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PubMed1993

Changes in right ventricular geometry and heart rate early after hemi-Fontan procedure.

To document and quantitate changes in right ventricular (RV) geometry and heart rate, we prospectively examined 35 consecutive patients with hypoplastic left heart syndrome under steady-state conditions (chloral hydrate sedation) before and after a bidirectional cavopulmonary anastomosis (hemi-Fontan) procedure. Right ventricular end-diastolic volume (RVEDV) was calculated as the product of RV cavity areas in two orthogonal planes divided by RV maximal length in either plane. After the hemi-Fontan procedure, RVEDV decreased by 33% from 33 +/- 13 to 22 +/- 11 mL (mean +/- standard deviation). Indexed RVEDV decreased from 86 +/- 37 to 57 +/- 28 mL/m2. The RV wall thickness at the diaphragm in subcostal frontal view (RVWD) increased by only 11% from 8 +/- 0.2 to 9 +/- 0.2 mm (p = not significant), but RVWD/RVEDV increased by 111% from 0.36 +/- 0.22 to 0.76 +/- 0.69 mm/mL (p = 0.002). The RV anterior wall thickness in subcostal sagittal view (RVWA) increased by only 13% from 7 +/- 0.2 to 8 +/- 0.2 mm (p = not significant), but RVWA/RVEDV increased by 103% from 0.31 +/- 0.20 to 0.63 +/- 0.54 mm/mL (p = 0.002). In 11 of 35 patients (31%), resting heart rate did not change (118 +/- 14 versus 108 +/- 9 beats/min; p = not significant); however, in 24 of 35 patients (69%), heart rate increased significantly (108 +/- 9 versus 127 +/- 10 beats/min; p = 0.05). In conclusion, RV wall thickness is high before the hemi-Fontan procedure and increases slightly in the first postoperative week.(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomosis, Surgical↗
PubMed1991

Hypoplastic left heart syndrome: hemodynamic and angiographic assessment after initial reconstructive surgery and relevance to modified Fontan procedure.

After undergoing initial reconstructive surgery for hypoplastic left heart syndrome performed between August 1985 and March 1989, 59 patients (age range 3 to 27 months, mean 13.8 +/- 4.5) underwent elective cardiac catheterization in anticipation of a modified Fontan procedure. Five important hemodynamic and anatomic features considered to be components of successful reconstructive surgery were specifically addressed. 1) Interatrial communication: Only two patients had a measured pressure difference of greater than 4 mm Hg across the atrial septum. 2) Tricuspid valve function: Angiography demonstrated significant tricuspid valve regurgitation in only five patients (moderate in two and severe in three). 3) Aortic arch: Pressure tracings from the right ventricle to the descending aorta revealed a gradient greater than 25 mm Hg in only two patients. 4) Pulmonary vasculature: Ten patients had a calculated pulmonary vascular resistance greater than 4 U.m2; 51 (86%) of the 59 patients had no evidence of distortion (stenosis or hypoplasia) of either the left or the right pulmonary artery. 5) Right ventricular function: Five patients had an end-diastolic pressure in the right ventricle greater than 12 mm Hg and two patients had qualitative assessment of decreased ventricular function. Comparison of catheterization data between survivors and nonsurvivors of the subsequent modified Fontan procedure showed that only significant tricuspid regurgitation is a possible predictor of poor outcome. After first stage reconstructive surgery for hypoplastic left heart syndrome, most survivors have favorable anatomy and hemodynamics at follow-up cardiac catheterization for a subsequent Fontan procedure.

Aorta, Thoracic↗
PubMed1992

[A successful two-staged Fontan procedure for tricuspid atresia with bilateral superior venae cavae and left pulmonary arterial stenosis].

A 10-year-old boy with tricuspid atresia (type I-b), bilateral superior venae cavae and left pulmonary arterial branch stenosis was treated successfully by a two-staged Fontan procedure. At 7 years of age, left Glenn shunt was performed after creation of a communicating vessel between the right and left superior vena cava, using a GORE-TEX graft of 10 mm of diameter. Three years later, after having confirmed graft patency, Fontan procedure was achieved without any problems. We think the innominate vein creation by an artificial material was a key design for the following successful two-staged Fontan procedure in this case.

