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PubMedDate not supplied

Postoperative graft thrombosis in Fontan procedure.

Fontan repair could be used as the definitive palliation in many forms of complex cyanotic congenital heart disease. But thrombosis can occur after a modified Fontan operation (right atrium-right ventricular connection with a conduit). Appropriate management of this complication includes thrombolytic therapy, thrombectomy and revision (if surgically remediable causes of the thrombosis are identified) or redo operation. Two repair operations were performed for the treatment of thrombosis of the right side of the heart in patients in whom we had previously performed Bjork modification (right atrium-right ventricular connection with a conduit). The thromboses occurred 6 and 9 years after the operation, respectively. In both cases, the redo Fontan operation was successfully performed using a dacron tube graft. Patients were anticoagulated after the operation. Risk of thrombosis of the right side of the heart after the Fontan repair may be minimized with the use of prophylactic anticoagulation in high-risk patients soon after the operation.

Child, Preschool↗

PubMed1991

The role of vasopressin and atrial natriuretic factor in postoperative fluid retention after the Fontan procedure.

The Fontan procedure results in right atrial distention and is complicated by fluid retention. Since systemic fluid balance may be hormonally mediated in part and related to right atrium size, we measured plasma atrial natriuretic factor and plasma arginine vasopressin levels in 19 patients undergoing the Fontan procedure and in 12 control patients undergoing other types of heart operations. Preoperative plasma atrial natriuretic factor levels were higher in patients undergoing the Fontan procedure than in control patients (95 +/- 16 pg/ml preoperatively versus 50 +/- 8 pg/ml; p less than 0.05) and increased in patients undergoing the Fontan procedure to 330 +/- 48 pg/ml by postoperative day 2 (p less than 0.05) but not in control patients. Increased plasma atrial natriuretic factor levels could enhance capillary transudation, but elevated plasma atrial natriuretic factor levels should also enhance diuresis and prevent fluid retention. Vasopressin levels, however, were also increased in patients undergoing the Fontan procedure (from 9 +/- 2 pg/ml preoperatively to 144 +/- 37 pg/ml at end of operation) and were higher and remained elevated longer than in control patients undergoing heart operations (37 +/- 7, 20 +/- 4, 16 +/- 6 pg/ml on postoperative days 1, 2, and 3 to 10 for the Fontan group compared with 15 +/- 4, 4 +/- 1, 4 +/- 2 pg/ml for control patients). Vasopressin levels were highest in the Fontan group with the most severe fluid retention and effusions (for example, 51 +/- 10 pg/ml versus 23 +/- 4 pg/ml, on postoperative day 1). Increased vasopressin and atrial natriuretic factor could act synergistically to result in the development of effusions after the Fontan procedure when atrial natriuretic factor-induced capillary transudation is combined with vasopressin-induced antidiuresis.

Atrial Natriuretic Factor↗

PubMed1985

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.

Blood Vessel Prosthesis↗

PubMed2000

Influence of ventricular morphology on outcome after the Fontan procedure.

The modified Fontan procedure has gained wide acceptance in the treatment of various congenital heart defects. Determination of risk factors for mortality remains an important issue for optimizing patient selection for the Fontan procedure. Conflicting results have been reported about whether ventricular morphology is a risk factor in these patients. Survival free of Fontan takedown or cardiac transplantation was assessed in the first 500 patients undergoing the Fontan procedure at our institution. This survival was correlated with ventricular morphology as evaluated by angiography. Both multivariate and univariate analyses indicated ventricular morphology was predictive of early survival free of Fontan takedown or cardiac transplantation following the procedure. However, there was no statistical evidence for ventricular morphology being a risk factor for mortality in patients alive 6 months after the procedure. Ventricular morphology is a risk factor for early survival in patients undergoing a Fontan procedure, with left ventricular morphology associated with a better early survival than right ventricular morphology.

Adolescent↗

PubMed1992

Quantification of flow through an interatrial communication. Application to the partial Fontan procedure.

The partial Fontan procedure has become an accepted alternative for the high-risk candidate. Creation of a small right-to-left shunt will lower the systemic venous pressure and improve systemic cardiac output while maintaining an acceptable systemic arterial saturation. However, because of variations in patient size and postoperative transpulmonary gradient, proper sizing of the residual defect is difficult. We have therefore conducted a series of experiments on a model that simulates the blood flow across interatrial defects of varying sizes at several pressure gradients. We used porcine blood to develop guidelines for the sizing of the residual defect. Our results demonstrate a linear relationship between flow and pressure gradient across all hole sizes tested. In addition, there was a linear relationship between atrial septal defect size and flow at each pressure gradient. Our data show that the Gorlin formula predictions overestimated flow by 10% to 40%. It is evident from these data that relatively small changes in the size of the atrial septal defect or in the pressure gradient result in significant changes in flow. Therefore we advocate the use of an adjustable interatrial communication such as the snare-controlled adjustable atrial septal defect for patients undergoing partial Fontan procedures.

