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At least 307 records · Page 17Linked to original sources
PubMed1988

Modified Fontan procedure for biventricular hearts with complex forms of double-outlet right ventricle.

The surgical management of patients with biventricular hearts and complex double-outlet right ventricle with noncommitted ventricular septal defect, hypoplastic ventricles, common atrioventricular orifice, straddling tricuspid valve, and straddling mitral valves is controversial. More recently at our institution, 23 of these patients underwent Fontan-type procedures as an alternative to the intraventricular tunnel repair. In all cases, the presence of more than one associated anomaly would have prevented adequate biventricular correction. The ventricular septal defects were of the inlet type in 14 patients, inlet and subpulmonary in one, multiple in one, subpulmonary in three, and subaortic in four. Twenty-one patients had atrioventricular junction anomalies, and 14 had hypoplastic ventricles (left ventricle in 11 and right ventricle in three). Pulmonary stenosis was present in 13 patients and pulmonary atresia in two. At operation, an extracardiac conduit was used in two patients and an atriopulmonary direct anastomosis in 21. There were six hospital deaths (26%). Seventeen patients had a mean follow-up period of 25 months. At the follow-up evaluation, nine patients were assigned to New York Heart Association Class I and seven to Class II; one patient assigned to Class IV died at 9 months postoperatively. Cumulative mortality was 30.4% (seven deaths). Univariate analysis identified hypoplastic left ventricle with any type of ventricular septal defect and atrioventricular junction as the only incremental risk factor for early and late outcome (p less than 0.01), which also influenced functional status and use of drug therapy (p less than 0.01) at the time of follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2005

Severe hemoptysis in a child after the Fontan procedure.

There is an increased incidence of pulmonary hemorrhage and hemoptysis among patients with congenital heart disease (CHD). The pathophysiology of pulmonary hemorrhage in CHD includes pulmonary hypertension, pulmonary venous congestion, aorto-pulmonary collaterals, pulmonary arteriovenous malformations, and dilated bronchial arteries. We present the case of a 6-year old boy who required treatment for massive hemoptysis after staged palliation for hypoplastic left heart syndrome (HLHS). Effective management of this life threatening entity is described as well as the anesthetic implications of performing rigid bronchoscopy in a patient with the Fontan circulation and massive hemoptysis.

Anesthesia, General↗
PubMed1991

The Fontan procedure: a historical review.

Complete bypass of the right ventricle was first performed successfully and subsequently reported by Fontan and Baudet for patients with tricuspid atresia. By eliminating congenital and surgical shunts, ventricular volume overload and pulmonary hypertension were avoided. A logical extension of its predecessor, the partial right heart bypass procedure introduced by Glenn, the Fontan operation has great application for patients with various forms of univentricular heart. It is one of the historic advances in the surgical treatment of congenital heart disease.

Cardiac Surgical Procedures↗
PubMed1996

Percutaneous coil embolization and balloon-expandable stenting; new treatments in intractable cardiac failure after Fontan procedure: a case report.

Successful percutaneous coil embolization of aortopulmonary collaterals and balloon-expandable stent implantation of left pulmonary artery stenosis in a child with hypoplastic right heart syndrome were reported, Presently, interventional cardiac catheterization is a very useful treatment strategy in congenital heart diseases unamenable to or inaccessible for surgery.

Catheterization↗
PubMed2006

Redirection of hepatic drainage for treatment of pulmonary arteriovenous malformations following the Fontan procedure.

The development of unilateral pulmonary arteriovenous malformations in patients after total cavopulmonary connection with an extracardiac conduit has been reported. Unequal distribution of hepatic venous flow to the lung is theorized to be the causative factor. We report the surgical management of pulmonary arteriovenous malformations in a patient with heterotaxy syndrome, single ventricle, and interrupted inferior vena cava. The patient had previously undergone a total cavopulmonary connection with an extracardiac conduit draining hepatic venous flow to the right branch pulmonary artery. In the subsequent operation, we redirected the extracardiac conduit to the innominate vein. This operation provided the affected lung with hepatic venous blood without exposing the patient to the morbidity associated with cardiopulmonary bypass.

Arteriovenous Malformations↗
PubMed1987

Pulmonary artery connection in the Fontan procedure. Flexible polytetrafluoroethylene conduit for expansion.

From clinical experiences with the Fontan operation in six cases, a few practical contrivances and operative steps are described. We stress that meticulous care should be taken not to cause any stenotic complication in the outflow tract toward the pulmonary artery, particularly by traction of the right atrial appendage only to achieve a direct anastomosis. Conduit repair would be necessitated by cases in order to expand the indicative criteria vertically as well as horizontally. For conduit material, we used consecutively nonvalved polytetrafluoroethylene that was reinforced by handmade stainless steel wire ring (Gore-Tex, Inc) or by spirally built-in stent (IMPRA, Inc). The latter was eventually useful in obtaining natural curving of the conduit without kinking or compression. Some contrivances in anastomosing a conduit were also proposed to achieve an excellent result. We believe these practical contrivances will serve for expansion of the indicative criteria and promise improved operative outcome.

