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PubMed1987

Intermediate follow-up and predicted survival after the modified Fontan procedure for tricuspid atresia and double-inlet ventricle.

We reviewed the records of 135 patients with tricuspid atresia and 120 patients with double-inlet ventricle who underwent modified Fontan procedure from 1973 to June 1985. This represents 67% of the total number of patients undergoing the procedure during this period. There were 32 (12%) in-hospital deaths (14 tricuspid atresia, 18 double-inlet ventricle) and 23 late deaths (13 tricuspid atresia, 10 double-inlet ventricle). For patients surviving to hospital dismissal, the 5 year survival was 87%. Follow-up data (mean 3.9 years) were obtained for 157 patients who survived at least 1 year after operation. At last follow-up, of the 157 patients, 92% were in NYHA class I or II, 92% claimed to be improved clinically by the operation, and 98% could work or attend school. Fifty percent were not taking medication at last follow-up. Some degree of edema or ascites was found in 16%. The study showed that (1) the modified Fontan procedure can be performed with low in-hospital mortality (12%) in patients with tricuspid atresia and double-inlet ventricle, (2) hospital survivors have an estimated 5 year survival of 87%, (3) subjectively, functional status is good for hospital survivors at intermediate follow-up, and (4) fluid retention continues to be a problem in a minority (16%) of patients.

Blood Vessel Prosthesis↗

PubMed1991

[Modified Fontan procedure, is the right atrial pump function necessary or not? Experimental approach using simulation model].

The effect of pulsatile pulmonary flow and the characteristics of pulmonary arterial pressure (PAP) trace were examined using a model simulating the right heart following the modified Fontan procedure. An inlet overflow tank (preload), valveless axial pulsatile pump, Windkessel model (afterload), and an outlet overflow tank (left atrium) were connected in series. Standard conditions were: flow 2.00 l/m, with 12 mmHg preload, 3.0 wood units resistance and left atrial pressure at 6 mmHg. The pump rate was set at 80 bpm. Simulated PAP and pulmonary flow (PF) waves produced by this model closely resembled those obtained from patients who had undergone the modified Fontan procedure. Parameters except preload were fixed and changes in PF were examined at varying preload of 8, 12, 15 and 17 mmHg. At the various preload levels, PF increased to values greater than the PF for the same conditions in the non-pulsatile state. With a preload value of 8 mmHg, PF increased from 1.10 l/m (non-pulsatile) to a maximum of 1.55 l/m at a peak PAP of 14 mmHg. With a preload of 12 mmHg, the PF increased from 2.00 l/m (non-pulsatile) to 2.65 l/m at 19 mmHg peak PAP. A preload of 15 mmHg resulted in an increase in PF from 2.65 l/m (non-pulsatile) to 3.30 l/m at 22 mmHg peak PAP, and when the preload was 17 mmHg the PF increased from 3.15 l/m (non-pulsatile) to 3.80 l/m at 25 mmHg peak PAP. The afterload of this model was fixed so the increase in PF with pulsation suggests a decrease in resistance.(ABSTRACT TRUNCATED AT 250 WORDS)

Atrial Function, Right↗

PubMed1994

Early and intermediate results of the Fontan procedure at moderately high altitude.

At higher elevations, alveolar hypoxia increases pulmonary vascular resistance and may limit the cardiac output of individuals without a subpulmonary ventricle. Thus, we reviewed the outcome of definitive palliation for tricuspid atresia and other forms of single ventricle in 60 consecutive Fontan patients living at a mean elevation of 1,370 m (range, 910 to 2,130 m). There were four early deaths (6.7%; 70% confidence limits, 4.1% to 10.7%) and six late deaths. Kaplan-Meier actuarial survival (+/- standard error) is 79.6% +/- 8.2% at 5 years. Survival was significantly decreased in patients with a preoperative mean pulmonary arterial pressure greater than or equal to 15 mm Hg unless the Fontan procedure was performed with a residual fenestration. Exercise tolerance was significantly worse at higher elevations in a subgroup of patients who have traveled to altitudes of 1,680 to 3,350 m. We conclude that the early and intermediate results of the Fontan procedure at moderately high altitude are similar to results reported at sea level. However, exercise tolerance may be impaired at higher elevations in many patients.

Adolescent↗

PubMed2000

Evolution of the Fontan procedure in a single center.

