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PubMed1991

[Hemodynamic changes and ventricular function before and after Fontan procedure for univentricular heart].

Ventricular function and hemodynamic parameters before and after Fontan operation were studied in patients with univentricular heart (UVH) of left ventricular (LV) type (9 cases, ages: 6-66, mean 13 years) and right ventricular (RV) type (13 cases, ages: 6-17, mean 9 years) without atrioventricular valve regurgitation. The preoperative ejection fraction (EF) was poor and only 44% of LV type and 23% of RV type met the Choussat's criterion (EF greater than or equal to 60%). There were 2 hospital deaths each in LV type and in RV type, but their causes could not be attributed to preoperative parameters of ventricular function. In survivors, the EF reduced from 62.9 +/- 7.0% before Fontan procedure to 43.4 +/- 12.6% after the procedure in LV type (p less than .05) and from 54.5 +/- 7.0% to 47.0 +/- 9.0% in RV type (p less than .01), although the ventricular end-diastolic pressure (EDP) also reduced from 12.7 +/- 2.7 mmHg before to 6.6 +/- 1.9 mmHg after the operation in LV type (p less than .05) and 10.4 +/- 2.6 mmHg to 5.5 +/- 2.4 mmHg in RV type (p less than .05). the ventricular end-diastolic volume (EDV) also reduced from 185 +/- 35% to 126 +/- 58% of expected normal volume in LV type (p less than .05) and 173 +/- 28% to 99 +/- 18% of expected normal volume in RV type (p less than .01).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1997

Modified Fontan procedure in ninety-nine cases of atrioventricular valve regurgitation.

Between January 1985 and August 1995, among 242 patients who underwent a modified Fontan procedure, 99 had atrioventricular valve regurgitation ranging in degree from 1 to 4, for which concomitant repair of the atrioventricular valve regurgitation was done in the majority of cases. In all but 4 cases the atrioventricular valve was repaired mainly by circular annuloplasty and valve replacement was not done in any case. Although the hospital mortality rate was significantly higher in cases with atrioventricular valve regurgitation (12/99, 12%) than in cases without (4/143, 3%; p < 0.0037, chi 2 test), actuarial survival in atrioventricular valve regurgitation was 84% for years 5 through 10. The degree of atrioventricular valve regurgitation before operation was 1.6 +/- 0.7 on average: in 49 cases with higher than grade 2 regurgitation before operation there was a significant decrease to 0.4 +/- 0.49 (p < 0.0001) after operation in short-term survivors. Patients with atrioventricular valve regurgitation can be treated with reasonable risk, provided proper repair of the valve is done. Circular annuloplasty is a simple and uniformly effective method to control regurgitation even in cases of common atrioventricular valve.

Adolescent↗
PubMed1996

Intermediate results of the extracardiac Fontan procedure.

BACKGROUND: Fourteen children (ages 2 to 14 years) and 1 adult (32 years) have undergone a modification of the Fontan procedure in which an extracardiac lateral tunnel or conduit is used in combination with staged or simultaneous bidirectional Glenn shunt(s). METHODS: Extracardiac lateral tunnels (n = 9) were constructed using a polytetrafluoroethylene patch (n = 7), pericardial patch (n = 1), or in situ pericardial flap (n = 1). Extracardiac lateral conduits (n = 6) were constructed using nonvalved homografts (n = 2) or polytetrafluoroethylene tube grafts (n = 4). Fenestrations were created in 4 patients (2 each in extracardiac lateral tunnel and extracardiac lateral conduit patients). Aortic cross-clamping was completely avoided in 12/15 patients (aortic cross-clamping in 2 patients for atrial septal defect enlargement and 1 for Damus-Kaye-Stansel procedure). RESULTS: There have been no operative deaths. Prolonged postoperative chest tube drainage (> 2 weeks) has been rare (n = 1). At follow-up (range, 6 to 54 months; mean, 27.5 months), all patients are in New York Heart Association class I or II and remain in normal sinus rhythm. Late protein-losing enteropathy was seen in 1 patient and was successfully treated by percutaneous creation of a stented fenestration from the extracardiac tunnel to the systemic atrium. Late catheterizations reveal unobstructed extracardiac lateral tunnel function and low pulmonary pressures (range, 11 to 13 mm Hg). Advantages of the extracardiac Fontan include (1) avoidance of aortic cross-clamping in most patients, (2) the hemodynamic benefits of total cavopulmonary connection, (3) avoidance of atriotomy and intraatrial suture lines, (4) preservation of sinus rhythm and no arrhythmias at 2 year follow-up, (5) drainage of the coronary sinus to low pressure atrium, (6) allowance for early/late fenestrations, (7) prevention of baffle leaks and intraatrial obstruction, and (8) allowance for growth (tunnel procedures only). CONCLUSIONS: We recommend this extracardiac procedure for all suitable patients undergoing surgical conversion to the Fontan circulation.

