospital deaths in the younger group (P = 0.009), and much older age at BCPS was associated with late deaths in the older group (P = 0.027). In this study population, early performance of BCPS is applicable for patients who have undergone prior palliation and for those in whom primary BCPS is the first surgical intervention, even for patients with haemodynamic instabilities.

Results of Fontan operation in patients with congenitally corrected transposition of great arteries†.

The purpose of this study was to examine the outcome after the Fontan operation in patients with congenitally corrected transposition of great arteries with ventricular septal defect and pulmonary stenosis (ccTGA-VSD-PS). Patient- and procedure-related variables were analysed in 23 patients with ccTGA-VSD-PS operated between April 2003 and April 2015. The mean age was 14.07 ± 6.38 years (range 4-23, median 11 years), with 82% patients being male (19/23). Dextrocardia was present in 52% (12/23) of patients and left superior vena cava was present in 26% (6/23) of patients. Most patients underwent extracardiac Fontan (n = 18), whereas in 5 patients lateral tunnel Fontan was performed. All patients received polytetrafluoroethylene grafts of size 18-22 mm for extracardiac Fontan. In 8 patients, conduits were fenestrated to reduce the intraconduit pressure. The mean hospital stay was 15.7 ± 11.24 days (5-60, median 14 days). The most common cause for prolonged hospital stay was pleural effusion in 5 patients (21.7%). One 7-year old patient developed conduit thrombosis, intracranial bleed, seizures and died. The mean follow-up was 46.4.4 ± 32.2 months (range 8-142, median 42 months) and was available for 21 patients (91.3%). There was 1 mid-term non-cardiac death after 3 years of operation. Of the total, 85.7% (18/21) patients in follow-up are in NYHA class I, whereas 3 patients are in class II. The actuarial event-free survival rate was 81.8 ± 13.2% at 10 years. In ccTGA-VSD-PS patients with non-routable VSD and in those with difficult options for biventricular repair, the Fontan approach provides satisfactory mid-term palliation.

Early result of patients undergoing transcatheter completion of Fontan and Fenestrated extracardiac Fontan

Introduction Fontan surgery and its modifications are well-recognized palliation for univentricular types of heart lesions. Nowadays palliation can be achieved by combined surgical and transcatheter approaches, which offer good immediate and early results for the high-risk patients. Method Between September 2006 through January 2012, 18 patients underwent transcatheter completion of Fontan (gp 1), 28 patients underwent surgical fenestrated extracardiac Fontan (gp 2). GP 1: patients were ventelated and angiogram and hemodynamic assessment followed by opening of the SVC-RA junction and fdevice closure of the ASD II, some with use of covered stent. GP 2: extracardiac Fontan is done with Fenetration by single Surgeon. Result Mean age 4.8 year for (gp1), 3.5 year for (gp2) mean weight is 20 kg& 14 kg respectively, median median sat is 95% and 88% ( p 0.07) , median pulmonary artery pressure is 15 mmhg and 12 mmhg , hospital stay is 2 days and 13 days ( p 0.05) ,7 patients has pleural effusion and 7 has atrial tachy cardia from gp 2 none from gp 1, median follow up 4.8 year. Conclusion We conclude that Trans catheter completion of Fontan approach in selected patient is safe and considered an alternative approach.

