The beneficial effects of total cavopulmonary conversion and arrhythmia surgery for the failed Fontan

Postoperative Fontan patients can develop hemodynamic abnormalities and refractory atrial arrhythmias resulting in significant morbidity and mortality. We present our experience with total cavopulmonary artery conversion and arrhythmia surgery. Between 1994 and 2001, 41 patients underwent total cavopulmonary artery conversion and arrhythmia surgery. Significant hemodynamic lesions were repaired concomitantly: aortic aneurysm (n = 1), atrioventricular valve insufficiency (n = 8), and pulmonary artery stenosis (n = 9). Thirty-five patients were in New York Heart Association class III or IV. Mean age at original Fontan was 7.5 ± 6.5 years, at Fontan conversion, 18.7 ± 9.0 years. Arrhythmia surgery for atrial re-entry tachycardia evolved from isthmus cryoablation (n = 10) to right-sided maze (n = 17). Maze-Cox III was used for 14 patients with atrial fibrillation. Atrial (n = 34) and dual chamber (n = 5) pacemakers were placed. Mortality and reoperation for bleeding rates are 0%. Chest tubes were removed on postoperative day 9.0 ± 6.0. Mean hospital stay was 11.8 ± 6.6 days. Three patients required cardiac transplantation at 8 days, 9 months, and 33 months postoperatively. There was one long-term death from acute myocardial infarction 2 years postoperatively. For the entire series, arrhythmia recurrence is 12.2% (5/41). Only 9.8% of patients (4/41) receive chronic antiarrhythmic medications; these patients were among the first eight in the series. Most patients are in New York Heart Association I or II. Bruce protocol in 12 patients showed increased tolerance (P < .05) Total cavopulmonary artery conversion with concomitant arrhythmia surgery is excellent therapy for patients with failed Fontan. It is safe, improves New York Heart Association class, improves exercise tolerance, and the incidence of recurrent arrhythmias is low. Copyright © 2002 by W.B. Saunders Company     

The failing Fontan circulation: successful conversion of atriopulmonary connections

OBJECTIVES: Symptoms from low cardiac output or refractory atrial arrhythmias are complicating atriopulmonary (classical) Fontan connections. We present our experience of converting such patients to total cavopulmonary connections with and without arrhythmia surgery. METHODS: Between 1997 and 2002, 15 patients (mean age, 19.7 +/- 7.0 years) underwent conversion operations 12.7 +/- 3.5 years after atriopulmonary Fontan operations. Preoperative New York Heart Association functional class was I in 2 patients, II in 2 patients, III in 6 patients, and IV in 5 patients. Four patients underwent intracardiac lateral tunnel conversion alone, and 11 received extracardiac total cavopulmonary connection, right atrial reduction, and cryoablation. RESULTS: No mortality occurred. One patient had conduit obstruction in the immediate postoperative period requiring replacement, and another required a redo operation for endocarditis. Average hospitalization was 17.9 +/- 9.38 days; chest drains were removed on median day 4 (range, 1-29; mean, 7.4 +/- 7.58 days). At follow-up (mean, 42.6 +/- 22.1 months), late atrial arrhythmias had recurred in 3 of 4 patients with intracardiac total cavopulmonary connections (without ablation) and 1 of 11 patients with extracardiac total cavopulmonary connections with ablation. All patients are in New York Heart Association class I or II. Exercise ability (Bruce protocol) improved 69% from a mean of 6.18 +/- 4.01 minutes to 10.45 +/- 2.11 minutes (P <.05). Need for antiarrhythmic agents decreased postoperatively (patients receiving < or =1 antiarrhythmic: 9 preoperatively vs 15 at long-term follow-up, P <.05). No patient has required transplantation. Protein-losing enteropathy, which was present in 1 patient, improved transiently with conversion. There was 1 late death from gastrointestinal hemorrhage. CONCLUSIONS: Fontan conversion can be achieved with low mortality and improvement in New York Heart Association class and exercise ability. Concomitant arrhythmia surgery reduces the incidence of late arrhythmias.     

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.     