Adolescent↗
PubMed1996

Late conduit occlusion after modified Fontan procedure with classic Glenn shunt.

A number of patients with complex congenital heart disease who were initially palliated with a classic Glenn shunt subsequently underwent modified Fontan procedures. This report discusses the operative management of complete occlusion of right atrial-to-right ventricular conduits in patients with patent classic Glenn shunts. The literature is reviewed regarding similar patients, and alternative treatment strategies are discussed.

Adult↗
PubMed1990

A modification of the Fontan procedure incorporating anomalies of systemic and pulmonary venous return.

A modification of the Fontan procedure is described in which separation of anomalous systemic and pulmonary venous pathways is accomplished without the need for construction of a complex intraatrial baffle. The feasibility of this simplified surgical technique is predicated on the presence of a left superior vena cava draining to a coronary sinus. The design of the procedure and results in two patients with polysplenia syndrome and a constellation of systemic and pulmonary venous anomalies are presented. Early and late results have been gratifying, and continued exploration of the technique seems warranted.

Adolescent↗
PubMedDate not supplied

Enlarged left vitelline vein remnant as a cause of cyanosis after the Fontan procedure: resolution with an Amplatzer vascular plug.

A 6-year-old girl with heterotaxy and a functional single ventricle had persistent cyanosis 4 years after a fenestrated Fontan procedure. Cardiac catheterization revealed a large venous fistula from a left-sided hepatic vein to the coronary sinus, resulting in desaturation. The anomalous vein was occluded with an Amplatzer vascular plug.

Child↗
PubMed1989

Surgical treatment of complex congenital heart disease with modified Fontan procedure.

Between October 1973 and mid-January 1987, 500 patients underwent a modified Fontan operation at the Mayo Clinic. This included 163 patients with tricuspid atresia, 154 patients with double inlet ventricle, and 183 patients with other complex single ventricle malformations. There have been 80 hospital deaths (16%). In the last 5 years there have been 39 deaths (11.7%). Lowest mortality was seen in patients with tricuspid atresia (11% overall, 5.8% last 5 years) and highest mortality was seen in patients with complex lesions. Younger age at operation, elevated pulmonary artery pressure, elevated right atrial pressure following operation, and prolonged bypass times were significant risk factors. The modified Fontan procedure provides effective palliation for complex congenital cardiac anomalies which have no other satisfactory treatment. Criteria for operability have been extended beyond those originally proposed for this operation. The operative mortality continues to decline with increasing experience, late results are good, and longevity appears to be increased. The Fontan operation has been increasingly utilized as the definitive surgical palliation for patients with functional single ventricle. At the Mayo Clinic, over 600 patients have undergone a modified Fontan operation since our first case in October 1973. This presentation gives the results of the first 500 cases and describes the various preoperative and perioperative factors which may influence a successful outcome.

Adolescent↗
PubMed2000

Early results of the fontan procedure in one hundred consecutive patients with hypoplastic left heart syndrome.