Pulmonary artery growth in univentricular physiology patients

A Fontan-type operation, i.e. a connection of the systemic veins and pulmonary arteries without subpulmonary ventricle, with different surgical techniques, is nowadays the only treatment option for patients with a functionally univentricular heart (UVH). Understanding the development of pulmonary arteries in patients who are considered for the Fontan procedure is important clinically. To evaluate the development of pulmonary arteries in patients with univentricular circulation. Between 1995 and 2007, 111 patients underwent a bidirectional Glenn procedure. In all patients, preoperative catheterisation was performed to assess the anatomy and haemodynamics of UVH, especially the size of the pulmonary arteries. Ninety nine patients were included in the bidirectional Glenn group; 62 of these underwent repeat catheterisation before Fontan completion. The late results, after one stage extracardiac total cavopulmonary anastomosis performed in 24 patients between 1992 and 2002, were reinvestigated (one-stage Fontan group). We assessed the changes in the McGoon ratio and Nakata index for the whole cohort of patients. McGoon ratio is the sum of the diameter of pulmonary arteries divided by the diameter of the aorta. Nakata index is the sum of the cross-sectional area of the pulmonary arteries divided by the body surface area. During cardiac catheterisation prior to Glenn procedure, the mean Nakata index was 351.9 (range 131.2-886) mm2/m2 and was higher in patients with increased pulmonary flow (p = 0.0135). Mean McGoon ratio was 2.5 (range 1.1-4.9). An average 40.3 months after Glenn procedure, the Nakata index and McGoon ratio decreased significantly to 226.4 ± 125 mm²/m² (p < 0.003), and to 2.14 ± 0.58 (p < 0.008) respectively. In the group of patients after one-stage Fontan in late follow-up, mean 7.4 years after procedure, the Nakata index decreased from 318.7 ± 159.1 mm²/m² to 120 ± 40 mm²/m² (p < 0.0001) and McGoon ratio from 2.4 ± 0.6 to 1.4 ± 0.27 (p < 0.0001). Only size of pulmonary arteries before Glenn procedure, in the bidirectional Glenn group, or before Fontan operation, in the one-stage Fontan group,were inversely correlated with the changes of size of pulmonary arteries (p = 0.0015 and p = 0.0012). The relative decrease of the size of pulmonary arteries in the inter-stage period (between bidirectional Glenn anastomosis and Fontan completion) and after Fontan completion may indicate that pulmonary artery sizes should probably not bean absolute limiting factor in the decision on treatment of functionally UVH patients, especially at the stage of Fontan approach.

Is the Extracardiac Conduit the Preferred Fontan Approach for Patients With Univentricular Hearts?

With improvements in technology, operative technique, and critical care medicine, more and more children with congenital heart disease are surviving into adulthood. As they age, these patients are providing physicians with new challenges, and we are realizing the long-term consequences of our previous interventions. This oftentimes has changed the mode of treatment for subsequent children. Response by Khairy and Poirier on p 2515 Fontan palliation for single-ventricle patients was first described in 1971.1 The original approach was in the form of a classic atriopulmonary Fontan connection, bypassing the nonfunctional right ventricle. After recognizing the long-term complication of an atriopulmonary connection (right atrial dilation, arrhythmias, and thrombus formation), modifications have been made. The atriopulmonary connection has been abandoned in favor of modified Fontan anatomy and physiology. The modified Fontan can be performed by the use of an extracardiac conduit approach or a lateral tunnel approach. The extracardiac Fontan uses an external conduit to anastomose the inferior vena cava into the pulmonary arteries, whereas a lateral tunnel Fontan uses a baffle within the right atrium (Figure 1). Because the systemic venous pathway is completely separated from the right atrium with the extracardiac Fontan, it is more challenging for the electrophysiologist to access the heart for ablation. My aim is to demonstrate why “the extracardiac conduit is the preferred Fontan approach for patients with univentricular hearts.” Figure 1. Left , Lateral tunnel Fontan shows the Fontan pathway inside of the right atrium. Right , Extracardiac Fontan shows the conduit completely separated from the right atrium. The extracardiac Fontan has several advantages over the lateral tunnel Fontan. Minimizing cardiopulmonary bypass and ischemia may optimize early postoperative outcomes. In multiple studies, both prolonged cardioplegic arrest and extended cardiopulmonary bypass times have been associated with an increased risk of early postoperative death or Fontan failure.2– …

Functional Total Anomalous Pulmonary Venous Drainage by Left Atrium-to-Superior Vena Cava Shunt

Total anomalous pulmonary venous connection is occasionally associated with a functional single ventricle and is thought to be a risk for the staged Fontan approach. We report a patient with a very rare presentation of mitral atresia and intact atrial septum with physiologically total anomalous pulmonary venous drainage by a connecting vein between the left atrium and the superior vena cava. A surgical atrioseptectomy as a functional repair of total anomalous pulmonary venous connection and pulmonary artery banding was successfully performed as the first palliation for Fontan completion.

