成人功能性单心室的一期Fontan手术治疗

目的:总结和分析成人功能性单心室Fontan手术治疗的早、中期结果。方法: 自2001年11月~2010年11月,共15例成人功能性单心室患者行一期Fontan手术治疗,术前心功能NYHA Ⅱ级4例,NYHA Ⅲ级11例;手术适应证为肺动脉发育良好,肺动脉平均压≤15 mmHg;手术方法为心外管道法Fontan手术,6例行人工血管和右心房开窗吻合,随访3月～6年。结果: 围术期死亡2例,术后平均拔除气管插管时间11 h（6～576 h）,平均住院时间13 d（7～44 d）,平均中心静脉压13 mmHg（7～16 mmHg）,平均动脉血氧饱和度96％（87%～99％）;12例患者心功能得到明显改善,4例心功能NYHA Ⅰ级,8例心功能NYHA Ⅱ级,1例心功能仍为NYHA Ⅲ级。结论: 一期心外管道法Fontan手术治疗成人功能性单心室手术早期和中期疗效良好。     

Arrhythmias after the Fontan procedure.

OBJECTIVE--To study the determinants and outcome of arrhythmias after the Fontan type operation. DESIGN--Retrospective analysis of data in patients operated on between 1972 and 1986 (follow up 5-19 years (mean 12 years)). PATIENTS--All 60 patients undergoing a Fontan type procedure at the National Heart Hospital, London, during the study period (mean age (SD) 12.3 (6.8) years). RESULTS--Postoperative arrhythmias occurred in 34 patients (57%), and 11 (58%) of 19 early postoperative deaths (within seven days) were related to arrhythmias. Early arrhythmias occurred in 19 (32%) patients of whom 11 (58%) died. All patients with early atrial fibrillation and His bundle tachycardia died and only preoperative atrial fibrillation recurred early. There was a higher incidence of early arrhythmias, which were less well tolerated, in double inlet single ventricle patients (9/19) than in those with tricuspid atresia (8/37). There were no other preoperative determinants of early arrhythmias or deaths from early arrhythmia. Late (after seven days) arrhythmias occurred in 15 (37% of hospital survivors). They had higher right atrial (RA) pressures both early and late after operation and had lower ventricular ejection fractions late after operation. Of those with atrial arrhythmias 86% had RA obstruction and 57% had an RA thrombus or pulmonary embolism at presentation; this was also confirmed in two patients in whom late sudden deaths occurred. Atrial fibrillation early after reoperation for RA obstruction was fatal. The actuarial arrhythmia free survival for hospital survivors was 60% at 10 years. CONCLUSIONS--Early postoperative arrhythmias were poorly tolerated, particularly atrial fibrillation and His bundle tachycardia. Previous atrial fibrillation was a relative contraindication to this procedure. Late postoperative arrhythmias were associated with higher RA pressures measured both early and late after operation and worse late ventricular function. Late arrhythmias may be the first manifestation of RA obstruction, which must be sought. RA thrombus was common in patients with atrial arrhythmias and should be treated early with anticoagulants.     

High frequency of arrhythmias after Fontan operation indicates earlier anticoagulant therapy

BACKGROUND: As patients began to survive for longer periods following modified Fontan operations (conventional atrio-pulmonary connection), the late morbidity after this procedure became increasingly apparent. The purpose of the present study was to evaluate late sequelae of modified Fontan operations in long-term survivors (n=14) at our institute. METHODS AND RESULTS: The cohort consisted of patients who underwent a modified Fontan operation between 1981 and 1990. Thus, all patients were examined at least 10 years postoperatively in this study. Early mortality, within 30 days of the operation, was 17.6% (three of 17 patients died from low output syndrome). Excluding these early deaths, the cumulative survival rate at 5 and 10 years was 100 and 79%, respectively. Arrhythmias including atrial fibrillation or flutter were the main late causes of morbidity. The arrhythmia-free rate at 5 and 10 years was 77 and 50%, respectively. Although the quality of life was considered good because all patients (n=11) who survived for 10 years or more were in class I or II according to the New York Heart Association classification, most of them in fact suffered from potentially life-threatening arrhythmias. CONCLUSIONS: Meticulous attention to and utilization of established treatment strategies for arrhythmias including anti-arrhythmics, anticoagulants, catheter ablation or re-operation converting the circulation to the total cavopulmonary connection must be considered in long-term survivors following the modified Fontan operation. The fact that no one knows when the thrombogenic arrhythmias occur suggests anticoagulants should be initiated in the early postoperative period.     

