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PubMed1997

[Thoracic epidural anesthesia for cholecystectomy in a patient after Fontan procedure].

A 12 year-old boy who had received modified Fontan procedure was scheduled for cholecystectomy. Anesthesia was maintained with thoracic epidural anesthesia (Th8-9) and O2-N2O-sevoflurane under mechanical ventilation through the endotracheal tube. Transeshophageal echo cardiography and mixed venous oxygen saturation (SvO2) were used for hemodynamic monitoring. The combined use of epidural anesthesia and volatile anesthetics decreased central venous pressure, left ventricular end diastolic volume, left ventricular ejection fraction and SvO2. These hemodynamic problems were easily solved by infusion therapy and a low dose of dopamine. On the other hand, there were also some hemodynamic benefits such as inhibition of tachycardia and suppression of an increase in pulmonary vascular resistance due to surgical stress. Moreover, patient returned to normal spontaneous breathing with complete analgesia during the early phase after surgery. CVP and SvO2 increased to preoperative values in the recovery room. From these results, we conclude that satisfactory results can be obtained with epidural anesthesia for upper abdominal surgery after Fontan procedure.

Anesthesia, Epidural↗
PubMed1998

[The conversion of a modified Fontan procedure to a total extracardiac cavo-pulmonary conduit. The Medico-Surgical Cardiology Group].

BACKGROUND: After a modified Fontan procedure with atriopulmonary or atrioventricular conduit, some patients present stress intolerance, supraventricular arrhythmia, recurrent pleuropericardial or ascitic effusions, and protein-losing enteropathy, all of which are signs that the previous procedure has failed. The aim of this study was to evaluate the midterm outcome after surgical therapy for this condition. MATERIAL AND METHODS: Between August 1994 and December 1997, nine patients (6 males and 3 females), age 10 to 39 (mean 21.5) years, underwent conversion of previous modified Fontan procedure to total extracardiac cavo-pulmonary connection. Time from the previous procedure was 6 to 18 years (mean 10). Diagnosis was tricuspid atresia with pulmonary stenosis (n = 2), double-inlet left ventricle and concordant ventriculoarterial connection (n = 3), double-inlet left ventricle and discordant ventriculoarterial connection (n = 3), Holmes heart (n = 1). Nine patients presented decreased stress tolerance, seven had arrhythmia, five had pleuropericardial effusions and two had protein-losing enteropathy. In all but one patient, right atrial pressure was higher than 15 mmHg, while in six patients the cardiac index was less than 2 l/min/m2. A polytetrafluoroethylene non-valved conduit was interposed between the inferior vena cava and the right pulmonary artery for conversion in all patients. A bidirectional cavo-pulmonary anastomosis (modified Glenn) was associated in all patients. Evaluation was done by NYHA Class and by an arbitrary score assigned to patients based on 7 parameters. RESULTS: There was no perioperative mortality. All patients were clinically improved at a mean follow-up of 24 months (range: 3 to 46). No patient had effusions, and the arrhythmias disappeared in 4 patients and were controlled by medical therapy in one. The two patients with protein-losing enteropathy improved markedly within 30 days and the score dropped below 10 points. CONCLUSIONS: The conversion of the modified Fontan procedure to total extracardiac cavo-pulmonary connection improves clinical condition by decreasing the right atrium-pulmonary gradient and right atrial preload, and by providing a laminar cavo-pulmonary flow without any need for intracardiac anastomoses. This procedure should be undertaken early in this subset of patients, before ventricular failure ensues.

Adolescent↗
PubMed1995

[Fontan procedure after pulmonary artery banding--surgical results and new anatomical repair for tricuspid atresia].