Outcome of symptomatic patients undergoing extracardiac Fontan conversion and cryoablation

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 2 patients with protein-losing enteropathy. METHODS: Ten patients (mean age 21.1 +/- 7.0 years) underwent extracardiac Fontan conversion, cryoablation, and pacemaker placement between November 1999 and April 2002 (13.1 +/- 4.1 years after the original atriopulmonary connection). Eight patients were in New York Heart Association class III and 2 were in New York Heart Association class IV. Nine patients had clinically important intra-atrial reentry tachycardia refractory to medical therapy. RESULTS: Follow-up was between 3.1 and 32.6 months (16.8 +/- 9). One death occurred at 7 days after surgery due to sepsis and multisystem organ failure. The second death occurred at 48 days from complications of protein-losing enteropathy. The second patient with protein-losing enteropathy had improved New York Heart Association classification, cessation of albumin transfusions, and a normal stool alpha antitrypsin level (down from 4.1 mg/g preoperatively). Five patients improved to New York Heart Association class I and 3 patients to New York Heart Association class II. Sustained arrhythmias could not be induced in any patient. Seven patients are on no antiarrhythmics. One patient had recurrence of intra-atrial reentrant tachycardia 11 months postoperatively, which required electrical cardioversion; this patient's symptoms are currently well controlled on 1 medication. CONCLUSION: Extracardiac Fontan, cryoablation, and pacemaker placement reduced atrial arrhythmias and improved New York Heart Association classification in all surviving patients. 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.     

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.     

Aortic regurgitation with dilation of ascending aorta and right coronary artery occlusion by a rudimentary aortic cusp

BACKGROUND: Atrial arrhythmia remains one of the major complications in the longer term after the Fontan procedure. METHODS: Conversion to total cavopulmonary connection was carried out concomitantly with surgical intervention for atrial arrhythmia in 4 patients undergoing the Fontan procedure by atriopulmonary connection and having continual atrial fibrillation or flutter in the longer term after the initial procedure. RESULTS: The surgical intervention restored sinus rhythm. Transient atrial fibrillation occasionally occurred after the reoperation in 1 patient in whom duration of preoperative arrhythmic period had been 6 years, and defibrillation was needed twice. In the other 3 patients, no episodes of paroxysmal arrhythmia have been noted. Subsequent to renewal of the Fontan circulation, cardiac index increased, with systemic venous pressure decreasing. All 4 patients are currently doing well with their functional status of New York Heart Association functional class I. CONCLUSIONS: Combination of conversion to total cavopulmonary connection and concomitant surgical intervention for atrial arrhythmia is effective, when used appropriately and in a timely manner in patients with atrial arrhythmia in the longer term after the initial Fontan procedure by atriopulmonary connection.     

Fontan conversion with novel direct ablation after childbirth: Report of a case

A 38-year-old woman underwent atriopulmonary Fontan surgery at age 18 years and subsequently successfully delivered a girl by cesarean section at age 34. Her condition later deteriorated due to atrial tachyarrhythmia and progressed to New York Heart Association (NYHA) class IV heart failure. Her treatment, at age 36, comprised total cavopulmonary connection conversion, direct right atrial ablation with bipolar radiofrequency devices, the creation of an atrial septal defect, and placement of a dual-chamber permanent pacemaker. Three years after the conversion, her condition has improved to NYHA class I.     

Improved early results with the Fontan operation in adults with functional single ventricle