Preliminary analysis of arrhythmias after the Fontan operation with extracardiac conduit compared with intra-atrial lateral tunnel.

BACKGROUND: It is to be expected that avoidance of the atrial suture line during extracardiac Fontan operation (ECFO) decreases the risk of postoperative arrhythmias. METHODS: Two groups of consecutive patients (23 with lateral tunnel (LTFO) and 24 with extracardiac conduit) who underwent successful Fontan operations between 5/93 and 10/98 were comparatively analyzed. All patients had postoperatively standard ECG and 24-hour monitoring. During follow-up, all patients had 2 - 8 (mean 3) standard ECG recordings per year and 76 % of the patients a 24-hour Holter ECG once a year. RESULTS: Median follow-up after ECFO and LTFO was 2.4 years and 4.5 years, respectively. The incidence of supraventricular tachyarrhythmias and bradyarrhythmias after ECFO versus LTFO was lower early after operation and during follow-up (p < 0.05). In follow-up, 20 ECFO patients (91 %) versus 11 patients after LTFO (52 %) remained in sinus rhythm (p < 0.01). Pacemaker insertion was required in 7 (33 %) LTFO patients but none of the ECFO patients (p < 0.01). CONCLUSIONS: The extracardiac Fontan operation decreases the incidence of early and medium-term postoperative arrhythmias. Comparative long-term results are not yet available.     

Arrhythmias after the Fontan procedure

Objective—To study the determinants and outcome of arrhythmias after the Fontan type operation.Design—Retrospective analysis of data in patients operated on between 1972 and 1986 (follow up 5–19 years (mean 12 years)).Patients—All 60 patients undergoing a Fontan type procedure at the National Heart Hospital, London, during the study period (mean age (SD) 12·3 (6·8) years).Results—Postoperative arrhythmias occurred in 34 patients (57%), and 11 (58%) of 19 early postoperative deaths (within seven days) were related to arrhythmias. Early arrhythmias occurred in 19 (32%) patients of whom 11 (58%) died. All patients with early atrial fibrillation and His bundle tachycardia died and only preoperative atrial fibrillation recurred early. There was a higher incidence of early arrhythmias, which were less well tolerated, in double inlet single ventricle patients (9/19) than in those with tricuspid atresia (8/37). There were no other preoperative determinants of early arrhythmias or deaths from early arrhythmia. Late (after seven days) arrhythmias occurred in 15 (37% of hospital survivors). They had higher right atrial (RA) pressures both early and late after operation and had lower ventricular ejection fractions late after operation. Of those with atrial arrhythmias 86% had RA obstruction and 57% had an RA thrombus or pulmonary embolism at presentation; this was also confirmed in two patients in whom late sudden deaths occurred. Atrial fibrillation early after reoperation for RA obstruction was fatal. The actuarial arrhythmia free survival for hospital survivors was 60% at 10 years.Conclusions—Early postoperative arrhythmias were poorly tolerated, particularly atrial fibrillation and His bundle tachycardia. Previous atrial fibrillation was a relative contraindication to this procedure. Late postoperative arrhythmias were associated with higher RA pressures measured both early and late after operation and worse late ventricular function. Late arrhythmias may be the first manifestation of RA obstruction, which must be sought. RA thrombus was common in patients with atrial arrhythmias and should be treated early with anticoagulants.     

Arrhythmias after the Fontan procedure

Objective—To study the determinants and outcome of arrhythmias after the Fontan type operation.

Design—Retrospective analysis of data in patients operated on between 1972 and 1986 (follow up 5–19 years (mean 12 years)).

Patients—All 60 patients undergoing a Fontan type procedure at the National Heart Hospital, London, during the study period (mean age (SD) 12·3 (6·8) years).