BACKGROUND: Surgical approaches to single ventricle variants include staged, fenestrated, and completed Fontan operations. This study compares outcomes with these modifications of the Fontan operation at a single center. METHODS: Preoperative risk factors and operative results were analyzed by multivariate techniques in 129 patients undergoing modified Fontan operations since March 1988. RESULTS: Overall early and late mortality was 5.4% and 0.8%, respectively. Before 1993, completed Fontan operation using right atrial to pulmonary artery anastomosis without fenestration was performed in the majority of patients (44 of 58; 76%). During this period, 10 of 17 patients at high risk had completed Fontan with three takedowns. In 1994, the staged hemi-Fontan and modified Fontan with a lateral tunnel anastomosis and with or without small fenestration (2.5 to 4 mm) were introduced. The majority of patients at high risk during this period underwent hemi-Fontan followed by fenestrated Fontan with no takedowns. Late atrial dysrhythmias occurred in 6 patients (4.7%), generally with larger fenestrations or right atrial to pulmonary anastomoses. Three patients (2.3%) had a stroke, 2 with large (> or = 4 mm) fenestrations. Of 38 fenestrations, 32 (84%) closed spontaneously by 1 year. No protein-losing enteropathy occurred. Most patients (118 of 121) were in New York Heart Association class I/II 4.5 years postoperatively. By multivariate analysis, only Down's syndrome (p < 0.001) predicted early mortality, whereas both Down's syndrome and a systemic right ventricle decreased late survival (p < 0.006). CONCLUSIONS: Proper selection of patients for modifications of the Fontan procedure resulted in excellent early and late survival with a low incidence of atrial dysrhythmia and stroke. Midterm functional outcomes were excellent.

Adolescent↗

PubMed2002

[Extracardiac fontan procedure in adult: report of a case].

We report a case of a 28-year-old female who underwent an extracardiac Fontan procedure. The subject was diagnosed as an atrioventricular septal defect (Rastelli classification: type C), a double outlet right ventricle, pulmonary artery stenosis, a hypoplasty of left ventricle, total anomalous venous return (Darling: Ib + IIb), and atrial flutter. She underwent a Blalock shunt and an aorto-pulmonary shunt at the ages of 3 and 9 years, respectively. Under a total CPB, an extracardiac total cavo-pulmonary connection (TCPC), using a 26 mm Hemashield graft, was completed. The postoperative course was uneventful. The complicated atrial anatomy and atrial arrhythmia indicated TCPC in this adult patient.

Adult↗

PubMed2001

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.

Adolescent↗

PubMed1997

Update on the modified Fontan procedure.

There have been multiple modifications for surgical palliation of functional single ventricle since the initial report by Fontan and Baudet in 1971. A modified Fontan procedure has been offered to patients with a variety of complex cyanotic heart disease at younger ages, and has resulted in decreased mortality. Reducing morbidity continues to be a challenge. These issues are multiple and include effusions, arrhythmia, ventricular function, exercise ability, progressive cyanosis from pulmonary arteriovenous malformations, systemic to hepatic venovenous malformations or atrial level shunting, thromboembolism, and protein-losing enteropathy. This article reviews clinical contributions published in the past year.

Child, Preschool↗

PubMed2002

Trans-aortic His bundle ablation with permanent ventricular pacing via the coronary sinus in L-transposition of great arteries with classic Fontan procedure.

We report findings in a patient with 1-transposition of great arteries with single ventricle and classic Fontan procedure who required both His bundle ablation for palliation of refractory atrial tachycardia and placement of a transvenous dual-chamber atrio-ventricular permanent pacemaker for hemodynamic support. A method for retrograde trans-aortic His bundle ablation in this congenital anomaly and subsequent placement of permanent dual chamber pacemaker using the right atrium and coronary sinus as an access to the ventricle is described.

Bundle of His↗

PubMed1998

Sinus node function after a systematically staged Fontan procedure.