BACKGROUND: A growing number of adults with functional single ventricles are presenting as candidates for first-time and redo-Fontan operations. This study describes the clinical presentation and early operative results of adults who have undergone Fontan modifications. METHODS: Between July 1995 and April 2003, 23 patients (>18 years old) had Fontan operations. We retrospectively reviewed their perioperative courses. RESULTS: Twenty-three Fontan operations (first-time [n = 8] and redo [n = 15]) were performed with no early or late deaths. No patient has required reoperation. One patient has been listed for orthotopic heart transplantation. The overall mean age is 23 years (18 to 41 years); mean follow-up, 30 months; median postoperative hospital stay, 8 days (4 to 34 days); and median duration of chest tube drainage, 4 days (2 to 12 days). The postoperative New York Heart Association (NYHA) functional class was improved in 22 of 23 patients. Eight first-time Fontan operations (7 of 8 nonfenestrated) were performed; lateral tunnel (n = 7) and extracardiac conduit (n = 1). Two patients had preoperative arrhythmias. New onset arrhythmias (ventricular tachycardia and sinus node dysfunction), requiring treatment, occurred in two patients. Fifteen redo-Fontan operations (all nonfenestrated) were performed; lateral tunnel (n = 5) and extracardiac conduit (n = 10). Fifteen patients had preoperative arrhythmias, thirteen of which had intraatrial reentry tachycardia (IART) and required antiarrhythmic medications. Concomitant intraoperative radiofrequency ablation (RFA) (n = 11) and cryoablation (n = 1) procedures were performed. In the immediate postoperative period, there was IART recurrence in five patients (post-RFA [n = 4] and postcryoablation [n = 1]). At latest follow-up, no patient is being treated with antiarrhythmic medications. Two patients had new onset atrial arrhythmias that required treatment. CONCLUSIONS: The Fontan operation can be performed in adults with minimal morbidity and improved NYHA functional class. New onset arrhythmias requiring treatment are sources of perioperative morbidity. Complete arrhythmia resolution of the preoperative arrhythmia may not be achieved in the immediate postoperative period in redo-Fontan patients. However, modification (intraoperative radiofrequency ablation-right atrial debulking) of the atrial tachycardia circuits in the redo-Fontan patients can result in complete resolution of preoperative atrial tachyarrhythmias at early follow-up.     

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.     

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 Extracardiac Total Cavopulmonary Connection For Definitive Conversion to the Fontan Circulation: Summary of Early Experience and Results

A bstract Between July 1991 and March 1993, five children (ages 2 to 6 years) with complex congenital heart disease have undergone a new operation for conversion to the Fontan circulation. This procedure combines a bidirectional Glenn shunt with an extracardiac lateral tunnel (ELT) to carry systemic venous return to the pulmonary arteries (PAs). The ELT was constructed so that the circumference consists of Gore-Tex (2/3) and lateral epicardial atrial wall (1/3). The ELT can be performed with all varieties of single ventricle physiology, as in our patients with tricuspid atresia (n = 3), dextrocardia (n = 1), and situs inversus with levocardia (n = 1). PA reconstruction was required in four patients. At follow-up from 1 to 20 months, all patients are in New York Heart Association Class I and in normal sinus rhythm. Postoperative catheterization has revealed low PA pressures (< 12 ± 1 mmHg) and angiography has shown excellent ELT function with brisk flow into the PAs bilaterally. All patients maintain an O₂ saturation > 94% on room air. The advantages of this new extracardiac modification of Fontan's operation are: (1) aortic cross-clamping is not usually required; (2) incorporation of lateral atrial wall in ELT allows for growth while permitting construction of a fenestration or adjustable atrial septal defect in high risk patients; (3) absence of atriotomy and intraatrial suture lines may decrease late risk of arrhythmias; (4) early or late baffle leaks cannot occur; (5) intraatrial obstruction from the baffle cannot occur; (6) coronary sinus remains in low pressure atrium; and (7) hydrodynamic benefits of the total cavopulmonary connection are preserved. We recommend this procedure for patients undergoing surgical conversion to the Fontan circulation.     

Long-term results of atriopulmonary fontan connection for complex cardiac anomalies