Results—Postoperative arrhythmias occurred in 34 patients (57%), and 11 (58%) of 19 early postoperative deaths (within seven days) were related to arrhythmias. Early arrhythmias occurred in 19 (32%) patients of whom 11 (58%) died. All patients with early atrial fibrillation and His bundle tachycardia died and only preoperative atrial fibrillation recurred early. There was a higher incidence of early arrhythmias, which were less well tolerated, in double inlet single ventricle patients (9/19) than in those with tricuspid atresia (8/37). There were no other preoperative determinants of early arrhythmias or deaths from early arrhythmia. Late (after seven days) arrhythmias occurred in 15 (37% of hospital survivors). They had higher right atrial (RA) pressures both early and late after operation and had lower ventricular ejection fractions late after operation. Of those with atrial arrhythmias 86% had RA obstruction and 57% had an RA thrombus or pulmonary embolism at presentation; this was also confirmed in two patients in whom late sudden deaths occurred. Atrial fibrillation early after reoperation for RA obstruction was fatal. The actuarial arrhythmia free survival for hospital survivors was 60% at 10 years.

Conclusions—Early postoperative arrhythmias were poorly tolerated, particularly atrial fibrillation and His bundle tachycardia. Previous atrial fibrillation was a relative contraindication to this procedure. Late postoperative arrhythmias were associated with higher RA pressures measured both early and late after operation and worse late ventricular function. Late arrhythmias may be the first manifestation of RA obstruction, which must be sought. RA thrombus was common in patients with atrial arrhythmias and should be treated early with anticoagulants.

     

Predictors of rhythm disturbances and subsequent morbidity after the Fontan operation

The electrocardiographic, hemodynamic and surgical data of 30 patients who underwent a Fontan operation between 1977 and 1986 were retrospectively reviewed to identify the incidence and predictors of immediate and late postoperative arrhythmias and associated morbidity in long-term survivors. Of 4 patients who died less than 1 year after operation (mortality 13%), 1 death was related to an arrhythmia. Three patients were not in sinus rhythm before operation and were excluded from the statistical analysis that examined predictors of arrhythmias. The remaining 23 long-term survivors have been followed 6.3 +/- 2.6 years (mean +/- standard deviation) since surgery and all remain in New York Heart Association functional class I or II. Ten patients (43%) developed immediate postoperative arrhythmias (less than or equal to 30 days) whereas 11 (48%) had late arrhythmias. With up to 10.7 years of follow-up, the proportion of patients free from late arrhythmias continues to decline. Arrhythmias included bradyarrhythmias, atrial tachyarrhythmias, the tachy-brady syndrome and supraventricular ectopic activity. Immediate postoperative arrhythmias predicted late arrhythmias (p = 0.022). The preoperative electrocardiogram was the only variable useful in predicting both immediate and late postoperative arrhythmias. A more negative P-wave deflection in lead V1 (-2.4 +/- 0.7 vs -1.4 +/- 1.2 mV, p = 0.02) predicted patients with immediate postoperative arrhythmias, whereas both greater P-wave duration and a more negative deflection in this lead predicted late arrhythmias (103 +/- 14 vs 83 +/- 20 ms, p = 0.01, and -2.5 +/- 0.8 vs -1.3 +/- 1.0 mV, p = 0.005, respectively).(ABSTRACT TRUNCATED AT 250 WORDS)     

Is it necessary to routinely fenestrate an extracardiac fontan?

OBJECTIVES: This study was conducted to assess the need for, and use of, fenestration of an extracardiac conduit Fontan. BACKGROUND: Fenestration of a Fontan connection has been proposed as a means of improving outcomes of single ventricle palliation. The benefit of fenestration is likely to be greatest in the early postoperative period when patients may experience increased pulmonary vascular resistance and decreased ventricular function due to the effects of cardiopulmonary bypass, aortic cross-clamping and positive pressure ventilation. However, there are potential drawbacks to fenestration. The utility of fenestration with extracardiac Fontan operation has not been determined. METHODS: Since 1992, 81 patients have undergone a modification of the Fontan procedure in which an extracardiac inferior cavopulmonary conduit is used in combination with a previously staged bidirectional Glenn anastomosis. We conducted a retrospective review of these patients. RESULTS: Fenestration was performed selectively in 32 patients (39%), including only 2 of the last 38 (5%). In seven patients, a fenestration was placed or clipped in the early postoperative period without cardiopulmonary bypass. There were two operative deaths. Prolonged (>2 weeks) pleural drainage occurred in 13 patients, 8 with fenestration and 5 without. In addition to undergoing earlier Fontan in our experience, patients who had a fenestration placed had significantly higher preoperative pulmonary vascular resistance, significantly higher common atrial pressure after Fontan and significantly lower post-Fontan systemic arterial oxygen saturation. Fontan pressure did not differ between nonfenestrated and fenestrated patients. At follow-up ranging to five years, there were two late deaths and no patients developed protein losing enteropathy. CONCLUSIONS: Fenestration is not necessary in most Fontan patients when an extracardiac conduit technique is performed as described in this article, and therefore, should not be performed routinely with the extracardiac conduit Fontan. The need for fenestration should be assessed after cardiopulmonary bypass when hemodynamics can be evaluated accurately. Fenestration can be placed and revised easily without bypass and with minimal intervention in patients with an extracardiac conduit Fontan.     

