Transvenous catheter ablation of a right atrial automatic ectopic tachycardia.

A 10 year old patient with right atrial automatic ectopic tachycardia underwent successful transvenous catheter ablation after endocardial mapping. Tachycardia had been present for 7 years before ablation and associated with development of dilated cardiomyopathy. A single 50 J discharge at the point of earliest atrial activation was followed by the establishment of sinus rhythm that remains at 10 months' follow-up. This case represents the first reported successful catheter ablation of an atrial automatic ectopic tachycardia. Specific anatomic factors favoring successful ablation and improvement in technique are discussed.

The automatic implantable cardioverter-defibrillator in young patients.

An international survey identified 40 patients less than 20 years old who underwent surgical implantation of an automatic implantable cardioverter-defibrillator (AICD). There was a history of aborted sudden cardiac death or sustained ventricular tachycardia in 92.5% of these patients. Twenty-two patients (55%) had structural heart disease; dilated and hypertrophic cardiomyopathy were the most common diagnoses. Eighteen patients (45%) had primary electrical abnormalities including seven with the congenital long QT syndrome. There were no perioperative deaths associated with device implantation. Concomitant drug therapy was administered to 75% of the patients. Defibrillator discharge occurred in 70% of the patients, with 17 patients (42.5%) receiving at least one appropriate shock. There were two sudden and two nonsudden deaths at 28.2 months' median follow-up. Sudden death-free survival rates by life table analysis at 12 and 33 months were 0.94 and 0.88, respectively. Total survival rates at 12 and 33 months were 0.94 and 0.82, respectively. The AICD represents an effective treatment approach for young patients with life-threatening ventricular tachyarrhythmias.

A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects.

OBJECTIVES: This study sought to define 1) the risk of sudden death after operation for common congenital heart defects; and 2) factors associated with an increased risk of sudden death. BACKGROUND: Although the prognosis for patients with congenital heart defects is improved by surgical treatment, they remain at a well recognized but poorly defined risk of late sudden death. METHODS: This population-based study evaluated all patients < 19 years old undergoing surgical treatment of common forms of congenital heart disease in the state of Oregon between 1958 and 1996. Patients were identified retrospectively through 1958, with prospective biannual follow-up beginning in 1982. The incidence and cause of late sudden death were evaluated for 3,589 patients surviving operation for the following defects: atrial, ventricular and atrioventricular septal defects; patent ductus arteriosus; pulmonary stenosis; aortic stenosis; coarctation of the aorta; tetralogy of Fallot; and D-transposition of the great arteries. RESULTS: There were 41 unexpected late sudden deaths during 45,857 patient-years of follow-up, an overall event rate of 1/1,118 patient-years. Thirty-seven of the 41 late sudden deaths occurred in patients with aortic stenosis, coarctation, transposition of the great arteries or tetralogy of Fallot, an event rate of 1/454 patients-years. In contrast, only four sudden deaths occurred among the other defects, an event rate of 1/7,154 patient-years (p < 0.01). The risk of late sudden death increased incrementally 20 years after operation for tetralogy of Fallot, aortic stenosis and coarctation. However, risk was not dependent on patient age at operation or surgical era. The causes of sudden death were arrhythmia in 30 patients, circulatory (embolic or aneurysm rupture) in 7 and acute heart failure in 4. CONCLUSIONS: The risk of late sudden death for patients surviving operation for common congenital heart defects is 25 to 100 times greater than an age-matched control population. This increased risk is primarily represented by patients with cyanotic or left heart obstructive lesions. The risk of sudden death appears to be time dependent, increasing primarily after the second postoperative decade.

Clinical course of persistent junctional reciprocating tachycardia.

