Fontan conversion with arrhythmia surgery.

OBJECTIVE: Hemodynamic abnormalities and refractory atrial arrhythmias in patients late after the Fontan operation result in significant morbidity and mortality. We reviewed our experience with Fontan conversion and concomitant arrhythmia surgery. METHODS: Between January 1996 and February 2004, 16 patients underwent Fontan conversion and arrhythmia surgery. Mean age at the initial Fontan operation was 5.1+/-3.5 (range: 2-15) years and mean age at Fontan conversion was 17.0+/-5.8 (range: 6-30). The initial Fontan operations were atriopulmonary connections in 14 patients, extracardiac lateral tunnel in 1, and intracardiac lateral tunnel in 1. The types of arrhythmia included atrial flutter in 10 patients and atrial fibrillation in 3. Fontan conversion operation was performed with intracardiac lateral tunnel in 5 patients and extracardiac conduit in 11. Arrhythmia surgery included isthmus cryoablation in 10 patients and right-sided maze in 3. RESULTS: There has been no mortality. At Fontan conversion operation, 7 patients required permanent pacemaker. All patients have improved to New York Heart Association class I or II. With a mean follow-up of 26.9+/-30.6 (range:1-87) months, 16 patients had sinus rhythm, 2 patients had transient atrial flutter which was well controlled, and 2 patients required permanent pacemaker during follow-up. CONCLUSIONS: Fontan conversion with concomitant arrhythmia surgery and permanent pacemaker placement is safe, improves New York Heart Association functional class, and has a low incidence of recurrent arrhythmias. In most patients, concomitant permanent pacemakers are needed.     

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.     

A successful conversion of failed Fontan circulation to total cavopulmonary connection in a case of tricuspid atresia without pulmonary stenosis

A 12-year-old boy was first admitted to Tokyo Metropolitan Children’s Hospital at 2 days of age with the diagnosis of tricuspid atresia without pulmonary stenosis, normally related great arteries, ventricular septal defect, and persistent left superior vena cava (PLSVC). He underwent pulmonary artery banding at the age of 1 year and atrial septectomy at 2.9 years. At the age of 6.3 years, he underwent successful Bjork operation in conjunction with closure of a trial and ventricular septal defects. Late after surgery, however, he developed progressive intolerance to exercise (NYHA III), cardiomegaly, and depressed ST segments in the left precordial leads. Catheter examination at the age of 11.9 years disclosed moderately elevated pressures (15 mmHg) in the right atrium and pulmonary artery, depressed left ventricular function with ejection fraction (EF) of 0.48, and marked dilatation of the coronary sinus (CS). Right atriography revealed retrograde filling of the dilated CS up to the junction with PLSVC. With supposition that the combined elevation of the volume and pressure loads on CS had disturbed both coronary circulation and ventricular function, he, at the age of 12 years, underwent take down of the Fontan circulation and conversion to the total cavopulmonary connection (TCPC). His postoperative recovery was uneventful and, at 2.5 years after the latest surgery, he is leading a normal life. The findings of the chest roentgenogram and electrocardiogram reverted to normal and EF returned to the normal value (0.66). This experience draws us to conclude that conversion to TCPC is a safe and effective mean to salvage the patients with failed Fontan circulation. This experience also suggests that dilatation of CS is a predictor of the failed or failing Fontan circulation.     

Management of late complications after classic Fontan procedure by conversion to total cavopulmonary connection.

Classic Fontan procedures, such as atriopulmonary connection or atrioventricular connection, are associated with right atrial dilatation and several late complications. Seven patients with univentricular heart who presented with exercise intolerance (n = 7), severely dilated right atrium (n = 7) with pulmonary vein compression (n = 3), atrial arrhythmias resistant to medical treatment (n = 4), cyanosis (n = 4), diffuse effusions and oedema (n = 1), and protein-losing enteropathy (n = 1), underwent conversion to total cavopulmonary connection 5.8-14.4 years after a previous atriopulmonary connection (n = 6) or atrioventricular connection (n = 1). A 14-year-old boy who, preoperatively, was in ventricular failure and a very poor state died early after conversion because of low cardiac output. All survivors had either marked or partial clinical improvement with regression of cardiomegaly, absence of pulmonary vein compression or cyanosis, and recovery of sinus rhythm. Conversion to total cavopulmonary connection appears to be effective in the treatment of late complications after classic Fontan procedures. It should be considered early in symptomatic patients, before significant ventricular dysfunction and clinical deterioration ensue.     