We examined our surgical experiences to determine the appropriate condition of pulmonary artery banding (PAB) for the Fontan procedure. From 1974 through 1992, thirteen patients underwent Fontan procedure following PAB at Tokyo Women's Medical College. Of these 6 had tricuspid atresia (TA), 5 had single ventricle, or 2 had other complex malformations. PAB was performed at the age of 1 to 14 months (mean 2.5 months). With monitoring pulmonary artery (PA) pressure and systemic arterial oxygen tension (PaO2), PA mean pressure decreased 43.6 to 18.8 mmHg and PA circumferences at the banding site decreased from 53.5 to 26.0 mm after PAB. This degree of PAB was tighter compared with the value estimated by Trusler's formula (27.9 mm) in most patients. After PAB, 5 patients required 8 additional palliative surgery including 3 systemic to pulmonary shunt, 3 rebanding, 1 palliative RVOTR to promote development of pulmonary vascular bed and 1 atrial septectomy to prevent pulmonary hypertension. Hemodynamic data before Fontan operation showed a tendency of higher PA pressure (17.5 vs 31.5 mmHg), and pulmonary vascular resistance (2.4 vs 5.5 Wood Unit) in non-survivors than in survivors. For patients with tricuspid atresia and high pulmonary vascular resistance, a new operation "Anatomical Repair" utilizing hypoplastic right ventricle with the translocation of pulmonary or aortic valve in tricuspid position was developed and successfully applied in three patients. In conclusion, initial tight PAB during early infancy and often repeated palliative surgery for development of adequate pulmonary vascular bed is the most important for maximizing the chance of subsequent successful Fontan procedure.

Age Factors↗
PubMed1988

Dysrhythmias after the modified Fontan procedure.

The cardiac rhythm before and after the modified Fontan procedure was reviewed in 24 patients. Transient atrial dysrhythmias were common in the immediate postoperative period. Late postoperative premature atrial contractions were detected by ambulatory monitoring in 20 of 23 patients; eight (34.8%) had supraventricular tachycardia. Late ventricular dysrhythmia was detected in 18 of 23 patients: ten had low-grade ventricular dysrhythmias and eight (34.8%) had multiform premature ventricular contractions. Five of the latter had couplets and one of these five plus another had ventricular tachycardia. Seven patients with supraventricular tachycardia and five patients with ventricular dysrhythmia required antiarrhythmic medication. Asymptomatic bradycardia was detected in five patients (21.7%). One patient had intermittent second-degree atrioventricular block. No specific risk factors predicted dysrhythmias. Thus, cardiac dysrhythmias were common in patients after the modified Fontan procedure, but were well tolerated in most patients. No sudden deaths or syncopal episodes have occurred during a mean follow-up of 5 years. One patient's death was related to severe left ventricular dysfunction. Permanent pacing has not been required in any patient.

Adolescent↗
PubMed1997

Atrial flutter in a pediatric patient in the immediate period after the Fontan procedure: control with oral propafenone.

A 2-year-old male underwent Fontan procedure as the third stage palliative repair for hypolastic left heart syndrome. On postoperative day number 15, he developed atrial flutter unresponsive to multiple attempts at cardioversion and therapeutic levels of digoxin and procainamide. Oral propafenone was administered and within 6 hours no further arrhythmias or side effects were noted. This case suggests that oral propafenone can be used with efficacy for the treatment of atrial flutter encountered in the immediate postoperative period following the Fontan procedure.

Administration, Oral↗
PubMed2006

Fontan procedure: old lessons and new frontiers.

The Fontan procedure is an operation created for patients with single ventricular physiology. These patients have the potential to survive well into adulthood, however new problems can arise. Various topics, including physiological constraints, patient care, morbidity and clinical outcomes are discussed. The aim of this review is to identify current topics within the care of the Fontan patient population for the physician not comfortable with their unique physiology and complications.

Arrhythmias, Cardiac↗
PubMed2003

[Fontan procedure for asplenia syndrome].

