Special problems in Fontan-type operations for complex cardiac lesions

Four patients with complex cardiac lesions, who underwent successfully a Fontan type of operation are presented, each case representing a particular problem in surgical management. The first exhibited displacement of a trileaflet straddling and overriding left atrioventricular valve in addition to tricuspid atresia, while the second one had unilateral lung perfusion. In the third case, a stenosis had developed near the origin of the right pulmonary artery, and pulmonary vascular resistance could not be determined prior to the definite palliation procedure, whereas the fourth patient presented with stenosis of the left atrioventricular valve. Surgery was planned after detailed evaluation of the morphologic and hemodynamic features by means of echocardiography and cardiac catheterization. Our results illustrate the common tendency to extend the limits for modified Fontan procedures, which also includes revision of the original criteria for selection.     

The Fontan procedure for tricuspid atresia: early and late results of a 25-year experience with 216 patients.

OBJECTIVES: We assessed the operative and late mortality and the present clinical status of 216 patients with tricuspid atresia who had a nonfenestrated Fontan procedure performed at the Mayo Clinic in the 25-year period 1973 to 1998. BACKGROUND: The Fontan operation eliminates the systemic hypoxemia and ventricular volume overload characteristic of prior forms of palliation. However, it originally did so at the cost of systemic venous and right atrial hypertension, and the long-term effects of this "price" were unknown when the procedure was initially proposed. METHODS: We reviewed the clinical records of the 216 patients retrospectively. These were arbitrarily grouped into early (1973 through 1980), middle (1981 through 1987) and late (1988 through 1997) surgical eras. Patient outcome was also analyzed according to age at surgery. Operative and late mortality rates were determined and present clinical status was ascertained in 167 of 171 surviving patients. RESULTS: Overall survival was 79%. Operative mortality steadily declined and was 2% (one of 58 patients) during the most recent decade. Late survival also continues to improve. Age at operation had no effect on operative mortality, and late mortality was significantly increased only in patients who were operated on at age 18 years or older. Eighty-nine percent of surviving patients are currently in New York Heart Association class I or II. CONCLUSIONS: The initial 25-year experience with the nonfenestrated Fontan procedure for tricuspid atresia has been gratifying, with most survivors now leading lives of good quality into adulthood. These results justify continued application of this procedure for children born with tricuspid atresia.     

Correction of tricuspid atresia.

Although Fontan and Baudet in 1971 described a physiological correction procedure for tricuspid atresia, very few successful operations have been reported. Two patients corrected 20 and 10 months ago at the Brompton Hospital are presented. These two patients exhibit many of the problems in the management of tricuspid atresia. The first patient aged 20 had undergone three previous palliative operations, a Blalock-Taussig shunt, a Glenn procedure, and an infundibular resection, and therefore presented a number of operative problems. In contrast the second patient, aged 8, whose condition had deteriorated considerably over the previous year, had had no previous surgical treatment. At operation he was found, in addition, to have a partial atrioventricular canal. Details of the operative procedures and the patients' postoperative course are described. The criteria for selection of patients for the Fontan operation are discussed as are the possible long-term hazards of homograft failure, atrial dysrhythmias, and hepatic dysfunction. The successful outcome of these two patients suggests that palliative surgery in infancy should allow for this form of correction in later life.     

Correction of tricuspid atresia.

Although Fontan and Baudet in 1971 described a physiological correction procedure for tricuspid atresia, very few successful operations have been reported. Two patients corrected 20 and 10 months ago at the Brompton Hospital are presented. These two patients exhibit many of the problems in the management of tricuspid atresia. The first patient aged 20 had undergone three previous palliative operations, a Blalock-Taussig shunt, a Glenn procedure, and an infundibular resection, and therefore presented a number of operative problems. In contrast the second patient, aged 8, whose condition had deteriorated considerably over the previous year, had had no previous surgical treatment. At operation he was found, in addition, to have a partial atrioventricular canal. Details of the operative procedures and the patients' postoperative course are described. The criteria for selection of patients for the Fontan operation are discussed as are the possible long-term hazards of homograft failure, atrial dysrhythmias, and hepatic dysfunction. The successful outcome of these two patients suggests that palliative surgery in infancy should allow for this form of correction in later life.     