Extracardiac Fontan conversion, cryoablation, and pacemaker placement for patients with a failed Fontan

OBJECTIVE: We review our experience with Fontan conversion and cryoablation in patients with an atriopulmonary Fontan in low cardiac output from arrhythmia or venous obstruction, including two patients with protein losing enteropathy. METHODS: Fifteen patients (mean age 25.0 +/- 8.4 years) underwent extracardiac Fontan conversion, cryoablation, and pacemaker placement between November 1999 and December 2004. Twelve patients were in NYHA class III and three were in NYHA class IV. Twelve had clinically important intraatrial reentry tachycardia refractory to medical therapy. RESULTS: Follow-up was between 2 and 62 months (mean 38.4 +/- 17.7). One death occurred at seven days after surgery due to sepsis and multisystem organ failure. The second death occurred at five days from myocardial depression following surgery. One patient with PLE preoperatively died to malnutrition and sepsis on POD number 52. The second patient with protein losing enteropathy had improved NYHA classification, cessation of albumin transfusions, and a normal stool alpha antitrypsin level (down from 4.1 mg/g preoperatively). All surviving patients improved NYHA classification to class I or II. Sustained arrhythmias could not be induced in any patient. One patient had recurrence of intraatrial reentrant tachycardia eleven months postoperatively that required electrical cardioversion and is currently well controlled on one medication. The other patients are not on any antiarrhythmic medical therapy. CONCLUSION: Extracardiac Fontan, cryoablation, and pacemaker placement reduced atrial arrhythmias and improved NYHA classification. In selected patients, this operation offers improvement in clinical outcome and is an alternative to transplantation. Protein losing enteropathy may not be a contraindication to performing Fontan conversion with cryoablation.     

Total cavopulmonary conversion and maze procedure for patients with failure of the Fontan operation.

OBJECTIVES: Hemodynamic abnormalities and refractory atrial arrhythmias in patients late after the Fontan operation result in significant morbidity and mortality. We review our experience with conversion to total cavopulmonary artery connections and arrhythmia surgery. METHODS: Between 1994 and 2001, 40 patients underwent Fontan conversion and arrhythmia surgery. Significant hemodynamic lesions such as aortic aneurysm (n = 1), atrioventricular valve insufficiency (n = 8), and pulmonary arterioplasty (n = 9) were repaired concomitantly. Thirty-four patients were in New York Heart Association class III or IV. Mean age at the original Fontan operation was 7.5 +/- 6.5 years and mean age at Fontan conversion was 18.7 +/- 9.0 years. Arrhythmia surgery has evolved from isthmus cryoablation in 10 patients to right-sided maze in 16 patients for atrial reentry tachycardia. The maze-Cox III operation was used for 14 patients with atrial fibrillation. Atrial (n = 33) and dual-chamber (n = 5) pacemakers were placed. RESULTS: There has been no early mortality. Chest tubes were removed on postoperative day 9.0 +/- 6.0. Hospital stay was 11.8 +/- 6.6 days. Three patients required cardiac transplantation at 8 days, 9 months, and 33 months postoperatively. There was 1 death 2 years postoperatively from acute myocardial infarction. For the entire series, arrhythmia recurrence is 12.5%, with only 10% of patients receiving long-term antiarrhythmic medications; these patients were among the first 8 patients in our series. Most patients are in New York Heart Association class I or II. Bruce protocol in 12 patients showed increased tolerance (P <.05). CONCLUSIONS: Fontan conversion to total cavopulmonary connection with concomitant arrhythmia surgery is excellent therapy for patients whose Fontan repair has failed. Fontan conversion is safe, improves New York Heart Association class, improves exercise tolerance, and has a low incidence of recurrent arrhythmias.     

The beneficial vortex and best spatial arrangement in total extracardiac cavopulmonary connection

OBJECTIVE: Total extracardiac cavopulmonary connection is an established procedure, but the best spatial arrangement remains controversial. On the basis of our clinical experience with total extracardiac cavopulmonary connection, we performed quantitative and qualitative flow analysis on total extracardiac cavopulmonary connection models simulating the two most frequent arrangements applied to our patients to determine the most favorable hydrodynamic pattern. METHODS: We selected two main groups among 110 patients who underwent total extracardiac cavopulmonary connection, those with left-sided inferior vena cava anastomosis (type 1) and those with facing superior and inferior vena cava anastomoses (type 2). Blown-glass total extracardiac cavopulmonary connection phantom models were constructed on the basis of nuclear magnetic resonance and angiographic images. Flow measurements were performed with a Nd:YAG Q-switched laser and a particle imaging velocimetry system. A power dissipation study and a finite-element numeric simulation were also carried out. RESULTS: When applying superior and inferior vena caval flow proportions of total systemic venous return of 40% and 60%, respectively, a vortex was visualized in the type 1 phantom that rotated counterclockwise at the junction of the caval streams. This apparent vortex was not a true vortex; rather, it represented a weakly dissipative recirculating zone modulating the flow distribution into the pulmonary arteries. The power dissipation and finite-element numeric stimulation confirmed the beneficial nature of the apparent vortex and a more energy-saving pattern in the type 1 phantom than in the type 2 phantom. CONCLUSION: Total extracardiac cavopulmonary connection with left-sided diversion of the inferior vena caval conduit anastomosis is characterized by a central vortex that regulates the caval flow partitioning and provides a more favorable energy-saving pattern than is seen with the total extracardiac cavopulmonary connection with directly opposed cavopulmonary anastomoses.     

