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The usefulness of the response to single and double ventricular premature complexes (VPCs) introduced during reciprocating tachycardia (RT) in predicting the location of a left free wall accessory pathway was studied in 55 patients with the Wolff-Parkinson-White syndrome. One VPC introduced from the right ventricle into narrow QRS RT when the His bundle was refractory resulted in retrograde atrial preexcitation in 25 of 55 (45%) patients, while 30 (55%) showed no preexcitation. Double VPCs produced retrograde atrial preexcitation in 9 of 26 patients not responding to a single VPC. No difference in RT cycle length, AH, HV or ventriculoatrial intervals was found between those patients who did or did not show retrograde atrial preexcitation. The response to single and double VPCs during RT was related to the location of the AP. The average distance of the AP from the crux determined by intraoperative epicardial mapping in the 41 patients who underwent surgery was 2.7 +/- 0.7 mapping units (left posterolateral region) in patients showing retrograde atrial preexcitation with a single VPC, 3.6 +/- 0.7 units (at the lateral left ventricular margin) in those responding to double VPCs and 4.3 +/- 0.8 units (beyond the LV margin) in those showing no response. Left bundle--branch block (LBBB) aberrancy during RT resulted in an average 60 +/- 14 ms prolongation of the ventriculoatrial interval in 40 patients, including 5 in whom LBBB was seen only after procainamide infusion. VPCs introduced into LBBB RT resulted in significant retrograde atrial preexcitation in 6 additional patients in whom no response during normal QRS RT was observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Standard electrocardiograms from 87 consecutive patients with tachycardia of left bundle branch block configuration were analyzed retrospectively for features that might be characteristic of tachycardia utilizing a nodofascicular Mahaim fiber. The study group consisted of 13 patients with nodofascicular tachycardia, 34 with supraventricular tachycardia and aberrant conduction over the His-Purkinje system, 22 with ventricular tachycardia and 18 with antidromic tachycardia utilizing a right-sided accessory atrioventricular pathway. Six variables present during tachycardia of left bundle branch block configuration were predictive of a nodofascicular fiber: cycle length between 220 and 450 ms, QRS axis of 0 to -75 degrees, QRS duration 0.15 second or less, R wave in lead I, rS wave in precordial lead V1 and a precordial transition from a negative to a positive QRS complex after lead V4. All six criteria were present in 16 of the 87 patients. No patient with ventricular tachycardia satisfied these criteria, whereas 3 of 34 with supraventricular tachycardia, 1 of 18 with antidromic tachycardia and 12 of 13 with tachycardia using a nodofascicular fiber did. It is concluded that analysis of the surface electrocardiogram during tachycardia may suggest the presence of a nodofascicular fiber.

The dynamic response of squared conduction velocity, theta 2, to repetitive stimulation in canine Purkinje fibers with quinidine was studied using a double-microelectrode technique. With stimulation, a frequency-dependent monoexponential increase in conduction delay (CD) and a decline in theta 2 were observed. The exponential rates and changes in steady-state CD and theta 2 were frequency- and concentration-dependent. The overall drug uptake rates describing blockade and the interpulse recovery interval were linearly related and steady-state values of theta 2 were linearly related to an exponential function of the stimulus intervals. Based on first-order binding, the frequency- and concentration-dependent properties of quinidine were characterized by the apparent binding and unbinding rates of 14.2 +/- 5.7 X 10(6) mol-1.s-1 and 63 +/- 12 s-1 for activated and 14.8 +/- 1.0 X 10(2) mol-1.s-1 and 0.16 +/- 0.03 s-1 for resting states. The recovery time constant extracted from the pulse train interpulse interval was 5.8 +/- 1.5 s compared with 5.1 +/- 0.6 s determined from a posttrain test pulse protocol. This study demonstrates that the kinetics of drug action can be derived from measures of impulse propagation. This provides a basis for characterizing frequency-dependent properties of antiarrhythmic agents in vivo and suggests the plausibility of a quantitative assessment of drug binding and recovery rates in man.

