Underwent Fontan Completion Research Articles

Does the off-pump Fontan procedure ameliorate the volume and duration of pleural and peritoneal effusions?☆

We initiated an off-pump Fontan procedure by using temporary bypass from the inferior vena cava to the atrium and advanced the procedure in selected patients by simply cross-clamping the inferior vena cava. We aimed to investigate whether the off-pump Fontan procedure could ameliorate the volume and duration of pleural and peritoneal effusion. We retrospectively reviewed 74 patients (aged <4 years) who underwent Fontan completion between January 2001 and December 2006. The patients were classified into the following two groups: a cardiopulmonary bypass group in which cardiopulmonary bypass was required (n=27) and an off-pump group in which the procedure was completed without the use of cardiopulmonary bypass (n=47). A propensity score was used to control the treatment selection bias for the use of cardiopulmonary bypass. Fourteen patients from each group were successfully matched. Both bilateral pleural and peritoneal drainage tubes were placed in all the patients. The total volume of the effusion was measured at 6, 12, 24, 48, and 72h postoperatively and was corrected for body weight (kg) and intervals (h). Significantly reduced effusion (ml/kg/h) was noted in the off-pump group compared to the cardiopulmonary bypass group at 12h (cardiopulmonary bypass group, 8.6 [4.8-11.5]; off-pump group, 2.5 [1.2-5.4]; p=0.006) and at 48h (cardiopulmonary bypass group, 6.1 [2.6-9.9]; off-pump group, 1.4 [0.9-3.1]; p=0.008). The off-pump Fontan procedure may reduce the volume of postoperative pleural and peritoneal effusion.

Bidirectional Glenn and Antegrade Pulmonary Blood Flow: Temporary or Definitive Palliation?

We sought to investigate the role of the bidirectional Glenn with antegrade pulmonary blood flow in the surgical history of children with univentricular hearts. A series of 246 patients, from three joint institutions, having univentricular heart with restricted but not critical pulmonary blood flow received a bidirectional cavopulmonary shunt with additional forward pulmonary blood flow. All patients have been studied according to their progression, or not, to Fontan operation. Two hundred and eight (84.5%) patients underwent bidirectional cavopulmonary anastomosis as primary palliation. Twenty patients (8.1%) with previous pulmonary artery banding were also enrolled in the study. Patients who had received additional pulmonary blood flow through a previous systemic to pulmonary artery shunt for the critical pulmonary blood flow were excluded. No in-hospital death occurred. Follow-up was complete at 100%. Mean follow-up was 4.2 +/- 2.8 years (range, 6 months to 7 years). During the observational period 73 (29.7%) patients, considered optimal candidates, underwent Fontan completion for increasing cyanosis and (or) hematocrit and (or) fatigue with exertion. Three patients expired after total cavopulmonary connection (3 of 73; 4.1% mortality rate). The remaining 173 (70.3%) patients are alive with initial palliation. All patients were still well palliated with an arterial oxygen saturation at rest about 90%. According to our experience and results, bidirectional Glenn with antegrade pulmonary blood flow may be an excellent temporary palliation prior to a Fontan operation, which can be performed at the onset of symptoms. Bidirectional Glenn may also be the best possible palliation for a suboptimal candidate for Fontan.

Fontan Completion in Infants

Since the implementation of the Fontan procedure, several clinical factors have been linked to outcome. A study of the outcome after Fontan completion was undertaken with particular attention to the influence of age and cardiac diagnosis. Review of all patients (n = 107) undergoing Fontan completion between January 1998 and July 2005 to identify predictors of outcome: early death, prolonged effusions, and prolonged hospital stay. Median age was 13 months (range, 11 to 35) and median weight was 9.4 kgs (6.7 to 15.1). Hypoplastic left heart syndrome was present in 61 patients, and stage I Norwood was the initial palliation in 69. An interim superior cavopulmonary connection was performed in all. A lateral tunnel was used, and a deliberate right to left shunt was created in 99 patients. Mean transpulmonary gradient and pulmonary artery pressure were 5.7 +/- 1.5 mm Hg and 11.6 +/- 2.2 mm Hg, respectively. Median time to extubation was 5 hours (range, 2.5 to 184). Median duration of pleural effusion was 3 days (1 to 58) and was greater or equal to 14 days in 13 patients (12%). Overall mortality was 4.5% (5 of 107). Variables associated with poor outcome included associated noncardiac diagnosis (p < 0.05), elevated transpulmonary gradient (p = 0.03), and pulmonary artery pressure (p < 0.02). Hypoplastic left heart syndrome was the only variable associated with prolonged effusive complications. Fontan completion can be performed with good results in the first year of life independent of anatomic diagnosis. Significant noncardiac pathology, and a higher pulmonary artery pressure were predictive of worse outcome. Hypoplastic left heart syndrome was associated with prolonged effusions.