Criss-cross heart: anesthetic considerations for total cavopulmonary shunt (Fontan procedure)

We report the anesthetic technique used for interatrial septal defect a 2-year-old girl weighing 11 Kg who presented with crossed atrioventricular connection (criss-cross heart), transposition of the great vessels, interatrial and interventricular septal defects, and subpulmonary and pulmonary valve stenoses. The patient was proposed for total cavopulmonary anastomosis with basal arterial oxygen saturation (SapO2) at 65%. Anesthesia was induced with inhaled agents and after intubation, hyperventilation was induced to achieve an end-tidal carbon dioxide pressure around 27 mm Hg. General anesthesia was provided in combination with spinal infusion of morphine chloride (100 microg Kg(-1)) for pain control. A Fontan procedure was carried out uneventfully with cardiopulmonary bypass. Milrinone was used at the extracorporeal circuit pump outlet. The patient was transferred to the pediatric intensive care unit where she was extubated without complications 90 minutes after admission. Anesthetic management is based on maintaining adequate preloading doses by administering volume, inhaled and/or intravenous agents, or use of vasoconstrictors and adjustment of ventilator parameters to modify pulmonary or systemic vascular resistance. We were able to maintain normal arterial pressure in our patient and provide adequate preloading through hyperventilation to reduce pulmonary vascular resistance.     

Prophylaxis of thromboembolic complications after the Fontan operation (total cavopulmonary anastomosis)

OBJECTIVES: Thrombotic events have been reported as a major cause of morbidity after the Fontan procedure. There is no consensus concerning the postoperative mode and duration of anticoagulation prophylaxis. In a retrospective study, we evaluated the results of a prophylactic regimen on the basis of the surgical technique, potentially predisposing risk factors, and specific sequelae. METHODS: We evaluated 142 surviving patients after total cavopulmonary anastomosis (mean follow-up was 91.1 +/- 43.9 months). Prophylactic antithrombotic treatment was initiated in 86 patients with partial prosthetic venous pathway with acetylsalicylic acid; 45 patients with complete autologous tissue venous pathway or partial prosthetic venous pathway received no anticoagulation, and 11 patients received warfarin sodium (Coumadin). During long-term follow-up, 22 patients (12 after acetylsalicylic acid medication) crossed over to warfarin. RESULTS: Thrombotic events occurred in 10 patients (7%), with systemic venous thrombus formation in 8 (5.6%), stroke in 2 (1.4%), and a peak incidence during the first postoperative year. Eight of 10 patients were receiving heparin therapy mainly for prolonged postoperative immobilization. During follow-up, none of the 74 patients receiving acetylsalicylic acid and 1 of 40 patients without medication presented with thrombus formation. Under warfarin medication, 1 of 28 patients had an asymptomatic thrombus. Expected freedom from a thromboembolic event was 92% at 5 years and 79% at 10 years. There was no association with coagulation factor abnormalities. Protein-losing enteropathy was present in 4 of 10 patients. CONCLUSION: A prophylactic anticoagulation strategy that considers the surgical technique and potential predisposing circumstances proved effective in the prevention of late thrombotic complications after total cavopulmonary anastomosis. There is no need for routine anticoagulation during long-term follow-up after Fontan-type surgery in pediatric patients.     

Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience

BACKGROUND: Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. METHODS: Thirty-one patients (19.9 +/- 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. RESULTS: There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. CONCLUSIONS: Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.     

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 is 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.     

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.     

