Single-stage Fontan procedure: early and late outcome in 124 patients

INTRODUCTION AND OBJECTIVES: The Fontan procedure was designed to palliate complex congenital heart disease with univentricular physiology. A retrospective study was made to document the determinants of early (/= 31 days) mortality with the modified Fontan procedure performed in one-stage over a 22-year period. MATERIAL AND METHODS: Between 1978 and 2000, 102 atriopulmonary, 16 cavopulmonary, and 6 Kawashima type anastomoses were performed to palliate complex congenital heart defects in 124 patients with a mean age of 7.3 4.7 years. Forty-five patient and procedure-related variables were analyzed in relation to mortality. All events were verified. RESULTS: There were 29 early (23%) and 20 late (16%) deaths. Estimated survival at 30 days, 2 years, 5 years, and 20 years was 78, 75, 66, and 50%, respectively. Subaortic stenosis, protein-losing enteropathy, and arrythmia were observed in 8, 5 and 33 patients, respectively, after surgery. Univariate and multivariable analysis indicated that left ventricular end-diastolic pressure (>/= 13 mmHg), mean pulmonary pressure (>/= 19 mmHg), mitral stenosis/atresia, atrioventricular valve regurgitation, visceral heterotaxia, absence of fenestration, risk factors criteria, duration of extracorporeal circulation, and operative technique were associated with early mortality. Reoperation, arrhythmia, and pacemaker implantation were predictors of late death. Forty percent remained free from surgical or catheter reintervention after Fontan operation at 20 years. CONCLUSIONS: The outcome of Fontan procedure is profoundly affected by patient-related variables (ventricular function and pulmonary circulation). Postoperative arrhythmia and reoperation shortened the lifespan of the Fontan circulation model in patients with atriopulmonary connections. Total cavopulmonary anastomosis improves the physiology of univentricular circulation. In the light of our findings, the modified Fontan procedure (one or two stages) should be performed early in life to better preserve ventricular and pulmonary vascular function.     

Surgical evaluation of the modified Fontan procedure.

From 1980 to 1990 152 patients underwent Fontan operation at our institution. The following patient groups were identified: 1. patients with tricuspid atresia (n = 82, 54.0%); 2. patients with single ventricle (n = 31, 20.3%); 3. patients with a wide variety of non correctable, complex cardiac malformations (n = 39, 25.7%). In 27.0% of the patients a primary Fontan operation was performed. 45.0% of the patients received a previous shunt to increase pulmonary blood flow and in 29.4% of the patients a pulmonary artery band was placed to reduce pulmonary flow. Overall mortality was not significantly different in patients with previous palliative procedures (19.4%, n = 18) as compared to 17.4% (n = 6) in patients with primary Fontan operation. Risk of death was high in the group with complex cardiac malformations (28.2%, n = 11) and in patients with single ventricle (19.4%, n = 6). Early mortality was considerably less in patients tricuspid atresia (8.5%, n = 7). Postoperatively patients with incorporation of the residual right ventricular chamber and pulmonary valve (Fontan-Bjoerk) showed a significant (p less than 0.05) lower incidence of pleural effusion as compared to patients with other modifications of the Fontan procedure. Actuarial survival rate of all patients is 83.8 +/- 3.1% (mean +/- SEM) at ten years. The modified Fontan procedures are providing an accepted surgical method for patients with otherwise non correctable cardiac malformations.     

The Fontan procedure

The Fontan procedure has afforded improved surgical repair for several complex congenital cardiac defects, including tricuspid atresia and single ventricle. Through surgical creation of a connection between the RA and the RV or PA, adequate pulmonary perfusion can be achieved without an RV. Although it is not an anatomic connection, the Fontan procedure is a more physiologic approach than the previously used shunt procedures. Systemic venous return and PVR are effectively separated within the heart, pulmonary blood flow is assured through an RA-to-PA connection, and ventricular volume overload is avoided. The procedure has been effective in relieving cyanosis and has resulted in improved levels of exercise tolerance after surgery.     

