Single-stage versus two-stage modified Fontan procedure

We compared surgical outcomes of the single-stage and two-stage modified Fontan procedures to clarify clinical superiority. Of 28 children undergoing a modified Fontan procedure from October 1995 to October 2005, 15 had a 1-stage and 13 had a 2-stage operation. In the 2-stage group, pulmonary artery growth was evaluated before and after the first stage. Operative mortality was 26.6% in the 1-stage group and 0% in the 2-stage group. The benefits of a previous bidirectional Glenn shunt were decreased cyanosis and volume overload, but there was no significant difference in pulmonary artery growth reflected in pulmonary artery indices before and after the bidirectional Glenn procedure. Older children underwent a 2-stage modified Fontan procedure and had better outcomes in terms of lower mortality, improved oxygen saturation, decreased volume load, and less deterioration of atrioventricular valve regurgitation.     

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.     

Outcome of early and late onset Fontan operation in patients with univentricular heart repair

ObjectiveTo evaluate our experience in the Fontan procedure comparing those below and above 6 years of age.MethodsA review of our clinical database was conducted to identify the patients who received extracardiac Fontan between 2002 and 2010. All demographic, echocardiographic, surgical, haemodynamic and follow-up data were collected. The overall mortality was defined as death occurring from the time of surgery to the most recent follow-up. Early postoperative death was defined as death occurring during admission or within 30 days from the operation. Seventy-six patients with functionally univentricular hearts were included in the study. Patients were divided into two groups. Group A included patients who had received extracardiac Fontan at the age of 6 years or less, whereas group B included patients who had received extracardiac Fontan at an age of more than 6 years.ResultsThe overall hospital mortality was 7.9% (10.2% in group A and 5.9% in group B). No statistically significant difference was seen between the two groups regarding the postoperative mortality including thrombosis, stroke, chylothorax, bleeding, pericardial effusion, wound infection, serious postoperative arrhythmias and protein losing enteropathy. On the other hand the mechanical ventilation duration, duration of hospital and ICU stay, duration of the chest tubes and the postoperative saturation was not significant between the two groups.ConclusionsThe age of the patient at the time of Fontan surgery does not affect the results, in terms of both morbidity and mortality.     

Plasma brain natriuretic peptide and systemic ventricular function in asymptomatic patients late after the Fontan procedure

While increased plasma brain natriuretic peptide (BNP) levels have been documented late after the Fontan procedure, its significance remains unclear. We sought to test the hypothesis that plasma BNP levels reflect systemic ventricular function after the Fontan procedure by interrogating the relationship between plasma BNP level and indices of ventricular function. The plasma BNP levels and systemic ventricular function, as determined by conventional and tissue Doppler assessments, acoustic quantification (AQ), and myocardial performance index (MPI), of 35 asymptomatic Fontan patients were compared to those of 34 control subjects who had previous repair of ventricular septal defect. When compared with controls, Fontan patients had significantly higher plasma BNP levels (median 21 pg/ml, range 5–397 vs median 15 pg/ml, range 5–62, P = 0.04). Their systolic and diastolic ventricular function was impaired as evidenced by reduced systemic ventricular early diastolic (E) inflow velocity, early to late (A) diastolic inflow velocity ratio, left and right annular early diastolic (e), late diastolic (a), and systolic (s) velocities, AQ-derived ventricular fractional area change, peak emptying rate, and peak filling rate, and increased AQ-derived atrial filling fraction and MPI (all P < 0.05). Plasma BNP levels correlated negatively with E and A velocities, left-sided annular a velocity, and right-sided annular e, a, and s velocities, and positively with AQ-derived atrial filling fraction (all P < 0.05). In conclusion, our findings suggest that plasma BNP levels reflect primarily the diastolic function of the systemic ventricle in asymptomatic Fontan patients.     

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.     