BACKGROUND: Historically the Fontan operation in patients with single ventricle asplenia syndrome has been associated with high mortality. We studied whether recent modifications of the surgical technique have improved outcome. METHODS: A retrospective review of 11 patients with asplenia syndrome who underwent a Fontan operation between 1996 and 2002 was performed. RESULTS: Anomalies of pulmonary venous return included 5 patients (46%) and 4 patients with an isolated hepatic venous return from inferior vena cava (36%). The type of Fontan procedure included 6 patients with an extracardiac tube-graft, 3 with a lateral tunnel modification, 1 with an intra-atrial tube graft, and 1 with an intra-extra atrial tube graft. A fenestration was placed in 3 patients (27%). There were no early deaths and 2 late hospital deaths (18%) due to tachyarrythmia and failed Fontan circulation. There were no late deaths and no complications during follow-up periods (mean 3 years). Of 21 patients diagnosed with asplenia syndrome between 1990 and 2000, overall survival was 57% and 12 patients underwent a Fontan-type operation. CONCLUSIONS: The Fontan operation can now be performed in patients with asplenia syndrome with good survival. Fontan staging, appropriate choice of Fontan modification, aggressive treatment of concomitant malformations, and use of a fenestration contribute to improved outcome.

Child↗
PubMed1998

Management of protein-losing enteropathy after the Fontan procedure.

Enteric loss of protein with the sequelae of edema, immunodeficiency, and hypercoagulability is being diagnosed with increasing frequency after Fontan procedure. The precise pathophysiological mechanism is unknown; however, protein-losing enteropathy (PLE) after Fontan procedure is likely related to a hemodynamic derangement that is not easily detectable via standard hemodynamic measures presently obtainable in the cardiac catheterization laboratory. Treatment options include (1) symptomatic relief via diuretics, supplemental albumin infusion, and dietary change to high protein/high medium-chain triglyceride intake, (2) hemodynamic improvement via afterload reduction (angiotensin-converting enzyme inhibitors), repair of branch pulmonary artery stenoses, or coil embolization of aortopulmonary collaterals, (3) intestinal cell membrane stabilization via high-dose steroids or heparin infusion, and (4) attempt at alteration of the primary hemodynamic derangement via fenestration of the systemic venous baffle or via heart transplantation. Further understanding of the cause of PLE after Fontan procedure is needed before more effective treatment options can be used. Copyright 1998 by W.B. Saunders Company

Journal Article↗
PubMed2003

Effect of beraprost sodium on pulmonary vascular resistance in candidates for a Fontan procedure: a preliminary study.

OBJECTIVE: Although long-term prostacyclin(PGI2) therapy in patients with severe pulmonary hypertension (PH)reduces pulmonary vascular resistance (PVR), there have been no reports on its therapeutic effects in patients with mild PH. We investigated the chronic effect of beraprost sodium (BPS), an oral PGI2 analog, in children with mild PH. METHODS: We studied 20 patients who were destined for a Fontan procedure with a mean pulmonary arterial pressure(PAP) of>20 mmHg and/or PVR of>3.0 Wood units. Both the PAP and the PVR in these cases were too high for patients to undergo a successful Fontan procedure. Seven patients received BPS (PG group) and 13 did not (control group). All patients underwent repeat cardiac catheterization to examine pulmonary hemodynamics. RESULTS: In the PG group, the pulmonary-to-systemic flow ratio (Qp/Qs) did not change after BPS administration(1.1 +/- 0.6 vs 1.3 +/- 0.9);however, the mean PAP decreased significantly (25.3 +/- 8.2 vs 19.9 +/- 6.5 mmHg; P < 0.05),as did PVR (3.7 +/- 1.3 vs 2.3 +/- 0.9 Wood units; P < 0.05), whereas the pulmonary artery (PA) index increased significantly (312 +/- 136 vs 375 +/- 165; P < 0.05). In the control group, the mean PAP decreased significantly (24.9 +/- 4.7 vs 19.8 +/- 6.3 mmHg; P < 0.05)and the PA index increased significantly (295 +/- 72 vs 362 +/- 114; P < 0.05). No significant changes in Qp/Qs (1.5 +/- 0.8 vs 1.4 +/- 0.6)or PVR (2.9 +/- 1.3 vs 2.5 +/- 0.8 Wood units) were observed. CONCLUSION: We conclude that long-term BPS administration probably reduces PVR in potential candidates for a Fontan procedure with mild PH. This treatment would reduce the risks associated with the Fontan procedure and would also improve its outcome.