OBJECTIVES: The purpose of this study was to review a large, single institutional experience with the Fontan procedure for patients with hypoplastic left heart syndrome. METHODS: One hundred consecutive patients with "classic" hypoplastic left heart syndrome underwent Fontan palliation between February 1992 and April 1998. Patient demographic, morphologic, and procedural variables were examined and analyzed. In particular, two different surgical techniques were used: technique I (February 1992 to December 1995) employed cardiopulmonary bypass and moderate systemic hypothermia, and technique II (December 1995 to April 1998), profound hypothermia and circulatory arrest. A retrospective review of medical records was performed and variables were examined and analyzed. RESULTS: Hospital survival for the entire cohort was 89% (95% CI 83%-95%). The technique of operation, cardiopulmonary bypass time, and aortic crossclamp time were each strongly associated with survival. Survival for patients treated by technique I was 79% (95% CI 68-91%; n = 48) and for those treated by technique II, 98% (95% CI 94%-100%; n = 52). Cardiopulmonary bypass and crossclamp times were also highly correlated with time to extubation and length of intensive care unit stay. Preoperative pulmonary artery pressure was correlated with survival; preoperative oxygen saturation, right atrial pressure, pulmonary vascular resistance, pulmonary artery size, extent of aortopulmonary artery collaterals, and echocardiographic estimates of ventricular function and tricuspid regurgitation were not correlated with survival. CONCLUSIONS: Our recent experience with Fontan palliation for patients with hypoplastic left heart syndrome suggests that it is attended by low perioperative mortality. The precise operative technique used appears to be an important determinant of outcome, with the duration of cardiopulmonary bypass and crossclamping being particularly significant.

Child, Preschool↗
PubMedDate not supplied

Successful bleomycin pleurodesis in a patient with prolonged pleural effusion after extracardiac fontan procedure.

Pleural effusions after modified Fontan operations are unpredictable and contribute significantly to the morbidity and prolonged hospitalization. Chemical pleurodesis is widely performed in adults for malignant pleural effusions, but has found less chance of usage for benign cases or the cases with unidentified etiology. Although it is a safe and easily performed procedure, the use of bleomycin for the treatment of pleural effusions in the pediatric age population has been rarely reported in the literature. In this manuscript, we present a case with prolonged pleural effusion after extracardiac Fontan operation, which was successfully treated with bleomycin pleurodesis.

Bleomycin↗
PubMed1981

Modified Fontan procedure. Methods to achieve direct anastomosis of right atrium to pulmonary artery.

Experience with Fontan operations suggests that techniques which utilize autogenous structures exclusively to achieve right atrium-pulmonary circuit connection may be preferred. Valves probably are not required in the circuit and in some cases have become obstructive with time. A large-diameter direct anastomosis of the right atrium to the pulmonary artery nearly always is possible and provides a modification of the Fontan procedure which combines favorable qualities of simplicity, all autogenous material, and posterior position which is free of the risks of compression. There also may be improved hemodynamics with this modification. Options to achieve this anastomosis depending on relations of the great arteries, previous operations, and other anatomic variations are described in eight patients.

Adolescent↗
PubMed1986

Right atrial thrombosis: rare complication of the modified Fontan procedure.

A four-year-old boy with DORV and hypoplastic left ventricle underwent a modified Fontan procedure. He had a very difficult postoperative course complicated by continued pleural effusions. Eventually he died during a last ditch effort to ligate the thoracic duct at the right diaphragm. At autopsy, an obstructing giant thrombus was found in the right atrium. This rare complication probably was due to multiple factors, but we now anticoagulate all Fontan patients routinely in an effort to prevent this fatal problem.

Child, Preschool↗
PubMed2001

Factors related to pleural effusions after Fontan procedure in the era of fenestration.

