Rescue cardiac transplantation for early failure of the Fontan-type circulation in children

OBJECTIVE: Early failure of the Fontan-type circulation is a potentially fatal complication. We review our experience with cardiac transplantation in children presenting with end-stage heart failure in this scenario. METHODS: We performed a retrospective review. Between 1985 and 2003, 6 children aged less than 16 years were referred for cardiac transplantation. The indication for cardiac transplantation was end-stage cardiac failure early after the completion of the Fontan-type operation. RESULTS: All 6 patients listed for transplantation underwent cardiac transplantation; the median interval to transplantation from the operation was 36 days (range, 6-180 days). Four patients had undergone the Fontan procedure, and 2 had one-and-a-half-ventricle repair. All 6 patients were ventilated and inotrope dependent, with varying degrees of multiorgan dysfunction. One patient was bridged to transplantation with extracorporeal membrane oxygenation. The median age at transplantation was 7.1 years (range, 3-12.5 years), and weight was 18.9 kg (range, 11-35 kg). One patient died on the operating table (graft failure and hemorrhage). In 5 survivors the median intensive care unit stay was 10 days (range, 8-61 days). On follow-up of 6 to 81 months, there have been no subsequent deaths, and the quality of life in survivors is good. CONCLUSION: Rescue cardiac transplantation in the setting of an early failure of the Fontan-type circulation and end-stage cardiac failure is an effective treatment option and can be performed with acceptable early mortality and encouraging short-term to medium-term results.     

Early and medium-term results after modified Fontan operation in adults.

OBJECTIVE: Single ventricle palliation is rarely performed in adults and the results are less optimal than in children. In this article we analyze our experience with the modified Fontan operation in this age group. METHODS: Data of 15 consecutive patients with single ventricle with a mean age of 26 (range 16-38) years, who underwent Fontan operation between 3/92 and 1/2000 were retrospectively analyzed. Five patients had previously had an aortopulmonary shunt in childhood and two patients had previously received a bi-directional cavopulmonary shunt as adults. Eleven patients were preoperatively in NYHA class III and four in class II. The main factors for the selection of the patients before surgery were well-developed pulmonary arteries with lower lobe index 120+30 mm/m(2), pulmonary artery pressure <18 mmHg, good cardiac function and enddiastolic systemic ventricular pressure <12 mmHg. The lateral tunnel Fontan operation (LTFO) was performed in ten patients and extracardiac Fontan operation (ECFO) in five. A fenestration 4-5 mm in size was constructed in all patients with LTFO and in three of five patients with ECFO. RESULTS: There was one intraoperative and one late death (total mortality 13%). The mean extubation time and hospital stay were 24 h and 21 days, respectively. Severe postoperative complications were observed in three patients (20%). Two LTFO patients out of a total of eight patients (53%) with perioperative arrhythmias received a permanent pacemaker due to bradyarrhythmia. During the median follow-up of 5.0 (range 2.3-10.1) years, four patients developed arrhythmias; one of them had new onset bradyarrhythmia after LTFO and required permanent pacemaker implantation. The median postoperative oxygen saturation was 93% (range 90-98%). NYHA class improved significantly in 12 survivors. Cardiac catheterization (0.5-4 years postoperatively, n=12) showed excellent Fontan hemodynamics in all patients. CONCLUSIONS: The modified Fontan operation can be performed in adults with acceptable early and midterm mortality and morbidity and leads to either complete or marked relief of cyanosis and enhanced exercise tolerance in all survivors. Postoperative arrhythmias are one of the main drawbacks but the incidence of arrhythmias after ECFO seems to be lower. The long-term follow-up has yet to be established.     