Arteriovenous Shunt, Surgical↗

PubMed1986

Long-term results after Fontan procedure and its modifications.

The Fontan procedure is an effective method of treatment for patients with tricuspid atresia, univentricular heart, and other complex lesions. Modifications of the Fontan procedure have been developed to treat various anatomic malformations. From 1975 to 1984, 31 patients (17 male and 14 female) have undergone 35 Fontan operations. The youngest patient was 4 and the oldest 26 years of age (average 12.2 years). Sixteen patients had tricuspid atresia, and 15 univentricular heart. Twenty-three patients had undergone 37 palliative operations before the Fontan procedure. The original Fontan procedure was performed in 10 patients until 1977, and there were three early deaths (30%). After that time, the operative technique was modified and the early mortality dropped to 12% (3/25 patients). A valved prosthetic conduit was used in 11 patients (right atrium to pulmonary artery in five; right atrium to right ventricle in six), a valveless conduit in 14 patients (right atrium to pulmonary artery in nine; right atrium to right ventricle in five), and direct anastomosis (right atrium to pulmonary artery) in 10 patients. Postoperative cardiac catheterization was performed in 20 patients and revealed excellent results in 15. The remaining five had reduced oxygen saturation; three of them had had a Glenn anastomosis 8 and 10 years before the Fontan procedure, and one had an anomaly of the coronary sinus with a shunt to the left atrium. This patient required reoperation. Three other patients underwent reoperation because of calcified stenotic and/or outgrown conduits. Postoperative mean right atrial pressure varied from 10 to 20 mm Hg. The hemodynamic ventricular parameters were within the normal range. We conclude that in patients with tricuspid atresia, univentricular heart, and other complex cardiac lesions, inactivating the right ventricular pump function by means of the Fontan procedure does not adversely affect the systemic ventricle. Although the response to exercise was abnormal, the clinical condition of these patients was good to excellent.

Adolescent↗

PubMed1998

Complement activation and cytokine generation after modified Fontan procedure.

BACKGROUND: The modified Fontan procedure separates the systemic and pulmonary circulations in patients born with a functional single ventricle. Delayed recovery is frequently observed after this procedure. It was our hypothesis that complement activation or cytokine generation may contribute to the pathophysiology of this problem. METHODS: We measured activated complement C3, thromboxane B2, interleukin-6, and tumor necrosis factor-alpha levels by immunoassay in 16 patients undergoing Fontan procedure. Patient plasma samples were obtained preoperatively, on initiation of cardiopulmonary bypass, after administration of protamine, and 1, 4, 8, and 24 hours postoperatively. RESULTS: There was no early or late mortality in this cohort of patients. Low cardiac output developed in 3 of 16 patients, and pleural effusions developed in 5. The median length of hospital stay was 9 days. Activated complement C3 levels increased from a baseline of 1,486 +/- 564 to 4,600 +/- 454 ng/mL after cardiopulmonary bypass and administration of protamine, and returned to baseline by 24 hours. The level of interleukin-6 increased from 42 +/- 32 to 176 +/- 22 pg/mL and at 24 hours remained elevated at 71 +/- 15 pg/mL. Neither thromboxane B2 nor tumor necrosis factor-alpha levels increased significantly. CONCLUSIONS: The data demonstrate threefold to four-fold increases in activated complement C3 and interleukin-6, indicating that both humoral and cellular systems are affected. It is our conclusion that complement and cytokine activation may contribute to the delayed recovery observed after Fontan procedure.

Antibody Formation↗

PubMed1993

Tricuspid atresia and univentricular heart after the Fontan procedure.

Patients undergoing the Fontan operation must be selected carefully. The best results are obtained in those who fulfill the criteria of Choussat et al. Other treatment options are now available. Patients known to be at high risk for the Fontan procedure should be considered for cavopulmonary anastomoses or cardiac transplantation. It is unknown whether performing the Fontan operation at an earlier age will help prevent the long-term problems with ventricular dysfunction. Long-term follow-up of all patients following the Fontan procedure is mandatory with noninvasive assessment of ventricular function and the anastomotic site. Arrhythmias should be managed aggressively with prompt restoration of sinus rhythm, when possible, ideally with antiarrhythmic agents with little or no negative inotropic action. Periodic assessment of serum proteins should be performed. Although the operative risk of the Fontan operation has continued to improve over the last few years, and in one series has been as low as 8%, long-term complications continue and reinforce the concept of the Fontan operation being a palliative rather than a curative procedure.