Blood Vessel Prosthesis↗
PubMed2001

Double inlet right ventricle versus other types of double or common inlet ventricle: its clinical characteristics with reference to the Fontan procedure.

OBJECTIVE: To determine the clinical features in patients with double inlet right ventricle (DIRV) and separate atrioventricular (AV) valves in terms of the Fontan procedure. METHODS: Surgical procedures were carried out in 31 patients with this rare malformation. Their clinical characteristics were compared with similar features in 45 patients with double inlet left ventricle (DILV), 93 with common inlet right ventricle (CIRV), and 20 with common inlet left ventricle (CILV). RESULTS: Pulmonary atresia with the right and the left pulmonary arteries being confluent was seen in 58% of patients with DIRV, and pulmonary atresia with non-confluent pulmonary arteries in 15% (P=0.0001). The incidence of these findings was significantly higher when compared with DILV (P=0.0001). Reflecting these morphologic features, constructions of systemic-to-pulmonary shunts and extensive plasty to the pulmonary arteries were more frequently needed in patients with DIRV than in the others (P=0.04, 0.0001). The AV valves have become moderately or severely regurgitant in three with DIRV (10%). The Fontan procedure has thus been employed in 21 with DIRV (64%), this incidence being significantly higher than that seen in the CIRV (37%) group (P=0.01). Survival rate of 95% at 10 years in this group was as excellent as that in the DILV group, and obviously better than that in either CIRV or CILV (P=0.002). CONCLUSIONS: Although multiple palliative procedures might be frequently needed to maintain pulmonary perfusion, the Fontan circulation can be justifiably established in patients with DIRV, and regurgitation across the AV valves was not very common.

Child↗
PubMed1990

Plasma atrial natriuretic peptide after the Fontan procedure and total cavopulmonary connexion.

Plasma atrial natriuretic peptide was measured in 10 children undergoing the Fontan procedure and 3 children undergoing total cavopulmonary connexion. There was no significant difference in pre-operative plasma levels, but post-operative levels were significantly higher 48 hours after cardiopulmonary bypass in the Fontan group. There was no significant difference in plasma arginine vasopressin levels either pre- or post-operatively. Post-operative pleural effusions occurred in only 2 of the 10 patients undergoing the Fontan procedure, but were present in all 3 of those undergoing total cavopulmonary connexion. The release of atrial natriuretic peptide is an appropriate homeostatic response to volume loading and the impairment of this response in the early post-operative period may be of clinical importance.

Arginine Vasopressin↗
PubMed1990

Assessment of the intrapulmonary ventilation-perfusion distribution after the Fontan procedure for complex cardiac anomalies: relation to pulmonary hemodynamics.

In 12 patients who underwent the Fontan procedure for complex cardiac anomalies, lung scanning with xenon-133 was performed to assess the intrapulmonary ventilation-perfusion distribution, and comparison was made with a control group. All data were then analyzed in relation to either pre- or postoperative pulmonary hemodynamic data. In ventilation scans, the intrapulmonary distribution in the right lung was almost normal. In perfusion scans, an abnormal increased upper to lower lobe perfusion ratio greater than the normal value found in the control group was noted in seven patients (58.3%). There was a significant correlation (p less than 0.02) between the upper to lower lobe perfusion ratio and postoperative pulmonary vascular resistance. Furthermore, this perfusion ratio correlated inversely with the preoperative (p less than 0.005) and postoperative (p less than 0.02) right pulmonary artery area index, defined as the ratio of cross-sectional area to the normal value. Of five patients with less than 90% arterial oxygen saturation, four showed an abnormal distribution of pulmonary blood flow greater than the normal perfusion ratio. No patient had evidence of a pulmonary arteriovenous fistula by the echocardiographic contrast study. These results suggest that abnormal distribution of pulmonary blood flow to the upper lung segment may develop in patients after the Fontan procedure, and that insufficient size of the pulmonary artery before operation and the consequent postoperative elevation of pulmonary vascular resistance may be responsible for this perfusion abnormality.