BACKGROUND: Sinus node dysfunction has been previously reported to occur in 13% to 16% of patients after the Fontan operation. Although there is concern that an intermediate cavopulmonary connection may increase the risk of sinus node dysfunction, previous studies have not reported on patients routinely staged to a Fontan operation. This study sought to determine the early and late incidences of sinus node dysfunction in patients systematically and uniformly staged to a Fontan operation after a prior hemi-Fontan. METHODS AND RESULTS: To determine the early incidence of sinus node dysfunction, hospital records and perioperative ECGs were reviewed in all 287 patients having had a staged Fontan operation between January 1990 and December 1995. A cross-sectional analysis was performed on 220 of 239 surviving patients (92%) to determine the late incidence of sinus node dysfunction. Sinus node dysfunction was present in 7% of the patients before and in 15% after the hemi-Fontan. Although most patients (81%) regained normal sinus node function between the 2 stages, 23% had sinus node dysfunction in the early postoperative period after the Fontan. Of the 95 patients followed for > 4 years after the Fontan operation, 44% had sinus node dysfunction. However, at a mean follow-up of 3.5 +/- 1.7 years, only 16 patients (6.7%) had received a pacemaker and only 10 (4.1%) had documented atrial flutter. CONCLUSIONS: Perioperative sinus node dysfunction is common after both the hemi-Fontan and the Fontan procedures. Although many patients regain sinus node function between the 2 stages, late sinus node dysfunction is common and more likely to occur in patients with early sinus node dysfunction and those with longer follow-up.

Adolescent↗

PubMed1992

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.

Adolescent↗

PubMed1996

[Continuous systemic venous oxygen saturation monitoring immediately after Fontan procedure].

Changes in cardiac output (CO), systemic venous oxygen saturation (SvO2), systemic oxygen consumption, and urinary output immediately after Fontan procedure were measured in 10 patients at the intensive unit (ICU) to assess the effects of aorusal from anesthesia, hypothermic management, and respiratory condition. The measurements were taken at the following phases; phase A in deep sedation under hypothermia (33-35 degrees C rectal temperature) and controlled ventilation; phase B in mild sedation under normothermia and controlled ventilation; phase C when awake under normothermia and assisted ventilation; phase D when awake under assisted ventilation; phase D when awake under normothermia immediately after extubation; and phase E 24 hours after extubation. Oxygen delivery (O2 Del.) and fractional oxygen fractions were calculated in each phase. Two patients whose SvO2 values were below 55% during the postoperative course needed reoperation for atrioventricular valve regurgitation in one case for PV stenosis in the other case. CO increased significantly (p < 0.05) after extubation (phase D), compared with that of controlled ventilation (phase B). Under hypothermia (phase A), urinary output was relatively higher with lower CO. There was a significant correlation between SvO2 and CO (R = 0.61) in phase A, however there was no correlation in phase E. Fractional oxygen extraction in phase A was significantly lower than in phase B. In conclusion, the continuous SvO2 measurements reflected real-time changes in cardiac output in the immediate post-Fontan patients. Induced hypothermia was beneficial in increasing urinary output presumably through the correction of maldistribution of cardiac output in post-Fontant patients. Arousal from anesthesia and spontaneous ventilation seemed advantageous for increasing cardiac output, hence, early extubation should be encouraged in the management of post-Fontan patients.

Blood Gas Monitoring, Transcutaneous↗

PubMed1994

Prolonged high frequency jet ventilation and milrinone therapy following modified Fontan procedure.

An 11-year-old boy who underwent a modified Fontan procedure required surgical re-exploration the next day. Profound cardiogenic shock developed and he required high frequency jet ventilation and milrinone therapy for 15 days. After 11 days of high frequency jet ventilation he developed a tracheal mucous plug leading to a hypoxic cardiac arrest from which he was successfully resuscitated.

Cardiotonic Agents↗

PubMed2000

Right-to-left shunt through the cardiac veins after the Fontan procedure.

We found right-to-left shunts through the cardiac veins postoperatively in 2 patients who had undergone the Fontan procedure. In one of the patients, channels were present through the cardiac veins independent of the coronary sinus. In the other patient, an atretic orifice for the coronary sinus, coupled with a persistent left-sided superior caval vein, complicated the postoperative course.

Blood Gas Analysis↗

PubMed2000

Plastic bronchitis occurring late after the Fontan procedure: treatment with aerosolized urokinase.

OBJECTIVE: To describe the use of aerosolized urokinase in a patient with plastic bronchitis after a Fontan procedure. DESIGN: Case report. SETTING: Pediatric intensive care unit in a university-affiliated children's hospital. PATIENTS: Report of one patient with acute respiratory failure secondary to plastic bronchitis. INTERVENTIONS: Aerosolized urokinase, multiple bronchoscopies, corticosteroids, mucolytics, bronchodilators, and atrial pacing. MEASUREMENTS AND MAIN RESULTS: Airway obstruction secondary to recurring casts improved with the treatments. Histologic analysis of the casts demonstrated less fibrin after treatments with aerosolized urokinase. No adverse events were noted. CONCLUSIONS: The addition of aerosolized urokinase to this patient's treatment regimen helped to resolve life-threatening airway obstruction secondary to fibrin casts.

