Cardiac arrhythmias and conduction disturbances in autoimmune rheumatic diseases

Rhythm and conduction disturbances and sudden cardiac death (SCD) are important manifestations of cardiac involvement in autoimmune rheumatic diseases (ARDs). In patients with rheumatoid arthritis (RA), a major cause of SCD is atherosclerotic coronary artery disease, leading to acute coronary syndrome and ventricular arrhythmias. In systemic lupus erythematosus (SLE), sinus tachycardia, atrial fibrillation and atrial ectopic beats are the major cardiac arrhythmias. In some cases, sinus tachycardia may be the only manifestation of cardiac involvement. The most frequent cardiac rhythm disturbances in systemic sclerosis (SSc) are premature ventricular contractions (PVCs), often appearing as monomorphic, single PVCs, or rarely as bigeminy, trigeminy or pairs. Transient atrial fibrillation, flutter or paroxysmal supraventricular tachycardia are also described in 20-30% of SSc patients. Non-sustained ventricular tachycardia was described in 7-13%, while SCD is reported in 5-21% of unselected patients with SSc. The conduction disorders are more frequent in ARD than the cardiac arrhythmias. In RA, infiltration of the atrioventricular (AV) node can cause right bundle branch block in 35% of patients. AV block is rare in RA, and is usually complete. In SLE small vessel vasculitis, the infiltration of the sinus or AV nodes, or active myocarditis can lead to first-degree AV block in 34-70% of patients. In contrast to RA, conduction abnormalities may regress when the underlying disease is controlled. In neonatal lupus, 3% of infants whose mothers are antibody positive develop complete heart block. Conduction disturbances in SSc are due to fibrosis of sinoatrial node, presenting as abnormal ECG, bundle and fascicular blocks and occur in 25-75% of patients.     

Atypical Atrioventricular Nodal Reentry Tachycardia with Atrioventricular Block Mimicking Atrial Tachycardia:

AVNRT Mimicking Atrial Tachycardia, Introduction : Fast-intermediate form AV nodal reentry tachycardia (AVNRT) sometimes may mimic atrial tachycardia or atrial flutter and render the diagnosis difficult when the tachycardia rate is fast and AV block occurs during tachycardia.
Methods and Results : A 45-year-old woman with paroxysmal supraventricular tachycardia was referred to this institution. Initially, the tachycardia was thought to be an atrial tachycardia because of: (1) a short cycle length of the tachycardia with 2:1 and Wenckebach AV block; (2) a difference in the atrial activation sequence during tachycardia and during ventricular pacing; and (3) failure of burst ventricular pacing to affect the atrial rate and the atrial activation sequence during tachycardia. An accurate diagnosis of fast-intermediate form AVNRT was subsequently made based on the finding that the tachycardia was induced following delivery of a third ventricular extrastimulus, which showed a sequence of V-A-H and a change on atrial activation sequence of the induced beat. Successful radiofrequency ablation was achieved only after accurate diagnosis of the tachycardia was made.
Conclusion : Fast-intermediate form AVNRT sometimes may masquerade as atrial tachycardia. Accurate diagnosis is mandatory for successful ablation therapy.     

The Response of Paroxysmal Supraventricular Tachycardia to Overdrive Atrial and Ventricular Pacing:

Pacing During Supraventricular Tachycardia. Introduction: Standard electrophysiologic techniques generally allow discrimination among mechanisms of paroxysmal Supraventricular tachycardia. The purpose of this study was to determine whether the response of paroxysmal Supraventricular tachycardia to atrial and ventricular overdrive pacing can help determine the tachycardia mechanism. Methods and Results: Fifty-three patients with paroxysmal Supraventricular tachycardia were studied. Twenty-two patients had the typical form of atrioventricular (AV) junctional (nodal) reentry, 18 patients had orthodromic AV reentrant tachycardia, 10 patients had atrial tachycardia, and 3 patients had the atypical form of AV nodal reentrant tachycardia. After paroxysmal Supraventricular tachycardia was induced, 15-beat trains were introduced in the high right atrium and right ventricular apex sequentially with cycle lengths beginning 10 msec shorter than the spontaneous tachycardia cycle length. The pacing cycle length was shortened in successive trains until a cycle of 200 msec was reached or until tachycardia was terminated. Several responses of paroxysmal Supraventricular tachycardia to overdrive pacing were useful in distinguishing atrial tachycardia from other mechanisms of paroxysmal Supraventricular tachycardia. During decremental atrial overdrive pacing, the curve relating the pacing cycle length to the VA interval on the first beat following the cessation of atrial pacing was flat or upsloping in patients with AV junctional reentry or AV reentrant tachycardia, but variable in patients with atrial tachycardia. AV reentry and AV junctional reentry could always be terminated by overdrive ventricular pacing whereas atrial tachycardia was terminated in only one of ten patients (P < 0.001). The curve relting the ventricular pacing cycle length to the VA interval on the first postpacing beat was flat or upsloping in patients with AV junctional reentry and AV reentry, but variable in patients with atrial tachycardia. The typical form of AV junctional reentry could occasionally be distinguished from other forms of paroxysmal Supraventricular tachycardia by the shortening of the AH interval following tachycardia termination during constant rate atrial pacing. Conclusions: Atrial and ventricular overdrive pacing can rapidly and reliably distinguish atrial tachycardia from other mechanisms of paroxysmal Supraventricular tachycardia and occasionally assist in the diagnosis of other tachycardia mechanisms. In particular, the ability to exclude atrial tachycardia as a potential mechanism for paroxysmal Supraventricular tachycardia has important implications for the use of catheter ablation techniques to cure paroxysmal Supraventricular tachycardia.     

Phasic coronary arterial flow velocity during arrhythmias in man

The influence of cardiac arrhythmias on coronary arterial flow velocity studied by means of a Doppler catheter flowmeter system is described in 47 patients. The arrhythmias examined included atrial and ventricular extrasystoles, atrial fibrillation, pacemaker-induced atrial tachycardia, paroxysmal atrial tachycardia, ventricular tachycardia, Wenckebach second degree atrioventricular block and complete heart block.     

Comparison of adenosine and verapamil for termination of paroxysmal junctional tachycardia

The effects of intravenous adenosine and intravenous verapamil on paroxysmal junctional tachycardia were compared in 20 patients undergoing invasive cardiac electrophysiologic study. In 13 patients the diagnosis was of a reentrant tachycardia using an extranodal accessory connection (atrioventricular [AV] reentrant tachycardia); 5 of these patients had overt preexcitation in sinus rhythm, 4 had concealed accessory connections and 4 had latent or intermittent preexcitation. In 7 patients the diagnosis was of an AV nodal reentrant tachycardia. Administration of adenosine resulted in termination of tachycardia in all 20 patients at a mean dose of 0.125 mg/kg (range 0.05 to 0.20). Although termination of tachycardia was frequently accompanied by atrial and ventricular premature complexes, no significant arrhythmias were observed after conversion. Administration of verapamil (0.145 mg/kg) resulted in termination of tachycardia in 19 of 20 patients but was followed by symptomatic arrhythmias in 2: preexcited atrial flutter in 1 patient and preexcited atrial tachycardia in another. Latent or intermittent preexcitation was unmasked in 4 of 4 patients immediately after termination of tachycardia by adenosine. Termination of tachycardia by verapamil revealed preexcitation in only 1 of these 4 patients. Analysis of results in terms of successful termination of tachycardia, absence of significant arrhythmias after conversion and unmasking of latent or intermittent preexcitation reveals that adenosine therapy was satisfactory in all 20 patients, whereas verapamil was satisfactory in only 14 of the 20 patients (p less than 0.05). All 6 of the patients with unsatisfactory responses to verapamil had AV reentrant tachycardia. These results suggest that adenosine has particular advantages over verapamil as acute treatment for patients presenting with an AV reentrant tachycardia.     

Syncopes and brief spells of feeling faint: value of Holter monitoring?