Lung function and aerobic capacity in adult patients following modified Fontan procedure

OBJECTIVE—To examine cardiopulmonary performance in 52 adult patients with a Fontan circulation.
DESIGN—Retrospective cohort study. Values of maximum oxygen uptake (VO₂max), maximum heart rate (HRmax), forced vital capacity (FVC), and forced expiratory volume in one second (FEV₁) were compared with predictive values for different age groups. Patients were further subdivided into those with a pulmonary artery connection (RA-PA) or right atrium to right ventricle conduit (RA-RV).
RESULTS—At late follow up (median 10 years, range 1 to 26 years), patients with Fontan circulation had greatly diminished VO₂max, HRmax, FVC, and FEV₁ compared with predicted values. Early age at surgery had a positive impact on aerobic capacity. The FEV₁:FVC ratio indicated restrictive lung function. No differences were found with respect to any variable between patients with RA-PA connections and those with RA-RV connections.
CONCLUSIONS—Patients with a Fontan circulation have greatly diminished values of aerobic capacity and a restrictive pattern of lung function. Patients with an early surgical procedure obtained higher values of VO₂max. The theoretical benefits of including the right ventricle in a Fontan circulation were not apparent.


Keywords: congenital heart disease; exercise test; lung function; aerobic capacity     

Arrhythmias after the Fontan procedure

Objective—To study the determinants and outcome of arrhythmias after the Fontan type operation.

Design—Retrospective analysis of data in patients operated on between 1972 and 1986 (follow up 5–19 years (mean 12 years)).

Patients—All 60 patients undergoing a Fontan type procedure at the National Heart Hospital, London, during the study period (mean age (SD) 12·3 (6·8) years).

Results—Postoperative arrhythmias occurred in 34 patients (57%), and 11 (58%) of 19 early postoperative deaths (within seven days) were related to arrhythmias. Early arrhythmias occurred in 19 (32%) patients of whom 11 (58%) died. All patients with early atrial fibrillation and His bundle tachycardia died and only preoperative atrial fibrillation recurred early. There was a higher incidence of early arrhythmias, which were less well tolerated, in double inlet single ventricle patients (9/19) than in those with tricuspid atresia (8/37). There were no other preoperative determinants of early arrhythmias or deaths from early arrhythmia. Late (after seven days) arrhythmias occurred in 15 (37% of hospital survivors). They had higher right atrial (RA) pressures both early and late after operation and had lower ventricular ejection fractions late after operation. Of those with atrial arrhythmias 86% had RA obstruction and 57% had an RA thrombus or pulmonary embolism at presentation; this was also confirmed in two patients in whom late sudden deaths occurred. Atrial fibrillation early after reoperation for RA obstruction was fatal. The actuarial arrhythmia free survival for hospital survivors was 60% at 10 years.

Conclusions—Early postoperative arrhythmias were poorly tolerated, particularly atrial fibrillation and His bundle tachycardia. Previous atrial fibrillation was a relative contraindication to this procedure. Late postoperative arrhythmias were associated with higher RA pressures measured both early and late after operation and worse late ventricular function. Late arrhythmias may be the first manifestation of RA obstruction, which must be sought. RA thrombus was common in patients with atrial arrhythmias and should be treated early with anticoagulants.

     

The fenestrated Fontan procedure.

In 90 patients with characteristics placing them at increased risk for a Fontan operation, a fenestration was created in the atrial baffle at the time of the Fontan repair. The rational was to allow a right to left shunt which would maintain cardiac output and limit right atrial pressure in the presence of conditions which limit pulmonary blood flow. Early mortality was 4/90 (4%), with an additional two patients having the Fontan repair taken down to a bidirectional cavopulmonary anastomosis. Postoperative right atrial pressures were low (average 13 mm Hg), as was the incidence of prolonged pleural effusions (13%). At short-term (average 13 months) follow-up, 77% of patients have had closure of the fenestration, and 92% are in New York Heart Association Class I. We conclude that baffle fenestration with subsequent transcatheter closure results in decreased mortality and morbidity among high risk patients undergoing a Fontan repair, and that the high functional level at short-term follow-up justifies continued aggressive management of such patients.     