To evaluate the long-term results of atriopulmonary Fontan connection, we reviewed the outcome of 20 consecutive patients (single ventricle 8, double outlet right ventricle 8, tricuspid atresia 3, mitral atresia 1; mean age, 13 +/- 11) who underwent this operation between 1981 and 1997 at our institution. Glenn shunt preceded Fontan operation in 1 patient. Four patients had a concomitant bidirectional Glenn anastomosis at the time of the Fontan operation. Follow-up was 100% complete, and total cumulative follow-up of all patients was 241 patient-years (maximum, 20 years). Three hospital deaths and 5 late deaths were observed, resulting in 58 +/- 11% of cumulative survival at 20 years after the operation. Sudden death probably due to arrhythmias was the major cause of the late death, and occurred in 3 patients. Reoperation was required in 5 patients (2%/patient-year) at 1 to 17 years postoperatively. Re-closure of an atrioventricular valve was performed for failure of the valve closure in 3 patients at 1 to 6 years postoperatively. Reconstruction of the stenotic atriopulmonary connection (APC) was concomitantly required in 2 of the 3 patients. Pacemaker implantation was required for bradycardia with atrial fibrillation in 1 patient at 17 years. One patient with atrial flutter developed intraright atrial thrombosis at 12 years after the initial Fontan operation, and this patient underwent conversion to extracardiac conduit cavopulmonary connection with right atrial maze procedure. Atrial tachyarrhythmias are most common complications, and occurred in 12 patients (5.0%/patient-year), resulting in 40 +/- 13% of the cumulative free rate at 15 years after the operation. Thus, failure of the atrioventricular valve closure and stenosis of APC were important early to intermediate complications after the atriopulmonary Fontan connection. On the other hand, atrial tachyarrhythmias were inevitable, and were most common late complications leading to intra-right atrial thrombosis, which was successfully resolved by conversion of the conventional atriopulmonary Fontan anastomosis to extracardiac conduit cavopulmonary connection.     

The impact of ventricular morphology on midterm outcome following completion total cavopulmonary connection.

OBJECTIVE: This study was undertaken to compare the early and midterm outcome following completion total cavopulmonary connection (TCPC) in patients with a single functional ventricle of left or right morphology. METHODS: Between August 1996 and July 2001, 103 patients underwent completion TCPC following an interim superior cavopulmonary connection. The single functional ventricle was of left (n=44, 42%) or right ventricular morphology (n=59, 58%). The TCPC was performed using an extracardiac conduit (n=84, 82%) or a lateral atrial tunnel (n=19, 18%), and was fenestrated in 53 patients (51%). Outcomes studied included duration of pleural effusions and in-patient hospitalisation; early mortality, reoperation and reintervention; actuarial survival, freedom from reoperation and reintervention; and current functional status. These were assessed according to a series of preoperative, operative and postoperative variables. Follow-up was complete with a median interval of 17 months (range, 21 days-5.2 years). RESULTS: Early mortality was 1.9% (n=2) and one other patient required takedown of the Fontan circulation. There was one late death. Five-year survival with a Fontan circulation (+/-1 SEM) was 95.6+/-2.5%. Forty-two patients (41%) had prolonged pleural drainage (> or =14 days) and 41 patients (40%) had a prolonged hospital stay. Five-year freedom from reoperation and reintervention (+/-1 SEM) were 92.2+/-5.0 and 73.4+/-6.0%, respectively. The Fontan procedure was associated with an improved functional class (P<0.005) and all current survivors (n=99) are in either New York Heart Association classes I or II. Multivariate analysis identified left atrial isomerism as the single risk factor for death (P<0.05). Independent risk factors for prolonged hospital stay included a morphologic right ventricle (P<0.05), increased postoperative pulmonary artery pressures (P<0.005) and an unfenestrated Fontan procedure (P<0.01). CONCLUSIONS: In this contemporary series, the modified Fontan procedure was characterised by low early mortality, excellent midterm survival, and improved functional class independent of the morphology of the single functional ventricle. Nevertheless, a morphologic right ventricle was a risk factor for prolonged in-patient hospitalisation and may yet influence long term survival.     

Total cavopulmonary anastomosis using atrial septal flap: technique and early results