Cardiac Surgical Procedures↗

PubMed1993

Incidence and management of dysrhythmias after Fontan procedure.

The modified Fontan procedure for univentricular heart disease results in the full spectrum of significant cardiac arrhythmias: tachycardias and bradycardias. The tachycardias are primarily supraventricular in origin: 1. atrial flutter, 2. primary atrial tachycardia, 3. atrial fibrillation, and 4. accelerated junctional rhythm or junctional tachycardia; however, ventricular tachyarrhythmias occur also, but less frequently. Bradycardia usually is a result of 1. sinoatrial node dysfunction resulting in junctional rhythm or 2. less commonly, atrioventricular conduction abnormalities, such as second degree and third degree atrioventricular block. Finally, the combination of atrial tachyarrhythmias and sinoatrial node dysfunction can occur in a small but significant number of patients after Fontan surgery. A complete array of medical and pacemaker therapeutic options is necessary for the physician to treat these difficult arrhythmias successfully.

Anti-Arrhythmia Agents↗

PubMed1980

Cardiovascular status after repair by Fontan procedure.

Patients surviving a Fontan operation experience dramatic symptomatic improvement, but concern remains about the long-term results of this operation. The clinical course and postoperative hemodynamic findings in 5 long-term survivors of the Fontan procedure from our institution are presented. Attention is drawn to 3 patients who required reoperation: 1 immediately for residual mild pulmonary stenosis, 1 for late onset complete heart block, and 1 for conduit valve stenosis. Review of our patients and those described in the literature reveals that all have ascites and pleural effusions as a results of high venous pressures but that this is usually a temporary problem. Late onset of obstruction to right atrial emptying has been reported in several patients in addition to ours. This raises serious concerns about the long-term fate of cloth conduits and porcine valves in this application. Although normal sinus rhythm has been thought to be essential for adequate pulmonary perfusion in these patients, it is interesting that several patients have tolerated atrial tachyarrhythmias, junctional rhythms, and even complete heart block without serious ill effects. We conclude that the Fontan procedure is extremely effective in relieving symptoms at an operative risk that is now acceptable, but these patients require very careful long-term follow-up because they are subject to a number of long-term complications.

Adolescent↗

PubMed1992

Factors influencing survival of patients with heterotaxy syndrome undergoing the Fontan procedure.

OBJECTIVES: This study was undertaken to determine those factors that may influence survival in patients with heterotaxy syndrome undergoing the Fontan procedure. BACKGROUND: The Fontan procedure remains the preferred palliative procedure for patients with heterotaxy syndrome. Although the mortality rate has improved for patients without this syndrome undergoing the Fontan procedure, it remains high for patients with heterotaxy syndrome. METHODS: The medical records of 20 consecutive pediatric patients with asplenia (n = 12) and polysplenia (n = 8) who underwent the Fontan procedure between January 1, 1986 and December 31, 1990 were reviewed. Anatomic and hemodynamic data were collected, as well as data on types of surgical palliative procedures and on outcome of the Fontan procedure. RESULTS: There were two early and two late deaths for a total mortality rate of 20% in the patients with heterotaxy syndrome, as compared with 8.5% for the patients without this syndrome who underwent the Fontan procedure during the same time period. Factors that significantly increased the risk of the Fontan procedure in these patients were 1) preoperative findings of greater than mild atrioventricular valve regurgitation, b) hypoplastic pulmonary arteries, and c) mean pulmonary artery pressure greater than or equal to 15 mm Hg after 6 months of age. Systemic and pulmonary venous anomalies coupled with single-ventricle anatomy were not significant risk factors for determining a poor outcome of the Fontan procedure. CONCLUSIONS: This study suggests that the outcome of the Fontan procedure in patients with heterotaxy syndrome may be improved by early protection of the pulmonary vascular bed, despite the existence of other cardiac anomalies.

Abnormalities, Multiple↗

PubMed1985

Permanent cardiac pacing after the Fontan procedure.