OBJECTIVE: The purpose of this study is to review the clinical course of persistent junctional reciprocating tachycardia (PJRT) in 21 patients spanning a wide age range to examine the electrophysiologic characteristics of the conduction system in these patients with PJRT, particularly in regards to its incessant nature and to evaluate the long-term response to radiofrequency ablation. BACKGROUND: Persistent junctional reciprocating tachycardia is uncommon, occurring in 1% of patients with supraventricular tachycardia. Its presentation, course and treatment are incompletely characterized. METHODS: The clinical, electrocardiographic, electrophysiologic and echocardiographic data of 21 patients with PJRT were reviewed. RESULTS: In 9 of these 21 patients, the mean tachycardia cycle length increased significantly (p < 0.0001) as the patients grew, from a mean tachycardia cycle length of 308+/-64 ms in the patients less than 2 years, 414+/-57 ms in the patients between 2 years and 5 years, to 445+/-57 ms in the patients greater than 5 years, primarily due to slowing of retrograde conduction in the accessory pathway. Persistent junctional reciprocating tachycardia was associated with impaired ventricular function in 11, improving spontaneously in 4 and, after successful ablation of the accessory pathway, in 7. All patients except one were uncontrolled on one or more medications. Ablation of the accessory pathway was successful in 19 of 21 patients. CONCLUSIONS: We conclude that PJRT is characterized by an onset in early childhood and by an age-related prolongation of the tachycardia cycle length mediated primarily through conduction delay in the concealed, retrogradely conducting accessory pathway. Ablation of the accessory pathway provides definitive treatment for PJRT.

Atypical forms of supraventricular tachycardia due to atrioventricular node reentry in children after radiofrequency modification of slow pathway conduction.

OBJECTIVES: This study was performed to investigate the prevalence, mechanisms and clinical significance of supraventricular tachycardias inducible in children or adolescents after radiofrequency modification of slow pathway conduction for the treatment of atrioventricular (AV) node reentrant tachycardia. BACKGROUND: Limited data have been reported with regard to the physiology of AV node reentrant tachycardia in young patients. Radiofrequency catheter ablation allows evaluation of the effects of selective modification of the different pathways involved in AV node reentrant tachycardia. METHODS: Selective modification of slow pathway conduction was performed in 18 young patients (12.9 +/- 3.4 years old) with typical (anterograde slow-retrograde fast) AV node reentrant tachycardia. Radiofrequency energy was applied across the posteromedial or midseptal tricuspid annulus, guided by slow pathway potentials and anatomic position. Programmed stimulation was performed after modification of slow pathway conduction defined as noninducibility of typical AV node reentrant tachycardia. RESULTS: Modification of slow pathway conduction was achieved in each patient, with a median of four applications of radiofrequency energy. However, atypical forms of supraventricular tachycardia were inducible in 9 of 18 young patients after slow pathway modification: AV node reentrant tachycardia with 2 to 1 AV block (seven patients); anterograde fast-retrograde slow AV node reentrant tachycardia (five patients); and sustained accelerated junctional tachycardia (two patients). In comparison, atypical forms of tachycardia were inducible in only 2 of 59 adult patients with AV node reentrant tachycardia undergoing slow pathway modification in the same laboratory (p = 0.01). Additional applications of radiofrequency energy to the posteromedial tricuspid annulus rendered AV node reentrant tachycardia with 2 to 1 block and the fast-slow form of AV node reentrant tachycardia noninducible. Junctional tachycardia terminated spontaneously in both patients. During 9.8 +/- 3 months of follow-up, slow-fast AV node reentrant tachycardia has recurred in one patient, whereas fast-slow AV node reentrant tachycardia has occurred in two patients, both with inducible fast-slow tachycardia after the initial modification of slow pathway conduction. CONCLUSIONS: Initial applications of radiofrequency energy may selectively modify the anterograde conduction of slow pathway fibers in young patients with AV node reentrant tachycardia. This may result in AV node reentrant tachycardia with 2 to 1 AV block or a reversal of the reentrant circuit (fast-slow tachycardia). Induction of these tachyarrhythmias indicates that further applications of radiofrequency energy are required for the successful modification of slow pathway conduction in young patients. The increased prevalence of inducible atypical arrhythmias among young patients suggests differences in the anatomic or electrophysiologic substrate of AV node reentrant tachycardia that may evolve as a function of age.

Analysis of programmed stimulation methods in the evaluation of ventricular arrhythmias in patients 20 years old and younger.

