The clinical course of acquired complete heart block in children with acute myocarditis.

A case of biopsy-proven myocarditis in a 7-year-old complicated by advanced atrioventricular (AV) block prompted a review of the medical literature to determine the prognosis for recovery of AV conduction or need for pacemaker implantation. A total of 40 patients younger than 20 years of age were identified, with return of AV conduction within 7 days in 27 patients (67%), permanent pacing in 11 patients (28%) with persistent AV block, and death in 2 patients (5%).

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.

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.

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.

Recipient to donor conduction of atrial tachycardia following orthotopic heart transplantation.

We report a case of atrial tachycardia in a 60-year-old male 8 years postorthotopic heart transplantation. At electrophysiology study, the clinical rhythm was found to arise from the remnant of the recipient atrium and was successfully terminated by delivery of radiofrequency energy. Surgical scars formed at the anastomosis of the recipient and donor atrium during the time of orthotopic heart transplantation are thought to electrically isolate the two areas. Although rarely recognized, dysrhythmias originating from the recipient atrial remnant may occur more often than previously thought.

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 effect of electrode size on transvenous defibrillation energy requirements: a prospective evaluation.

Recent technological advances have resulted in high success rates for implantation of nonthoracotomy defibrillation lead systems. Further decreases in defibrillator size, facilitating pectoral placement, will depend in part on lowering defibrillation energy requirements. The purpose of this study was to determine if endocardial defibrillation energy requirements are influenced by electrode size. Thirteen adult mongrel dogs were studied under general anesthesia. A 9 Fr integrated bipolar pace/sense/defibrillation electrode (cathode) was positioned transvenously at the RV apex. The second defibrillation electrode (anode) was positioned at the junction of the RA and SVC. Two diameters of the proximal electrode, 7 Fr and 11 Fr, were sequentially tested in random order in each animal. The DFT for each electrode was determined using a 50-V up-down method. Energy, leading edge voltage, and current, current distribution, and total resistance were measured. The mean defibrillation voltage threshold with the 11 Fr proximal electrode was significantly less than with the 7 Fr proximal electrode (551.1 +/- 76.5 V vs 588.5 +/- 54.6 V, P < 0.01). Similarly, the mean DFT with the 11 Fr electrode was less than with the 7 Fr electrode (20.7 +/- 5.7 J vs 23.3 +/- 4.4 J, P < 0.01). Lower DFTs were found using the larger electrode in 11 of the 13 animals studied. However, there was no difference in defibrillation lead impedance between the two electrode systems. Endocardial defibrillation energy requirements may be lowered with a larger diameter proximal electrode. The mechanism by which this occurs may be due to a more even distribution of current gradients with the larger electrode. Determination of the optimal electrode size requires evaluation in humans, as this may allow further reduction in defibrillation energy requirements and defibrillator size.

Inadvertent atrioventricular block during radiofrequency catheter ablation. Results of the Pediatric Radiofrequency Ablation Registry. Pediatric Electrophysiology Society.

BACKGROUND: Inadvertent atrioventricular block is a complication of radiofrequency ablation. The present study is an analysis of the incidence, significance, and factors associated with inadvertent atrioventricular block during radiofrequency catheter ablation in childhood and adolescence. METHODS AND RESULTS: The records of the Pediatric Radiofrequency Ablation Registry were reviewed. Between January 1, 1991, and April 1, 1994, atrioventricular block occurred in 23 of 1964 radiofrequency ablations (1.2%): 14 as third-degree block (3 transient) and 9 as second-degree block (5 transient). Atrioventricular block occurred from 5 seconds to 2 months (mean, 4.1 days; median, 15 seconds) after the onset of the energy application. Eight transient cases lasted 1 hour to 1 month (mean, 9.4 days; median, 7 days). Inadvertent atrioventricular block was related to the ablation anatomic site: 3 of 111 (2.7%) anteroseptal, 11 of 106 (10.4%) midseptal, and 2 of 197 (1.0%) right posteroseptal sites (P = .0007) for anteroseptal, P = .0001 for midseptal, and P = .17 for right posteroseptal versus nonright septal sites). Five of 314 (1.6%) ablations for atrioventricular nodal reentrant tachycardia resulted in atrioventricular block (P = .004 versus nonright septal sites). Compared with a matched subgroup, radiofrequency ablation experience was the only significant risk factor (32.7 versus 106.6, P = .002) for the occurrence of atrioventricular block. CONCLUSIONS: Inadvertent atrioventricular block may occur during or late after radiofrequency catheter ablation. It is associated with ablations for (1) anterior and midseptal accessory pathways and atrioventricular nodal reentry and (2) relative institutional inexperience.

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.

Balloon aortic valvuloplasty for aortic stenosis in neonates, children, and young adults.

Balloon aortic valvuloplasty is a safe and effective treatment for aortic stenosis in neonates, children, and young adults. The indication for balloon valvuloplasty in children is a peak systolic gradient > 70 mmHg or > 50 mmHg in any patient with symptoms. Published results indicate that the procedure results in an acute reduction in gradient ranging from 49%-70%, and this reduction appears to persist through at least an intermediate follow-up. The percentage reduction in gradient is similar for neonates as well as older children. In neonates, it is imperative to evaluate the size of the left ventricle prior to balloon valvuloplasty since mortality is highest in those with variants of hypoplastic left heart syndrome. Though morbidity and mortality is higher in the neonatal age group, the results are similar to those following surgical intervention. In children older than 1 month of age, the major complication is the development of aortic regurgitation, although it usually appears to be well-tolerated. Previous surgical valvotomy is not a contraindication to balloon aortic valvuloplasty. In the current era, we believe that balloon aortic valvuloplasty should be considered as the first option in neonates, children and young adults with significant aortic valve obstruction.

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.