Fontan-type procedures: residual lesions and late interventions

BACKGROUND: The purpose of this study was to determine the type and incidence of hemodynamic and electrophysiological abnormalities requiring surgical or catheter-based interventions in a single-center long-term experience. METHODS: Eighty-eight patients with a follow-up of at least 5 years (mean follow-up, 9.6 +/- 2.6 years) after Fontan-type procedures were included. All patients had undergone cardiac catheterization either as part of the regular postoperative protocol or because of symptomatic atrial tachycardia or increasing cyanosis. RESULTS: Freedom from reoperation for up to 5 years was documented for 82% of patients and decreased to 76% after 8 years. Late reoperations included conversion of an atriopulmonary anastomosis to a total cavopulmonary anastomosis in 2 patients with atrial dysrhythmia and implantation of an extracardiac conduit in 1 patient with left atrial isomerism and intrapulmonary arteriovenous malformations after a Kawashima operation. Decline in sinus node function with symptomatic bradycardia required pacemaker therapy in 10 patients (11%). Transcatheter interventions included fenestration occlusion in 5 of the 11 patients with initial baffle fenestration. In 6 of 17 patients with aortopulmonary collaterals, coil occlusion was indicated to reduce future systemic ventricular volume load. Various systemic venous collaterals were documented in 11 patients and required coil occlusion in 2. One patient with symptomatic protein-losing enteropathy underwent transcatheter fenestration creation without sustained relief of symptoms. Freedom from transcatheter interventions decreased from 94% to 82% after 5 and 10 years, respectively. CONCLUSIONS: During long-term follow-up, reoperations are rare and mainly involve Fontan conversion to either a lateral-tunnel or extracardiac-conduit procedure. Detailed angiographic evaluation on a routine basis allows identification of the vascular sites of origin of aortopulmonary collateral vessels and systemic venous collaterals potentially developing during long-term follow-up. Transcatheter interventions including fenestration occlusion and occlusion of venous collaterals and aortopulmonary collaterals were performed to maintain and improve the Fontan circulation in clinically symptomatic and asymptomatic patients. During long-term follow-up after Fontan-type operations, a regular postoperative cardiac catheterization protocol is recommended.     

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)     

Functional status in adolescents and adults with Fontan circulation

OBJECTIVE: We determined functional status in adolescents and adults with Fontan circulation. METHODS: Functional status was studied in 25 patients surviving more than 2 years after the definitive procedure and currently no younger than 18 years old. Age at operation was 2 to 44 years old, and follow-up was 12 +/- 5 years. To achieve Fontan circulation, atriopulmonary connection was used in 14 patients, and total cavopulmonary connection in 11 patients. RESULTS: One patient undergoing atriopulmonary connection died suddenly 6 years after the Fontan procedure due to pulmonary thromboembolism. New York heart association functional status was class I in 23, and class II in 2, at the latest follow-up. Catheterization done 6.5 +/- 6.8 years after the Fontan procedure showed that systemic venous pressure was statistically higher (p = 0.019) in the atriopulmonary connection group (13 +/- 3 mmHg) than in the total cavopulmonary connection group (10 +/- 3 mmHg). Exercise tests in 19 patients showed reduced tolerance in all, with maximal oxygen intake being 24.4 +/- 5.1 ml/kg/min. Serum glutamic oxaloacetic transaminase and glutamic pyruvic transaminase were elevated above normal in 11 (44%). Arrhythmia was noted over longer terms in 4 patients undergoing atriopulmonary connection; in 3, atriopulmonary connection was converted to total cavopulmonary connection, and surgical intervention for atrial arrhythmia was successful. CONCLUSION: Although functional status in adolescents and adults with Fontan circulation was good, arrhythmia and liver dysfunction in such subjects could lead to morbidity.     

Fontan completion without surgery.