Modified Fontan Operation in Patients with Anomalies of Systemic and Pulmonary Venous Connection

The presence of anomalies of the systemic and pulmonary venous connection associated with single ventricle anomalies has been considered a contraindication for the Fontan operation (FO). The aim of this study is to outline the technical considerations associated with the presence of anomalies of systemic and/or pulmonary venous connections and to identify the risk factors for mortality in this group of patients undergoing the modified FO. Between 1989 and 2004, 63 patients (median age, 3.2 years) with anomalous systemic or pulmonary venous connection underwent a Fontan procedure at our institution. Nine patients had a combination of anomalous systemic and pulmonary venous connection, 49 patients had anomalous drainage only from the systemic circulation, and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 25 patients. Previous palliative operations had been performed in 51 patients (81%). There was 1 early death, and 2 patients required take down of Fontan procedures. Two patients required reoperation for revision of the atrial baffle. At a mean follow-up of 4.6 +/- 3.4 years, there have been 5 late deaths (8%) and 45 patients (71%) have undergone Fontan completion. Actuarial survival was 92% at 1 year and 91% at 5 and 10 years-not significantly different from the overall survival of the Fontan patients. We conclude that the modified FO can be successfully performed in patients with anomalous systemic or pulmonary venous connections, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients with normal connections.

Improving Outcomes in Functional Single Ventricle and Total Anomalous Pulmonary Venous Connection

Background We have previously reported that the outcome of infants with functional single ventricle and total anomalous pulmonary venous connection is poor relative to that of other single ventricle patients. Younger age at initial operation and obstructed total anomalous pulmonary venous connection were found to be risk factors for mortality. A review of our recent experience was undertaken to determine whether results in these patients are improving. Methods Medical records of 18 patients admitted after 1997 were reviewed (group B) and compared with the previous group of 73 patients admitted between 1984 and 1997 (group A). Data were analyzed using a Cox proportional hazards model. Results Median age at first operation was the same for both groups. The incidence of obstructed total anomalous pulmonary venous connection was not significantly different between groups (29% versus 33%, p = 0.70). Early survival is significantly improved for group B compared with group A ( p = 0.015). Only group and younger age at initial operation were found to be risk factors for mortality. In the current group, 5 patients have undergone superior cavopulmonary connection with one death, 3 have undergone Fontan completion with no deaths, and heart or heart-lung transplantation was performed in 5 patients with two deaths. Conclusions Early survival in patients with single ventricle and total anomalous pulmonary venous connection has improved significantly in recent years, but intermediate survival is still approximately 50%. Selective management using staged reconstructive surgery and transplantation may result in improved survival. Further study may identify risk factors for mortality and improve selection of appropriate patients for each therapy.

Systemic to pulmonary arterial shunts in neonates

The major strategy for palliation of cyanotic lesions in neonates is the systemic to pulmonary arterial shunt. Between May 1995, and December 2002, 48 consecutive neonates underwent systemic to pulmonary arterial shunts for cyanosis with reduced pulmonary blood flow. The mean age was 11.6 days (±SD 7.38) and the mean weight, 3.2kg (±SD 0.52). The babies were classified into three groups: Group I-Tetralogy-pulmonary Atresia (n=18), Group II-single Ventricle-Pulmonary atresia without (n=19) and with (n=5) isomerism, Group III-Pulmonary Atresia with Intact ventricular septum (n=6). Diagnosis was made by 2D echocardiography. Indication for cardiac catheterization was delineation of pulmonary anatomy/ductus laterality (n=4) or balloon atrial septostomy (n=4). The surgical procedure was a modified Blalock-Taussig shunt on the side of the situs. Post-operatively, no anti-coagulation or anti-platelet medication was employed. There was no mortality. Four cases required revision of the shunt in the immediate post-operative period for shunt thrombosis. The mean follow up was 17.54 months (±SD 8.36). In Group I, nine patients have undergone total correction with or without a conduit, while three required new arterial shunts for shunt/pulmonary artery stenosis. In Group II, nine patients have undergone bi-directional Glenn with atrial septectomy (n=2) and pulmonary artery plasty (n=4) and one patient underwent Fontan completion. In Group III, two patients underwent bi-directional Glenn and two had pulmonary valvotomy with/without right ventricular outflow tract widening. All the remaining babies are waiting for the second/final stage palliation or total correction. Systemic to pulmonary arterial shunts in neonates is a gratifying and reasonably safe surgical procedure. Most babies become candidates for eventual univentricular/bi-ventricular repair.

