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.     

改良Fontan术后心律失常及危险因素

目的分析改良Ｆｏｎｔａｎ手术后心律失常及其危险因素，以期降低术后心律失常的发生率。方法对１９８４～１９９６年间１１１例患者术后心律失常发生类型及发生因素进行分析。结果房性心动过速（房速）发生率最高，占心律失常的４３．９％；非阵发性交界性心动过速次之，占２８．８％。房速者术后中心静脉压显著升高，右房与肺动脉连接者术后房速及非阵发性交界性心动过速的发生率均显著高于全腔静脉与肺动脉连接者。结论术后中心静脉压明显升高及右房与肺动脉连接术式是影响术后心率失常的主要危险因素。     

Cardiac transplantation after the Fontan or Glenn procedure

OBJECTIVES: The purpose of this study was to review the clinical course and outcome of cardiac transplantation after a failed Glenn or Fontan procedure. BACKGROUND: Late complications of the Glenn or Fontan procedure, including ventricular failure, cyanosis, protein-losing enteropathy, thromboembolism, and dysrhythmias often lead to significant morbidity and mortality. If other therapies are ineffective, cardiac transplantation is the only therapeutic recourse. Transplantation in this unique population presents significant challenges in the operative and perioperative periods. METHODS: The anatomic diagnoses, previous operations, clinical status, and indications for transplantation were characterized in patients transplanted after a Glenn or Fontan procedure. Outcomes after transplantation, including postoperative complications and mortality, were reviewed. Comparisons were made between survivors and nonsurvivors. RESULTS: Primary orthotopic cardiac transplantation was performed in 35 patients (mean age 15.7 +/- 8.5 years) with a mean follow-up of 54 +/- 46 months. A total of 11 patients had undergone a Glenn shunt and 24 patients a Fontan procedure. Indications for transplantation were a combination of causes including ventricular dysfunction, failed Fontan physiology, and/or cyanosis. Ten patients died 相似文献   

Surveillance for liver complications after the Fontan procedure

The physiological consequences of the Fontan circulation impose risk for hepatic dysfunction and may culminate in hepatic fibrosis, cirrhosis, and hepatocellular carcinoma. Consensus regarding appropriate surveillance modalities to diagnose liver disease in Fontan patients is lacking, in part due to the relative lack of strong evidence and prospective studies in this patient population. The goal of this paper is to critically review the current evidence and provide recommendations for the surveillance of hepatic complications in the post‐Fontan patient population.     

Pathogenetic mechanisms of venous congestion after the Fontan procedure

BACKGROUND: The hemodynamic status after a Fontan type procedure for definitive palliation of functionally univentricular hearts is dominated by a high central venous pressure, which seems to be one of several factors responsible for venous congestion appearing as a frequent complication in the early and late postoperative course. The purpose of our study was to find other hemodynamic parameters correlating with the presence of venous congestion and effusions in these patients. METHODS: We compared the hemodynamic data of 18 patients who had an uneventful long-term course after a Fontan type procedure with the respective data of 10 patients who developed symptoms of venous congestion in the immediate postoperative period. Based on a theoretical model, we developed an algorithm to calculate mean hydrostatic capillary pressure from mean arterial pressure, systemic vascular resistance index and central venous pressure. RESULTS: Pulmonary vascular resistance index (2.1 +/- 1.0 mmHg L-1 min m2), mean left atrial pressure (9.7+/-4.0 mmHg) and cardiac index (3.6+/-0.6 l/min/m2) are mainly normal in patients with venous congestion in the immediate postoperative period, but mean hydrostatic capillary pressure is significantly higher compared to patients without venous congestion (24.3+/-3.1 vs 18.3+/-4.0 mmHg). Lower mean hydrostatic capillary pressures in these patients are due to a highly significant increase of systemic vascular resistance index (18.6+/-4.2 versus 33.6+/-6.6 mmHg L-1 min m2) and a concomitant decrease of cardiac index to 2.4+/-0.3 l/min/m2. CONCLUSIONS: The increase of mean hydrostatic capillary pressure, caused by high central venous pressures but also by relatively low systemic vascular resistance indexes, seems to be the hemodynamic key parameter responsible for venous congestion and effusions in patients after a Fontan type procedure in the immediate postoperative period.     