Introduction The Fontan procedure has undergone many modifications to avoid atrial arrhythmias and thrombus formation. We used patient’s interatrial septum as a flap to direct the inferior venacaval blood to the superior venacava. Methods Seventeen patients, aged 1 to 17 years, underwent modified total cavopulmonary anastomosis. Interatrial septum was used to create the inner half of the atrial tunnel, outer half being formed by right atrial free wall. Post-operatively, all patients underwent echocardiography. Seven patients underwent 24 hour ambulatory Holter monitoring and 6 patients underwent cardiac catheterization and cineangiography. Results There was one early death due to low cardiac output. One patient had transient supraventricular arrhythmia. Two patients had singnificant pleural effusion. Holter Monitoring reveled sinus rhythm in all 7 patients studied. Follow up ranged from 18 to 60 months and patients were evaluated as they came for follow up. Long term follow up is currently being compiled. There was one late death from a non-cardiac cause. The remaining patients were in New York Heart Association (NYHA) Class I or II. All patients were in sinus rhythm. Echocardiography and cineangiography revealed absence of obstruction or leak. Conclusions Total cavopulmonary anastomosis using autogenous atrial septum is a useful modification for classical cavopulmonary anastomosis and provides good early results.     

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.     

Long-term results of the fenestrated Fontan operation. Progress of patients with patent fenestrations.

The fenestrated Fontan operation was introduced as a modification of the "completed" Fontan operation for patients with high risk factors, and low operative mortality has frequently been reported. However, use of the umbrella device is now restricted, and this procedure should be performed without subsequent closure. In this paper, we review our clinical experience with this procedure and discuss ongoing problems. Sixteen patients (4 tricuspid atresia and 12 other cardiac anomalies including 5 cases of univentricular heart) underwent the fenestrated Fontan operation (7 atriopulmonary and 9 total cavopulmonary connection). All of them have some risk factors for a completed Fontan operation. There were three early deaths of the 16. Two experienced an anticipated thromboembolic accident, one of which involved the pulmonary aspect while the other involved the arterial aspect. Patients who survived the operation have progressed well and have a clinical status of New York Heart Association class I, with the exception of one late death due to congestive heart failure. There have been no thromboembolic accidents in this group during the late follow-up period. Spontaneous closures of the fenestrations were noted in two patients. The late mean Qp/Qs value in patients with patent fenestrations was 0.80 +/- 0.1, SaO2 was 88.8 +/- 5.6%, and right atrial pressure was 9.7 +/- 3.8 mmHg. No major problems have been encountered in patients with a patent fenestration over extended periods. A modified Fontan operation to fit a permanently open fenestration may be considered as a final surgical option for certain high-risk patients.     

Reversed remodelling in dilated cardiomyopathy by passive containment surgery is associated with decreased circulating levels of endothelin-1.

OBJECTIVE: To evaluate the influence on circulating levels of endothelin-1 and big endothelin-1 in relation to echocardiographic findings and functional assessment, by passive containment surgery in heart failure patients with dilated cardiomyopathy. METHODS: Thirteen patients with dilated cardiomyopathy subjected to cardiac surgery received the Acorn Cardiac Support Device. Patients with ischemic dilated cardiomyopathy (n=6) underwent coronary artery bypass surgery receiving one to three bypass grafts. In the idiopathic dilated cardiomyopathy group (n=7), mitral valve plasty was performed in five patients while two patients received the cardiac support device only. Circulating plasma levels of endothelin-1 and big endothelin-1 were measured in all patients before surgery and 12 months after surgery. Concomitantly New York Heart Association functional class and 6-min walk were evaluated and cardiac dimensions measured with echocardiography. RESULTS: Following surgery there was a significant decrease in circulating plasma levels of endothelin-1 (5.9+/-0.6 pM preoperatively vs 4.3+/-0.3 pM postoperatively, P<0.05). New York Heart Association functional class improved (2.8+/-0.2 preoperatively vs 1.8+/-0.2 postoperatively, P<0.05). The 6-min walk increased (384+/-24 m preoperatively vs 465+/-33 m postoperatively, P<0.05). There was also a decrease in left ventricular end diastolic diameter (69+/-2mm preoperatively vs 62+/-2mm postoperatively, P<0.05) and left ventricular end systolic diameter (60+/-2mm preoperatively vs 54+/-3mm postoperatively, P<0.05). Linear correlation revealed a relationship between decreased left ventricular end diastolic diameter and decreased endothelin-1 levels (R=0.56; P<0.05). CONCLUSIONS: Following passive containment surgery using the Acorn Cardiac Support Device there is a decrease in circulating levels of endothelin-1 concomitant with a decrease in cardiac dimensions and function improvement.     