Acute Disease↗

PubMed1983

Modified Fontan procedure in patients with previous ascending aorta-pulmonary artery anastomosis.

The Fontan procedure has proved useful in the surgical treatment of patients with tricuspid atresia, univentricular heart, and other complicated cardiac anomalies. Previous palliation of these patients with ascending aorta-right pulmonary artery anastomosis (Waterston shunt) may impose severe restrictions in the operability of these patients because of severe distortion, narrowing, and scarring of the proximal right pulmonary artery. A modification of the Fontan operation is described in which repair is facilitated by transecting the right pulmonary artery at the site of anastomosis, trimming away the distorted narrowed segment, and anastomosing the resulting cuff to the lateral aspect of the superior vena cava. The right atrial appendage is anastomosed to the rudimentary right ventricle or the main pulmonary artery directly or via a tubular Dacron graft. The technique has been applied successfully in four consecutive cases.

Adolescent↗

PubMedDate not supplied

Modified Fontan procedure for left atrial isomerism: alternative technique.

Anomalous systemic or pulmonary venous connections increase the risk and technical difficulty of the modified Fontan procedure. This report describes an alternative technique of total diversion of systemic venous return to the pulmonary artery in a child with left atrial isomerism, incorporating an extracardiac conduit between the hepatic veins and the right pulmonary artery.

Double Outlet Right Ventricle↗

PubMed2004

Endocardial atrial pacing lead implantation and midterm follow-up in young patients with sinus node dysfunction after the fontan procedure.

The purpose of the study was to investigate the results of endocardial lead implantation, lead performance, and follow-up in young patients after the Fontan procedure. A retrospective study was conducted with patients who had endocardial atrial pacing for SND and intact AVN function after Fontan from two pediatric centers. Patient demographics, pacing, and sensing data of endocardial atrial leads were analyzed at the time of pacemaker implantation and follow-up visits. Fifteen patients (weight 42.6 +/- 35 kg) had transvenous endocardial atrial lead implantation at an average age of 11.4 +/- 6.5 years. Active-fixation leads were used in all patients and steroid elution was present in 12 (80%) patients. Adequate P wave sensing was obtained in patients with sinus rhythm (n = 10); the remaining four patients had junctional rhythm without measurable P waves. Lead failure was not observed in any patient during the follow-up period of 2.9 +/- 2.1 years. The energy threshold at implantation was 1.46 +/- 1.5 microJ, 1.54 +/- 0.75 microJ at 3 months, 0.62 +/- 0.45 microJ at 1 year, 0.72 +/- 0.65 microJ at 2 years, 0.75 +/- 0.55 microJ at 3 years, and 0.8 +/- 0.85 microJ at 5 years postimplant. The lead impedance was 648 +/- 298 omega at implantation, 714 +/- 163 omega at 3 months, 744 +/- 195 omega at 1 year, 734 +/- 198 omega at 2 years, 800 +/- 142 omega at 3 years and 830 +/- 200 omega 5 years postimplant. Anticoagulation therapy (aspirin n = 5, warfarin n = 8) was continued by 13 patients. Complications consisted of a pneumothorax at implantation and a transient ischemic attack in one patient 4 years after pacemaker implant. Endocardial atrial leads offer low energy thresholds and can be implanted relatively safely in Fontan patients.

Adolescent↗

PubMed2006

Extracardiac Fontan procedure bridging the vertebra for apico-caval juxtaposition.

For 5 patients with univentricular heart associated with apico-caval juxtaposition, an extracardiac Fontan procedure was carried out using an artificial graft bridging the vertebra to avoid graft compression by the vertebra and the ventricle. For 2 patients representing nonconfluency between the inferior caval vein and the hepatic vein, a hand-made, shoe-tree graft was used. Postoperatively all patients are doing well without a stenotic venous pathway. This extracardiac operation using an artificial graft bridging the vertebra may be advantageous for univentricular heart associated with apico-caval juxtaposition to prevent a postoperative stenotic venous pathway.

Adolescent↗