Anomalous pulmonary venous return in the staged palliation of functional univentricular heart defects

Background. Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic, pulmonary, or systemic and pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure. Methods and Results. Between March 1990 and March 1997, 32 patients with functional single ventricle and anomalous pulmonary venous return underwent operation at our institution. Five of 25 patients who underwent neonatal palliation died in the early postoperative period, all of whom had obstructed anomalous pulmonary venous return. Twenty-one patients have undergone bidirectional cavopulmonary shunt, including 7 in whom this was the primary palliative procedure. There was one early and two late deaths after the bidirectional Glenn procedure, two in patients with asplenia syndrome and none in patients with previously obstructed pulmonary venous return. Seven patients have undergone Fontan completion, 5 with an extracardiac conduit. There was one early death and one take-down to a classic Glenn shunt, both in patients who did not undergo the extracardiac conduit Fontan operation. Conclusions. Anomalous pulmonary venous return can significantly complicate the management of the single-ventricle patient, with the major impact on survival coming in the neonatal period. Palliation with the aim of performing an extracardiac conduit Fontan procedure allows greater latitude and more streamlined management in this group of patients.

Additional pulmonary blood flow with the bidirectional Glenn anastomosis: does it make a difference?

Background. The bidirectional cavopulmonary shunt has become a mainstay in the palliation of patients with a functional single-ventricle heart. However, there remain a number of unresolved issues regarding this procedure, many of which concern the response of the pulmonary vasculature to this unique circulatory physiology. Among the issues of debate are the role and effects of an additional source of pulmonary blood flow. Methods. Between January 1990 and April 1997, 160 patients underwent bidirectional cavopulmonary anastomosis. Median age at operation was 7.8 months, and age ranged from 24 days to 43 years. An additional source of pulmonary blood flow was included in 93 patients (58%). A retrospective review of our experience with this cohort was performed with a focus on the role of additional pulmonary blood flow. Results. Eight patients (5%) died in the early postoperative period, and the overall early failure rate (death or take-down) was 7.5% (n = 12). Eleven other patients underwent early reoperation to decrease (n = 8) or increase (n = 3) the amount of pulmonary blood flow. Early survivors were followed up for a median of 23 months, during which time 5 patients died and 30 patients underwent Fontan completion. Including early and late mortality, actuarial survival rates at 1 and 2 years were 91% and 88%, respectively. Conclusions. The bidirectional cavopulmonary shunt is a useful procedure in the early or intermediate-term management of patients with a functional univentricular heart. However, there is much still to be learned about this unique physiologic system. The role of accessory pulmonary blood flow remains unclear, as does the use of the bidirectional cavopulmonary shunt as long-term palliation. Pulmonary arteriovenous fistulas are a serious concern, especially in young patients with heterotaxy syndrome.

Bidirectional Cavopulmonary Shunt in Patients With Anomalies of Systemic and Pulmonary Venous Drainage

Background. Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic or pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure.Methods. Between March 1990 and December 1995, 36 patients with anomalous systemic or pulmonary venous drainage underwent bidirectional cavopulmonary shunt. A combination of anomalous systemic and pulmonary venous drainage was present in 12 patients, whereas 19 patients had anomalous drainage only from the systemic circulation and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 18 patients. The median age at operation was 11 months, and bidirectional cavopulmonary shunt was the first surgical procedure performed in 10 of these patients. Techniques of repair are described.Results. There were two early deaths and one bidirectional cavopulmonary shunt was taken down, for mortality and failure rates not significantly different than those for all patients undergoing bidirectional cavopulmonary shunt during this time period (n = 117). At a mean follow-up of 19.9 months, there have been three late deaths and 11 patients have undergone Fontan completion. Actuarial survival was 87% at 1 year and 81% at 3 years. Among all patients undergoing bidirectional cavopulmonary shunt during this time period, neither heterotaxy syndrome nor anomalies of systemic or pulmonary venous return were significantly associated with decreased survival or poor outcome.Conclusions. Bidirectional cavopulmonary shunt can be performed in patients with anomalous systemic or pulmonary venous drainage, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients undergoing bidirectional cavopulmonary shunt. In this report, we describe our experience with this group of patients, primarily focusing on outcomes and technical issues that pertain to the use of bidirectional cavopulmonary shunt as a preparatory procedure for the extracardiac conduit Fontan operation.(Ann Thorac Surg 1997;63:1676–84)