High energy external cardioversion for refractory atrial fibrillation in postoperative tetralogy of fallot.

Long-term complications of surgical repair of Tetralogy of Fallot include atrial arrhythmias. These can be difficult to treat, and loss of sinus rhythm can lead to profound hemodynamic consequences in the presence of residual structural abnormalities. We describe the first report of high-energy external cardioversion in a 46-year-old man with repaired tetralogy of Fallot with atrial fibrillation refractory to conversion with normal energy. This represents an alternative to internal cardioversion or rate control for these patients.

Severe sinus bradycardia in a patient with Rett syndrome: a new cause for a pause?

Rett syndrome is a progressive disorder seen primarily in young females. It is characterized by autonomic dysfunction affecting many organ systems. Although sudden death is common in these patients, little is known about the cardiovascular manifestations of this dysautonomia. Earlier reports focused on the role of sympathetic overactivity manifest as reduced heart rate variability and prolonged QT intervals indicating a propensity for lethal ventricular arrhythmias. Bradyarrhthmias and sinus node dysfunction, however, have not previously been reported. We describe a 2-year-old with Rett syndrome and severe sinus bradycardia requiring a pacemaker implant. This case represents the first description of a new cardiovascular manifestation of Rett syndrome and may provide an explanation for sudden and unexpected death in some of these patients.

Afterload reduction therapy in patients following intraatrial baffle operation for transposition of the great arteries.

Patients with intraatrial baffle procedure for transposition of the great arteries (TGA) have diastolic dysfunction, decreased exercise capacity, stroke volume response and elevated systemic vascular resistance (SVR) during exercise. Angiotensin-converting enzyme (ACE) inhibitors improve exercise capacity in adults with congestive heart failure by improving diastolic function and decreasing SVR. We tested the hypothesis that ACE inhibitors decrease SVR and improve exercise capacity in patients after intraatrial baffle procedure for TGA. We studied the effects of enalapril in nine patients with TGA s/p intraatrial switch (mean age, 13.8 +/- 3 years) 7 to 21 years (mean, 12 +/- 4 years) after intraatrial baffle procedure. Enalapril (0.5 mg/kg/day, maximum dosage 20 mg bid) was administered for 12 months. Patients exercised using a cycle ergometer ramp protocol (0.25 W/kg/min) before enalapril (baseline), 1 month, 6 months, and 12 months after treatment initiation. Heart rate, blood pressure, cardiac output, respiratory rate, minute ventilation, oxygen consumption (VO2), total exercise time, work, and power were measured. SVR, cardiac index, and stroke volume index (SVI) were calculated. Two-tailed paired Student's t-test was used to compare data to those of normal control patients and the patients' baseline data. Patients had lower resting heart rate, cardiac index, maximum heart rate, cardiac index (CI), SVI, VO2, exercise time, work, and power and higher maximal SVR at baseline compared to normal control patients. There was no significant difference in total exercise time, work, power, VO2 (rest/peak), SVR, SVI, and CI after 12 months of therapy compared to patients' baseline values. We conclude that short-term (<1 year) use of enalapril does not improve exercise performance in patients with TGA in whom the intraatrial baffle procedure has been performed.

Evaluation and treatment of pediatric patients with neurocardiogenic syncope.

Neurocardiogenic syncope is the most common form of syncope in children. The most widely accepted mechanism of its etiology is a vigorous contraction of the heart due to relative hypovolemia leading to stimulation of C-fibers and sympathetic withdrawal. Episodes usually occur with an upright position and are preceded by a typical prodrome. A classic history is paramount to the diagnosis. Tilt testing aids in the diagnosis in certain cases. The cornerstone of therapy is maintaining adequate hydration and avoiding situations that may lead to harm with syncopal events. Beta blockers, fludrocortisone, alpha adrenergic agents and disopyramide are commonly used to prevent syncope. Seratonin reuptake inhibitors, pacing with rate drop algorithms and tilt training are relatively new in the armamentarium of therapy.

Use of multisite electroanatomic mapping to facilitate ablation of intra-atrial reentry following the Mustard procedure.