Regression of Marked Myocardial Sinusoids Associated With Hypoplastic Left Heart Syndrome During Staged Fontan Approach

The anatomic subtype of mitral stenosis, aortic atresia, and myocardial sinusoids is reported to be associated with increased mortality for staged repair for hypoplastic left heart syndrome. We herein report a very rare case of this subtype of hypoplastic left heart syndrome presenting with marked myocardial sinusoids in the left ventricle, which nearly disappeared after successful Fontan completion. Our successful experience suggests marked myocardial sinusoids per se are not a risk factor for surgical palliation in hypoplastic left heart syndrome.

Coagulation abnormalities and liver function after hemi-Fontan and Fontan procedures — the importance of hemodynamics in the early postoperative period☆

The causes of coagulation abnormalities and thromboembolic complications during staged Fontan approach in patients with single ventricle remain unclear. This study was designed to evaluate the coagulation profile in the early postoperative period after hemi-Fontan and Fontan procedures with relationship to liver function and hemodynamic variables. The prospective study on 43 patients after hemi-Fontan (group 1) and 37 patients after Fontan procedure (group 2) was carried out. Coagulation profile (factor VII, factor VIII, ATIII, fibrinogen, prothrombin), liver function (total serum protein, albumin, AST, ALT, bilirubin), and hemodynamic variables were assessed on postoperative day 1 and 5 and compared to preoperative measures. Factor VIII concentration was significantly higher on first postoperative day in both groups. On postoperative day 5 the concentration of factor VIII was significantly decreased in group 1 whereas constant in group 2. The concentration of factor VII, ATIII, fibrinogen, and prothrombin was significantly decreased on first and increased on fifth postoperative day after both hemi-Fontan and Fontan procedures. The increase in bilirubin concentration was more distinctive after Fontan operation (p=0.003) with lower AST in this group (p<0.0001). The single ventricle function, pO2 and central venous pressure had significant influence on factor VIII (p=0.034), factor VII (p=0.012), ATIII (p=0.006), and prothrombin (p=0.024) concentrations in group 2 with no significant influence in group 1. The distinctive causes of coagulation abnormalities during staged Fontan approach are hemodynamic changes and temporary liver dysfunction. Elevated concentration of factor VIII and significant influence of hemodynamics on coagulation profile could contribute to postoperative thromboembolic complications.

The role of the Fontan procedure in the surgical treatment of congenital heart malformations with double-outlet right ventricle.

The Fontan procedure in one of its many modifications is applicable to the surgical treatment of those patients with double-outlet right ventricle who are not amenable to bi-ventricular correction. Included in this group are patients with significant hypoplasia of one of the ventricles, patients with distant (non-committed) ventricular septal defect, patients with significant straddling of the atrioventricular valves, patients with abnormal atrioventricular connections (such as absence of one of the valves), or common atrioventricular valve connection. The presence of complex associated lesions such as those seen in the heterotaxia syndromes may, by virtue of the surgical risk of attempts at bi-ventricular repair, make the Fontan approach a suitable option for these patients. The presence of a subaortic conus and the potential for subaortic obstruction, a frequent findings in patients with double outlet right ventricle, plays an important role in the preparation and conduct of the Fontan operation. Copyright 2000 by W.B. Saunders Company

Commentary: Functionality after the fontan procedure—perceived reality and the pondering prince

Commentary: Functionality after the fontan procedure—perceived reality and the pondering prince

The Fontan Procedure for Pulmonary Atresia With Intact Ventricular Septum: Operative and Late Results

Objectives. The goals of the study were to evaluate the operative and late mortality associated with the Fontan procedure in patients with pulmonary atresia and an intact ventricular septum and to obtain follow-up information on the current clinical status of surviving patients.Background. Between 1979 and October 1, 1995, 40 patients with the anomaly had a nonfenestrated Fontan procedure performed at the Mayo Clinic. Because there are no previously published reports involving a series of this size in which the Fontan approach was used for this condition, a review of patient outcomes was thought to be of value.Methods. The medical records of the 40 patients were reviewed retrospectively, and 34 were determined to be alive. The status of the survivors as of late 1995 was then ascertained by direct examination, questionnaire or telephone follow-up.Results. There were three operative deaths and three late deaths. The current ages of the 34 survivors ranged from 4 to 30 years (median 13). Thirty-three of the 34 survivors were thought to be in New York Heart Association functional class I or II, and all but three of these patients, of school age or older, were either full-time students or working full time. The three adults who were not employed thought they were capable of working but were not doing so because of socioeconomic reasons. More than half of the patients were not receiving cardiovascular medications.Conclusions. These overall gratifying early and late results encourage continued application of this operation for appropriately selected patients with this complex congenital cardiovascular anomaly.(J Am Coll Cardiol 1997;29:1359–64)