Radionuclide evaluation of lung perfusion after the Fontan procedure

Lung perfusion was evaluated in 19 patients in whom a Fontan operation had been performed at a mean age of 3.7 years. First pass and equilibrium data were acquired during the lung particle perfusion scan 0.5 to 7.9 years (mean 3.7 years) following the Fontan procedure. Abnormalities of lung perfusion were documented in 8 patients. Minimal underperfusion of small areas of either right or left lung were noted in 4 patients, while the remaining 4 had evidence of major perfusion defects, involving both lungs. The perfusion defects were localized, in the majority of cases, on the side where a palliative procedure had been performed before the Fontan operation: it is of note that all our patients without palliative procedures did not show abnormalities in lung perfusion. Major abnormalities of lung perfusion seemed related to possible intimal thrombosis or emboli due to prolonged polycythemia or to pulmonary vessel distortion due to long-standing shunts.     

Right-to-left shunt through the cardiac veins after the Fontan procedure

We found right-to-left shunts through the cardiac veins postoperatively in 2 patients who had undergone the Fontan procedure. In one of the patients, channels were present through the cardiac veins independent of the coronary sinus. In the other patient, an atretic orifice for the coronary sinus, coupled with a persistent left-sided superior caval vein, complicated the postoperative course.     

Arrhythmias after Fontan operation: comparison of lateral tunnel and extracardiac conduit

Background

Arrhythmias are frequent causes of morbidity and mortality in patients with single ventricle physiology after Fontan operation. The aim of this study was to evaluate which type of Fontan procedure—lateral tunnel (LT) or extracardiac conduit (EC)—provides superior outcomes related to the problem of early postoperative and 1-year follow-up arrhythmias.

Methods

We retrospectively analyzed the incidence, types, and duration of rhythm disorders in 101 consecutive patients who received either LT (n = 60) or EC (n = 41) between April 1997 and March 2006 in Slovak Children's Cardiac Center, Bratislava (Slovakia). Weight, age, sex, and the type of heart morphology did not differ significantly between the 2 groups. The rhythm was monitored and documented perioperatively and postoperatively with standard electrocardiogram (ECG) recording and continual ECG monitoring. Duration of extracorporeal circulation, duration of aortic crossclamp and hemodynamic variables were analyzed with respect to the development of early arrhythmias in both groups. Twenty-four-hour ECG Holter monitoring (DMS 300-7, Holterreader, Producer DMS, Nevada, USA) was used to detect arrhythmias at the 1-year follow-up.

Results

Early postoperative rhythm abnormalities were identified in 31 patients (52%) who underwent LT and in 22 patients (54%) who underwent EC. The most frequent type of rhythm disturbance was junctional rhythm in both groups. The bivariate analysis revealed that there was no significant difference in the incidence, type, or duration of early onset arrhythmias between the 2 groups. Although, there was no significant difference in the duration of arrhythmia since the admission form the operating room. The need of aortic crossclamp was significantly lower in EC group (P < .001). However, this did not correlate with lower incidence of early onset arrhythmias with EC modification. At the 1-year follow-up, the prevalence of arrhythmias was similar in both groups.

Conclusions

Extracardiac conduit as compared with LT does not provide superior outcomes related to the problem of early and 1-year onset arrhythmias. Other factors than the risk of early postoperative and early follow-up arrhythmias should be considered in surgical preference of modification strategy.     

The Fontan procedure

The Fontan procedure has afforded improved surgical repair for several complex congenital cardiac defects, including tricuspid atresia and single ventricle. Through surgical creation of a connection between the RA and the RV or PA, adequate pulmonary perfusion can be achieved without an RV. Although it is not an anatomic connection, the Fontan procedure is a more physiologic approach than the previously used shunt procedures. Systemic venous return and PVR are effectively separated within the heart, pulmonary blood flow is assured through an RA-to-PA connection, and ventricular volume overload is avoided. The procedure has been effective in relieving cyanosis and has resulted in improved levels of exercise tolerance after surgery.     

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.     

Influence of ventricular morphology on outcome after the Fontan procedure

The modified Fontan procedure has gained wide acceptance in the treatment of various congenital heart defects. Determination of risk factors for mortality remains an important issue for optimizing patient selection for the Fontan procedure. Conflicting results have been reported about whether ventricular morphology is a risk factor in these patients. Survival free of Fontan takedown or cardiac transplantation was assessed in the first 500 patients undergoing the Fontan procedure at our institution. This survival was correlated with ventricular morphology as evaluated by angiography. Both multivariate and univariate analyses indicated ventricular morphology was predictive of early survival free of Fontan takedown or cardiac transplantation following the procedure. However, there was no statistical evidence for ventricular morphology being a risk factor for mortality in patients alive 6 months after the procedure. Ventricular morphology is a risk factor for early survival in patients undergoing a Fontan procedure, with left ventricular morphology associated with a better early survival than right ventricular morphology.     

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.