Child↗
PubMed1991

Total cavopulmonary anastomosis versus conventional modified Fontan procedure.

The total cavopulmonary anastomosis, lateral tunnel Fontan, has been advocated as a preferred method for Fontan type repair. From 1987 to July 1990, 39 patients underwent total cavopulmonary anastomoses (group 1) and 39 patients underwent modified Fontan procedures (group 2); patients receiving adjustable atrial septal defects were excluded. Diagnoses in group 1 included tricuspid atresia in 5 patients, single ventricle in 32, and pulmonary atresia and intact ventricular septum in 2. Diagnoses in group 2 included tricuspid atresia in 20, single ventricle in 17, hypoplastic left heart syndrome in 1, and pulmonary atresia and intact ventricular septum in 1. There were no significant differences in age, weight, cross-clamp time, duration of inotropic support, postoperative effusions, or hospital stay between the two groups. Early mortality in group 1 was 7.7% (3/39) and in group 2, 2.6% (1/39). There was no difference in the incidence of early dysrhythmias or early pacemaker placement. Late mortality was 2.8% in group 1 and 8% in group 2 with a mean follow-up of 18 and 25 months, respectively. Follow-up in group 1 revealed 33 patients in normal sinus rhythm and 1 patient with episodes of supraventricular tachycardia; no additional patients have required pacemakers. Follow-up in group 2 revealed 27 patients in normal sinus rhythm and supraventricular tachycardia in 4 patients; 5 additional patients have required pacemaker placement. There is no apparent difference in early outcome between the total cavopulmonary anastomosis and the conventional modified Fontan. However, there appears to be an increased incidence of late dysrhythmias and the need for pacemaker placement in the conventional modified Fontan group compared with the lateral tunnel group.

Adolescent↗
PubMed2002

Staged Fontan procedure for mitral atresia associated with severe tricuspid regurgitation, pulmonary hypertension, and pulmonary artery distortion.

Optimal initial palliation and a subsequent staged approach is mandatory for high-risk Fontan candidates. We describe the case of mitral atresia with severe tricuspid regurgitation and pulmonary hypertension successfully managed by repeated palliation from the neonatal period and 2-stage Fontan surgery. A 1-month-old boy diagnosed with mitral atresia and double-outlet right ventricle underwent pulmonary artery banding at 1 month of age, followed by repeated pulmonary artery banding accompanied by tricuspid annuloplasty and atrial septal defect enlargement at 6 months. Because of the presence of pulmonary artery distortion, right ventricular dysfunction, and borderline pulmonary vascular resistance, a hemi-Fontan procedure was conducted with extended pulmonary artery plasty when the boy was 3 years and 8 months old. Cardiac catheterization done 3 months after showed improvement in risk factors, and the final Fontan operation (total cavopulmonary connection) was successfully done in conjunction with repeated tricuspid annuloplasty when the boy was 4 years and 5 months old. The patient remains in excellent clinical condition at the last follow-up 5 years after the final Fontan procedure with sinus rhythm and good ventricular function.

Fontan Procedure↗
PubMed1997

Pulmonary atresia with intact ventricular septum: results of the Fontan procedure.