Ablation of intra-atrial reentrant tachycardia following Mustard or Senning procedures has low success rates. The Biosense Carto system was used to map intra-atrial reentry in a 22-year-old woman who had undergone a Mustard procedure. A line of block was created connecting a Mustard baffle suture line to the tricuspid valve annulus, which terminated the arrhythmia and prevented its reinitiation. Multisite electroanatomic mapping was invaluable in defining atrial anatomy and the intra-atrial reentrant pathway, and in creating a contiguous line of block. This mapping may improve ablation success rates in patients following the Mustard or Senning repair.

Significance of a Q wave in lead I in the newborn.

We present 11 healthy newborns whose electrocardiograms had a pure Q wave in lead I and who did not have a myocardial infarction clinically. We propose that in the healthy newborn, a pure Q wave in lead I may be due to increased right ventricular mass, not myocardial infarction.

JT dispersion in Wolff-Parkinson-White syndrome: effect of eccentric ventricular depolarization on the dispersion of repolarization.

There is much interest in QT dispersion for noninvasive risk stratification of patients at risk of arrhythmias. However, little is known about the genesis of abnormal QT dispersion. In particular, whether eccentric ventricular depolarization, as seen in preexcitation, can lead to abnormal dispersion of repolarization is unknown. We studied 24 children aged 1-19 years (mean +/- SD, 11 +/- 5 years) with manifest preexcitation due to Wolff-Parkinson-White syndrome who had successful catheter ablation. Standard ECGs done preablation, early postablation (< 1 week), mid postablation (> 1 week, < 2 months), and late postablation (> 2 months) were reviewed. The QRS duration prior to ablation ranged from 90-160 ms (mean +/- SD, 123 +/- 21 ms). On the preablation ECG, the JT and JTc dispersions showed no relationship to the QRS duration (r = 0.04 and 0.07, respectively). There was no change in JT dispersion when the preablation (42 +/- 15 ms) ECG was compared to early (43 +/- 15 ms), mid (44 +/- 13 ms), and late postablation (48 +/- 19 ms) ECGs. There was no significant change in JTc dispersion as well. Thus, JT dispersion is unrelated to QRS duration and unaffected by catheter ablation in patients with Wolff-Parkinson-White syndrome. Eccentric ventricular depolarization does not lead to abnormal dispersion of repolarization.

Safety of transvenous atrial defibrillation: studies in the canine sterile pericarditis model.

BACKGROUND: It is recognized that a ventricular vulnerability period exists during which atrial shock delivery may induce a ventricular tachyarrhythmia. This study was designed to define the zone in which the ventricles are vulnerable to induction of ventricular tachyarrhythmia during delivery of atrial shocks in the sterile pericarditis canine model of atrial fibrillation. METHODS AND RESULTS: Two days after creation of sterile pericarditis, 24 dogs underwent either a four-part or five-part ventricular vulnerability protocol during which atrial shocks were delivered between transvenous catheters, one in the distal coronary sinus and one in the right atrial appendage. The protocol included part 1, shocks during induced atrial fibrillation; parts 2 through 4, shocks delivered synchronously with the last ventricular beat of one of the following three ventricular pacing protocols: constant ventricular rates (S1S1), short-long-short cycles (S1S2S3-V), and ventricular premature beats (S1); and part 5, shocks delivered synchronously with the last R wave resulting from an atrially paced short-long-short cycle (S1S2S3-A). Ventricular tachyarrhythmia was induced 122 times: 2 of 665 shocks in two dogs in part 1, 29 of 786 shocks in nine dogs in part 2, 67 of 734 shocks in 15 dogs in part 3, 24 of 919 shocks in five dogs in part 4, and none in part 5. All ventricular proarrhythmia resulted from shocks delivered during the T wave of a preceding ventricular beat. No episodes of ventricular tachyarrhythmia were induced by atrial shocks synchronized to R waves with the previous RR at intervals above the QT+60 ms interval (absolute interval >320 ms), with one exception, at the QT+100 ms interval (absolute interval 360 ms). CONCLUSIONS: With transvenous electrode catheters used to deliver atrial shocks, life-threatening ventricular rhythms were induced but were limited to a specific zone defined by the QT interval.

Radiofrequency catheter ablation of right ventricular outflow tachycardia in children and adolescents.