Arrhythmias after the Fontan procedure

Objective—To study the determinants and outcome of arrhythmias after the Fontan type operation.Design—Retrospective analysis of data in patients operated on between 1972 and 1986 (follow up 5–19 years (mean 12 years)).Patients—All 60 patients undergoing a Fontan type procedure at the National Heart Hospital, London, during the study period (mean age (SD) 12·3 (6·8) years).Results—Postoperative arrhythmias occurred in 34 patients (57%), and 11 (58%) of 19 early postoperative deaths (within seven days) were related to arrhythmias. Early arrhythmias occurred in 19 (32%) patients of whom 11 (58%) died. All patients with early atrial fibrillation and His bundle tachycardia died and only preoperative atrial fibrillation recurred early. There was a higher incidence of early arrhythmias, which were less well tolerated, in double inlet single ventricle patients (9/19) than in those with tricuspid atresia (8/37). There were no other preoperative determinants of early arrhythmias or deaths from early arrhythmia. Late (after seven days) arrhythmias occurred in 15 (37% of hospital survivors). They had higher right atrial (RA) pressures both early and late after operation and had lower ventricular ejection fractions late after operation. Of those with atrial arrhythmias 86% had RA obstruction and 57% had an RA thrombus or pulmonary embolism at presentation; this was also confirmed in two patients in whom late sudden deaths occurred. Atrial fibrillation early after reoperation for RA obstruction was fatal. The actuarial arrhythmia free survival for hospital survivors was 60% at 10 years.Conclusions—Early postoperative arrhythmias were poorly tolerated, particularly atrial fibrillation and His bundle tachycardia. Previous atrial fibrillation was a relative contraindication to this procedure. Late postoperative arrhythmias were associated with higher RA pressures measured both early and late after operation and worse late ventricular function. Late arrhythmias may be the first manifestation of RA obstruction, which must be sought. RA thrombus was common in patients with atrial arrhythmias and should be treated early with anticoagulants.     

The Fontan procedure in adults

SETTING—Tertiary adult congenital cardiac referral centre.
DESIGN—Retrospective cross sectional analysis.
OBJECTIVES—To report our 20 year experience with adult Fontan operations, and to compare late outcome in patients with single ventricle with definitive aortopulmonary or cavopulmonary shunt palliation.
PATIENTS AND MAIN OUTCOME MEASURES—Patients older than 18 years undergoing Fontan operation between 1 January 1982 and 31 December 1998 were identified. Mortality and late outcome were derived from hospital records. These patients were compared with a cohort of 50 adults with single ventricle who had not undergone a Fontan operation.
RESULTS—61 adults, median age 36 years (range 18-47 years), with a median follow up of 10 years (range 0-21 years) were identified. Actuarial survival was 80% at one year, 76% at five years, 72% at 10 years, and 67% at 15 years. Compared with before the Fontan operation, more patients were in New York Heart Association (NYHA) functional class I or II at the latest follow up (80% v 58%, p < 0.001). Systolic ventricular function deteriorated during follow up such that 34% had moderate to severe ventricular dysfunction at the latest follow up compared with 5% before Fontan (p < 0.001). Arrhythmia increased with time (10% before Fontan v 57% after 10 years, p < 0.001). Fontan patients had improved NYHA functional class, ventricular function, atrioventricular regurgitation, and fewer arrhythmias than the non-Fontan group at the latest follow up.
CONCLUSION—The Fontan operation in adults has acceptable early and late mortality. Functional class, systolic ventricular function, atrioventricular regurgitation, and arrhythmia deteriorate late after surgery but to a lesser degree than in non-Fontan patients with a single ventricle.


     

Outcome and assessment after the modified Fontan procedure for hypoplastic left heart syndrome.

BACKGROUND. We reviewed the outcome of 76 consecutive patients (age range, 5 months to 6 years; median age, 19 months) who underwent a modified Fontan procedure after initial palliative surgery for hypoplastic left heart syndrome (HLHS) between January 1984 and December 1989. METHODS AND RESULTS. Modifications of the Fontan procedure included transatrial baffle of pulmonary venous return to the tricuspid valve (n = 10) or inferior vena cava baffle within the right atrium to the superior vena caval-pulmonary artery anastomosis, with pulmonary artery augmentation (n = 66). Actuarial survival rates were 74% (1 month), 58% (12 months), 56% (2 years), and 52% (4 years). Of the 43 survivors, 25 patients have returned for postoperative cardiac catheterization at a medium of 13 months after the Fontan procedure. Mean +/- SD hemodynamic values were cardiac index, 2.8 +/- 0.6 l/min/m2; right arterial pressure, 11 +/- 2 mm Hg; pulmonary artery wedge pressure, 6 +/- 3 mm Hg; and arterial oxygen saturation, 94 +/- 3%. No patient had significant tricuspid or native pulmonary valve insufficiency. CONCLUSIONS. Survival after the Fontan procedure in patients with HLHS is comparable to survival after a Fontan procedure in patients with other complex congenital heart lesions. In the subgroup of patients with HLHS who survived both reconstructive surgery and a Fontan procedure and have been evaluated by cardiac catheterization after a Fontan procedure, the use of the right ventricle as the systemic ventricle yielded excellent intermediate results for Fontan physiology.     