BACKGROUND: Children with pulmonary atresia and an intact ventricular septum show a heterogeneous spectrum of cardiac anomalies. A biventricular repair is attainable in some; a Fontan procedure or a one-and-a-half ventricle is the only possible repair for others. Children with right ventricle-to-coronary artery connections, with or without right ventricle-dependent coronary artery blood flow, are a high-risk group. METHODS: Between May 1980 and December 1994, 22 children underwent a Fontan operation for the treatment of pulmonary atresia with an intact ventricular septum at The Hospital for Sick Children, Toronto. The mean age was 5.8 years (median, 4.9 years). All children had had at least one pre-Fontan palliative procedure; 19 had two, and 7 of these had three or more. Right ventricle-to-coronary artery connections were present in 15 children, including 5 with right ventricle-dependent coronary artery blood flow. Thromboexclusion of the right ventricle was done in 10 children, with 7 undergoing it before and 3 at the time of the Fontan procedure. RESULTS: There were three early deaths (13.6%) and one late death. The actuarial survival at 10 years after the Fontan operation was 80%. Early postoperative complications occurred in 4 children. Follow-up was completed in all children at a mean of 4 years (range, 1 to 12.5 years) after the Fontan operation. Atrial arrhythmia occurred in 3 children, and permanent pacemakers were required in 4. CONCLUSIONS: Results of the Fontan operation for the treatment of pulmonary atresia with an intact ventricular septum are satisfactory. Thromboexclusion of the right ventricle is indicated in the presence of right ventricle-to-coronary artery connections without right ventricle-dependent coronary artery blood flow. The right ventricle should not be decompressed or thromboexcluded in children with right ventricle-dependent coronary artery blood flow, and at the Fontan operation, saturated blood must enter the right ventricle.

Child, Preschool↗
PubMed1997

The Fontan procedure for pulmonary atresia with intact ventricular septum: operative and late results.

OBJECTIVES: The goals of the study were to evaluate the operative and late mortality associated with the Fontan procedure in patients with pulmonary atresia and an intact ventricular septum and to obtain follow-up information on the current clinical status of surviving patients. BACKGROUND: Between 1979 and October 1, 1995, 40 patients with the anomaly had a nonfenestrated Fontan procedure performed at the Mayo Clinic. Because there are no previously published reports involving a series of this size in which the Fontan approach was used for this condition, a review of patient outcomes was thought to be of value. METHODS: The medical records of the 40 patients were reviewed retrospectively, and 34 were determined to be alive. The status of the survivors as of late 1995 was then ascertained by direct examination, questionnaire or telephone follow-up. RESULTS: There were three operative deaths and three late deaths. The current ages of the 34 survivors ranged from 4 to 30 years (median 13). Thirty-three of the 34 survivors were thought to be in New York Heart Association functional class I or II, and all but three of these patients, of school age or older, were either full-time students or working full time. The three adults who were not employed thought they were capable of working but were not doing so because of socioeconomic reasons. More than half of the patients were not receiving cardiovascular medications. CONCLUSIONS: These overall gratifying early and late results encourage continued application of this operation for appropriately selected patients with this complex congenital cardiovascular anomaly.

Adolescent↗
PubMed1990

[A right atrial thrombus after Fontan procedure--a case report].

A 17-year-old male with tricuspid atresia who underwent a modified Fontan procedure died due to heart failure 4 years and 6 months after operation. At autopsy a thrombus was found in the right atrial appendage. From the experience, it might be recommended to excise the appendage when the modified Fontan procedure leaving the appendage was employed. In addition, anticoagulation therapy as well as regular echocardiographic examination for detecting thrombus formation should be considered after the operation.

Adolescent↗
PubMed1987

Surgical experience with the modified Fontan procedure.

During a 10 year period 109 patients (3 months to 47 years old) underwent modifications of the Fontan procedure for repair of classic tricuspid atresia (TA) (n = 58), univentricular atrioventricular connection (UVH) (n = 38), or other complex malformations (CM) (n = 13). Among patients with TA, an atriopulmonary connection was used in 19 (33%) and incorporation of the right ventricle with the Björk modification and with a right atrial-to-right ventricular valved conduit was used in 20 (34%) and in 19 (33%), respectively. Three of the latter 19 also underwent a combined Fontan-switch procedure. The hospital mortality rate was 13.8% (70% confidence limits, 9.3% to 18.3%) for patients with TA, 28.9% (70% confidence limits, 21.3% to 37.0%) for patients with UVH, and 7.7% (70% confidence limits, 0% to 15.4%) for patients with CM. Multivariate analysis identified with the following variables as risk factors for both early and late deaths: diagnosis of UVH, previous pulmonary artery banding (PAB), and postrepair right atrial pressure, and, in patients with TA, the use of a direct atriopulmonary connection (all variables, p less than .05). Morphometric lung biopsy scores were not different in patients with PAB, implicating the role of ventricular hypertrophy rather than pulmonary vascular disease as the mechanism for the adverse effect of PAB. Right atrial pressure was a predictor of serious late cardiac symptoms, which were present in 10% of eligible patients (p = .032). This review demonstrates a survival advantage with modifications of the Fontan procedure that incorporate the hypoplastic right ventricle in patients with TA.(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗
PubMed2004