Adolescent↗
PubMed2005

Nitric oxide in pulmonary arteriovenous malformations and Fontan procedure.

Pulmonary arteriovenous malformations are a well documented complication of superior cavopulmonary (Glenn) connections. We report the successful management of a case of severe hypoxemia in the early postoperative period of a patient who underwent the Fontan operation. The patient had previously been diagnosed with pulmonary arteriovenous malformations; the use of inhaled nitric oxide was followed up with reversal of life-threatening hypoxemia. At 6-month postoperative follow-up, the patient was asymptomatic with near normal aortic saturation.

Abnormalities, Multiple↗
PubMed1999

Equivalent survival following cavopulmonary shunt: with or without the Fontan procedure.

OBJECTIVES: In 1992, an analysis of our experience with the cavopulmonary shunt (CPS) demonstrated equivalent long-term survival, with or without subsequent conversion to a Fontan circulation. Before 1992 (era 1) intervention was deferred until mandated by clinical deterioration. Since 1992 (era 2), timing of both CPS and Fontan was compressed in an effort to improve survival. Survival following CPS is analyzed to ascertain whether Fontan confers any survival advantage over no further definitive intervention. METHODS: From 1962 to 1997 inclusive, 490 patients underwent CPS, excluding those who had a CPS concomitant with a Fontan. In 55 patients the CPS was performed at or after a biventricular repair (BVR), or after a Fontan, and these patients are excluded. The 435 patients remaining followed a surgical protocol which included a subsequent BVR (n = 28), or a subsequent Fontan operation (n = 220), or no further definitive surgery (CPS only, n = 187). Between eras the mean age at surgery decreased for all procedures. RESULTS: Long-term survival 20 years after a CPS in 435 patients is 56 +/- 5%. Survival at 20 years among the 220 patients who were subsequently converted to a Fontan circulation is 65 +/- 8% compared to 50 +/- 11% for the 187 patients who did not have a Fontan. However, most of their survival difference is because all early deaths after a CPS occurred in the non-Fontan group. Multivariable analysis demonstrated that proceeding to a Fontan did have a small survival advantage which was not evident by univariate analysis. Independent risk factors for death, at any time, are a common atrioventricular valve, or pulmonary artery banding. The era had no effect on survival. CONCLUSIONS: The single ventricle circulation appears to have a limited durability of, an average, 30-40 years. There is a slight survival advantage in converting patients after a CPS to a Fontan circulation. A marked reduction in age at CPS and at Fontan has, as yet, not improved survival.

Adolescent↗
PubMed1992

The fenestrated Fontan procedure.

In 90 patients with characteristics placing them at increased risk for a Fontan operation, a fenestration was created in the atrial baffle at the time of the Fontan repair. The rational was to allow a right to left shunt which would maintain cardiac output and limit right atrial pressure in the presence of conditions which limit pulmonary blood flow. Early mortality was 4/90 (4%), with an additional two patients having the Fontan repair taken down to a bidirectional cavopulmonary anastomosis. Postoperative right atrial pressures were low (average 13 mm Hg), as was the incidence of prolonged pleural effusions (13%). At short-term (average 13 months) follow-up, 77% of patients have had closure of the fenestration, and 92% are in New York Heart Association Class I. We conclude that baffle fenestration with subsequent transcatheter closure results in decreased mortality and morbidity among high risk patients undergoing a Fontan repair, and that the high functional level at short-term follow-up justifies continued aggressive management of such patients.

Adolescent↗
PubMed1994

Surgical alternatives to the Fontan procedure incorporating a hypoplastic right ventricle.

BACKGROUND: Frequently the definitive operation for patients with a right ventricle (RV) that is too small to support full cardiac output is a modified Fontan operation. However, other surgical options exist that incorporate a small RV in the atriopulmonary pathway when biventricular repair is not feasible because of RV or tricuspid valve hypoplasia. The risks and benefits of these options have not been well defined. METHODS AND RESULTS: Between 1988 and 1993, 8 patients (6 with pulmonary atresia and intact ventricular septum and 2 with tricuspid valve stenosis and RV hypoplasia) underwent a cavopulmonary connection, which allowed right atrial blood to flow either to the pulmonary artery via the RV or directly via the cavopulmonary anastomosis. Age at surgery ranged from 1.5 to 9 years. The proximal right pulmonary artery was ligated in 5 patients, and the atrial septal defect was closed during the same procedure in 7 of the 8 patients. The echocardiographic right ventricular-left ventricular volume ratio ranged from 9% to 25%, and tricuspid valve z-scores ranged from 0 to -4. There were no deaths at a median follow-up of 24 months (range, 7 to 61 months). Mild exertional limitation was evident in only one patient. Postoperative echocardiograms demonstrated pulsatile systolic flow across the RV outflow tract in 5 patients and low-velocity diastolic-systolic flow in a sixth patient with extreme tricuspid valve hypoplasia. At postoperative cardiac catheterization (6 patients) right atrial mean pressures ranged from 7 to 13 mm Hg and mixed venous saturations from 62% to 70%. CONCLUSIONS: Right atrial decompression via a superior vena cava-to-pulmonary artery anastomosis allows incorporation of a small RV into the pulmonary circulation and closure of the atrial septum, with excellent results to date.