The Fontan operation with a fenestrated patch—rationale and results

AbstractA fenestration in the atrial septum may be temporarily beneficial in high–risk patients undergoing the modified Fontan operation. The venous system is decompressed into the left atrial cavities resulting in an improvement in the systemic output at the expense of a moderate arterial desaturation due to a right–to–left shunt. Two groups of patients undergoing the modified Fontan operation were compared. Those treated with the classic operation (non–fenestrated Fontan group, n=56) had lower prevalence of preoperative risk factors for the Fontan operation than those undergoing the Fontan operation with a fenestrated patch (n=91). Nevertheless, early results of mortality, repair take–down and morbidity (duration of pleuropericardial effusions) were comparable or better in the fenestrated Fontan group. By regression analysis the presence of a fenestrated patch was the major determinant in the reduction in the amount and duration of postoperative effusion. Of the early 73 survivors in the fenestrated Fontan group, fifty tolerated the closure of the fenestration, which occurred either spontaneously (five cases) or was achieved by a clamshell device during cardiac catheterization. Eleven patients failed the first attempt at fenestration closure, but this was successful at the second attempt a few months later. Coil embolization of arterial collaterals to the lung circulation and balloon dilatation of pulmonary artery stenoses were required in some cases. Other patients following the Fontan operation with a fenestrated patch showed an improvement of their ventricular function which was initially impaired. This favorable experience demonstrates the usefulness of the “fenestrated Fontan approach” in patients who are suboptimal candidates for the classic operation, provided that the preoperative risk factors are potentially reversible.

Pediatric cardiac surgery.

The treatment of many forms of congenital heart disease has continued to advance, and numerous reports have appeared in the last year to document these improvements. Increased information regarding the "natural history" of repaired congenital heart disease has also been reported. Based both on improving results of surgical treatment and on the problems with delayed reparative operations, there has been continued interest in primary early repair of an increasing number of defects, including atrioventricular canal, tetralogy of Fallot, pulmonary atresia with intact ventricular septum, transposition of the great arteries, and truncus arteriosus. Advances have also been reported in transplantation for complex congenital heart disease and in the "physiologic correction" of various forms of single ventricle by the Fontan approach. A number of experimental reports on the protection of both the heart and the brain during ischemia point out areas where knowledge is incomplete and where further research is needed. These developments suggest that the overall outlook for the patient with congenital heart disease has continued to improve.

An Operation for the Hypoplastic Left Heart Syndrome: Preliminary Report

A successful first-stage operation for physiological correction of hypoplastic left heart syndrome is reported. This consists of creating a proximal communication between the pulmonary artery and the aorta while perfusing the lungs through a 3 mm orifice in a flap of pulmonary artery wall. The flap is turned in to partition the main pulmonary artery. We believe this operation has more potential for subsequent correction using the Fontan approach than previously reported first-stage procedures. Thus, further trial is indicated in selected patients.

Fontan procedure for tricuspid atresia.

A Fontan procedure has been performed on 29 patients for tricuspid valvular atresia. The age range was 8 months to 33 years (median 10 years), the pulmonary vascular resistance ranged from 1.8-6.1 units . m2 (mean 3.3 units . m2), and the mean pulmonary arterial pressure ranged from 13-45 mm Hg (mean 21 mm Hg). Twenty-nine previous operations had been performed in 23 patients. Fourteen other associated cardiopulmonary anomalies were present in 12 patients. There were four hospital deaths (13.8%), all in patients with complicating features. Among the last 22 consecutive patients who have undergone operation, one died (4.5%). Complete atrioventricular block necessitated pacemaker implantation in one patient. No late deaths occurred. Of the 19 patients followed 3 months or more from the time of operation, eight have no restriction of exercise capacity, nine have only mild restriction, and two have a poor result. The Fontan approach to tricuspid atresia has several theoretical advantages over previously used shunts or pulmonary artery banding, the operative mortality in patients who have suitable anatomy and hemodynamics is low, and the results have been good.