OBJECTIVES: The current study reviews the safety and efficacy of radiofrequency catheter ablation for the treatment of right ventricular outflow tachycardia in children and adolescents and describes a modified method for mapping the tachycardia focus. BACKGROUND: Although radiofrequency catheter ablation has proved highly effective for the treatment of supraventricular tachycardia during childhood and adolescence, its application in children with idiopathic right ventricular outflow tachycardia has been limited. METHODS: Six children (mean [+/- SD] age 10.6 +/- 2.4 years, range 6 to 16) with right ventricular outflow tachycardia underwent seven radiofrequency catheter ablation procedures. The mean tachycardia cycle length was 323 +/- 24 ms (range 300 to 360). Two multipolar catheters were positioned in the right ventricular outflow tract to map the tachycardia focus. RESULTS: Radiofrequency catheter ablation was successful in five (83%) of the six children (95% confidence interval 36% to 99%). At successful ablation sites, local endocardial activation time preceded the surface QRS onset by 46 +/- 5 ms (range 37 to 57), and there was concordance of the 12-lead pace map and the electrocardiogram (ECG) in 11 (one patient) to 12 ECG leads (four patients). One patient developed complete right bundle branch block during radiofrequency catheter ablation. There were no additional complications and no clinical recurrences over a mean follow-up period of 12.7 +/- 3.8 months (range 9 to 22). CONCLUSIONS: These results suggest that radiofrequency catheter ablation is a safe and effective treatment for right ventricular outflow tachycardia during childhood and adolescence. In addition, tachycardia mapping may be enhanced by use of a multipolar right ventricular outflow catheter technique.

Atrial defibrillation using temporary epicardial defibrillation stainless steel wire electrodes: studies in the canine sterile pericarditis model.

OBJECTIVES: This study sought to determine whether temporary epicardial wire electrodes can be used safely and effectively to defibrillate the atria with low energy shocks in the absence of anesthesia. BACKGROUND: Atrial fibrillation after open heart surgery is a significant clinical problem. METHODS: Twelve dogs with sterile pericarditis were studied. In the first group (6 dogs, bilateral thoracotomy group), a wire electrode, insulated except for the distal 6 cm, was placed on the epicardial free wall of each atrium. Each end of the bare wire was then sutured to the parietal pericardium. In the second group (6 dogs, median sternotomy group), the wire electrodes were kept in place by a double loop of Prolene placed around the distal tip of the bare wire and sewn to the overlying parietal pericardium. In the bilateral thoracotomy group, atrial defibrillation thresholds (defined as < 90% and > 10% successful defibrillation of 20 shocks at a given delivered energy) were obtained in anesthetized dogs using the wire electrodes with the chest closed and open and using two transvenously placed catheters with coil electrodes in the distal 6 cm (one in the coronary sinus and the other in the right atrial appendage) with the chest open. In the median sternotomy group, thresholds were obtained in minimally sedated animals without reopening the chest. A 25% increase above threshold shock was also used to determine a new percent success. After 4 days, the wire electrodes were removed by pulling on the external ends. At the time of removal, blood pressure and heart rate were monitored for 30 min, after which dogs were killed and their hearts sent for histopathologic study. For all dogs, chest radiographs were obtained postoperatively and on study days. RESULTS: Atrial defibrillation using the wire electrodes was successful in all dogs at a mean (+/- SE) voltage of 112 +/- 9 V, with an energy level of 0.46 +/- 0.07 J and an impedance of 59.3 +/- 5 ohms. The mean percent success at the atrial defibrillation threshold was 36 +/- 5%. The 25% increase in defibrillation voltage improved the mean percent success to 73% (mean energy 0.66 +/- 0.19 J). No clinical or hemodynamic complications were observed during shock delivery, and no ventricular arrhythmias were induced during the shocks. No complications followed wire electrode removal. Histopathologic analysis showed no structural damage. CONCLUSIONS: The atrial defibrillation threshold obtained using temporary epicardial wire electrodes for atrial defibrillation is < 1 J in dogs. Atrial defibrillation using temporary epicardial wire electrodes can be performed safely, quickly and reliably without the need for anesthesia or antiarrhythmic agents. The wire electrodes can be removed without adverse hemodynamic or structural consequences. These data provide a basis for testing atrial defibrillation using epicardial wire electrodes in patients after open heart surgery.