Adolescent↗
PubMedDate not supplied

Ethnic differences in coagulation factor abnormalities after the Fontan procedure.

We tested the hypothesis that Chinese patients have a coagulation profile that is less prothrombotic than that of Caucasian counterparts after the Fontan procedure by determining the type and prevalence of anticoagulant and procoagulant deficiencies in Chinese patients and comparing the findings to those previously reported in Caucasian series. The liver function and coagulation factors were assessed in 21 ethnic Chinese patients, aged 17.0 +/- 5.6 years, at 10.7 +/- 4.0 years after the Fontan procedure. The results were compared to those of 21 age-matched Chinese controls with minor congenital heart disease. The prevalence of coagulation factor deficiencies in our patients was further compared to that reported in Caucasian patients. When compared with controls, patients had significantly lower protein C (p = 0.014), factors II (p = 0.024), V (p < 0.001), VII (p < 0.001), IX (p = 0.036), and X (p < 0.001), and higher bilirubin (p = 0.001) levels. The prevalence of protein C deficiency was 9.5%, whereas those of factor II, V, VII, IX, and X deficiencies were 0, 66.7, 9.5, 0, and 57.1%, respectively. When compared with Caucasian data, our data showed a significantly lower prevalence of protein C, total protein S, antithorombin III, factor II, and factor VII deficiencies. Furthermore, the previously reported increase in factor VIII levels was not found. In contrast, the prevalence of factor X deficiency was higher in our patients. This study provides the first evidence of ethnic differences in coagulation factor abnormalities after the Fontan procedure. The imbalance between procoagulant and anticoagulant pathways in Chinese patients favors a bleeding, rather than a thrombotic, tendency.

Adolescent↗
PubMed1994

Resuscitation after modified Fontan procedure.

As the survival rate after modified Fontan procedure is increasing, cardiopulmonary resuscitation may be needed in such patients for other causes not related to their cardiac status. The state-of-the-art cardiopulmonary resuscitation with external chest compression may not be sufficient to revive such patients, and the addition of external abdominal compressions may help in the final outcome, as is reported here.

Abnormalities, Multiple↗
PubMed2005

The off-pump Fontan procedure by simply cross-clamping the inferior caval vein.

BACKGROUND: We previously used the Fontan procedure, when applicable, by placing a temporary bypass from the inferior vena cava to the atrium. Alternatively, we have striven to achieve the procedure even without use of a temporary bypass in patients having collaterals between the inferior vena cava and the superior vena cava, so as to simplify the procedure. The azygous vein was intentionally left patent at the bidirectional Glenn procedure in the most recent 9 patients, expecting reasonable venovenous communication at Fontan completion. Surgical results will be described for the preliminary experience. METHODS: Since 2001, this alternative technique has been attempted in 34 patients undergoing the staged Fontan procedure, and eventually used in 22. Duration between the staged procedures was 4 to 108 months (median, 10 months). We considered that the technique was feasible unless femoral venous pressure exceeded 20 mm Hg immediately after cross-clamping the inferior vena cava. Although catheterization before the Fontan completion illustrated development of venovenous collaterals in 14 patients, oxygen saturation remained greater than 80% throughout the period of the bidirectional Glenn physiology. RESULTS: In all 22 patients, the extracardiac channel was readily reconstructed with an excellent surgical field of view, without operative mortality. On cross-clamping the inferior vena cava, the systemic circulation could be well maintained by administration of dopamine. Oxygen saturation immediately became approximately 97% to 100%. Maximal pressure gradient was 11 +/- 5 mm Hg between the superior vena cava and the femoral vein. Postoperatively, serum concentration of enzymes did not critically increase (maximal aspartate transaminase, 96 +/- 89 U/L; alanine transaminase, 65 +/- 59 U/L; total bilirubin, 1.8 +/- 1.1 mg/dL; creatine kinase, 437 +/- 230 U/L). CONCLUSIONS: This alternative technique, when feasible under the current criteria, was simple and did not provide any clinically significant impediments.

Child↗
PubMed1994

Acute hemodynamic effects of adjustable atrial septal defect closure in the lateral tunnel Fontan procedure.