Cardiac Catheterization↗
PubMed1985

Outcome of the Fontan procedure in patients with tricuspid atresia.

Between 1973 and 1983, 90 patients with tricuspid atresia underwent the Fontan operation at the Mayo Clinic. Operative mortality was 12% overall and decreased to 7% during the second 5 year period. At postoperative follow-up of 65 patients (1 to 10 years, mean 4), 50 (86%) of 58 present survivors were in excellent condition. There had been seven late deaths. Catheterization 0.5 to 8 years (mean 2.7) postoperatively in 25 patients disclosed a mean right atrial pressure of 14 mm Hg (range 6 to 21) and a mean cardiac index at rest of 3.0 (range 1.8 to 4.2). Mean left ventricular end-diastolic pressure was 8.5 mm Hg postoperatively compared with 16.5 mm Hg preoperatively; the decrease was secondary to a decreased ventricular volume load (mean volume load index 10.2 preoperatively, 3.0 postoperatively). We recommend that patients with tricuspid atresia have the Fontan operation by age 6 years to protect myocardial function.

Adolescent↗
PubMed1991

Results of the Fontan procedure for patients with univentricular heart.

One hundred twenty-four consecutive patients with univentricular heart undergoing the Fontan operation were reviewed. Patients with tricuspid atresia or biventricular heart with hypoplasia of one ventricle were excluded. Eighty-four patients had left ventricular morphology. Atrioventricular connection was double-inlet (n = 76), common (n = 29), absent left atrioventricular connection (n = 14), and absent right atrioventricular connection (n = 5). Actuarial survival was 77% (70% confidence limits, 73% to 81%) at 1 year, 66% (70% confidence limits, 60% to 72%) at 5 years, and 49% (70% confidence limits, 36% to 61%) at 10 years, indicating a continuing risk for premature death. Multivariate analysis identified preoperative ventricular function and hypertrophy as risk factors for survival. High postrepair right atrial pressure (greater than 15 mm Hg) emerged as a strong intraoperative predictor of survival. Logistic regression analysis of these factors predicts high probability of death for certain subgroups of patients after the Fontan operation. Forty-four percent (n = 53) of these original 124 patients are alive and in New York Heart Association class I at follow-up. Thirty-eight percent (n = 33) of survivors have worse ventricular function than preoperative. Long-term survival is disappointing. Certain identifiable subgroups of patients with univentricular heart have unacceptable risks for the Fontan operation and should have alternate management. High postrepair right atrial pressure is an ominous sign, and if it persists the Fontan should be fenestrated or taken down.

Adolescent↗
PubMed1988

Hemodynamic responses to isolated increments in heart rate by atrial pacing after a Fontan procedure.

The physiologic role of the right ventricle has long been a subject of interest to physiologists. The Fontan operation provides a human model for studying the circulation in series devoid of a subpulmonic right ventricle. The hemodynamic response to isotonic exercise in this setting has been established, and differs appreciably from normal. However, the physiologic response to an increase in heart rate (atrial pacing) as an isolated variable has not been examined and compared to atrial pacing in hearts with two concordant subarterial ventricles. Accordingly, we compared the supine bicycle exercise response to rate-equivalent right atrial pacing in nine patients after atriopulmonary anastomoses (the Fontan operation) for single ventricle or tricuspid atresia. Cardiac index increased 77% with exercise (rest 2.6 L/min/m2; exercise 4.6 L/min/m2) but decreased 12% with atrial pacing (rest 2.5 L/min/m2; pacing 2.2 L/min/m2). Pulmonary arterial oxygen saturation declined significantly during exercise (rest 68%; exercise 31%) and during atrial pacing (control 72%; pacing 64%). The mean increment in pulmonary arterial pressure was 1.3 times greater with exercise (rest 14 mm Hg; exercise 20 mm Hg) than with pacing (control 12 mm Hg; pacing 16 mm Hg). Peak systemic arterial systolic pressure increased 14% with exercise but was unchanged by pacing. Systemic and pulmonary vascular resistances fell with exercise but changed insignificantly during atrial pacing. Stroke volume rose slightly with exercise but fell significantly with pacing.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