A retrospective study of Holter monitoring of 250 patients referred for syncope and short spells of dizziness suspected of being cardiac in origin was undertaken to assess the diagnostic value of the investigation. The arrhythmias observed were classified in 3 groups, significant, suspect and physiological with respect to their true or potential severity and to previously reported results of Holter monitoring in healthy subjects. The following arrhythmias were classified as significant: supraventricular tachycardia with a ventricular rate greater than or equal to 200 bpm; sustained ventricular tachycardia (greater than 30 s and greater than or equal to 150 bpm), bradycardia (less than bpm), sinus arrest (waking greater than 2 s sleeping greater than or equal to 6 s), complete AV block with wide QRS complexes and pacemaker dysfunction. The following arrhythmias were classified as suspect: paroxysmal supraventricular tachycardia with a ventricular rate less than 200 bpm, salvos of ventricular tachycardia (120 greater than 150 bpm); R/T phenomenon and doublets (greater than or equal to 50/24 hours), sinus arrest of 2 to 6 seconds during sleep, complete AV block with narrow QRS complexes or second degree Mobitz II block. This classification led to a diagnosis of certitude in 20 patients (5.7%) with significant arrhythmias concomitant with syncope or a minor form in only 5 cases, supraventricular tachycardia (4 cases), ventricular tachycardia (4 cases), AV block (5 cases), sinus arrest (3 cases), pacemaker dysfunction (4 cases); a diagnosis of presumption in 74 patients (21.1%) with suspect arrhythmias in the absence of syncope or minor equivalent.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cryoablation of a Nodoventricular Mahaim Fiber

An 11-year old female presented with paroxysmal tachycardia and was diagnosed with a Mahaim fiber during electrophysiologic study. A preexcited tachycardia and the typical variety of AV nodal reentry tachycardia were induced at different times. During preexcited tachycardia, the His bundle electrogram followed the ventricular electrogram, and, introduction of atrial premature beats at different coupling intervals, advanced the peri-AV nodal atrial tissue, with no change in the ventricular cycle length, leading to a diagnosis of an antidromic tachycardia due to a nodoventricular fiber. Cryoablation at a mid-septal location under three-dimensional guidance successfully eliminated both tachycardias without detrimental effects to the AV node.     

Transcoronary chemical ablation of atrioventricular node for the treatment of paroxysmal supraventricular tachycardia--a case report

We applied transcoronary chemical ablation of the atrioventricular (AV) node to a patient with uncontrollable paroxysmal supraventricular tachycardia (PSVT). Through an angioplasty dilatation catheter. 99% ethanol at a dose of 1.0 ml was selectively infused into the AV nodal artery. Complete AV block with junctional escape rhythm occurred. Two weeks later, the treadmill exercise test was performed according to a modified Bruce protocol. The patient tolerated for 12 min, and the heart rate increased to 85 beats/min. His bundle electrocardiogram showed that the AV block resulted from atrio-Hisian block. Neither atrial nor ventricular extrastimulus could induce the tachycardia. It appeared that chemical ablation was a good method for controlling medically resistant PSVT. Elevation of serum creatine kinase was observed when ethanol overflowed during the ablation procedure. Occlusion of branches of the AV artery and mild hypokinesis in the inferobasal wall of the left ventricle were seen.     

Use of intravenous adenosine in sinus rhythm as a diagnostic test for latent preexcitation

In a proportion of patients with left free wall accessory connections, preexcitation is apparent only during atrial arrhythmias or atrial pacing (latent preexcitation). These patients may be at risk of a rapid ventricular response to atrial fibrillation despite the absence of preexcitation in sinus rhythm. The ability of intravenous adenosine to unmask latent preexcitation was evaluated in 22 patients with a history of documented supraventricular tachycardia and a normal electrocardiogram during sinus rhythm. Preexcitation was unmasked in response to adenosine in 4 patients: all 4 were shown to have latent preexcitation at electrophysiologic study. In 12 patients atrioventricular (AV) nodal conduction delay or block was induced without preexcitation after adenosine (first-degree AV block in 8, second-degree block in 4): at subsequent electrophysiologic study none of these patients was found to have latent preexcitation. Five patients had little or no PR prolongation in response to adenosine: of these, 2 were shown to have latent preexcitation at electrophysiologic study. Atrial fibrillation was induced in 1 patient and a narrow complex regular tachycardia in another after intravenous adenosine. Intravenous adenosine during sinus rhythm is capable of producing AV nodal conduction delay or block in 73% of patients with a history of supraventricular tachycardia: in these patients adenosine provides a diagnostic test that is both 100% sensitive and 100% specific for latent preexcitation. In those patients in whom adenosine does not produce AV conduction delay or block, further investigation is required to establish or refute the diagnosis of latent preexcitation.     