Transcatheter interventions in the early postoperative period after the Fontan procedure

Objective : To analyze the safety and clinical impact of interventional cardiac catheter procedures in the management of early postoperative problems after completion of an extracardiac Fontan procedure. Background : The mortality after Fontan procedure has consistently decreased over the last decade. The role of interventional catheterization to address early postoperative problems in this setting has not been studied systematically. Methods : Over a 9.7‐year period, 289 patients underwent an extracardiac fenestrated Fontan procedure with two early deaths (0.7%) and takedown in four (1.4%). Twenty‐seven patients (9.3%) underwent 32 interventional cardiac catheter procedures at a median interval of 12.2 (1–30) days. The median weight was 14.5 (13.5–25) kg. The case notes and procedure records were reviewed retrospectively. Results : Fontan pathway obstructions were treated in 11 patients with stent implantation with good results and no complications. Stent fenestration of the Fontan circulation was performed in 16 patients with one episode of transient hemiparesis and one episode of pericardial effusion. Three patients underwent initial balloon dilatation of branch pulmonary arteries or fenestration with little effect and underwent stent treatment 6 (5–9) days later. One patient had device closure of a large atrial fenestration. In one patient, residual anterograde pulmonary blood flow was occluded using a device. There were no deaths and in‐hospital course was improved in all. Conclusion : Interventional cardiac catheter procedures can be performed safely and effectively in the early postoperative period after Fontan completion to address hemodynamic problems. These techniques contribute significantly to achieve a very low mortality and address morbidity after Fontan completion. © 2010 Wiley‐Liss, Inc.     

Relation between preoperative left ventricular muscle mass and outcome of the Fontan procedure in patients with tricuspid atresia

The relation between preoperative left ventricular muscle mass and clinical outcome of the Fontan procedure was evaluated retrospectively in 22 patients with tricuspid atresia who were selected for this physiologic surgical correction by conventional hemodynamic criteria. Patients were divided into two groups: group A (excellent or good outcome) and group B (poor outcome or death) based on the clinical course assessed up to 9.5 years postoperatively. Thirteen of 22 group A patients did not have prolonged, clinically significant, systemic venous hypertension and were not on long-term diuretic drug therapy. Nine of 22 group B patients either had clinically significant systemic venous hypertension, required long-term diuretic drug therapy or died (3 patients). Age at surgery, pulmonary arteriolar resistance, left ventricular ejection fraction, end-diastolic volume, end-diastolic pressure, systemic oxygen saturation and pulmonary to systemic blood flow ratio (Qp/Qs) were not statistically different between the two groups. Left ventricular muscle mass, both in group A patients (92 +/- 31 g/m2) and in group B patients (146 +/- 61 g/m2), was greater than the normal mean value (p less than 0.01 and p less than 0.001, respectively). Left ventricular muscle mass in group B was significantly greater than in group A (p less than 0.01). Furthermore, left ventricular muscle mass/end-diastolic volume (mass/volume) ratio, reflecting the extent of left ventricular hypertrophy relative to volume overload, was significantly greater in group B (1.1 +/- 0.28) than in group A (0.84 +/- 0.21) (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

The Fontan procedure in adults

SETTING—Tertiary adult congenital cardiac referral centre.
DESIGN—Retrospective cross sectional analysis.
OBJECTIVES—To report our 20 year experience with adult Fontan operations, and to compare late outcome in patients with single ventricle with definitive aortopulmonary or cavopulmonary shunt palliation.
PATIENTS AND MAIN OUTCOME MEASURES—Patients older than 18 years undergoing Fontan operation between 1 January 1982 and 31 December 1998 were identified. Mortality and late outcome were derived from hospital records. These patients were compared with a cohort of 50 adults with single ventricle who had not undergone a Fontan operation.
RESULTS—61 adults, median age 36 years (range 18-47 years), with a median follow up of 10 years (range 0-21 years) were identified. Actuarial survival was 80% at one year, 76% at five years, 72% at 10 years, and 67% at 15 years. Compared with before the Fontan operation, more patients were in New York Heart Association (NYHA) functional class I or II at the latest follow up (80% v 58%, p < 0.001). Systolic ventricular function deteriorated during follow up such that 34% had moderate to severe ventricular dysfunction at the latest follow up compared with 5% before Fontan (p < 0.001). Arrhythmia increased with time (10% before Fontan v 57% after 10 years, p < 0.001). Fontan patients had improved NYHA functional class, ventricular function, atrioventricular regurgitation, and fewer arrhythmias than the non-Fontan group at the latest follow up.
CONCLUSION—The Fontan operation in adults has acceptable early and late mortality. Functional class, systolic ventricular function, atrioventricular regurgitation, and arrhythmia deteriorate late after surgery but to a lesser degree than in non-Fontan patients with a single ventricle.