The purpose of this study was to systematically evaluate programmed ventricular stimulation in patients less than 21 years of age undergoing electrophysiologic testing. A standardized protocol was applied in 55 consecutive patients (mean age 14 years) with the following clinical presentations: sustained ventricular tachycardia (VT) (n = 17); ventricular fibrillation (VF) (n = 7); syncope with heart disease (n = 10); nonsustained VT (n = 6); and syncope with an ostensibly normal heart (n = 15). The stimulation protocol consisted of 1 and 2 ventricular extrastimuli during sinus rhythm, followed by 1 to 4 (S2, S3, S4, S5) extrastimuli during pacing at 2 ventricular sites. Of the 17 patients with sustained VT, 12 had induction of the arrhythmia (sensitivity = 71%). Overall, 18 of 55 patients had inducible sustained VT, with this response significantly enhanced by use of S4 or S5 protocols (p = 0.02). Although no syncope patients with an ostensibly normal heart had inducible sustained VT, 7 had polymorphic nonsustained VT in response to ventricular stimulation. The mean number of extra-stimuli preceding the induction of nonsustained or sustained VT or VF did not differ. The induction of VF in 5 cases during this study was preceded in each case by extrastimuli intervals less than or equal to 190 ms. Thus, data indicate that aggressive stimulation protocols appear to be required for induction of sustained VT in most young patients, nonsustained polymorphic VT as a response to aggressive programmed stimulation is of uncertain significance, and that coupling intervals less than or equal to 190 ms may correlate with the induction of VF.

Bradycardia-mediated tachyarrhythmias in congenital heart disease and responses to chronic pacing at physiologic rates.

The coexistence of bradycardia and a tachyarrhythmia may preclude effective pharmacologic treatment of 1 arrhythmia without paradoxic aggravation of the other. This study evaluated the potential relation between the 2 types of arrhythmias and the effect of conventional modes and rates of pacing for bradycardia on the frequency of the associated tachyarrhythmias. Twenty-one young patients, aged 2 to 19 (mean 11) years with congenital heart disease and a tachyarrhythmia occurring in the setting of chronic bradycardia were studied. The effects of pacing were evaluated by comparison of the number of episodes of clinical tachycardia during the 12-month intervals before and after pacemaker implantation. During these intervals, antiarrhythmic drug therapy was not altered. Patients were analyzed as independent groups, based on the type of tachyarrhythmia: supraventricular (n = 5), atrial flutter (n = 9) and ventricular (n = 7). The modes of chronic pacing were AAI (n = 4), DDD (n = 6) and VVI (n = 11). The prevention of bradycardia by pacing was associated with a significant decrease in the frequency of supraventricular (p = 0.008) and ventricular (p = 0.02) tachyarrhythmias. However, the frequency of atrial flutter was not altered. Prevention of tachycardia was more frequently associated with the AAI and DDD modes of pacing compared to VVI (p = 0.08). Pacing represents an effective therapy for certain tachyarrhythmias associated with chronic bradycardia, although critical modes may be required.

Comparison of radiofrequency catheter ablation procedures in children, adolescents, and adults and the impact of accessory pathway location.

Radiofrequency (RF) catheter ablation is an accepted treatment for supraventricular tachycardia. However, the determinants of success, difficulty, or risk of complication associated with ablation have not been defined. This study evaluated patient age and location of the accessory or extranodal pathway as determinants of these procedural variables. Patients were stratified by age, with those aged 2 to 12 years classified as children, those aged 13 to 19 years as adolescents, and those > or = 20 years as adults. Locations were defined as right, septal, or left free wall accessory pathways, or extranodal slow pathways associated with atrioventricular node reentrant tachycardia. A total of 443 RF ablation procedures performed in 413 patients were evaluated. All procedures were performed in the same laboratory by the same group of physicians. Success rates for ablation of supraventricular tachycardia did not differ among the 3 age groups, ranging from 93% to 95%. Procedural aspects, including total procedure time, fluoroscopy time, and number of applications of RF energy also did not differ by age group. However, analysis of outcome and procedural complexity with respect to pathway location demonstrated that ablation of right free wall and septal accessory pathways was significantly more difficult than left free wall or slow pathway (success rates of 85% and 88% vs 97% and 98%, respectively, p = 0.01 and 0.02), irrespective of age. Additionally, right free wall pathways required significantly greater procedure time (mean = 5.1 hours), fluoroscopy time (mean = 78 minutes), and RF applications (median = 16) than ablations performed at other sites.(ABSTRACT TRUNCATED AT 250 WORDS)

Safety and efficacy of radiofrequency modification of slow pathway conduction in children < or = 10 years of age with atrioventricular node reentrant tachycardia.