Incidence, outcome, and risk factors for stroke after the Fontan procedure.

We reviewed records on patients who underwent hemi-Fontan and Fontan procedures over a 13-year period at a single institution to examine the incidence and outcome of stroke and to attempt to identify risk factors for stroke. A history of pulmonary artery banding was associated with an increased risk of stroke.

Adolescent↗
PubMed1995

Cardiac anatomy in patients undergoing the Fontan procedure.

BACKGROUND: Controversies in nomenclature, especially of hearts with "single ventricle," have clouded discussion and understanding of the anatomy. Many patients with such malformations are submitted to the Fontan procedure as definitive surgical palliation. The spectrum of anatomy among these patients is wide and deserves analysis in an effort to provide a simple framework for description and to eliminate confusion. METHODS: We reviewed 138 successive patients undergoing the Fontan operation at one institution to demonstrate the variability in pathology. RESULTS: Only 89 patients (65%) had a univentricular type of atrioventricular connection. All but 5 patients had two ventricular chambers. Among the 49 patients with biventricular atrioventricular connections, 43 had a hypoplastic ventricle that precluded a biventricular repair. CONCLUSIONS: Full understanding of the malformations that may preclude a "biventricular" repair and hence necessitate a Fontan procedure requires knowledge of the different forms of univentricular atrioventricular connection that, although usually associated with two ventricular chambers, are seldom amenable to a "two-pump repair." Understanding of those types of "biventricular heart" that preclude a two-pump repair (eg, severe hypoplasia of the left ventricle or the right ventricle) or are associated with high risks (eg, straddling atrioventricular valve) is also important.

Fontan Procedure↗
PubMed1995

Modification of the Fontan procedure. Superior vena cava to left pulmonary artery connection and inferior vena cava to right pulmonary artery connection with adjustable atrial septal defect.

BACKGROUND: A modification of the Fontan procedure with unidirectional cavopulmonary connection is described in which the superior vena cava (SVC) is connected to the left pulmonary artery (PA) and the inferior vena cava (IVC) is connected to the right PA via a lateral tunnel with a snare-controlled, adjustable atrial septal defect (ASD). This allows matching of the SVC and IVC flows with the lung of appropriate size. The obligatory left Glenn shunt provides an adequate arterial oxygen saturation, and the elevation in SVC pressure is well tolerated. The adjustable ASD allows selective decompression of the IVC that maintains cardiac output and reduces fluid accumulation in the serous cavities. METHODS AND RESULTS: Since March 1992, we have performed this procedure in 18 patients. There were 17 children and 1 adult. Median age was 3 years and 9 months (range, 13 months to 36 years). Six patients had been staged with a previous bidirectional Glenn shunt. Preoperative cardiac catheterization revealed a PA pressure of 13 +/- 2 mm Hg and a transpulmonary gradient of 5 +/- 3 mm Hg. Ventricular function was satisfactory in all patients. At the completion of bypass, the pressures in the SVC and IVC were 16 +/- 4 mm Hg and 10 +/- 3 mm Hg, respectively (P < .01). The left atrial pressure was 6.0 +/- 3.0 mm Hg and the arterial O2 saturation on 100% oxygen was 93 +/- 3%. There was one death as a result of intractable atrial arrhythmias. The remaining 17 patients had a mean hospital stay of 9.7 days (6 to 18 days). The length of pleural drainage was 7 +/- 3 days. The ASD was adjusted in 11 patients before discharge. Oxygen saturation at discharge was 85.4 +/- 4%. Nine patients had repeat catheterization. The ASD was completely closed in 6 patients, an average of 2.5 months after surgery (range, 3 weeks to 5 months). After ASD closure, the arterial oxygen saturation was 96 +/- 3%, and the SVC and IVC pressures were both 13 +/- 3 mm Hg. CONCLUSIONS: The Fontan procedure with unidirectional cavopulmonary connection and adjustable ASD has several advantages that may reduce mortality and morbidity for the high-risk Fontan candidate.