     

Lung function and aerobic capacity in adult patients following modified Fontan procedure

OBJECTIVE—To examine cardiopulmonary performance in 52 adult patients with a Fontan circulation.
DESIGN—Retrospective cohort study. Values of maximum oxygen uptake (VO₂max), maximum heart rate (HRmax), forced vital capacity (FVC), and forced expiratory volume in one second (FEV₁) were compared with predictive values for different age groups. Patients were further subdivided into those with a pulmonary artery connection (RA-PA) or right atrium to right ventricle conduit (RA-RV).
RESULTS—At late follow up (median 10 years, range 1 to 26 years), patients with Fontan circulation had greatly diminished VO₂max, HRmax, FVC, and FEV₁ compared with predicted values. Early age at surgery had a positive impact on aerobic capacity. The FEV₁:FVC ratio indicated restrictive lung function. No differences were found with respect to any variable between patients with RA-PA connections and those with RA-RV connections.
CONCLUSIONS—Patients with a Fontan circulation have greatly diminished values of aerobic capacity and a restrictive pattern of lung function. Patients with an early surgical procedure obtained higher values of VO₂max. The theoretical benefits of including the right ventricle in a Fontan circulation were not apparent.


Keywords: congenital heart disease; exercise test; lung function; aerobic capacity     

Current management of late failure after classic Fontan modifications: Fontan conversion

BACKGROUND: Re-establishment of Fontan circulation by eliminating the drawbacks of classic Fontan modifications has been proposed recently to improve the functional class and quality of life of patients with failed Fontan circulation. METHODS: Five patients underwent extracardiac and lateral tunnel Fontan conversion due to failure of the Fontan circulation, after classic Fontan modifications. All of the patients were female and mean age was 10.2 +/- 2.2 years (range: 8-14 years). Previous Fontan modifications were atrio-pulmonary anastomosis in 3 and Kawashima operation in 2 patients. Time to re-operation for Fontan conversion was 6 +/- 1.5 years (range: 4-8 years). Indications for re-operation were right heart failure, right atrial dilatation, and intractable atrial arrhythmias in patients with previous atrio-pulmonary anastomosis, progressive hypoxia and exercise intolerance in patients with previous Kawashima operation due to pulmonary arteriovenous fistulas. Previous atrio-pulmonary anastomoses were converted to intra-atrial or lateral tunnel Fontan operation with modified right atrial Maze procedure. Extracardiac Fontan completion was carried out after previous Kawashima operations by redirection of hepatic veins to the lungs. RESULTS: There was no mortality and no major morbidity. All patients were discharged from the hospital in good condition and in sinus rhythm. No prolonged or recurrent effusions were observed. On follow-up, all patients were in sinus rhythm and had NYHA class I functional capacity. In two Kawashima patients, SpO(2) gradually increased from 60 % to 90 % six months after the operation. DISCUSSION: We suggest that Fontan conversion should be considered in patients with previous atrio-pulmonary anastomosis, when right atrial dilatation or intractable atrial arrhythmias with deteriorating functional status develops. Redirection of hepatic venous flow to lungs induces regression of pulmonary arteriovenous fistulas and improves arterial saturation in patients with previous Kawashima operation.