This study evaluated procedural considerations, risks, and long-term efficacy of radiofrequency modification of slow pathway conduction for treatment of atrioventricular node reentrant tachycardia in children < or = 10 years of age. Using a combined anatomic and electrographic mapping approach, modification of slow pathway conduction was achieved in 25 consecutive patients, although 4 had some form of transient atrioventricular block, indicating the need for caution in patient selection, catheter manipulation, and ablation.

The learning curve for radiofrequency ablation of tachyarrhythmias in pediatric patients. Participating members of the Pediatric Electrophysiology Society.

The results of radiofrequency ablation for treatment of supraventricular tachyarrhythmias have been reported to improve with increasing experience; however, the precise nature of the learning curve in children is unknown. From November 1990 to October 1993, 1,546 consecutive procedures from the Pediatric Radiofrequency Ablation Registry were categorized into deciles based on number of prior pediatric procedures at the submitting institution. Negative exponential models were tested for strength of relation between volume of prior experience and 4 measures of outcome: success rate, complication rate, fluoroscopy time, and procedure time. Negative exponential curves described the experience-outcome relations well (r = 0.81 to 0.97). Learning rates were most rapid for successful ablation of left free wall accessory pathways, and slowest for right free wall pathway ablation. These models suggest that, given enough experience, procedural success rates > 90% (regardless of pathway location) and fluoroscopy and procedure times averaging < 40 minutes and 250 minutes, respectively, can be achieved in pediatric patients.

Use of implantable cardioverter-defibrillators in the congenital long QT syndrome.

We surveyed the use of implantable cardioverter-defibrillators in patients with congenital long QT syndrome. The implantable cardioverter-defibrillator was used primarily in high-risk persons and appeared safe and effective over a mean 31-month follow-up.

Unmasking accessory pathway conduction with adenosine-induced atrioventricular nodal block after radiofrequency catheter ablation.

Radiofrequency catheter ablation is very effective in eliminating conduction over accessory pathways in patients with Wolff-Parkinson-White syndrome. However, accessory pathway conduction recurs in approximately 5 to 9 percent of patients in the weeks to months following ablation. We describe two cases in which intravenous adenosine revealed persistent accessory pathway conduction after apparently successful ablation, thus providing an indication for the delivery of further ablative therapy. Adenosine may improve the long-term efficacy of radiofrequency catheter ablation of accessory pathways by manifesting latent accessory pathway conduction.

Pharmacologic management of arrhythmias in patients with congenital heart disease.

The management of cardiac arrhythmias has evolved rapidly over the past decade. This includes the development of more effective antiarrhythmic medications as well as catheter- and device-based therapies. Antiarrhythmic medications remain the primary treatment modality for most acute arrhythmias; however, the long term use of these medications may be accompanied by severe adverse effects. For this reason, antiarrhythmic medications are increasingly used in conjunction with other forms of therapy, such as catheter ablation or pacemaker implantation. Patients with congenital heart disease often have an increased propensity for cardiac arrhythmias due to both inherent conduction system abnormalities and impaired ventricular function. The purpose of this review is to examine the currently available antiarrhythmic drugs and assess their role in the treatment of arrhythmias in patients with congenital heart disease. It is important to emphasize that patients with congenital heart disease often have hemodynamic limitations and may be at an increased risk for developing adverse effects with antiarrhythmic agents. An awareness of the arrhythmias associated with congenital heart disease, the natural history of these arrhythmias, and the potential benefit of treatment with antiarrhythmic medications versus other forms of therapy provides a rational basis for therapy in this challenging population of patients.