OBJECTIVES: This study evaluated the acute hemodynamic changes with atrial septal defect closure in the postoperative period in patients undergoing the Fontan procedure. BACKGROUND: The adjustable atrial septal defect is a modification of the Fontan procedure designed to improve cardiac output and reduce systemic venous hypertension during the postoperative period. Limited information is available on the effects of inter-atrial shunting on the physiology of direct cavopulmonary connection. METHODS: In 11 patients (aged 9 months to 14.5 years), the atrial septal defect was closed 8 h to 4.6 days (mean 1.7 days) postoperatively. Indications for closure included mean right atrial pressure < 15 mm Hg or arterial oxygen saturation < 80%, or both. RESULTS: Data presented are mean values +/- 1 SD. Mean right atrial pressure was 13.4 +/- 3.0 mm Hg on admission to the intensive care unit, 10.0 +/- 2.0 mm Hg (p = 0.02) immediately before closure and 11.4 +/- 2.8 mm Hg (p = 0.02) after closure. There was a significant decrease in cardiac output, as calculated from arteriovenous oxygen saturation difference (26 +/- 9%, p = 0.003), Doppler aortic flow (19 +/- 9%, p = 0.0002) and ventricular volumes by two-dimensional echocardiography (20 +/- 8%, p = 0.0001). Arterial oxygen saturation increased from 82 +/- 5% to 94 +/- 4% (p = 0.0001), and arteriovenous oxygen saturation difference increased from 25 +/- 8% to 33 +/- 9% (p = 0.0001). Systemic oxygen delivery decreased from 727 +/- 354 to 655 +/- 325 ml/min per m2 (p = 0.02). One patient required reopening of the atrial septal defect. CONCLUSIONS: These data demonstrate that a controlled right to left atrial shunt improves cardiac output and systemic oxygen delivery and facilitates the postoperative management of patients after the Fontan procedure. Atrial septal defect closure increases systemic saturation to normal values and prevents potential systemic embolization but significantly decreases oxygen delivery and might limit exercise tolerance.

Adolescent↗
PubMed1998

[Anesthetic management for Fontan procedure without the use of cardiopulmonary bypass].

We examined the anesthetic management for Fontan procedure performed without the use of cardiopulmonary bypass (Group N, n = 7) and that for equivalent procedure under cardiopulmonary bypass (Group E, n = 10) retrospectively. In Group N, surgical repairs of major vascular system were performed while bypassing the superior or inferior vena cava to the right atrium. The use of anesthetics and vasoactive agents was similar in both groups. Patients in Group N had significantly less blood loss and were extubated significantly earlier than those in Group E. However, significant metabolic acidosis was noted in Group N when reconstruction of the vascular system was completed and so-called Fontan circulation was initiated. Fontan procedure without the use of cardiopulmonary bypass may have advantage of less impairment for the cardiac performance and the pulmonary vasculature. However, its anesthetic management is another challenge to the anesthesiologist and requires meticulous control of both optimum preload and vascular resistance of the pulmonary artery.

Anesthesia, General↗
PubMed1999

Abnormalities in liver function and coagulation profile following the Fontan procedure.