Life-threatening hemoptysis following the Fontan procedure

Two cases of life-threatening recurrent hemoptysis occurring 10 years after a Fontan operation are presented. Bleeding from aortopulmonary collateral vessels was responsible for this complication in both cases, and the importance of systematic selective angiography of all potential origins of such abnormal vessels, including those arising from the abdominal aorta, is highlighted. Although coil embolization of aortopulmonary collateral vessels is usually definitive, pulmonary lobectomy may be necessary. The present report demonstrates, for the first time, that rescue extracorporeal membrane oxygenation support can be used as a bridge to surgery in case of severe uncontrollable hemoptysis in such cases.     

Arrhythmias after the Fontan procedure.

OBJECTIVE--To study the determinants and outcome of arrhythmias after the Fontan type operation. DESIGN--Retrospective analysis of data in patients operated on between 1972 and 1986 (follow up 5-19 years (mean 12 years)). PATIENTS--All 60 patients undergoing a Fontan type procedure at the National Heart Hospital, London, during the study period (mean age (SD) 12.3 (6.8) years). RESULTS--Postoperative arrhythmias occurred in 34 patients (57%), and 11 (58%) of 19 early postoperative deaths (within seven days) were related to arrhythmias. Early arrhythmias occurred in 19 (32%) patients of whom 11 (58%) died. All patients with early atrial fibrillation and His bundle tachycardia died and only preoperative atrial fibrillation recurred early. There was a higher incidence of early arrhythmias, which were less well tolerated, in double inlet single ventricle patients (9/19) than in those with tricuspid atresia (8/37). There were no other preoperative determinants of early arrhythmias or deaths from early arrhythmia. Late (after seven days) arrhythmias occurred in 15 (37% of hospital survivors). They had higher right atrial (RA) pressures both early and late after operation and had lower ventricular ejection fractions late after operation. Of those with atrial arrhythmias 86% had RA obstruction and 57% had an RA thrombus or pulmonary embolism at presentation; this was also confirmed in two patients in whom late sudden deaths occurred. Atrial fibrillation early after reoperation for RA obstruction was fatal. The actuarial arrhythmia free survival for hospital survivors was 60% at 10 years. CONCLUSIONS--Early postoperative arrhythmias were poorly tolerated, particularly atrial fibrillation and His bundle tachycardia. Previous atrial fibrillation was a relative contraindication to this procedure. Late postoperative arrhythmias were associated with higher RA pressures measured both early and late after operation and worse late ventricular function. Late arrhythmias may be the first manifestation of RA obstruction, which must be sought. RA thrombus was common in patients with atrial arrhythmias and should be treated early with anticoagulants.     

Pulmonary arterial changes in patients dying after a modified Fontan procedure following pulmonary artery banding

Summary Pulmonary arterial changes were histometrically analyzed in four cases of postoperative death following a modified Fontan procedure in which pulmonary artery banding had previously been performed because of pulmonary hypertension. Case 1 was a 3-year-old girl with corrected transposition of the great arteries (TGA), ventricular septal defect, and double-inlet left ventricle; case 2 was a 6-year-old girl with single ventricle (SV) and complete TGA; case 3 was a 25-month-old boy with SV and doubleoutlet right ventricle; and case 4 was a 21-year-old man with tricuspid atresia. The cause of death in cases 1, 2, and 3 was pulmonary hypertensive crisis due to postoperative vasoconstriction of the small pulmonary arteries. Medial hypertrophy remained in half of the preacinar small pulmonary arteries although it was not observed in all the intraacinar arteries in cases 1 and 2, even after banding. The postoperative course of case 4 was uneventful despite multiple thromboembolism in the small pulmonary arteries. However, the patient died due to a thrombosed artificial valve. The results suggest that residual medial hypertrophy of the small pulmonary arteries was a major risk factor in these cases. Lung biopsy is recommended to determine the indications for the Fontan procedure in these hemodynamically critical cases.     