Permanent cardiac pacing after a Fontan procedure is complicated by complex cardiovascular anatomy. Of 332 patients undergoing the Fontan procedure at the Mayo Clinic, we evaluated 15 who postoperatively required permanent pacing (mean age 16.5 years, range 4 to 31 years). Underlying congenital cardiac defects included univentricular heart in nine patients, double-outlet right ventricle in three, and tricuspid atresia in three. The indication for pacing was postoperative heart block in seven patients, congenital heart block in five, postoperative sick sinus syndrome in two, and heart block because of previous operation in one. Pacemakers were implanted immediately postoperatively in 11 patients and 12 to 57 months later in four patients. VVI systems were used in nine patients, DDD in four, AAI in one, and a Medtronic Activitrax VVI in one. All ventricular leads were epicardial. Four atrial leads were transvenous endocardial and one was epicardial. Three patients died 4, 9, and 69 months later of causes unrelated to pacing. Among the 12 survivors, mean follow-up was 34 months (range 1 to 107 months). Two patients had a total of three episodes of loss of ventricular capture because of increased chronic thresholds. Our current approach to pacing after a Fontan procedure includes (1) intraoperative placement of temporary atrial and ventricular electrodes, (2) intraoperative attachment of a permanent ventricular epicardial lead for congenital or surgically induced high-grade atrioventricular block, (3) postoperative insertion of transvenous atrial leads if dual-chamber pacing is indicated, and (4) use of programmable pulse generators with high output capability.

Adolescent↗

PubMed1994

Reduction in triiodothyronine levels following modified Fontan procedure.

Diminished cardiac function is a common manifestation following the modified Fontan procedure. Since thyroid hormone has important effects on cardiovascular function, the present study was undertaken to evaluate changes in thyroid hormone levels following this operation. A control group consisting of children undergoing open heart procedures other than a Fontan procedure was also evaluated. Serum total and free triiodothyronine (T3), total and free thyroxine (T4), thyroid stimulating hormone (TSH), and thyroglobulin were measured by immunoassays. The Fontan group demonstrated an initial increase in free T4, while free T3, total T3, total T4, TSH, and thyroglobulin were reduced. Over the subsequent days, free T4 decreased to below the preoperative value. By the fifth and eighth postoperative days, free T3, total T3, free T4, and total T4 remained reduced, while TSH and thyroglobulin began increasing toward the preoperative levels. The control group also demonstrated decreases in free T3 and TSH. However, these values had returned to baseline by the fifth postoperative day. The results indicate that children undergoing open heart surgery have suppression of the pituitary-thyroid axis, and that this is prolonged in patients undergoing Fontan procedure. The decreased levels of T3 following Fontan procedure may have adverse effects on the recovery of patients undergoing this operation.

Atrial Function↗

PubMed2000

Intraoperative evaluation of pulmonary artery flow during the Fontan procedure by transesophageal Doppler echocardiography.

After the Fontan procedure, pulmonary artery (PA) flow is maintained without right ventricular pump function. We evaluated intraoperative PA flow velocity patterns using transesophageal Doppler echocardiography (TEE) immediately after cardiopulmonary bypass (CPB) in patients during Fontan or hemi-Fontan procedures. We studied 10 patients with single-ventricle physiology (age range, 5 mo to 3 yr 1 mo). Anesthesia was induced and maintained with fentanyl. After induction of anesthesia, a pediatric TEE probe was inserted into the esophagus. All patients had surgical repair involving direct anastomosis of the right atrium to the PA. Immediately after completion of CPB, adequacy of the atriopulmonary anastomosis was assessed and PA flow velocity was recorded. In all patients, the atriopulmonary anastomosis was clearly defined using a single-plane TEE probe, and PA flow recording was completed successfully. Intraoperative PA flow velocities showed two distinct patterns. Biphasic forward flows with peak velocities during systole and diastole were observed in six patients. The remaining four patients showed forward flows with flow reversals. The four patients demonstrating flow reversals showed significantly reduced fractional shortening (26.5+/-2.1% vs. 35.5+/-6.3%) and larger pressure gradient between the right atrium and left atrium (10.8+/-1.3 mm Hg vs 8.0+/-0.9 mm Hg) when compared to those without reverse flow. Two patients with reverse flow required reoperation because of hypotension. Because PA flow is influenced by pulmonary vascular resistance and left ventricular function, TEE assessed intraoperative PA flow should be further evaluated as a useful predictor of surgical outcome after a Fontan procedure.

Blood Pressure↗

PubMed1998

Hemodynamic effects of human atrial natriuretic peptide after modified Fontan procedure.