OBJECTIVE: To investigate liver function and coagulation disorders in patients with a Fontan circulation at different time intervals after surgery. DESIGN: Retrospective analysis of clinical data and cross sectional study relating liver function and coagulation profile to time since surgery, in 28 surviving patients after the modified Fontan procedure. PATIENTS: 20 patients (71%) with atriopulmonary anastomosis, seven (25%) with atrioventricular anastomosis, and one (4%) with total cavopulmonary connection. Follow up ranged from 2.0 to 21.8 years (mean 11.1). RESULTS: Abnormal liver function tests, mainly reflecting cholestasis, were present in 21 patients who had a significantly longer follow up (p < 0.01). Protein synthesis was normal in almost all patients. Coagulation profile showed abnormalities in 22 patients. "Procoagulant" abnormalities-that is, decreased plasminogen and protein C activity-were found in 11 and five patients, respectively. The extent of these abnormalities was less in patients with a longer follow up. Anticoagulant abnormalities were factor V deficiency in 16 patients and factor VII deficiency in 17, resulting in a prolonged prothrombin time in 19 patients. Thirteen patients had both pro- and anticoagulant abnormalities. A prethrombotic state was present in five patients, with a significantly longer mean time interval since surgery (p = 0.05). Thus, although the individual procoagulant indices decreased with increasing time intervals since surgery, a prethrombotic state was found particularly in patients with a long term follow up. CONCLUSIONS: Mild cholestasis was mainly present in Fontan patients with a long duration of follow up. Along with laboratory procoagulant abnormalities indicating a prethrombotic state, anticoagulant abnormalities were also present. The coagulation profile varied at different time intervals after surgery. Thus detailed evaluation should be performed regularly, and the use of anticoagulants should be considered in every patient. Long term prospective studies are needed to evaluate the individual fluctuations of coagulation profile over time following a Fontan procedure.

Adolescent↗
PubMed1982

Rest and exercise hemodynamics after the Fontan procedure.

Sixteen consecutive patients (12 with tricuspid atresia) underwent a Fontan procedure, with no operative deaths. There were three late deaths. Eleven of the survivors were electively catheterized 4-25 months postoperatively. Rest and exercise hemodynamics were measured in five patients, and resting hemodynamics alone were measured in three. All exercised patients were New York Heart Association class I Cardiac index was low at rest (2.3 +/- 0.61/min/m2) and during exercise (4.9 +/- 1.11/min/m2) due to a low stroke index both at rest (28 ml) and exercise (35 ml). Accordingly, mixed venous oxygen saturations were decreased (66% at rest and 31% during exercise). These values are significantly lower than those at rest and during exercise from 23 control patients of similar age and size. Heart rates, pulmonary vascular resistances, and left ventricular filling pressures appeared normal both at rest and during exercise. High right atrial pressure at rest (15 mm Hg) was associated with minimal conduit gradient (2 mm Hg). However, exercise increased the mean conduit gradient to 8 mm Hg, demonstrating significant functional conduit obstruction. As expected, the cardiovascular response to exercise is abnormal after the Fontan procedure, even in asymptomatic patients. This abnormal response may be exacerbated by conduit obstruction, and conduit obstruction may not be apparent during resting studies.

Adolescent↗
PubMed2006

Relevance of the measurement of the concentration of lactate in the serum subsequent to the Fontan procedure in small children.

BACKGROUND: To determine the clinical relevance of change in the level of lactate in the serum as a meanings of establishing the efficacy of the circulation immediately after the Fontan procedure in small children. METHODS: We measured the concentration of lactate in the serum consecutively in 30 patients undergoing the Fontan procedure, without fenestration, under the age of 2 years. Of these, we did not use cardiopulmonary bypass in 13. RESULTS: Concentrations gradually increased during the first several hours after establishment of the Fontan circulation, and then eventually decreased, reaching the normal range within 48 hours, with specific values of 0.9 plus or minus 0.3 pre-operatively, 2.3 plus or minus 1.1 immediately after creation of the Fontan circulation, 4.0 plus or minus 2.4 at 6 hours, 1.6 plus or minus 0.6 at 24 hours, and finally 1.3 plus or minus 0.4 millimoles per litre at 48 hours. This trend was irrespective of use or no use of cardiopulmonary bypass. The higher initial level in the intensive care unit was related to higher systemic venous pressure, higher transpulmonary pressure gradient, higher maximal level of alanine transaminase and blood urea nitrogen, and longer duration of peritoneal drainage, the latter as a monitor of sequestration of fluid rather than peritoneal dialysis. In patients with initial levels greater than 3.0 millimoles per litre, there was extended duration of endotracheal intubation. CONCLUSIONS: Postoperative changes in the levels of lactate in the serum subsequent to creation of the Fontan procedure were peculiar, not necessarily correlated with the cardiac output, but relevant to the clinical course.

Biomarkers↗
PubMed1981

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.

Adolescent↗