Steven B. Fishberger

Factors that influence the development of atrial flutter after the fontan operation

OBJECTIVES Atrial flutter is a frequent, potentially fatal complication of the Fontan operation, but risk factors for its development are ill defined. We evaluated clinical features that might predict the development of atrial flutter in patients who had a Fontan operation. METHODS We evaluated 334 early survivors of a Fontan operation done between April 1973 and July 1991 (mean follow-up, 5.0 +/- 3.8 years). Evaluation included electrocardiography, Holter monitor recordings, and chart review. Modifications of the Fontan operation included an extracardiac conduit (n = 43), an atriopulmonary anastomosis (n = 117), or a total cavopulmonary anastomosis (n = 174). Patient, time, and procedure-related variables were analyzed with respect to the development of atrial flutter. RESULTS Atrial flutter was identified in 54 (16%) patients at a mean of 5.3 +/- 4.7 years (range 0 to 19.7 years) after Fontan operation. Atrial flutter developed sooner and was more likely to occur in patients who were older at the time of Fontan operation (12.4 +/- 7.6 vs 6.3 +/- 5.2 years; p < 0.001), had a longer follow-up interval (8.7 +/- 3.9 vs 4.4 +/- 3.4 years; p < 0.001), had a prior atrial septectomy or pulmonary artery reconstruction (p < 0.01), and had worse New York Heart Association class symptoms (p < 0.02). The presence of sinus node dysfunction was associated with a higher incidence of atrial flutter (p < 0.001). Although there was a lower prevalence of atrial flutter in those patients with a total cavopulmonary anastomosis, the follow-up for this group was shorter. Anatomic diagnoses, perioperative hemodynamics, and other previous palliative operations were not associated with an increased incidence of atrial flutter. Multivariate analysis identified age at operation, duration of follow-up, extensive atrial baffling, and type of repair as factors associated with the development of atrial flutter after Fontan operation. CONCLUSION Atrial flutter continues to develop with time after the Fontan operation. Further follow-up is necessary to determine whether a total cavopulmonary anastomosis reduces the incidence of atrial flutter.

Functional outcome after the Fontan operation: Factors influencing late morbidity

OBJECTIVES The purpose of this study was to describe the functional outcome of a large number of patients after modifications of the Fontan operation and to investigate perioperative risk factors that might influence late functional state. METHODS A comprehensive cross-sectional review of the first 500 patients undergoing a Fontan operation at our institution was undertaken. Those surviving with an intact Fontan circulation were reviewed by questionnaire to assess functional status and medication history. Medical records, chest roentgenograms, echocardiograms, cardiac catheterizations, and laboratory investigations were also reviewed to assess postoperative status. RESULTS Three hundred sixty-three long-term survivors with an intact Fontan circulation were identified during cross-sectional follow-up. Median age at operation was 5.0 years (range 0.4 to 31 years), and median follow-up was 5.4 years (range 1.7 to 20 years). Most patients (91.1%) were in New York Heart Association class I or II. In a multivariate model, poor (class III or IV) functional state was associated with longer duration of follow-up (p < 0.001), a prior atrial septectomy (p = 0.03), and a prior main pulmonary artery-ascending aorta anastomosis (p = 0.05). CONCLUSIONS A poor functional outcome is uncommon after the Fontan operation but becomes more frequent with increasing duration of follow-up.

Staged operation to fontan increases the incidence of sinoatrial node dysfunction

Morbidity and mortality of total cavopulmonary connection (modified Fontan procedure) may be decreased in many patients with single ventricle in whom the risk of surgery is high by performing the operations in a staged fashion. Each operative intervention, however, exposes the sinoatrial node region to risk of injury, and a multistaged approach may increase the risk of altered sinoatrial node function in these patients. The purpose of this study was to compare the prevalence of perioperative arrhythmias in patients undergoing either a primary or staged approach to the Fontan operation. Records were retrospectively reviewed for all patients having a Fontan procedure between January 1988 and December 1992. Of 324 patients undergoing a Fontan operation, 227 had a Fontan operation without a prior cavopulmonary shunt (group 1) and 97 had a cavopulmonary shunt before a Fontan operation (group 2). Arrhythmias were classified as altered sinoatrial node function, supraventricular tachycardia, or atrioventricular block. The prevalence of both transient (resolving before hospital discharge) and fixed (persisting until hospital discharge) altered sinoatrial node function was similar for the two groups after cavopulmonary shunt or primary Fontan despite a heterogeneous patient population (group 1: 10.6%/4.4%; group 2: 10.3%/3.1%; p=0.28). Conversion from cavopulmonary shunt to Fontan in group 2 resulted in a higher prevalence of altered sinoatrial node function in the early postoperative period (transient: 23.7%; fixed: 23.7%; p < 0.001) and on follow-up (group 1: 7.7%; group 2: 16.7%; p < 0.02). In group 2, 40 of 82 patients without arrhythmia after first intervention (cavopulmonary shunt) had an arrhythmia after the second intervention (Fontan) (49%); of 14 with an arrhythmia after the first operation, 10 (71%) had one at the second intervention (p < 0.01). In conclusion, a multistaged operative pathway to Fontan reconstruction is associated with a higher early risk of altered sinoatrial node function. The occurrence of altered sinoatrial node function after cavopulmonary shunt is itself a risk factor for arrhythmia after the Fontan operation. Longer follow-up is needed to assess the full impact of this finding.

Long-term outcome in patients with pacemakers following the fontan operation*

Patients with pacemakers after Fontan surgery compared favorably with nonpaced patients with respect to survival. In patients with atrioventricular block, dual chamber pacing was superior to VVI pacing.