Every year, individuals with no history of heart disease succumb to sudden cardiac death (SCD). Pathologic examination of the hearts usually reveals various forms of heart disease as hypertrophic cardiomyopathy or coronary artery disease. In other cases, however, there is no obvious structural heart disease, and it is possible that some of these individuals died because of a cardiac arrhythmia involving an accessory pathway. If this were the case, the most likely scenario would be onset of atrioventricular reciprocating tachycardia (AVRT), degeneration of the AVRT into atrial fibrillation with a rapid ventricular response over the accessory pathway, and subsequent death caused by the development of ventricular fibrillation. Although these events have been documented, albeit rarely, during intracardiac electrophysiologic studies, in reality very little is known about the natural history of asymptomatic and untreated patients with Wolff-Parkinson-White (WPW) syndrome. In fact, SCD in a previously asymptomatic patient with WPW syndrome is probably relatively rare. Whether asymptomatic WPW patients should undergo electrophysiologic or pharmacologic testing to determine their 'potential' to develop serious cardiac arrhythmias is controversial. The present paucity of data concerning the natural history of WPW syndrome in asymptomatic patients militates against successful identification of those patients who are at risk for sudden death. Long-term prospective studies are necessary to clarify which asymptomatic patients with WPW syndrome require treatment.

Two types of arrhythmias are associated with the Wolff-Parkinson-White syndrome: those in which the accessory pathway is a required part of the reentrant circuit, e.g., orthodromic atrioventricular reciprocating tachycardia, and those that conduct over the accessory pathway but do not require its activation for maintenance of tachycardia, e.g., atrial flutter/fibrillation. Increased sympathetic tone shortens the refractoriness of atrial and ventricular tissue; however, conduction in the atrium and ventricle is not considered the limiting factor for maintenance of atrioventricular reciprocating tachycardia or conduction over the accessory pathway in atrial arrhythmias. Intravenous beta-adrenergic blockers given to patients in the resting state have a minimal to moderate effect in depressing atrioventricular nodal conduction, but have little or no effect on accessory pathway refractoriness or conduction in most patients. In patients presenting with atrioventricular reentry, intravenous administration of beta-adrenergic blocking drugs often is not effective to terminate tachycardia. However, long-term oral therapy with these agents may be beneficial, especially in patients in whom enhanced sympathetic tone is responsible for the initiation or maintenance of tachycardia.

The demonstrable value of EP studies for any given diagnostic or therapeutic category, in the last analysis, is largely a function of the subjects studied. Extrapolations from published data (that generally reflect a highly select patient population) to an individual patient can be fraught with error. Considerations of sensitivity and specificity must be balanced against the important need for information in patients at risk from life-threatening arrhythmias. We must never forget, however, that the EP substrate for any arrhythmia is not, as one might wish, a "black box" that should be expected to respond in a reproducible fashion to stimulation. The substrate is dynamic and subject to modification by change in autonomic tone, stretch, blood flow, basal rate, pH, electrolytes, oxygenation, and exposure to perhaps as yet undiscovered humoral mediators. The challenge to the clinical electrophysiologist is therefore not to exaggerate his efforts in one direction (i.e. programmed stimulation) while disregarding the other variables mentioned. Nor should we be disappointed and discard this approach because our expectations of an oversimplified model of arrhythmia testing are not fulfilled. Thus, in addition to careful stratification of patients, baseline studies should perhaps be carried out with more deliberate consideration of autonomic tone (exercise, isoproterenol), stretch (volume, handgrip, afterload), stress (physical and psychological), local anesthetic used, and body position. Only in this way will the scientific basis for acute and chronic EP testing be firmly established.

During the electrophysiologic study of 435 patients referred for evaluation of Wolff-Parkinson-White syndrome, 42 (10%) had preexcited reciprocating tachycardia (defined as a macro-reentrant tachycardia that used an accessory atrioventricular [AV] pathway for antegrade conduction). The ages of the patients ranged from 9 to 67 years (27 +/- 14). Thirty-three were male patients, nine female, and eight had Ebstein's anomaly. Preexcited reciprocating tachycardia cycle length was 220 to 430 msec (294 +/- 42). Significant hemodynamic compromise in the laboratory directly related to preexcitated reciprocating tachycardia occurred in only one patient. However, in 10 patients a transformation to atrial fibrillation was seen after a spontaneously occurring premature atrial contraction. Only 17 of the 42 patients with preexcited reciprocating tachycardia during electrophysiologic study had the same tachycardia documented clinically. These 17 patients were more often younger with multiple accessory pathways and with no history of orthodromic reciprocating tachycardia when compared with 25 patients in whom preexcited reciprocating tachycardia could be induced only in the laboratory. Preexcited reciprocating tachycardia was induced in the laboratory in 22 of 374 (6%) patients with single accessory pathways and in 20 of 61 (33%) of those with multiple accessory pathways. In the 20 patients with multiple accessory pathways, the spectrum of reentrant circuits included fusion over two or more accessory pathways or fusion over both an accessory pathway and the AV node. In the 22 patients with a single accessory pathway and true antidromic reciprocating tachycardia, all but two episodes were at least 4 cm from the AV node. No patient with true antidromic reciprocating tachycardia had a posterior septal accessory AV pathway. Only in patients with multiple accessory pathways was the posterior septal accessory AV pathway used as the antegrade limb.