OBJECTIVE: There are several modifications introduced in the preparation for a subsequent non-surgical transcatheter completion of the Fontan procedure. We report our experience with one type of the modification and the short-term results following its implementation. METHODS: During bidirectional cavopulmonary connection (BCPC) an intra-atrial lateral tunnel is additionally created, as intended for a Fontan procedure but fenestrated with a 10-14 mm aperture. The cardiac end of the superior vena cava (SVC) is then patched to maintain the physiology of BCPC. During the interventional transcatheter completion procedure, the SVC patch is perforated using radio-frequency (RF) energy, balloon-dilated, and stented as well. The aperture is closed with a device when required. Paired t-test was used to compare data before and after the Fontan completion. RESULTS: From June 2003 to February 2006, 16 patients (9 boys and 7 girls, mean age 12 months) underwent the surgical procedure described. The mean bypass time was 137 min and the mean ischemic time was 77 min. There were no operative deaths. One patient with bilateral SVC required a take down due to recurrent effusions. Ten months later, nine patients underwent completion (mean age 20 months, mean weight 10.6 kg). The stents were dilated to a mean diameter of 14.4mm. All except one aperture was closed with a device. The mean fluoroscopy time was 41 min. Oxygen saturation increased from 85 to 94% (p=0.001). Pulmonary artery pressures remained normal (16 mmHg before and 19 mmHg after, p=0.12). No patients required mechanical ventilation and none developed pleural effusions or arrhythmias. All were discharged from hospital within 6 days of the Fontan completion. Twenty-two months after Fontan, all were well. Echocardiography revealed no gradients across the stents. Two patients had minor leaks across the aperture. One underwent further stent dilatation a year later. CONCLUSIONS: Fontan completion without surgery is suitable in patients with single ventricles with lower mortality and morbidity, avoids multiple surgical interventions while maintaining the staged approach and allows for successive dilatation of the Fontan pathway to accommodate for growth.     

Orthotopic heart transplantation for failing single ventricle physiology.

OBJECTIVE: Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for failing single ventricle physiology. METHODS: Between 1988 and 2002, 25 patients (mean age 9.3+/-7.1 years) underwent OHT for complex congenital heart disease (CHD) with a functional right (15 patients) or left (10 patients) single ventricle. Palliative staging towards Fontan completion had been previously accomplished in 22 patients (88%). Transition to OHT occurred from a shunt stage in 10, a bi-directional cavopulmonary anastomosis (BDG) stage in nine, and after Fontan failure in six patients. RESULTS: Thirty-day survival was 68.0+/-9.3% with no additional mortality up to 14.1 years. OHT following BDG staging exhibited 100% long-term survival, as opposed to 66.7+/-15.7% for OHT after systemic-to-pulmonary shunt, and 33.3+/-19.2% for OHT following failing Fontan (p=0.032). Regression logistic modelling indicated failing Fontan circulation as predictor of higher mortality after OHT (p=0.041). Reintervention was necessary in four patients 40+/-11 months after OHT to address residual superior vena cava (two) and isthmic (two) stenosis. Overall freedom from reintervention was 88.3+/-8.1% at 5 years. CONCLUSIONS: OHT for structural CHD with single ventricle physiology entails substantial early mortality while BDG enables the best transition to heart transplant. OHT should be considered in the decision-making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted.     

Preoperative small pulmonary artery did not affect the midterm results of Fontan operation.

OBJECTIVE: It was well discussed, in the primary Fontan era, that small pulmonary arterial size could affect the results immediately after the Fontan procedure. The objective of the present study is to determine whether this feature remains as a risk factor in the era of the staged Fontan completion and poses functional impediments in the longer terms. METHODS: Between June 1991 and November 2004, the staged Fontan completion was carried out subsequent to the bidirectional Glenn procedure in consecutive 57 patients with a preoperative pulmonary artery index less than 250 mm2/m2 (Group-S; minimum index 104 mm2/m2). Clinical data were reviewed retrospectively. As background and reference information, similar data were collected in another consecutive 64 patients with larger pulmonary arteries who underwent the staged Fontan completion during the same period (Group-L; maximum index 697 mm2/m2). RESULTS: No patients died after the Fontan procedure in Group-S, while six early deaths in Group-L. No takedown of the Fontan circulation was carried out in either group. The latest catheterizations, at 2.8+/-2.7 years postoperatively, showed a pulmonary artery index significantly lower than the preoperative index (Group-S: 198+/-37-176+/-49 mm2/m2; P=0.0082, Group-L: 360+/-94-266+/-89 mm2/m2; P<0.0001). Hemodynamics in Group-S during the intermediate term were identical with those in Group-L in mean pulmonary arterial pressure (10+/-2 in Group-S and 10+/-3 mmHg in Group-L), mean atrial pressure for the systemic chambers (5+/-2 and 6+/-3 mmHg, respectively), mean transpulmonary gradient (5+/-2 and 4+/-2 mmHg, respectively), cardiac index (3.0+/-0.7 and 3.0+/-0.6l/min/m2, respectively), and arterial oxygen saturation (93+/-3% and 94+/-2%, respectively). Similarly, brain natriuretic peptides concentration in the serum (19.4+/-15.6 in Group-S and 28.3+/-37.2 pg/ml in Group-L) and peak oxygen consumption on exercise testing (24.8+/-4.5 and 24.0+/-6.3 ml/kg/min, respectively) were not inferior in Group-S to those in Group-L. Conclusions: The outcome after the Fontan completion, including functional ones in the intermediate term, was acceptable in patients having a preoperative PA index smaller than 250 mm2/m2. Pulmonary artery index decreased still further postoperatively, but did not obviously militate against functional efficacy of the Fontan circulation.     