Discontinuous Pulmonary Arteries Do Not Preclude Good Fontan Outcomes

Objective. Discontinuous pulmonary arteries are believed to portend poor outcomes for a single ventricle palliation leading to Fontan's operation. This is a single institutional review of patients with single ventricle and discontinuous pulmonary arteries who underwent pulmonary artery centralization as part of staged surgical palliation. Design. The study is a retrospective case series. Patients. From November 1997 to December 2005, 12 centralization procedures were performed on 12 single ventricle patients with discontinuous pulmonary arteries. The diagnoses at surgery were as follows: heterotaxy 67%, pulmonary atresia 75%, a single morphologic right ventricle 58%, a single morphologic left ventricle 33%, and functional single ventricle with atrial situs inversus 8%. Outcome Measures. The outcome was assessed by hospital survival, actuarial survival, and New York Heart Association (NYHA) classification at follow-up. Results. The overall actuarial survival following centralization is 100% (95% confidence interval = 0.698 to 1). Seventy-five percent of the patients have undergone a Fontan procedure. Median McGoon ratio pre-Fontan = 1.65 (range: 1–2.1). Median follow-up after Fontan = 4.4 years (range: 1.2–9 years). Overall actuarial survival following Fontan is 100% (95% confidence interval = 0.428–0.911). Following the Fontan, there have been no thromboembolic complications, protein-losing enteropathy, nor Fontan takedowns. One hundred percent of the Fontan patients are NYHA class I. Conclusions. This experience indicates that a resuscitative strategy for discontinuous pulmonary arteries can result in good outcomes after the Fontan procedure. The presence of discontinuous pulmonary arteries in patients with single ventricle physiology should not preclude a management strategy with the goal of Fontan candidacy.     

Management and outcome of infants and children with right atrial isomerism.

OBJECTIVES: To assess the current results and outcome of surgery in infants and children with right atrial isomerism and complex congenital heart disease. SETTING: Tertiary referral centre. METHODS: 20 consecutive children with right atrial isomerism and complex congenital heart disease underwent surgery over a 6 year period between August 1987 and July 1993. The results and outcome were analysed according to age, presentation, and surgical procedures. RESULTS: Patients were divided into two groups depending on age at presentation and initial surgery: group A comprised 11 patients who required surgical intervention in the first month of life (mean age 5 days); and group B comprised nine patients who required initial surgical intervention after the first month of life (mean age 6.8 months). Seven (64%) of the 11 patients in group A had obstructed pulmonary venous drainage and ten (91%) had pulmonary atresia. There were seven early deaths (64%), including the five patients who required systemic to pulmonary artery shunt and simultaneous repair of obstructed pulmonary veins. The long-term survival rate in this group was 18% (two of 11). Pulmonary venous obstruction was present in two (22%) of the nine patients in group B and four (44%) had pulmonary atresia. There were no early deaths. One patient died after a second palliative procedure. There was one late sudden death. Four patients had a Fontan operation with no deaths. Two of the remaining three patients meet the Fontan criteria. The long-term survival rate in this group was 78% (seven of nine). CONCLUSIONS: Surgical management of patients with right atrial isomerism who have complex congenital heart disease carries a high mortality and remains palliative. The overall survival rate was 45% (nine of 20); 18% in patients requiring surgery in the first month of life (group A) and 78% in patients requiring surgery after the first month of life (group B) (P < 0.001). Of the total of 20 patients, nine were potential candidates for a Fontan operation. Seven of these have undergone a Fontan procedure with five survivors.     

Fontan procedure--indication and clinical results

Twenty-six patients with tricuspid atresia (15), univentricular heart (7), and single ventricle (4) underwent 27 Fontan or modified Fontan procedures between 1975 and 1981. The age of the patients varied between 4 and 26 years. Twenty patients had had a total of 33 palliative operations prior to correction. The original Fontan procedure was performed in 10 patients from 1975 to 1977. According to the various anatomical findings modifications of the Fontan procedure, such as direct anastomosis or implantation of a valveless conduit, were introduced in 1977. Early mortality among all the patients was 22% (6 patients died). Three deaths occurred in the initial period 1975 to 1977. Among the last 20 patients (1978 to 1981) there were 3 early deaths. Three patients with single ventricle survived, one died due to pulmonary failure. There were 2 late deaths (sepsis, sudden cardiac death). Postoperative cardiac catheterization performed in 17 patients revealed excellent results in 13 patients; the remaining 4 displayed diminished arterial oxygen saturation, three of them had Glenn palliation prior to corrective surgery. Postoperative right atrial mean pressure varied from 10 to 23 mmHg. The left ventricular parameters were within the normal range.     

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.     