Long-term outcome and quality of life in adult patients after the Fontan operation

The first successful Fontan operation was performed in 1971, and this first cohort of Fontan patients is reaching adulthood with unclear outcome of this palliative procedure. We studied the mortality, morbidity, and quality of life in our adult Fontan patients. We examined all patients (n = 36) who underwent a Fontan procedure and were being seen in an adult outpatient clinic by using electrocardiography, exercise testing, and echocardiography. Quality of life was assessed by the Short Form 36 questionnaire. The mean follow-up period was 15 years (range 0 to 23). Of the initial 36 patients, 10 died (28%) at a mean of 10 years (range 0 to 21) after the Fontan operation and 1 patient underwent cardiac transplantation. Reoperations were performed in 21 patients (58%), and the most common reason was revision of the Fontan connection. Sustained supraventricular tachycardia was observed in 20 patients (56%) with an increased incidence of arrhythmias with longer follow-up. Thromboembolic events were detected in 9 patients (25%), 5 of whom had adequate anticoagulant levels at the time of event. The thromboembolic event was fatal for 3 patients. A total of 195 hospital admissions (mean 3.8 +/- 2.7, range 1 to 13) was recorded. Quality-of-life assessment showed physical functioning, mental health, and general health perception to be significantly lower for Fontan patients than for the normal Dutch population. Thus, we found high mortality and very high morbidity in adult patients after the Fontan operation. In particular, reoperations, arrhythmias, and thromboembolic events compromised quality of life.     

Arrhythmia Recurrence in Adult Patients with Single Ventricle Physiology Following Surgical Fontan Conversion

Objectives. To evaluate the incidence of atrial tachy-arrhythmia (AT) recurrence following conversion from right atrial-pulmonary artery (RA-PA) Fontan to total cavopulmonary connection (TCPC) in adults. Background. AT is a recognized sequel of Fontan palliation, especially in RA-PA Fontans, and is associated with significant morbidity. While catheter ablation achieves fairly reliable short-term success with low morbidity, conversion to TCPC with arrhythmia surgery is a highly effective treatment option for the classical Fontan patients with incessant AT. Methods. Single center retrospective review. Results. Twenty-seven adults underwent Fontan conversion from RA-PA to TCPC, mostly for AT indications (n = 24). Nine (33%) underwent conversion to a lateral tunnel (LT) and 18 (67%) to an extracardiac (EC) Fontan. Two patients died <30 days post-operatively. Both had liver failure and had been turned down for cardiac/liver transplantation. In-hospital complications occurred in 15/27 patients (55%), including recurrence of AT requiring cardioversion in six patients (22%) and persistent pleural effusions in 4 (15%). Mean follow-up was 4.2 years (range 3 months–14 years). Functional capacity improved from mean New York Heart Association (NYHA) class 1.8 pre-conversion to 1.2 post-conversion (P= 0.008). Twenty-one patients had concomitant arrhythmia surgery (MAZE in 12 patients with IART and Cox-MAZE in nine patients with A-Fib +/− IART). Of these, 3/21 (14%) had AT recurrence >3 months following conversion. Conclusions. Conversion from RA-PA Fontan to TCPC, with arrhythmia surgery, decreases AT recurrence and improves functional capacity. The risk of peri-operative mortality is highest in patients with cirrhosis. AT recurred in 14% of patients.     

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.     