Catheter ablation for cardiac arrhythmias

The clinical introduction of catheter ablation in 1981 revolutionized the treatment of cardiac arrhythmias. Implementation of radiofrequency as an alternative energy source, with the advantages of higher selectivity and less collateral damage, provided an expansion of catheter ablation therapy. Today the majority of arrhythmias can potentially be cured with catheter ablation therapy. The safety and efficacy of catheter ablation for treatment of AV nodal reentrant tachycardia, accessory pathway arrhythmias, focal atrial tachycardia, atrial flutter and idiopathic ventricular tachycardia, is well established. Catheter ablation for treatment of atrial fibrillation and ventricular tachycardia secondary to structural heart disease, remains an area of active research. In this article we will review the current state of knowledge about the technique, indications, and results of catheter ablation for the treatment of cardiac arrhythmias.     

Catheter Ablation for the Treatment of Paroxysmal Supraventricular Tachycardia

Catheter Ablation for PSVT. Radiofrequency catheter ablation has evolved into a front-line curative therapy for patients who have paroxysmal supraventricular tachycardia secondary to Wolff-Parkinson-White syndrome, AV nodal reentrant tachycardia, and atrial tachycardia. In patients with accessory pathways, cure rates exceed 90% in almost all anatomic locations. Equally high success rates are noted in patients with atriofascicular pathways and the permanent form of junctional reciprocating tachycardia. Complications secondary to catheter ablation of accessory pathways occur in 1% to 3% of patients and include cardiac perforation, tamponade, AV block, and stroke. In patients with AV nodal reentrant tachycardia, selective slow pathway ablation is curative in over 95% of patients with a very low risk of AV block. Atrial tachycardias originating in both the left and right atria can he successfully ablated in over 80% of patients. Given the overall effectiveness of this procedure, radiofrequency catheter ablation should be considered as front-line therapy in patients with recurrent or drug-refractory paroxysmal supraventricular tachycardia. Although an effective therapy, the risks and benefits of this procedure need to be assessed in all patients who are candidates for this procedure.     

Adenosine-Sensitive Atrial Reentrant Tachycardia Originating from the Atrioventricular Nodal Transitional Area

Adenosine-Sensitive AT from AVN Area. Introduction : Atrial tachycardia shows wide variations in its electrophysiologic properties and sites of origin. We report an atrial tachycardia with ECG manifestations and electrophysiologic characteristics similar to an atypical form of AV nodal reentrant tachycardia (AVNRT).
Methods and Results : This supraventricular tachycardia was observed in 11 patients. It was initiated by atrial extrastimulation with an inverse relationship between the coupling interval of an extrastimulus and the postextrastimulus interval. Its induction was not related to a jump in the AH interval, and its perpetuation was independent of conduction block in the AV node. Ventricular pacing during tachycardia demonstrated AV dissociation without affecting the atrial cycle length. A very small dose of adenosine triphosphate (mean 3.9 ± 1.2 mg) could terminate the tachycardia. The earliest atrial activation during tachycardia was recorded at the low anteroseptal right atrium with a different intra-atrial activation sequence from that recorded during ventricular pacing, where the tachycardia was successfully ablated in 9 of 10 attempted patients. Bidirectional AV nodal conduction remained unatttched after successful ablation.
Conclusion : There may he an entity of adenosine-sensitive atrial tachycardia probably due to focal reentry within the AV node or its transitional tissues without involvement of the AV nodal pathways. This tachycardia can he ablated without disturbing AV nodal conduction from the right atrial septum.     

Initiation of atrioventricular nodal reentrant tachycardia in patients with discontinuous anterograde atrioventricular nodal conduction curves with and without documented supraventricular tachycardia: Observations on the role of a discontinuous retrograde conduction curve