BACKGROUND: Significant pleural effusions after the Fontan operation prolong hospital stay, may increase the risk of infection, and may necessitate a pleurodesis procedure. METHODS AND RESULTS: From February 1991 to April 2000, 98 consecutive patients under the age of 18 years underwent the fenestrated Fontan procedure at Children's Hospital of Wisconsin. Ninety-four patients who survived at least 30 days after surgery were retrospectively evaluated for the following factors: age, ventricular morphology (right single ventricle, left single ventricle [RV/LV]), fenestration open (FO) or closed (FC) at end of operation, intracardiac Fontan (IF) or extracardiac Fontan (EF), days with chest tube output per day >5, 10, and/or 20 mL. kg(-1). d(-1) (CTO5, CTO10, and CTO20, respectively), need for pleurodesis, length of hospital stay (LOS), operation during winter respiratory viral season of November through March (ReVS+, ReVS-), and pre-Fontan mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR). In univariate analysis, the ReVS+ patients had prolonged LOS, greater chest tube output, and more pleurodesis (P<0.05), and PAP was related to CTO5 and CTO10 but not to CTO20 or LOS. No significant differences were found in LOS, CTO5, CTO10, CTO20, and need for pleurodesis between patients in RV/LV, FO/FC, IF/EF, or PVR groups. Patients <4 years of age had more instances of CTO20 (P<0.05). When we used ordinary least squares regression analysis with age, FO or FC, RV or LV, PAP, and ReVS+ or ReVS- to predict each of CTO5, CTO10, CTO20, and LOS, only ReVS+ or ReVS- and age were statistically significant in all models. CONCLUSIONS: Use of the Fontan procedure during the respiratory viral season appeared to be related to significant, prolonged pleural effusions and longer hospitalizations.

Adolescent↗
PubMed2000

The role of the Fontan procedure in the surgical treatment of congenital heart malformations with double-outlet right ventricle.

The Fontan procedure in one of its many modifications is applicable to the surgical treatment of those patients with double-outlet right ventricle who are not amenable to bi-ventricular correction. Included in this group are patients with significant hypoplasia of one of the ventricles, patients with distant (non-committed) ventricular septal defect, patients with significant straddling of the atrioventricular valves, patients with abnormal atrioventricular connections (such as absence of one of the valves), or common atrioventricular valve connection. The presence of complex associated lesions such as those seen in the heterotaxia syndromes may, by virtue of the surgical risk of attempts at bi-ventricular repair, make the Fontan approach a suitable option for these patients. The presence of a subaortic conus and the potential for subaortic obstruction, a frequent findings in patients with double outlet right ventricle, plays an important role in the preparation and conduct of the Fontan operation. Copyright 2000 by W.B. Saunders Company

Journal Article↗
PubMed1995

[Right atrial thrombosis; an unusual late complication of the Fontan procedure. Apropos of a case].

The authors report the case of a large right atrial thrombosis causing acute cardiac failure in an 18 year old patient with tricuspid atresia who had undergone 3 operations: Waterston shunt at 2 months of age, Fontan procedure at 3 years of age with reoperation at 13 years of age. The diagnosis was made at echocardiography and angiography. Surgical management comprised ablation of the thrombus and a tunneling of the right atrium between the inferior vena cava and the atriopulmonary conduit. The immediate postoperative course was complicated by a slowly regressive neurological deficit. The medium-term outcome (one year) is satisfactory with antiarrhythmic and anticoagulant therapy. A review of the literature showed that these thromboses are not exceptional in the early postoperative period for a variety of reasons. Secondary thrombosis is often related to arrhythmias, thus requiring clinical, electrocardiographic and echocardiographic follow-up of these patients and the prescription of antiarrhythmic and anticoagulant therapy. The diagnostic methods are discussed. Echocardiography may be sufficiently explicit to make potentially risky angiography unnecessary.

Adolescent↗
PubMed1982

Postoperative evaluation of the Fontan procedure by radionuclide angiography.

Seven patients were evaluated by radionuclide angiography (RNA) following Fontan-like procedures for complex cyanotic congenital defects. Both first-pass and ECG gated equilibrium angiography were performed in each. Residual right-to-left shunts, right atrial (RA) outflow and pulmonary arterial obstruction, and left ventricular dysfunction were demonstrated by these techniques. In addition, RA ejection fraction (RAEF) by gated equilibrium scan was measured in each patient. Relatively low RAEF was seen in conjunction with residual obstruction to pulmonary flow. RNA is useful in the postoperative evaluation of patients after Fontan procedures.

Adolescent↗