Results of the modified Fontan operation in adults

The results of the modified Fontan procedure were examined in 77 patients 18 years of age and older who underwent operation at this institution between October 1973 and December 1986. This series represents 16% of the patients undergoing the modified Fontan operation during that period. Of the 77 patients (46 men and 31 women), 29 had tricuspid atresia, 30 had double-inlet ventricle and 18 had other complex lesions. There were 5 (6%) hospital deaths, compared with 67 (17%) for patients of all ages undergoing the Fontan operation during the same period. Patient age 18 years and older was not a significant risk factor. There were 8 late deaths. Detailed follow-up data were available on 61 (95%) survivors from 5 months to 12 years (mean 4 years) postoperatively. Fifty-seven (93%) of the patients were in New York Heart Association class I or II, 20 (33%) were taking no medication and 11 (18%) were taking digoxin alone. Seventeen (28%) patients complained of persistent ascites or edema. The study indicated that (1) the modified Fontan operation can be performed with low mortality in adults, (2) the long-term outcome is favorable and (3) persistent ascites or edema may be a problem in some patients.     

Late postoperative cardiac arrhythmias after total cavopulmonary anastomosis and correlation with age of the patients at operation

Late postoperative arrhythmias are a common problem associated with significant morbidity following Fontan operations. We analyzed in our patients whether age at the time of a modified Fontan procedure (total cavopulmonary connection) has an influence on the frequency of late postoperative arrhythmias. We examined all 66 long-term surviving patients who underwent a total cavopulmonary connection (TCPC) in our center during a nine year period. At the time of TCPC, 31 children were younger (group I) and 35 children were older than 4 years (group II). The follow-up period was almost identical in both groups (group I: 4.29 years, group II: 4.52 years). We evaluated all ecgs performed at maximum intervals of 6 months and all Holter ecgs performed at intervals of 12 months. Atrial tachyarrhythmias (supraventricular tachycardias, atrial flutter) were registered in six patients (9%). All of these patients belonged to group II (17%). Ventricular arrhythmias (couplets, ventricular tachycardia) were found in 3% of the younger and 17% of the older patients. Abnormalities of sinus node function were registered in equal frequency in younger and older children (group I: 35%, group II: 31%), while pauses with a duration of > 2 s where present only in older children (group II: 14%). Pacemaker implantation or antiarrhythmic therapy became necessary in 10/66 patients (15%) affecting none of the children who underwent the TCPC < 4 years (group I) but 10/35 children (group II) who underwent the TCPC at an older age (29%). According to our findings in the medium-term follow-up after TCPC, atrial tachyarrhythmias and complex ventricular arrhythmias are registered less frequently in children who underwent the modified Fontan procedure at an age of less than 4 years. Although further studies will have to show whether these differences persist in the long-term follow-up, these findings represent an additional argument for the elective performance of modified Fontan operations at an age of less than 4 years.     

Clinical outcomes of acute myocarditis in childhood

OBJECTIVE: To describe clinical outcomes of a paediatric population with histologically confirmed lymphocytic myocarditis. DESIGN: A retrospective review between November 1984 and February 1998. SETTING: A major paediatric tertiary care hospital. PATIENTS: 36 patients with histologically confirmed lymphocytic myocarditis. MAIN OUTCOME MEASURES: Survival, cardiac transplantation, recovery of ventricular function, and persistence of dysrhythmias. RESULTS: Freedom from death or cardiac transplantation was 86% at one month and 79% after two years. Five deaths occurred within 72 hours of admission, and one late death at 1.9 years. Extracorporeal membrane oxygenation support was used in four patients, and three patients underwent heart replacement. 34 patients were treated with intravenous corticosteroids. In the survivor/non-cardiac transplantation group (n = 29), the median follow up was 19 months (range 1.2-131.6 months), and the median period for recovery of a left ventricular ejection fraction to > 55% was 2.8 months (range 0-28 months). The mean (SD) final left ventricular ejection and shortening fractions were 66 (9)% and 34 (8)%, respectively. Two patients had residual ventricular dysfunction. No patient required antiarrhythmic treatment. All survivors reported no cardiac symptoms or restrictions in physical activity. CONCLUSIONS: Our experience documents good outcomes in paediatric patients presenting with acute heart failure secondary to acute lymphocytic myocarditis treated with immunosuppression. Excellent survival and recovery of ventricular function, with the absence of significant arrhythmias, continued cardiac medications, or restrictions in physical activity were the normal outcomes.     