Extracardiac conduit with a limited maze procedure for the failing Fontan with atrial tachycardias

BACKGROUND: Atrial arrhythmias are a frequent late complication of Fontan procedures. Conversion to an extracardiac conduit combined with reducing right atrial size should improve hemodynamics and reduce the development of tachyarrhythmias. More effective control may be achieved by interrupting atrial arrhythmia circuits and atrial pacing. METHODS: Between May 1997 and October 2001, 6 patients underwent a revision of their intracardiac Fontan anastomosis. The conversion included an extracardiac conduit insertion, limited right atrial maze procedure, and pacemaker placement. Ages ranged from 14 to 34 years (mean, 22.8 years) at an average of 14.6 +/- 4.4 years after their original Fontan procedure. All of the patients had medically uncontrollable atrial tachyarrhythmias with markedly reduced exercise tolerance. RESULTS: All of the patients survived with an average hospital stay of 16.7 days. Exercise tolerance has improved in all 6 patients, and atrial tachycardias have either decreased (n = 2) or disappeared (n = 4). Two patients are taking antiarrhythmic medications other than digoxin. Follow-up is a mean of 28.5 months. CONCLUSIONS: Limited right atrial maze procedure, atrial size reduction, and pacemaker implantations are worthwhile additions to simple conversion to an extracardiac conduit in the failing Fontan.     

Nontransplant cardiac surgery for end-stage cardiomyopathy

OBJECTIVE: To treat end-stage cardiomyopathy, we evaluated endoventricular circular patch plasty, partial left ventriculectomy, and valvular reconstruction alone in our 2-year experience. METHODS: Among 86 patients with heart failure evaluated between December 1996 and February 1999, 33 patients with ischemic cardiomyopathy (25 men and 8 women; mean age 61 +/- 7.8 years; New York Heart Association class 3.5 +/- 0.5) were treated with endoventricular circular patch plasty combined with coronary bypass grafting (84%) and mitral reconstruction (36%). The other 53 patients with nonischemic cardiomyopathy (45 men and 8 women; mean age 48 +/- 14.3 years, New York Heart Association class 3.7 +/- 0.5), were treated by left ventricular reduction by partial left ventriculectomy (n = 37) or patch plasty (n = 3) and valve reconstruction alone (n = 13). The first 24 patients (group I) underwent ventriculectomy with or without valve reconstruction; the more recent 29 patients (group II) underwent left ventricular reduction (n = 16) or valve reconstruction alone (n = 13) on the basis of the intraoperative echocardiographic evaluation to observe changes of wall motion and thickness during cardiopulmonary bypass. RESULTS: Ischemic Group: Hospital mortality in elective (n = 26) and emergency (n = 7) operations was 4% and 43%, and 3 patients died in the late postoperative period. Mean New York Heart Association class and ejection fraction improved from 3.5 +/- 0.5 to 1.5 +/- 0.7 and from 23% +/- 7.7% to 36% +/- 8.6%, respectively. Left ventricular end-diastolic and end-systolic volume indexes decreased from 162 +/- 46 mL/m(2) to 110 +/- 39 mL/m(2) and from 130 +/- 47 mL/m(2) to 70 +/- 32 mL/m(2), respectively. Nonischemic Group: In 40 patients with left ventricular reduction, hospital mortality in elective (n = 33) and emergency (n = 7) operations was 6% and 86%, and 5 patients died in the late postoperative period. Mean New York Heart Association class and ejection fraction improved from 3.7 +/- 0.5 to 1.7 +/- 0.6 and from 18% +/- 6.4% to 31% +/- 5.9%. Left ventricular end-diastolic and end-systolic volume indexes decreased from 203 +/- 45 mL/m(2) to 110 +/- 37 mL/m(2) and from 164 +/- 40 mL/m(2) to 79 +/- 33 mL/m(2), respectively. In 13 patients undergoing valve reconstruction alone (12 mitral with or without tricuspid and 1 tricuspid plus left ventricular assist device), hospital mortality in elective (n = 9) and emergency (n = 4) operations was 0% and 50% with no late deaths. Mean New York Heart Association class and ejection fraction improved from 3.6 +/- 0.5 to 2.0 +/- 0.5 and from 22% +/- 6.0% to 30% +/- 14.5%, respectively. Mean left ventricular end-diastolic and end-systolic volume indexes decreased from 170 +/- 34 mL/m(2) to 150 +/- 50 mL/m(2) and from 140 +/- 38 mL/m(2) to 104 +/- 40 mL/m(2), respectively. Overall mortality decreased from 50% in group I to 10% in group II. The survival estimates at 2 years were 77% (confidence limits 57%-88%) in the ischemic group and 63% (confidence limits 47%-75%) in the nonischemic group (no significant difference). The analysis of our data showed that the factors influencing the surgical results for dilated cardiomyopathy were presence of severe mitral regurgitation, preoperative New York Heart Association functional class IV with emergency operation, and operative procedures with randomly performed partial left ventriculectomy without an intraoperative echo test. CONCLUSION: Endoventricular circular patch plasty, partial left ventriculectomy, and solo valve reconstruction can be performed with an acceptably low risk as elective operations. The selection of operative procedures in idiopathic dilated cardiomyopathy and avoidance of emergency surgery improved operative mortality and morbidity. Among patients who survived at least 1 year, there were no late deaths up to 30 months' follow-up.     