Bi-directional cavopulmonary shunt: is accessory pulsatile flow, good or bad?

OBJECTIVE: Evaluation of the effect and long-term outcome of accessory pulsatile blood flow versus classical bi-directional cavopulmonary connection (BCPC). METHODS: Retrospective review of the medical and surgical records. RESULTS: Two-hundred and five patients (119 boys, 86 girls) underwent BCPC from 1990 to 1996. Accessory pulsatile flow was present in 68%, flow being maintained through the pulmonary trunc in 46%, systemic-to-pulmonary artery shunt in 13% and mixed in 7%, or patent ductus arteriosus in 2%. Patients with accessory pulsatile flow had lower hospital mortality (3% versus 5%), while mean pulmonary artery pressure (14.1 versus 12.6 mmHg P = 0.050) and increase of oxygen saturation (12.4 versus 8.7, P = 0.034) were significantly higher. The period of artificial ventilation (1.9 day) and ICU stay (6 days) did not differ for both groups. Late mortality was higher following accessory pulsatile flow (6% versus 1%). At late follow-up patients with accessory pulsatile flow had significantly higher oxygen saturation (mean 85 +/- 4%, versus 79 +/- 4%; P < or = 0.005). If subsequent completion of Fontan is considered the optimal palliation and subsequent systemic to pulmonary artery shunt, arteriovenous fistula and transplantation is considered a failure, patients with accessory pulsatile flow had significantly more and earlier completion of the Fontan procedure (mean 1.7 +/- 2.4 years, versus 2.7 +/- 4.4 years; P = 0.008). Survival is not influenced by age at bi-directional cavopulmonary shunt surgery, left or right functional ventricular anatomy or previous palliative surgery. One patient with accessory pulsatile flow developed systemic-to-pulmonary collateral's eventually requiring lobectomy. CONCLUSION: Despite two different initial palliative techniques the outcome was not significantly different. Accessory pulsatile blood flow appeared not to be a contra-indication for a completion Fontan procedure. Moreover, the data suggest that after accessory pulsatile flow can safely be performed, at late follow-up oxygen saturation is higher, while, significantly more and earlier completion of Fontan occurred. Age at bi-directional cavopulmonary shunt, basic left or right ventricular anatomy or previous palliative surgery did not influence survival.     

Fontan procedure for asplenia syndrome

BACKGROUND: Historically the Fontan operation in patients with single ventricle asplenia syndrome has been associated with high mortality. We studied whether recent modifications of the surgical technique have improved outcome. METHODS: A retrospective review of 11 patients with asplenia syndrome who underwent a Fontan operation between 1996 and 2002 was performed. RESULTS: Anomalies of pulmonary venous return included 5 patients (46%) and 4 patients with an isolated hepatic venous return from inferior vena cava (36%). The type of Fontan procedure included 6 patients with an extracardiac tube-graft, 3 with a lateral tunnel modification, 1 with an intra-atrial tube graft, and 1 with an intra-extra atrial tube graft. A fenestration was placed in 3 patients (27%). There were no early deaths and 2 late hospital deaths (18%) due to tachyarrythmia and failed Fontan circulation. There were no late deaths and no complications during follow-up periods (mean 3 years). Of 21 patients diagnosed with asplenia syndrome between 1990 and 2000, overall survival was 57% and 12 patients underwent a Fontan-type operation. CONCLUSIONS: The Fontan operation can now be performed in patients with asplenia syndrome with good survival. Fontan staging, appropriate choice of Fontan modification, aggressive treatment of concomitant malformations, and use of a fenestration contribute to improved outcome.     