To evaluate factors playing a role in initiation of atrioventricular (AV) nodal reentrant tachycardia utilizing anterogradely a slow and retrogradely a fast conducting AV nodal pathway, 38 patients having no accessory pathways and showing discontinuous anterograde AV nodal conduction curves during atrial stimulation were studied. Twenty-two patients (group A) underwent an electrophysiologic investigation because of recurrent paroxysmal supraventricular tachycardia (SVT) that had been electrocardiographically documented before the study. Sixteen patients (group B) underwent the study because of a history of palpitations (15 patients) or recurrent ventricular tachycardia (one patient); in none of them had SVT ever been electrocardiographically documented before the investigation. Twenty-one of the 22 patients of group A demonstrated continuous retrograde conduction curves during ventricular stimulation. In 20 tachycardia was initiated by either a single atrial premature beat (18 patients) or by two atrial premature beats. Fifteen of the 16 patients of group B had discontinuous retrograde conduction curves during ventricular stimulation, with a long refractory period of their retrograde fast pathway. Tachycardia was initiated by multiple atrial premature beats in one patient. Thirteen out of the remaining 15 patients received atropine. Thereafter tachycardia could be initiated in three patients by a single atrial premature beat, by two atrial premature beats in one patient, and by incremental atrial pacing in another patient. In the remaining eight patients tachycardia could not be initiated. Our observations indicate that the pattern of ventriculoatrial conduction found during ventricular stimulation is a marker for ease of initiation of AV nodal tachycardia in patients with discontinuous anterograde AV nodal conduction curves.     

Differenzialdiagnostik und Therapie tachykarder Herzrhythmusstörungen

The 12-channel surface electrocardiogram (ECG) is an important diagnostic tool for diagnosis of arrhythmias and acute coronary syndrome. Supraventricular tachycardia (SVT) is a paroxysmal tachycardia as are sinus tachycardia, atrial tachycardia, atrioventricular (AV) nodal reentry tachycardia and tachycardia due to accessory pathways. All SVTs are characterized by a ventricular heart rate >?100 beats/min and small QRS complexes (QRS width <?0.12 s) during tachycardia. It is important to analyze the relation between p-wave and QRS complex, to look for an electric alternans as a main finding for an accessory pathway. Wide QRS complex tachycardia (QRS width ≥?0.12 s) occurs in SVT with aberrant conduction, SVT with bundle branch block or ventricular tachycardia (VT). In broad complex tachycardia, AV dissociation, negative or positive concordant patterns in V1–V6, a notch in V1 and qR complexes in V6 in tachycardia with left bundle branch block morphology, are findings indicating VT. In addition, an R/S relation <?1 in V6 favors VT when right bundle branch block tachycardia morphologies are present. By analyzing the surface ECG in the correct way with a systematic approach, the specificity and sensitivity of correctly identifying SVT or VT can be raised to >?95?%. Therapy of tachycardia is possible with few antiarrhythmic drugs (concept of the 5As), beta-blocking agents, cardioversion and defibrillation. Using these approaches termination of tachycardia is possible in the majority of cases with high success rates.     

Long-term follow-up of arrhythmias in patients with myotonic dystrophy treated by pacing: a multicenter diagnostic pacemaker study

OBJECTIVES: We hypothesized that pacemaker (PM) implantation in patients with myotonic dystrophy (MD) with a prolonged HV interval, even asymptomatic, may protect them against sudden death related to atrioventricular (AV) block. We sought to prospectively document the true incidence of AV block episodes in this high-risk population and accurately trace, in the long term, by the PM, the occurrence of arrhythmias that may remain undetected during conventional follow-up. BACKGROUND: Myotonic dystrophy is associated with a high risk of sudden death, commonly attributed to AV block or ventricular arrhythmias, but cardiac pacing is only recommended as a secondary prevention. METHODS: Patients with MD with an HV interval > or =70 ms, even in the absence of related symptoms, prospectively received a cardiac PM, including an algorithm capable of diagnosing episodes of bradycardia and tachyarrhythmias. RESULTS: The population consisted of 49 patients (45.5 +/- 8.9 years old) followed for 53.5 +/- 27.2 months. Paroxysmal arrhythmias were recorded in 41 patients (83.7%), consisting of complete AV block (n = 21), sino-atrial block (n = 4), or atrial (n = 25) or ventricular (n = 13) tachyarrhythmias. No patient died of AV block during follow-up, but 10 deaths occurred, 4 of them sudden. An arrhythmic cause could be excluded by postmortem PM interrogation in two cases of typical sudden death. CONCLUSIONS: Arrhythmias are common in patients with MD with infrahisian conduction abnormalities. The prophylactic implantation of a pacing system when the HV interval is > or =70 ms seems appropriate. The PM protects the patient against the clinical consequences of paroxysmal profound bradycardia and facilitates the diagnosis and management of frequent paroxysmal tachyarrhythmias.     