Adolescent↗
PubMed2003

Procoagulant and anticoagulant factor abnormalities following the Fontan procedure: increased factor VIII may predispose to thrombosis.

OBJECTIVE: Using age-matched controls, this study prospectively evaluated coagulation factor abnormalities and hemodynamic variables in children who had undergone the Fontan operation. METHODS: Coagulation factors were assayed in 20 children (mean age 6.4 +/- 2.9 years), at a mean 3.7 +/- 2.3 years after the Fontan procedure; 24 healthy children (mean age 6.8 +/- 2.8 years) were assayed as controls. Concentration of factors II, V, VII, VIII, IX, X; ATIII; plasminogen; proteins C and S; fibrinogen; serum albumin; and liver enzymes were measured. Normal reference intervals based on the control patients were determined using 95% confidence limits. Patient demographic, hemodynamic variables, and elapsed time after the Fontan procedure were evaluated as possible predictors of coagulation abnormalities. RESULTS: Concentrations of protein C; factors II, V, VII, X; plasminogen; and ATIII were significantly lower in Fontan patients compared with age-matched controls (P <.01); factor VIII was significantly elevated in 6 patients (35%), 2 of whom had a thromboembolic event. A higher superior vena cava pressure was predictive of an elevated factor VIII level (P =.003). No other specific hemodynamic variables were predictive of a procoagulant or anticoagulant abnormality. CONCLUSION: Procoagulant and anticoagulant factor levels were significantly lower in patients after the Fontan operation independent of hemodynamic variables peculiar to the Fontan circulation. Increased factor VIII level requires further evaluation as a cause of thrombosis in patients with Fontan physiology and may also indicate a subset of these patients in whom anticoagulation is indicated.

Adolescent↗
PubMed1990

Effect of right ventricular anatomy on the cardiopulmonary response to exercise. Implications for the Fontan procedure.

Incorporation of the right ventricle (RV) into the pulmonary circulation of patients with tricuspid atresia undergoing a Fontan procedure has been advocated. The consequences of this approach on the exercise function of these patients was studied by examining the effects of progressive and steady-state bicycle exercise tests performed by 11 patients with right atrial (RA)-RV Fontan anastomoses, seven patients with RA-pulmonary artery (PA) Fontan anastomoses, 13 patients after repair of tetralogy of Fallot, and 34 normal control patients. All patients were in New York Heart Association class I. The exercise function of the patients undergoing RA-RV and RA-PA Fontan procedures were similar. The achieved peak work loads 60% and 67% of control and peak oxygen consumptions 60% and 64% of control, respectively. Both groups also displayed excessive ventilation, elevated dead space/tidal volume ratios, and depressed cardiac output during steady-state exercise. In contrast, tetralogy of Fallot patients achieved peak work loads and oxygen consumptions 83% of control and maintained normal cardiac outputs and dead space/tidal volume ratios during steady-state exercise. These results suggest that the presence of an RV within the pulmonary circulation of the Fontan patient does not result in improved exercise function. This may be due to the development of obstructive gradients across the RA-RV conduits during exercise or to the RV's negative effect on left ventricular compliance. Moreover, in contrast with the postoperative tetralogy of Fallot patient, the hypoplastic RV of tricuspid atresia may not have sufficient myocardium to assume the active pumping function required by exercise.

Adolescent↗