Late pulmonary valve replacement after repair of tetralogy of Fallot

BACKGROUND: Pulmonary regurgitation appears to be well tolerated early after repair of tetralogy of Fallot; however, it may result in progressive right ventricular dilatation and dysfunction necessitating eventual valve replacement. Our objective was to review our experience with late pulmonary valve replacement after complete repair of tetralogy of Fallot.Methods and results: A total of 42 patients (16 female and 26 male) were operated on between July 1, 1974, and January 1, 1998. Mean age was 22 years (range 2-65 years). The mean interval between tetralogy repair and pulmonary valve replacement was 10.8 years (range 1.6 months-33 years). Mean follow-up was 7.8 +/- 6.0 years (maximum 23 years). Indications for pulmonary valve replacement included decreased exercise tolerance in 58%, right heart failure in 21%, arrhythmia in 14%, syncope in 10%, and progressive isolated right ventricular dilatation in 7%. Heterograft prostheses were used in 33 patients and homografts in 9. Five patients underwent isolated pulmonary valve replacement; concomitant procedures performed in 37 patients included tricuspid valve repair/replacement (n = 18), residual ventricular septal defect repair (n = 12), atrial septal defect closure (n = 4), pulmonary artery patch angioplasty (n = 17), and right ventricular outflow tract enlargement (n = 13). One patient died early (2%) of multiorgan failure. There were 6 late deaths, 3 of which were cardiac related. Survival was 95.1% +/- 3.4% and 76.4% +/- 8.9% at 5 and 10 years, respectively. Functional class of patients was improved significantly; preoperatively, 76% of patients were in New York Heart Association class III-IV, and after pulmonary valve replacement, 97% of surviving patients were in class I-II (P =.0001). Moderate to severe reduction in right ventricular function was noted on preoperative echocardiography in 59% and on late echocardiography in 18% (P =.03). Of the 5 patients who had supraventricular arrhythmias before pulmonary valve replacement, 1 had postoperative recurrence and the arrhythmia is controlled with antiarrhythmic therapy; the other 4 are in normal sinus rhythm at late follow-up. Eight patients subsequently underwent pulmonary valve re-replacement without early mortality at a mean interval of 9.0 +/- 4.2 years (range 3.8-16.8 years). Freedom from pulmonary valve re-replacement was 93.1% +/- 4.7% and 69.8% +/- 10.7% at 5 and 10 years, respectively. The only significant risk factor for re-replacement was young age at the time of the initial pulmonary valve replacement (P =.023). CONCLUSION: Late pulmonary valve replacement after tetralogy repair significantly improves right ventricular function, functional class, and atrial arrhythmias, and it can be performed with low mortality. Subsequent re-replacement may be necessary to maintain functional improvement.