BACKGROUND: Reduction of pulmonary vascular resistance and maintenance of urine output are important after the modified Fontan procedure. Atrial natriuretic peptide (ANP) has the effects of a vasodilator (including the pulmonary arteries) and a physiologic diuretic, and newly synthesized human ANP is available. We measured plasma ANP levels before and after the Fontan procedure and examined the effects of human ANP on hemodynamic parameters after the Fontan procedure. METHODS: Eight patients, aged 2 to 15 years, underwent the Fontan procedure (atriopulmonary connection). Blood samples were taken before and 3 hours after operation, and plasma ANP levels were measured by radioimmunoassay. The correlation between central venous pressure and ANP was examined. Human ANP was infused intravenously at a dosage of 0.1 microg x kg(-1) x min(-1) for 1 hour after the Fontan procedure under controlled ventilation and another blood sample was obtained. Urine volume and central venous pressure were measured, and pulmonary vascular resistance and the cardiac index were calculated by the thermodilution catheter method before and after human ANP infusion. One hour after human ANP infusion was discontinued, the evaluation was repeated. No other diuretics were given and the infusion rates of catecholamine were kept constant during these measurements. RESULTS: Plasma ANP levels before and after the Fontan procedure were 29.1 and 54.9 pg/mL, respectively, and a positive correlation was obtained between central venous pressure and plasma ANP levels (r = 0.661, p < 0.05). Human ANP infusion significantly decreased central venous pressure and pulmonary vascular resistance, and increased urine volume and the cardiac index, whereas the plasma ANP level was elevated to 617.5 pg/mL. Systemic blood pressure did not change significantly. CONCLUSIONS: Atrial natriuretic peptide is secreted in response to elevated central venous pressure after the Fontan procedure, but its concentration might not be sufficient. Human ANP can be a therapeutic choice after the Fontan procedure as a physiologic diuretic and a pulmonary vasodilator.

Adolescent↗

PubMed1988

Tricuspid atresia versus other complex lesions. Comparison of results with a modified Fontan procedure.

Several modifications of the Fontan principle are currently applied to the treatment of tricuspid atresia with low mortality. The use of these modifications in other malformations has most frequently been associated with less satisfactory results. At our institution, from June 1977 to October 1986, 35 consecutive patients, whose ages ranged from 8 months to 20 years (median age 3.4 years), underwent a modified Fontan procedure. Twenty patients with a median age of 3.2 years (group I) having tricuspid atresia (16 patients) or hypoplastic right heart syndrome (four patients) were treated by means of a right atrium-pulmonary artery anastomosis (12 patients) or right atrium-subpulmonary chamber connection (eight patients). Fifteen patients (group II) with a median age of 3.6 years, having a single left ventricle (10 patients), left atrioventricular valve hypoplasia or atresia (three patients), or double-outlet right ventricle (two patients), underwent right atrium-pulmonary artery anastomosis, together with a repositioning of the atrial septum to the right of the right atrioventricular valve, which thus left intact the inlet to the ventricle(s). The operative mortality rate was 25% in group I and 0% in group II. One patient in group I and one in group II died late postoperatively. All the 28 survivors are free of symptoms 3 months to 9 years after correction. According to our results, low risk can be associated with modified Fontan procedures in the treatment of complex heart malformations other than tricuspid or pulmonary atresia. Preserving the integrity of the entire inlet to the ventricle(s) by repositioning the interatrial septum, as done in group II malformations, might be helpful in improving the quality of the repair.

Adolescent↗

PubMed1996

Aortopulmonary collateral vessels and prolonged pleural effusions after modified Fontan procedures.

Pleural effusions after the modified Fontan procedure are unpredictable, increase morbidity, and prolong hospital stay. To assess the relation between preoperative characteristics and postoperative pleural drainage, we performed a retrospective study of 71 patients who underwent Fontan procedures. Analyses revealed no significant relation between duration of effusion and age at Fontan, preoperative oxygen saturation, pulmonary artery pressure, ventricular end-diastolic pressure, type of Fontan, or prior cavopulmonary anastomosis. Patients with significant aortopulmonary collateral vessels evidenced by angiographic opacification of the pulmonary arteries or veins had more prolonged pleural drainage. The duration of the pleural drainage was significantly less in patients who had aortopulmonary collateral occlusion.

Adolescent↗

PubMed1994

[Modified Fontan procedure for complex cardiac anomaly with nonconfluent pulmonary artery].

Modified Fontan procedure was successfully performed in two patients with complex cardiac anomaly and nonconfluent pulmonary artery. A nonconfluent portion of the pulmonary artery was interposed with a xenopericardial roll. In one patient, the central pulmonary artery was completely absent. Two-staged reconstruction of the central pulmonary artery was carried out through a left thoracotomy and a median sternotomy before the Fontan procedure. In the other patient, a juxtaductal segment of the pulmonary artery was nonconfluent. Correction of the nonconfluent pulmonary artery was performed concomitantly with the Fontan procedure. Fenestrated Fontan procedure seemed to be the beneficial modification for survival. Both patients have been day well during the midterm follow-up period.

Cardiac Surgical Procedures↗