Altered endothelial function following the Fontan procedure

OBJECTIVE: Thrombosis has been widely described after the Fontan procedure. The vascular endothelium plays a central role in the control of coagulation and fibrinolysis. The aim of this study was to investigate if patients undergoing a modified Fontan procedure have impaired endothelial function and fibrinolysis in the late postoperative course. PATIENTS AND METHODS: We compared 23 patients aged from 7 to 26 years with age-matched healthy volunteers, collecting blood samples prior to and following standardized venous occlusion testing. Plasma levels of von Willebrand factor antigen, tissue-type plasminogen activator antigen, plasminogen activator inhibitor-1, and D-dimer were measured with enzyme-linked immunosorbent assay. RESULTS: We found increased plasma levels of von Willebrand factor antigen in patients when compared to controls (p = 0.003). At the basal condition, concentrations of tissue-type plasminogen activator antigen and plasminogen activator inhibitor-1 antigen in the plasma, as well as their activity, were not significantly different between patients and controls. Following venous occlusion, concentrations of tissue-type plasminogen activator antigen in the plasma were significantly increased both in patients and controls, compared to pre-occlusion values. D-dimer was within the reference range. Multivariate discriminant analysis differentiated patients and their controls on the basis of differences for plasminogen activator inhibitor-1 and von Willebrand factor antigen (p = 0.0016). CONCLUSIONS: Our data suggest that patients with the Fontan circulation may have endothelial dysfunction, as indicated by raised levels of von Willebrand factor. Fibrinolysis seems to be relatively preserved, as suggested by appropriate response to venous occlusion.     

A model to simulate the haemodynamic effects of right heart pulsatile flow after modified Fontan procedure.

The effect of pulsatile pulmonary flow after the modified Fontan procedure was examined in a model that simulated the right heart. An inlet overflow tank (preload), axial pulsatile pump, Wind-Kessel model (afterload), and an outlet overflow tank were connected in series. The standard conditions were flow 2.00 l/min with 12 mm Hg preload pressure, 3.0 Wood units resistance, and an outlet overflow tank pressure at 6 mm Hg. The pump rate was set at 80 beats/min. The simulated pulmonary arterial pressure and pulmonary flow waves produced by this model closely resembled those obtained from patients who had undergone the modified Fontan procedure. All variables except the preload were fixed and changes in pulmonary flow were examined at preload pressures of 8, 12, 15, and 17 mm Hg. As the peak pulmonary arterial pressure increased so did pulmonary flow, until it was greater than during the non-pulsatile state. Because the afterload of this model was fixed, this result suggests that there was a concomitant decrease in resistance. This model indicates that pulsatile pulmonary blood flow is likely to have a beneficial effect on the pulmonary circulation after the modified Fontan procedure.     

A model to simulate the haemodynamic effects of right heart pulsatile flow after modified Fontan procedure.

The effect of pulsatile pulmonary flow after the modified Fontan procedure was examined in a model that simulated the right heart. An inlet overflow tank (preload), axial pulsatile pump, Wind-Kessel model (afterload), and an outlet overflow tank were connected in series. The standard conditions were flow 2.00 l/min with 12 mm Hg preload pressure, 3.0 Wood units resistance, and an outlet overflow tank pressure at 6 mm Hg. The pump rate was set at 80 beats/min. The simulated pulmonary arterial pressure and pulmonary flow waves produced by this model closely resembled those obtained from patients who had undergone the modified Fontan procedure. All variables except the preload were fixed and changes in pulmonary flow were examined at preload pressures of 8, 12, 15, and 17 mm Hg. As the peak pulmonary arterial pressure increased so did pulmonary flow, until it was greater than during the non-pulsatile state. Because the afterload of this model was fixed, this result suggests that there was a concomitant decrease in resistance. This model indicates that pulsatile pulmonary blood flow is likely to have a beneficial effect on the pulmonary circulation after the modified Fontan procedure.