Clinical Importance of Fontan Circuit Thrombus in the Adult Population: Significant Association With Increased Risk of Cardiovascular Events

BackgroundThe impact of Fontan circuit thrombus is poorly understood. The objectives of this study were to determine (1) the incidence of Fontan circuit thrombus and proportion of silent thrombus; (2) any association between Fontan circuit thrombus and markers of Fontan circulatory dysfunction; and (3) the association of Fontan circuit thrombus with adverse cardiac outcomes.MethodsWe conducted a retrospective review of adult patients who underwent the Fontan procedure (aged > 18 years) followed at St. Paul’s Hospital who underwent cardiac computed tomography or magnetic resonance imaging assessment (n = 67). Fontan circulatory dysfunction markers included clinical heart failure, N-terminal pro-brain natriuretic peptide, ventricular dysfunction, atrioventricular valvular regurgitation, refractory arrhythmias, declining exercise capacity, and hepatic/renal dysfunction. Adverse cardiac outcomes were death, heart transplantation, or surgery for Fontan revision or atrioventricular valve replacement.ResultsFontan circuit thrombus was present in 15 of 67 patients (22%): 41% (7/17) classic/modified Fontan and 16% (8/50) total cavopulmonary connection. Incidence was 36% among those suspected to have Fontan circuit thrombus; 14% in those with no clinical/echocardiographic suspicion; and clinically silent in 40% diagnosed with Fontan thrombus. The time from Fontan surgery to Fontan circuit thrombus diagnosis was 22 ± 6 years in the classic/modified group vs 14 ± 8 years in the total cavopulmonary connection group (P = 0.03. Fontan circuit thrombus was associated with adverse cardiac outcomes (27% [4/15] vs 8% [4/52], P = 0.02), but there was no difference in Fontan circulatory dysfunction markers.ConclusionGiven the incidence of Fontan circuit thrombus and association with adverse cardiac outcomes, routine surveillance of the Fontan circuit should strongly be considered. The identification of thrombus should lead to anticoagulation implementation/optimization, along with screening/intervention for reversible Fontan circulatory issues in an attempt to prevent adverse cardiac outcomes.     

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.     

Risk factors for arrhythmia and late death in patients with right ventricle to pulmonary artery conduit repair--Japanese multicenter study

BACKGROUND: Arrhythmia and late cardiac deaths are thought to be major complications in patients after right ventricle (RV) to pulmonary artery (PA) conduit repair, although the incidence and predictors of these complications remain unknown. The aim of this study was to clarify the incidence and risk factors for arrhythmia and late deaths in patients with the RV to PA conduit repair through a Japanese multicenter study. METHODS: Three hundred fifty-one hospital survivors who underwent the RV to PA conduit repair before 1995 were studied. RESULTS: Survival rate after repair was 92% at 10 years, 88% at 20 and 25 years, respectively. Late death was observed in 30 (8.5%) including 4 patients with sudden death (SD). Higher right ventricular pressure (p = 0.02), larger cardio-thoracic ratio after repair (p = 0.02) and higher incidence of brady- or tachy-arrhythmia and SD (9/30) were associated with late death. Six (1.7%) patients developed ventricular tachycardia or ventricular fibrillation (VT/Vf). There were 22 patients who had 23 new-onset supraventricular tachy-arrhythmia (SVT). Right ventricular hypertension (p = 0.04) was associated with VT/Vf or SD. Male sex (p < 0.01), absence of previously aorto-pulmonary shunt (p < 0.05), older age at repair (p < 0.01) or longer length of follow-up (p < 0.01) were associated with SVT. CONCLUSION: Arrhythmia and late sudden death are relatively common late after the RV to PA conduit repair. Our data support recent surgical strategies of earlier primary operation and timely reoperation for progressive right ventricular outflow stenosis that may reduce the incidence of late arrhythmias and SD.     