Long-Term Results of Right Latissimus Dorsi Cardiomyoplasty

A bstract Background : Initial experimental and clinical studies have shown that cardiomyoplasty using the right latissimus dorsi can improve left ventricular (LV) function early after operation. Methods : This study presents the long-term clinical results of this procedure. Between March 1991 and November 1992, 16 patients (12 men, 4 women; mean age 57; range 33–77 years) underwent operation. Survivors were evaluated at 6-month intervals for 2 years with right heart catheterization, radionuclide scans, exercise testing, and quality of life questionnaires. Results : The operative mortality was 6% (1/16), but 3 additional patients experienced sudden death within 6 months of operation. Survival was 63% (10/16) at 12 months and 50% (8/16) at 24 months. There were no significant changes in hemodynamic variables at 12 or 24 months after surgery. Left ventricular ejection fraction increased from 26.1 ± 5.3 to 33.4 ± 10.3 (p < 0.05) 6 weeks after operation, but was not different from baseline thereafter. The LV end-diastolic volume decreased significantly at 6 months from 306.1 ± 71 to 249.4 ± 69 mL (p < 0.01), and remained lower than the preoperative value in subsequent follow-up. Comparison of preoperative LV ejection fraction and LV stroke work index in 24-month survivors (7) and nonsurvivors (9) showed that survivors had better LV function before surgery. Functional status and quality of life were improved. Conclusions : Long-term results of right latissimus dorsi cardiomyoplasty are comparable to results using the left latissimus dorsi. Overall survival was limited by late sudden death, but survivors had improved functional capacity and stable LV function and size.     

Heart transplantation in patients with previous Fontan operations

OBJECTIVE: The clinical features and outcomes of patients undergoing heart transplantation after a failed Fontan operation are still debated. The aim of this study was to retrospectively evaluate our experience in 14 patients undergoing heart transplantation after previous Fontan-type operations. METHODS: From 1990 to 2002, 14 patients underwent heart transplantation in our institution after a previous Fontan procedure. The mean age at the time of the Fontan operation and at transplantation was 7.3 +/- 2.8 and 17.2 +/- 6.3 years, respectively. The indication for transplantation was protein-losing enteropathy in 7 patients, arrhythmia with ventricular dysfunction in 5 patients, and heart failure in 2 patients. All patients received basic immunosuppressive therapy with cyclosporine (INN: ciclosporin) and azathioprine without induction therapy or maintenance steroids. RESULTS: Two hospital deaths occurred: one patient died on the fifth postoperative day of graft failure, and the second died on the 17th postoperative day after an acute neurologic event. Two patients died later, one 23 months after transplantation of acute rejection and the other after 90 months of chronic rejection and endocarditis. One patient underwent successful reintervention 2 years after heart transplantation for pulmonary vein obstruction. The 10 surviving patients are in New York Heart Association class I, with a mean follow-up of 64.5 +/- 42 months. One of them was delivered of a healthy baby 5 years after transplantation. Patients with protein-losing enteropathy reached a normal protein level within a mean of 10 months (range, 6-18 months) after transplantation. Four patients required a temporary administration (3-6 months) of oral steroid therapy for recurrent rejection episodes. Currently, 7 patients are taking cyclosporine, and 3 are taking cyclosporine and azathioprine. The actuarial survival at 1, 5, and 10 years was 86% +/- 9%, 77% +/- 12%, and 62% +/- 17%, respectively. CONCLUSION: Heart transplantation is a good option for patients with a failing Fontan operation. We documented the reversibility of protein-losing enteropathy in all patients. No mortality caused by surgical complications was observed.     