Outcome of revision surgery for failed primary Souter-Strathclyde total elbow prosthesis

Total elbow arthroplasty is used for the treatment of the painful, destroyed elbow joint. With the increase in elbow replacement surgery in recent years, the number of revision surgeries will also increase. At our center, 236 primary Souter-Strathclyde total elbow prostheses have been placed. Twenty-four of these have been revised and were followed up for a mean of 74 months (range, 12-165 months). The postoperative clinical outcome after revision surgery can approximate the outcome after primary elbow replacement. Unfortunately, 8 elbows needed to be re-revised, 7 elbow prostheses loosened, and we had 1 early dislocation during follow-up. Three of the eight re-revised elbows had a third revision. Two other patients had a resection arthroplasty because of deep infection after the first revision. After 5 years, 73.8% of the revised elbow prostheses were still in situ. The lack of other satisfactory treatment options, combined with the satisfactory clinical results, justify revision surgery of elbow prostheses. The absence of intraoperative fracture during removal of the relatively small standard components and the availability of long-stemmed revision components, which facilitate fixation in the impaired bone, both support our preference for the Souter-Strathclyde prosthesis.     

心房内侧隧道Fontan术纠治小儿复杂先天性心脏病47例

目的 总结应用心房内侧隧道Fontan术治疗47例小儿复杂先天性心脏病（先心病）的经验。方法 47例病儿年龄1岁5个月－14岁;体重9－42kg。病种包括三尖闭锁10例,单心室17例,右室双出口14例,完全性大动脉转位2例和纠正性大动脉转位4例。结果 术后早期死亡9例,手术死亡率19.1％。1995年前手术死亡率26.3％（5／19例）,1995年后（含1995年）14.3%(含1995年）14.3%(4/28例）。晚期死亡1例。结论 改良Fontan术适应证已扩大至许多解剖上不能纠治、功能上仅有一个单心室腔的复杂性紫绀型先心病,为提高手术疗效须严格掌握手术适应证;板障上开窗可有效降腔静脉和右房压力,减少胸腔引流量,缩短住院,是高体循环心排出量;少数不能耐受Fontan术后循环生理改变 的难治性低心输出量病儿,应早期果断再手术改建为双向腔肺分流术（半Fontan术）     

Clinical results of conversion total hip arthroplasty after failed bipolar hemiarthroplasty

Total hip arthroplasty (THA) has been the most common surgery performed for complications of bipolar arthroplasty. We wanted to evaluate functional results and complications after conversion of bipolar hemiarthroplasty to THA in 25 patients followed for an average of 7.2 years. Indications for conversion included acetabular erosion with well-fixed femoral stem in 13 patients, acetabular erosion with femoral loosening in 8 patients, and periprosthetic fracture in 4 patients. The average Harris Hip Score improved from 41 (34 to 67) before conversion to 85 (65 to 95) at final follow-up. The average Western Ontario McMaster Universities Osteoarthritis Index (WOMAC) score improved from 81 (41 to 96) before conversion to 17 (8 to 36) at final follow-up. Pain component of WOMAC improved from an average of 15 (7 to 20) to 4 (0 to 11). The stiffness component of WOMAC improved from 6 (2-8) to 30 (0-4) and the function component improved from 59 (36-68) to 17 (8-36). The complications included 2 recurrent dislocations, 2 dislocations, 1 acetabular loosening, and 1 trochanteric nonunion. One patient required revision of acetabular component. The conversion THA after symptomatic bipolar arthroplasty can offer reliable pain relief and functional improvement. The perioperative complications approximate those of revision THAs.     

Immediate results of bidirectional cavopulmonary anastomosis and Fontan operations in adults

The purpose of this study is to analyze the immediate results of bidirectional cavopulmonary anastomosis (BCPA) and Fontan operations performed in adults, and to reveal the risk factors. During the years 1983-2010, 681 consecutive patients underwent BCPA or a Fontan operation. Fifty-three of 681 patients were more than 18?years of age. Twenty-nine adults underwent BCPA and 24 underwent a Fontan operation. Immediate results of surgical treatment were followed during the hospital period. The average number of exceeded 'operability' criteria by Choussat et al. [Choussat A, Fontan F, Besse P, Vallot F, Chauve A, Bricaud H. Selection criteria for Fontan procedure. In: Anderson RH, Shinebourne EA, editors. Pediatric Cardiology. Edinburgh: Churchhill Livingstone, 1977:559-566] was significantly higher in patients from the BCPA group compared to the Fontan group (1.3±0.8 vs. 0.9±0.7, P=0.034). Hospital mortality after BCPA in adults was 6.9% (2/29) and did not differ from children (7.1%, 19/268), P=0.634. Hospital mortality after Fontan operation in adults was 8.3% (2/24) and did not differ from children (11.9%, 43/360), P=0.419. The frequency of non-lethal hospital complications was higher in patients after a Fontan operation. Patients from the Fontan group more frequently developed arrhythmias and prolonged pleural effusions. Preoperative regurgitation at atrioventricular valves was an independent risk factor for hospital mortality and morbidity after a Fontan operation. BCPA and Fontan operations performed in adults are accompanied by good immediate results and considerably improves patients' condition.