Postoperative and long‐term outcomes in children with Trisomy 21 and single ventricle palliation

Objective: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long‐term outcomes in patients with T21 and SV palliation.
Design: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anom‐ aly following Fontan palliation for similar diagnoses. Kaplan‐Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) through 2014 for patients with adequate identifiers.
Results: We identified 118 children with T21 who underwent initial surgical SV pallia‐ tion. Among 90 (75.6%) patients surviving their first surgery, 66 (73.3%) underwent Glenn anastomosis and 25 (27.8%) completed Fontan palliation with in‐hospital sur‐ vival of 80.3% and 76.0%, respectively. Fifty‐three patients had sufficient identifiers for PCCC‐NDI‐OPTN linkage. Ten‐year survival, conditioned on discharge alive after the Fontan procedure, was 66.7% compared to 92.2% for 51 controls without genetic anomaly (P = .001). Median age at death for T21 patients following initial surgical SV palliation was 2.69 years (IQR 1.34‐7.12) with most deaths (89.2%) attributed to the underlying congenital heart disease (CHD).
Conclusions: Children with T21 and SV are at high risk for procedural and long‐term mortality related to their genetic condition and underlying CHD. Nevertheless, a se‐ lect group of patients can successfully complete Glenn or Fontan palliation, reaching satisfactory long‐term survival.     

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.     

Natural history of the ventricular septal defect in tricuspid atresia and its surgical implications.

We have previously reported isolated cases of anatomical and functional closure of ventricular septal defects in tricuspid atresia. To study this phenomenon further, clinical, angiographic, and pathological findings in 20 consecutive cases of tricuspid atresia were reviewed. Sixteen cases were found to have normally related great arteries (type I) and 4 had transposition (type II). In 6 of these patients there was evidence of closure of a ventricular septal defect; in 3 this was complete and in the other 3, partial. Five of these ventricular septal defect closures occurred in type I patients and one among type II. Increasing cyanosis and polycythaemia and/or disappearance of a previously heard murmur were observed in all patients; these signs are more conspicuous in complete closure than in partial. The incidence of closure of ventricular septal defect in tricuspid atresia is 38 per cent and approximates to the incidence of spontaneous closure of isolated ventricular septal defects. Progressive muscular "encroachment" of the margins of ventricular septal defects with subsequent fibrosis and covering by endocardial proliferation is the most likely mechanism of closure in tricuspid atresia. The factors initiating the closure of ventricular septal defect remain unknown, but because of its occurrence in patients both with and without previous shunt operations, it is reasonable to assume that closure of ventricular septal defect in tricuspid atresia is not initiated or accelerated by these surgical shunts. Recent developments in surgical technique permit total surgical correction of tricuspid atresia at least in a physiological sense, but these operations can be successfully performed only in older children (over 8 years). Thus, palliation is essential in younger patients until they reach the age for total correction. A Blalock-Taussig shunt is recommended in preference to a Glenn anastomosis because the latter may leave the left pulmonary circuit without blood supply if the ventricular septal defect closes. In type II cases, a large and non-restrictive ventricular septal defect is essential for survival of the patient after the Fontan operation. For this reason, the size of the ventricular septal defect should be evaluated before and at the time of surgical correction. If the ventricular septal defect is small in a type II case, resection of the ventricular septum or a complete bypass of the ventricular septal defect and right ventricle is necessary at the time of the Fontan operation.     

Growth of children with a functionally single ventricle following palliation at moderately increased altitude

The bidirectional Glenn and Fontan procedures are empirically performed as interim and definitive procedures in children with a functionally single ventricle. The optimal stage of palliation, nonetheless, remains unknown. During childhood, growth is a fundamental measure of response to therapy. Growth may be influenced by the degree of cyanosis, the volume load on the ventricle, and cardiac performance. Thus, the weight and stature of children with a functionally single ventricle who underwent a bidirectional Glenn procedure or a Fontan procedure were studied to determine the effect of each intervention on growth. Z scores for weight and stature were retrospectively determined prior to palliation, at yearly intervals for 4 years, and from long-term measurements until 18 years of age in all patients with at least 2 years of observation following palliation. Growth was evaluated in 54 patients with a bidirectional Glenn procedure, and 65 patients with a Fontan procedure. The Z scores for weight were improved after each method of surgical palliation. Stature, however, was improved only following the bidirectional Glenn procedure. Growth was impaired in patients who developed protein losing enteropathy. Weight improved only during the initial 2 years after the Fontan procedure in patients who had a surgical fenestration. Over the long-term, patients who underwent a Fontan procedure were more likely to have a Z score less than -2.0 for weight and stature than patients who underwent only a bidirectional Glenn procedure. Late mortality and the incidence of heart transplantation were increased in patients who experienced a decrease in their rate of growth, defined as a negative change of more than one Z score in weight or stature, following the Fontan procedure. In conclusion, at moderately increased altitude, children with a functionally single ventricle grow more appropriately following the bidirectional Glenn procedure than following the Fontan procedure. A decrease in the rate of growth is associated with a poor prognosis following the Fontan procedure.     