The Fontan-operation: from intra- to extracardiac procedure

PURPOSE: For treatment of univentricular heart, the Fontan operation has been established as the definitive palliation. The current controversy is mainly based on the high incidence of arrhythmias after an intra-atrial lateral tunnel Fontan operation. METHODS: From January 1995 until April 2002, 46 children underwent a Fontan-type operation with or without a small fenestration. In 33 patients (group I) an intracardiac tunnel and in 13 patients (group II) an extracardiac conduit procedure was performed. PRINCIPAL FINDINGS: There was no perioperative mortality. All patients showed postoperative a significant increase of arterial oxygen saturation, from 76 to 86% after surgery with fenestration, or to 90.5% without fenestration respectively. In patients with fenestration procedure, the saturation rose to 90% after closure of fenestrations 9 to 12 months after operation. CONCLUSIONS: Modified Fontan operations can be performed in normothermia on the beating heart with acceptable mortality. The extracardiac conduit Fontan procedure has the benefits of less surgical injury and a higher intraoperative flexibility.     

Impact of Fontan conversion with arrhythmia surgery and pacemaker therapy

Objective: In the long-term period after Fontan operation, atrial arrhythmia was one of the important factors to decide the postoperative quality of life. We reviewed the impact of Fontan conversion with arrhythmia surgery and pacemaker therapy. Methods: Thirty-eight patients underwent Fontan conversion using extracardiac conduit from 1992, and 22 patients with atrial arrhythmia underwent maze procedure simultaneously using cryoablation or radiofrequency ablation and epicardial DDD pacemaker implantation and 16 patients had regular ‘sinus’ rhythm before Fontan conversion. Mean follow-up period was 52 months. Pre- and postoperative clinical course were analyzed. Average weight, age at Fontan conversion, and years after first Fontan operation were 49.0 kg, 25.8 years old, 14.7 years, respectively. Nineteen percent of patients were in New York Heart Association class I (NYHA I), and 74% of patients were in NYHA II, and 7% were in NYHA III, respectively. Results: Except three early deaths, actual survival rate at 1 year and 5 years were 80% and 64%, respectively. In survivors, 80% of the patients obtained regular heart rhythm including artificial pacemaker rhythm, although only 43% of the patients had regular ‘sinus’ rhythm before the Fontan conversion. Postoperative average cardiothoracic ratio and oxygen saturation (SpO₂) were 50% and 94%, and 74% of patients were in NYHA I and 26% were in NYHA II, respectively, after Fontan conversion. Conclusions: Mid-term results of Fontan conversion with arrhythmia surgery and pacemaker therapy were acceptable. Restoration of regular rhythm might improve the postoperative NYHA status and the activity of the daily life.     

The role of bidirectional cavopulmonary shunt on selection of Fontan patients]

There are no objection against that pulmonary vascular resistance index (PVRI) is one of the most important factors for completion of successful application of Fontan-type operation. However, calculated PVRI in single ventricle physiology in often unreliable because of difficulty in accurate measurement of pulmonary blood flow, especially in patients with decreased pulmonary blood flow. Although the role of bidirectional cavopulmonary shunt (BCPS) in such patients has been increasingly recognized, the impact of BCPS on PVRI has not been fully understood. Between November 1993 and November 1996, 24 patients, aged between 0.54 and 22.2 years, with a wide variety of cardiac malformations underwent BCPS, and were followed up for the mean of 15.1 months. There were four hospital deaths (16.7%) and three deaths in follow-up (12.5%). Serial catheterization revealed that significant increase in mean arterial oxygen saturation from 75.8% to 83.9% (p = 0.005), and decrease in mean Nakata's index from 433 to 311 (p < 0.0001). PVRI calculated by using formulas derived from Ohm's law before BCPS (Pulmonary flow was derived from Fick formula) was highly (greater than 10 u.m2) or moderately (between 4 and 10 u.m2) elevated in 6 and 7 patients, respectively. However, PVRI in these patients was normal after BCPS. Fourteen out of 24 patients underwent total cavopulmonary connection (TCPC) with 8 to 15 months of interval from BCPS, and have been currently surviving, and in NYHA functional class I or II, except 2 patients who underwent take-down. In conclusion, early and midterm outcome after staged operation appears to be excellent. BCPS is a good interim procedure, in part because one can more properly select patients undergoing Fontan operations from the PVRI point of view.     