Impact of arrhythmia circuit cryoablation during Fontan conversion for refractory atrial tachycardia

Refractory atrial arrhythmias in late postoperative Fontan patients are usually associated with residual hemodynamic abnormalities and result in significant morbidity and mortality. Surgical revision of the Fontan anastomosis may improve hemodynamics without eliminating tachycardia. This study sought to assess the impact of surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion on the clinical recurrence of tachycardia. Sixteen consecutive atriopulmonary Fontan patients with refractory atrial arrhythmias underwent surgical conversion to lateral tunnel total cavopulmonary anastomosis (15) or Fontan revision (1 patient). The initial 4 patients underwent Fontan conversions alone, without specific arrhythmia surgery. The subsequent 12 patients underwent electrophysiologically guided cryoablation of the tachycardia circuits at the time of surgical conversion. The mean age at Fontan revision was 15.6 +/- 3.8 years. Cryoablation was directed to 3 identified major tachycardia circuits: the inferomedial right atrium, the superior rim of the prior atrial septal defect patch, and along the lateral right atrial wall. Transmural antitachycardia pacemakers were implanted in 11 of the 16 patients. There was no surgical mortality in either group, and all patients improved in functional classification. All patients not undergoing cryoablation experienced recurrent symptomatic tachycardia requiring antiarrhythmic therapy (median follow-up, 54 months) versus 2 of 12 patients receiving cryoablation (median follow-up, 25 months; p <0.02). Thus, surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion is highly effective in the management of refractory atrial arrhythmias, and is superior to Fontan conversion alone.     

Long-term results of the Fontan operation for double-inlet left ventricle

The purpose of this study was to quantify and determine predictors of long-term survival and functional outcome in patients with double-inlet left ventricle (DILV) after the Fontan operation. The Fontan operation has become the procedure of choice for DILV. Early survival has improved, but mortality and morbidity persist. Record review and follow-up questionnaires were used to ascertain the status of 225 patients with DILV who had Fontan operations from 1974 to 2001 at the Mayo Clinic. The median age at operation was 9 years. The median follow-up period was 12 years (range 3 months to 25 years). There were 22 deaths (9.3%) <30 days after the operation. Early mortality decreased to 3% (2 of 70 patients) after 1989. Overall late survival was 78% (159 of 203 patients). Actuarial survival for the 203 early operative survivors at 5, 10, 15, and 20 years was 91%, 80%, 73%, and 69%, respectively. Forty-nine percent (99 of 203) had additional surgical procedures after the Fontan operation. Other frequent late events were atrial flutter or fibrillation (57%), protein-losing enteropathy (9%), and thromboembolic events (6%). Current health status was described as good or excellent by 84% of patients, fair by 18%, and poor by 12%. In conclusion, the Fontan operation for DILV is now performed with a low operative mortality rate. Long-term survival has improved, and most patients have good functional status.     

Long-term anticoagulation therapy and thromboembolic complications after the Fontan procedure

BACKGROUND: The necessity for chronic anticoagulation of Fontan patients remains controversial. We determined the prevalence of thromboembolic complications after the Fontan procedure in relation to different long-term anticoagulation strategies. METHODS: The clinical outcomes, postoperative anticoagulation strategies and occurrence of thromboembolic complications in 102 ethnic Chinese patients who had undergone Fontan procedure between 1980 and 2002 were reviewed. RESULTS: The early and late surgical mortalities, all unrelated to thromboembolism, were 10.8% (11/102) and 5.8% (6/104), respectively. Of the 85 survivors, 46 (54%) were maintained on long-term warfarin therapy, 8 (9%) on aspirin prophylaxis while 31 (37%) were not on chronic anticoagulation. Four (4.5%) patients, two with and two without warfarin prophylaxis, developed thromboembolic complications at 0.14 to 7.7 years after the Fontan procedure (0.74%/patient-year). Three had a grossly dilated right atrium after atriopulmonary connection, two of whom had atrial fenestrations. The other had atrial tachycardia. Freedom from development of thromboembolic complications (mean+/-S.E.) at 1, 5 and 10 years after surgery was 97+/-19%, 96+/-2.5% and 92+/-4.2%, respectively. When compared with those on long-term warfarin therapy, patients without chronic anticoagulation were followed-up longer (p=0.001), more likely to have undergone atriopulmonary connection (p<0.001), less likely to have fenestrations (p=0.02) and cardiac arrhythmias (p=0.02) but not predisposed to increased risk of thromboembolism (p=1.00). CONCLUSION: The study supports the contention that chronic anticoagulation may not be required for majority of ethnic Chinese Fontan patients. Nonetheless, it may perhaps be considered in those with grossly dilated right atrium, cardiac arrhythmias and residual right-to-left shunts.