Myocardial Mechanics Before and After Ablation of Chronic Tachycardia

Chronic tachycardia has been shown to cause a congestive cardiomyopathy; however, previous methods of evaluating ventricular function are highly dependent on cardiac loading conditions. Mean velocity of fiber shortening and its relation to end‐systolic wall stress (ESS) is a preload independent index of contractility that incorporates afterload. We reviewed 33 patients (aged 6 months to 20 years; mean 9.7 years) with ectopic atrial tachycardia (EAT) (n = 19), permanent functional reciprocating tachycardia (PfRT) (n = 12), or ventricular tachycardia (n = 2). who underwent nonpharniacological elimination of tachycardia; 28 by radiofrequency ablation and 5 surgically. Ventricular function was evaluated by echocardiographic measurements of shortening fraction, mean velocity of shortening corrected for heart rate (VcFc), and afterload as ESS. Contractility, expressed as the stress‐velocity index, was determined by comparing the ESS/VcFc relation to the predicted normal VcFc for the measured ESS. Myocardial dysfunction was seen in 21 patients: 13 with EAT; 7 with PJRT; and 1 with ventricular tachycardia. In patients with EAT, the mean heart rate in tachycardia was significantly faster in those with dysfunction than in those without dysfunction (176.8 ± 32.2 vs 136.7 ± 28.2; P < 0.02). Of the 21 patients with dysfunction, full recovery was seen in 17 of 18 patients restudied after intervention (mean 17.5 ± 17.6 weeks), and the remaining patient improved markedly, but did not normalize entirely. Dysfunction, seen in 64% of young patients with chronic tachycardia, was due to depressed myocardial contractility, and is generally reversible within 3 months of definitive therapy.

Use of adenosine-sensitive nondecremental accessory pathways in assessing the results of radiofrequency catheter ablation

Adenosine can cause conduction block in about 20% of nondecremental accessory pathways. Along with atrial activation mapping, adenosine may help differentiate retrograde AV node conduction versus residual accessory pathway conduction after radiofrequency catheter ablation; however, it is important to test the accessory pathway response to adenosine before ablation, particularly with a concealed accessory pathway.

Congenital cardiac surgery without routine placement of wires for temporary pacing.

OBJECTIVE Temporary pacing wires have been associated with serious postoperative complications. Recommendations for their routine use after open heart surgery are decades old, and may not reflect current surgical techniques and outcomes. METHODS The electronic web-enabled medical records of all patients undergoing congenital cardiac surgery from February, 2002, through December, 2005, were reviewed, excluding patients undergoing implantation of pacemakers as a primary procedure, or those undergoing ligation of a patent arterial duct. RESULTS There were 1193 surgical procedures performed, 1041 with cardiopulmonary bypass. Median age of the patients was 5.8 months, with a range from 0 days to 54 years, weighing 6.2 kilograms, with a range from 1 to 114 kilograms. Mortality prior to discharge was 2.5%, and median postoperative stay was 6 days. No deaths were attributed to arrhythmias. Temporary pacing wires were placed 14 times (1.2%). Indications for placement included sinus nodal dysfunction in 8 patients, preoperative in 4 and intraoperative in 4, high degree atrioventricular block in 4 patients, and intraoperative atrial flutter in 2 patients. Of these patients, 4 (0.3%) eventually underwent permanent implantation of a pacemaker, 2 for persistent sinus nodal dysfunction, and 2 for persistent atrioventricular block. Postoperative junctional ectopic tachycardia requiring antiarrhythmic therapy occurred in 9 patients (0.8%). All recovered without incident, and none were treated with temporary pacing. CONCLUSIONS The diminished risk of unexpected postoperative arrhythmias in the current era alleviates the necessity for routine placement of temporary pacing wires. Those institutions with experienced surgical and cardiac critical care teams may be able to predict the need for temporary pacing wires preoperatively or intraoperatively.

Radiofrequency Ablation of Pediatric AV Nodal Reentrant Tachycardia during the Ice Age: A Single Center Experience in the Cryoablation Era

Background: Radiofrequency catheter ablation of atrioventricular nodal reentrant tachycardia (AVNRT) has proven to be an effective therapy in the pediatric population. However, concerns of inadvertent permanent AV nodal block have resulted in many pediatric programs adopting cryoablation as their primary ablation approach for AVNRT.

Electroanatomic Mapping of the Right Coronary Artery: A Novel Approach to Ablation of Right Free-Wall Accessory Pathways

Background: Catheter ablation of right free‐wall (RFW) accessory pathways continues to be associated with lower success and higher recurrence rates compared with other pathway locations. Reliably identifying the precise location of RFW accessory pathways often contributes to the difficulty in ablating these pathways. Improved localization of RFW accessory pathways has been described utilizing multielectrode right coronary artery (RCA) catheterization. This approach has not been widely adopted, in part due to concerns of prolonged catheter placement within the RCA. We describe the technique of creating a 3‐D electroanatomic map of the right atrioventricular groove, limiting the duration of a microcatheter within the RCA, to facilitate ablation of RFW accessory pathways.

Urgent Permanent Pacemaker Implantation in Critically Ill Preterm Infants

The management of complete heart block in premature low birth-weight infants, particularly those with hydrops fetalis, is challenging. We report emergent implantation of permanent epicardial pacemakers in the first 48 hours of life in two premature infants (one with hydrops fetalis) with birth weights of 1,400 grams and 1,000 grams.