Outcome of patients with double-inlet left ventricle or tricuspid atresia with transposed great arteries

OBJECTIVES: We sought to determine the long-term outcomes and risk factors for mortality in patients with double-inlet left ventricle (DILV) or tricuspid atresia with transposed great arteries (TA-TGA). BACKGROUND: Patients with DILV or TA-TGA are at risk of systemic outflow obstruction and a poor outcome. The impact of various management strategies on the long-term outcomes of these patients remains unknown. METHODS: We reviewed the outcomes of 164 consecutive pediatric patients with DILV or TA-TGA who underwent surgical palliation between 1983 and 2002. Patients with a Holmes heart or heterotaxy syndrome or who were lost to follow-up (n = 24) were excluded. Risk factors for mortality or the need for orthotopic heart transplantation (OHT) were assessed by multivariate analysis. RESULTS: There were 105 patients with DILV and 35 patients with TA-TGA. The overall mortality rate, including OHT, was 29%. Patients with DILV had a lower mortality rate than patients with TA-TGA (23% vs. 49%, p = 0.007). Multivariate analysis showed the presence of arrhythmia and pacemaker requirement as independent risk factors for mortality, whereas pulmonary atresia or stenosis and pulmonary artery banding were associated with decreased mortality. Gender, era of birth, aortic arch anomaly, and systemic outflow obstruction were not risk factors. The perioperative and overall mortality were similar between patients who underwent the Damus-Kaye-Stansel procedure beyond the neonatal period and those had subaortic resection. CONCLUSIONS: The mortality of patients with DILV or TA-TGA remains high. The outcomes of these patients are influenced by restriction of pulmonary blood flow, arrhythmia, and pacemaker requirement. Surgical palliation to relieve systemic outflow obstruction is not associated with a poor outcome.     

Results of the modified Fontan operation in adults

The results of the modified Fontan procedure were examined in 77 patients 18 years of age and older who underwent operation at this institution between October 1973 and December 1986. This series represents 16% of the patients undergoing the modified Fontan operation during that period. Of the 77 patients (46 men and 31 women), 29 had tricuspid atresia, 30 had double-inlet ventricle and 18 had other complex lesions. There were 5 (6%) hospital deaths, compared with 67 (17%) for patients of all ages undergoing the Fontan operation during the same period. Patient age 18 years and older was not a significant risk factor. There were 8 late deaths. Detailed follow-up data were available on 61 (95%) survivors from 5 months to 12 years (mean 4 years) postoperatively. Fifty-seven (93%) of the patients were in New York Heart Association class I or II, 20 (33%) were taking no medication and 11 (18%) were taking digoxin alone. Seventeen (28%) patients complained of persistent ascites or edema. The study indicated that (1) the modified Fontan operation can be performed with low mortality in adults, (2) the long-term outcome is favorable and (3) persistent ascites or edema may be a problem in some patients.     

Influence of age at time of surgery on pre-operative left ventricular mass and postoperative outcome of Fontan operation in children with tricuspid atresia and native pulmonary stenosis.

Purpose of this study was to examine the influence of early (less than two and half years) versus later (greater than four years) age at time of Fontan type palliation in tricuspid atresia with native pulmonary stenosis on outcome with special reference to left ventricular mass and function. Among the 21 patients with tricuspid atresia, twelve (group A) underwent a Fontan type palliation at a median age of one (.6 to 2.5) years and nine (group B) at a median age of 7.5 (4.8 to 28) years. Left ventricular mass was assessed by cross-sectional echocardiography in the apical two and four chamber view. Mass was calculated as difference between epicardial and endocardial volume x 1.05 (specific gravity of heart muscle). Mass divided by volume at end-diastole yielded the mass/volume index. There was a weak correlation between age and left ventricular mass with an r-value of 0.74. Ejection fraction was calculated from the endocardial volume measurements at end-diastole and end-systole. Patient data were compared to normal values previously established in 95 controls, who were age-matched for the patients. Immediately before surgery left ventricular mass was significantly higher in the nine patients, who underwent surgery at a later age. While postoperative stay in hospital and duration of treatment in the intensive care unit did not differ significantly between both groups, the incidence of pleural and pericardial effusions and the duration of insertion of drainage tubes for these effusions differed significantly with the group A patients (under two and half years of age) doing better.(ABSTRACT TRUNCATED AT 250 WORDS)     

Considerations in Critical-Care and Anesthetic Management of Adult Patients Living With Fontan Circulation