Pharmacological testing in the diagnosis of arrhythmias

Pharmacological testing has several indications in the diagnosis of arrhythmia. It is used for the diagnosis of bradycardia-related syncope either during non invasive tests as adenosine triphosphate (ATP) for the diagnosis of vasovagal syncope, but also for the diagnosis of sick sinus syndrome or isoproterenol infusion during the head up tilt test to induce a vasovagal syncope or during electrophysiological study to look for infrahisian AV block or organic sick sinus syndrome after injection of Ajmaline or to know if sick sinus syndrome or suprahisian AV block are reversible after atropine and are vagal-related. It is used for the diagnosis of supraventricular and ventricular tachycardia; isoproterenol is largely used generally during electrophysiological study. The infusion of isoproterenol is required in exercise-related arrhythmias, in arrhythmogenic right ventricular cardiomyopathy, in idiopathic ventricular tachycardia and in idiopathic dilated cardiomyopathy. ATP can be used to induce a vagal-related atrial fibrillation and may help to differentiate a reentry through accessory pathway or AV nodal re-entrant tachycardia. It is used for the detection and the evaluation of prognosis of some diseases at risk of sudden death. Isoproterenol infusion is required in the preexcitation syndrome to look for the shortening of accessory refractory period. Ajmaline or fleca?nide injection is mandatory in the family of a patient with a Brugada syndrome to detect the disease.     

Triggered activity as the proposed mechanism of left atrial tachycardia induced by premature ventricular beats.

In a 57-year-old woman with complex ventricular ectopy, a paroxysmal supraventricular tachycardia initiated by premature ventricular beats is presented. She underwent an electrophysiologic study. The tachycardia origin was localised to the left atrium. At the presence of retrograde dual atrioventricular nodal pathway, the atrial tachycardia was induced by programmed ventricular stimulation. Triggered activity was shown to be the likely mechanism of both atrial and ventricular arrhythmias.     

Subcostal M-mode echocardiography of the right atrial wall for differentiation of supraventricular tachyarrhythmias with aberration from ventricular tachycardia

Atrial contractions can be reliably detected by subcostal M-mode echocardiography of the right atrial wall. Recognition of various rhythm disturbances, especially tachyarrhythmias, is facilitated in the simultaneously recorded nondiagnostic ECG. Differentiation between supraventricular tachycardias with aberration and ventricular tachycardia with atrioventricular dissociation is alleviated. However, this technique is of limited value in the diagnosis of ventricular tachycardia with retroconduction and is not useful in differentiating supraventricular tachycardias with wide QRS from ventricular tachycardia when atrial fibrillation coexists.     

NASPE Plenary Lecture 2001. Catheter ablation: a personal perspective

Catheter ablation for control of cardiac arrhythmias was introduced 20 years ago. Since then, this technique has been applied successfully to virtually all cardiac rhythm disturbances. In this essay, some of the newer applications of ablative techniques for patients with AV nodal reentrant tachycardia, atrial flutter, and atrial fibrillation are emphasized. "AV nodal reentrant tachycardia" may involve a nodofascicular tract. A new classification of atrial flutter is proposed and various causes of atrial fibrillation are discussed.     

Patients with supraventricular tachycardia presenting with aborted sudden death: incidence, mechanism and long-term follow-up

A total of 13 (4.5%) of 290 patients with aborted sudden death had either documented (7; 54%) or strong presumptive evidence of supraventricular tachycardia that deteriorated into ventricular fibrillation. Six (46%) of the 13 had an accessory conduction pathway and either atrial fibrillation (5 patients) or paroxysmal atrioventricular (AV) reentrant tachycardia (1 patient) that deteriorated into ventricular fibrillation. Three patients with AV node reentrant tachycardia and four with atrial fibrillation and enhanced AV node conduction presented with supraventricular arrhythmias that deteriorated into ventricular fibrillation. Patients were treated with medical, surgical or catheter ablative procedures designed to prevent recurrences of supraventricular arrhythmias. Four patients received an implanted automatic defibrillator, but none had an appropriate device discharge. Over a follow-up period of 41.6 +/- 33.6 months, 12 patients are alive without symptomatic arrhythmias. One patient died because of severe chronic lung disease and heart failure. Supraventricular tachycardia was the cause of aborted sudden death in approximately 5% of patients referred for evaluation of sudden cardiac death. Treatment directed at prevention of supraventricular tachycardia was associated with an excellent prognosis. Current treatment techniques appear to obviate the need for automatic defibrillator therapy in these patients.