Fontan completion after balloon angioplasty of multiple pulmonary artery stenosis in a child with pulmonary atresia with intact ventricular septum; report of a case

A 5-year-old boy was referred to our institute for cardiac evaluation having been previously seen at another center. He had been diagnosed a pulmonary atresia with intact ventricular septum just after birth, and undergone Blalock-Taussig shunts. Although his central pulmonary artery pressure was high (mean pressure 26 mmHg) after bilateral Blalock-Taussig shunts, multiple peripheral pulmonary artery stenosis protected his pulmonary vascular bed from pressure load. We released the multiple pulmonary artery stenosis partly by catheter intervention and partly by surgical operation, and staged Fontan operation was completed. The surgery in combination with catheter therapies would expand the indication of Fontan-type operation, and it would contribute to the patients' postoperative prognosis.     

Evidence of pulmonary vascular disease after heart transplantation for Fontan circulation failure

OBJECTIVES: Elevated pulmonary vascular resistance may contribute to late Fontan circulation failure but is difficult to assess in such patients. Our aims were to assess outcomes of patients with failed Fontan circulation after heart transplantation and to determine whether elevated pulmonary vascular resistance might have contributed to the failure. METHODS: Fifteen patients (14 Fontan circulations, 1 Kawashima circulation) underwent transplantation. The most common indication was ventricular dysfunction (mean ventricular end-diastolic pressure 12.5 mm Hg). Patients with early failures (n = 4) required transplantation less than 1 year after the Fontan operation. Those with late failures (n = 11) underwent transplantation at least 1 year after the Fontan operation. Mean age at transplantation was 11.6 years. Mean Fontan-transplantation interval was 7.4 years. Mean pulmonary arterial pressure, transpulmonary gradient, and pulmonary vascular resistance before and after transplantation were assessed. Paired t tests of variable differences were used to compare variables. Survival was estimated by the Kaplan-Meier method. RESULTS: In-hospital mortality was 7%. There were 2 late events (1 death, 1 retransplantation) related to compliance or rejection issues. Graft survivals were 93%, 82%, and 82% at 3, 5, and 7 years, respectively. Posttransplantation pulmonary vascular resistance was elevated (>2.0 Wood units . m 2 ) in 11 of 14 survivors past initial hospitalization (mean 3.3 +/- 1.7 Wood units . m 2 ). Only patients with early Fontan failures (3 of 4) had normal posttransplantation pulmonary vascular resistance. In paired comparisons, posttransplantation transpulmonary gradient was increased by a mean of 6.8 mm Hg ( P < .0001) relative to pretransplantation value. CONCLUSIONS: Outcomes after heart transplantation for failed Fontan circulation were good. Mild-to-moderate pulmonary vascular disease was evident after heart transplantation for late failure. Elevated pulmonary vascular resistance is a likely contributor to Fontan circulation failure.     

Relationship between postoperative atrial volumes and prognosis after modified Fontan operation in patients with complex cardiac anomalies