The Fontan procedure is a staged palliation for various complex congenital cardiac lesions, including tricuspid atresia, pulmonary atresia, hypoplastic left heart syndrome, and double-inlet left ventricle, all of which involve a functional single-ventricle physiology. The complexity of the patients’ original anatomy combined with the anatomic and physiologic consequences of the Fontan circulation creates challenges. Teens and adults living with Fontan palliation will need perioperative support for noncardiac surgery, peripartum management for labour and delivery, interventions related to their structural heart disease, electrophysiology procedures, pacemakers, cardioversions, cardiac surgery, transplantation, and advanced mechanical support. This review focuses on the anesthetic and intensive care unit (ICU) management of these patients during their perioperative journey, with an emphasis on the continuity of preintervention planning, referral pathways, and postintervention ICU management. Requests for recipes and doses of medications are frequent; however, as in normal anesthesia and ICU practice, the method of anesthesia and dosing are dependent on the presenting medical/surgical conditions and the underlying anatomy and physiologic reserve. A patient with Fontan palliation in their early 20s attending school full-time with a cavopulmonary connection is likely to have more reserve than a patient in their late 40s with an atriopulmonary Fontan at home waiting for a heart transplant. Each case will require an anesthetic and critical care plan tailored to the situation. The critical care environment is a natural extension of the anesthetic management of a patient, with complex considerations for a patient with Fontan palliation.     

Atrioventricular Valve Failure in Fontan Palliation

Background

Atrioventricular valve failure (moderate or greater regurgitation, or valve operation) is a risk factor for adverse outcomes in patients undergoing Fontan palliation.

Single ventricle palliation: greater risk of complications with the Fontan procedure than with the bidirectional Glenn procedure alone

BACKGROUND: This study was performed to evaluate and compare the early, intermediate, and long-term outcomes of the bidirectional Glenn procedure and Fontan procedure in patients who live at moderately high altitude. METHODS: The outcome of each method of palliation for patients with a functionally single ventricle was retrospectively evaluated from a review of medical records. RESULTS: The bidirectional Glenn procedure was performed in 177 patients from October 1984 to June 2004. The Fontan procedure was performed in 149 patients from June 1978 to June 2004. Cardiovascular death or heart transplantation occurred in 8% of patients after the bidirectional Glenn procedure and 17% of patients after the Fontan procedure. Complications of systemic thromboembolic events, bleeding associated with anticoagulation therapy, protein losing enteropathy, and arrhythmias requiring implantation of a pacemaker, cardioversion, or radiofrequency ablation occurred in 7% of patients after the bidirectional Glenn procedure and 47% of patients after the Fontan procedure. Cardiovascular deaths and heart transplantation occurred less frequently when the Fontan procedure was performed in patients with a previous bidirectional Glenn procedure. However, the actuarial transplant-free survival and freedom from complications was not superior for a subgroup of patients who had a Fontan procedure after a bidirectional Glenn procedure in comparison to a subgroup of patients who had a bidirectional Glenn procedure alone. CONCLUSIONS: The bidirectional Glenn procedure can be used for long-term palliation of patients with a functionally single ventricle. Additional palliation with a Fontan procedure may increase the risk of stroke, protein losing enteropathy and arrhythmias without improving survival.     

Catheter ablation of multiple, surgically created, atrioventricular connections following Fontan-Bj?rk procedure.

An increased incidence of Wolff-Parkinson-White (WPW) syndrome with tricuspid atresia has been reported. Although atrioventricular accessory pathways may develop across suture lines after the Fontan-Bj?rk procedure, the presence of multiple acquired accessory pathways has only been described rarely. We report on a case of a female with tricuspid atresia who underwent the Fontan operation at 5 years of age. One year later, she developed a WPW pattern. Narrow complex tachycardias started at the age of 18. An electrophysiological study revealed the presence of three accessory pathway connections at the surgical anastomosis level. All of them were successfully ablated and there were no recurrences.     

Relief of an early pulmonary pathway obstruction with stenting after modified Fontan operation in an adult

The prognosis of Tricuspid Atresia, a rare congenital heart disease, has been changed by surgery. The criteria for Fontan operation have been well established in the literature and adult patients rarely fulfil these criteria; however, in very selected cases Fontan operation can be performed also in adults. A 33 year old woman with tricuspid atresia and previous palliation with classical right Glenn and with left modified Blalock-Taussing, underwent modified Fontan operation because increasing cyanosis and moderate exercise intolerance. Three weeks after operation the patient was readmitted with severe heart failure because of a tight obstruction at the anastomosis between right atrium and main-left pulmonary artery. The stenosis was treated with balloon and stenting achieving large pathway. Our experience confirms that after a modified Fontan, if cardiac failure occurs, an immediate full investigation have to be done.