We evaluated right and left atrial (RA and LA) volumes after modified Fontan operation in 6 patients, in 1 with mitral atresia and 5 with single ventricle. The age at operation was 10.2 +/- 2.5 (mean +/- SD) years. The interval between operation and study was 4.1 +/- 4.8 months. RA and LA maximum volume indexes (VImax) (ml/m2) were measured by angiography. One patient died 72 days after operation due to low output syndrome. In survived patients (n = 5), postoperative pulmonary arterial wedge pressure was 6 +/- 3 mmHg, right atrial pressure 13 +/- 5 mmHg, and end-diastolic pressure in the systemic ventricle 6 +/- 3 mmHg, and one who died had 18 mmHg, 25 mmHg, and 9 mmHg in each parameter, respectively. LAVImax in the survivors (53 +/- 18 ml/m2) were in the range of normal or above normal value. The nonsurvivor had extremely small LAVImax (13 ml/m2). RAVImax in the alive patients (25 +/- 11 ml/m2) was smaller than normal (data was not available in a non-survived patient). These results may indicate that a normal LA volume appears to be as important factor for the prognosis after modified Fontan operations with intraatrial routing for complex lesions regardless of the postoperative RA volume.     

Thromboembolic complications after Fontan procedures: comparison of different therapeutic approaches

BACKGROUND: Although patients after Fontan procedure have a high incidence of thromboembolic complications, anticoagulant therapy is not handled uniformly. We analyzed the frequency and clinical relevance of thromboembolism after Fontan procedure and compared different therapeutic approaches. METHODS: From 1986 to 1998, 101 patients (mean age, 7.3 +/- 8.1 years) underwent Fontan type procedure (modified Fontan, n = 40; total cavopulmonary connection, n = 61). In 85 of 87 survivors, transthoracic echocardiography was performed; and in 31 transesophageal echocardiography and/or angiography was performed. Mean follow-up was 5.7 +/- 3.5 years. Three groups with different anticoagulant regimen were compared: group I without medication (n = 45), group II with acetylsalicylic acid therapy (n = 14) and group III with Coumadin (n = 26). RESULTS: Thromboembolic events occurred in 13 of 85 patients (15.3%; 3.3 events/100 patient-years). Type of operation as well as other known risk factors had no influence on the rate of thromboembolism. Within the first postoperative year, seven of 13 events occurred. A second peak developed beyond 10 years of follow-up. Patients benefit significantly from Coumadin compared with those who did not receive any medication, with similar results in the entire population and the subgroup of patients with total cavopulmonary connection (log-rank, p = 0.031 and p = 0.033, respectively). With 4.2 events/100 patient-years, the cumulative event rate was substantially higher in group I than with 1.6 in group II and with 1.1 in group III. No relevant bleeding complications occurred. CONCLUSIONS: Thromboembolism is frequent after Fontan procedure with a peak during the first postoperative year and another peak beyond 10 years of follow-up. Coumadin is the most effective prophylactic therapy in preventing thromboembolism. Therefore, we suggest initial oral anticoagulation therapy in patients with Fontan type operation.     

Fontan procedure for hypoplastic left heart syndrome.

Since 1985, 354 neonates have undergone palliative reconstruction for hypoplastic left heart syndrome with 109 early deaths and 12 late deaths. Of the survivors, before 1989, 77 patients underwent a subsequent modified Fontan operation, consisting of baffling the atrial septal defect to the tricuspid valve (initial 25 patients) or intraatrial baffling of the inferior vena cava to the pulmonary arteries and superior vena cava (52 patients). There were 17 early deaths and three late deaths. Major serous effusions developed in 42 patients (54%) after Fontan operation. Since 1989, a staged approach to Fontan's operation was undertaken in an effort to reduce the volume load of the right ventricle as early as possible, to minimize the impact of rapid changes in ventricular geometry and diastolic function that can accompany a primary Fontan operation, and to reduce effusive complications. Thus, at a mean age of 6 months, 121 patients have undergone closure of aortopulmonary shunt, augmentation of central pulmonary arteries, and association of the superior vena cava with the branch pulmonary arteries (hemi-Fontan procedure). Of these, 61 patients have already undergone completion of the Fontan procedure with six early deaths and three late deaths. Major serous effusions developed in 28 patients (46%) with the staged Fontan. For perspective, the contemporary experience since January 1991 consists of 58 neonates who have undergone initial palliation with 11 deaths (19%), 17 patients who have undergone the hemi-Fontan procedure with one death (6%), and 21 patients who have undergone completion of the Fontan operation with one death (5%).(ABSTRACT TRUNCATED AT 250 WORDS)