Eight patients, aged 5 to 57 years, with uncontrolled symptomatic tachycardia for 2.5 to 41 years (mean 15) and significant left ventricular (LV) dysfunction in the absence of any other apparent underlying cardiac disease underwent evaluation. Incessant tachycardia was present for 0.5 to 6.0 years (mean 2.1) in 7 patients. One patient had an ectopic atrial tachycardia and 7 patients had an accessory atrioventricular pathway that participated in reciprocating tachycardia. Six patients underwent surgery; the ectopic focus was ablated in 1 patient and an accessory pathway was divided in 5 patients. One patient underwent open ablation of the His bundle and 1 patient underwent closed-chest ablation of the atrioventricular conduction system. Myocardial biopsy specimens were obtained from 5 patients, none of which yielded a specific diagnosis. Pretreatment radionuclide angiography demonstrated a mean ejection fraction (EF) of 19 +/- 9% (range 10 to 35%). Following tachycardia control a marked improvement in LV function was noted in 6 of 8 patients at rest and in 1 additional patient during exercise. The EF increased to 33 +/- 17% (range 16 to 56%) an average of 8 days after treatment and to 45 +/- 15% (range 22 to 67%) at late follow-up 3.5 +/- 40 months (mean 17) later (p less than 0.005). Seven patients remain asymptomatic 11 to 40 months (mean 22) after the corrective procedure and have resumed normal activities. These findings suggest that chronic uncontrolled tachycardia may result in significant LV dysfunction, which is reversible in some cases after control of the arrhythmia.

Cardiac illness in myotonic muscular dystrophy (MyD) is infrequent, but subclinical cardiac involvement in MyD is very common (found in 42 of 46 subjects) and may be responsible for sudden death. In this series, we found ECG abnormalities in 72%, left ventricular dysfunction in 70%, mitral valve prolapse in 37%, and sudden death in 4%. Four deaths during the study period were due to acute left ventricular failure, one to sepsis and respiratory insufficiency, and one was unexplained. We did not find ominous bradyarrhythmias or atrioventricular block, evidence of congestive heart failure, noninvasive evidence of coronary artery disease, or any correlation of type or amount of cardiac involvement with any clinical parameter such as age, sex, or severity of systemic dystrophy. We feel tachyarrhythmias may play as important a role in sudden death of myotonic muscular dystrophy subjects as bradyarrhythmias, and coronary artery disease in addition to cardiac dystrophy may produce arrhythmias and myocardial dysfunction in myotonic muscular dystrophy. In addition, some subjects have an unusual form of resting left ventricular dysfunction which improves with exercise. The most important problem in the clinical management of myotonic muscular dystrophy subjects is sudden death, and the solution does not appear to be empiric ventricular pacing. Our recommendations for prophylaxis of sudden death in myotonic muscular dystrophy are noninvasive investigation of coronary artery disease in subjects with significant risk factors, with angiography and surgery if indicated: detailed evaluation of syncopal and presyncopal events, including electrophysiologic testing, with pacemaker or antiarrhythmic drug therapy if indicated; and consideration of ventricular pacing of asymptomatic subjects if severe bradycardia or marked intraventricular conduction delay develops during follow-up, serial 12-lead ECGs. The documentation of tachyarrhythmias during sudden death and syncopal episodes in myotonic muscular dystrophy subjects makes ventricular pacing alone an uncertain modality for prevention of sudden death in subjects with only mildly lengthened PR or QRS intervals, and suggests a combination of pacemaker and antiarrhythmic drug therapy for the myotonic muscular dystrophy subject with syncope of no apparent cause.

Nonischemic ventricular tachycardia most commonly arises in the right ventricular free wall and is frequently refractory to medical therapy. Many different types of surgical procedures have been employed to treat medically refractory nonischemic ventricular tachycardia arising in the right ventricle, but the results of these procedures have been less than optimal. The majority of these surgical procedures have been directed toward ablation of the site (or sites) of origin of the tachyarrhythmia and have failed because of the frequent occurrence of multifocal or polymorphic ventricular tachycardia in these patients. We first employed localized surgical isolation procedures to control nonischemic ventricular tachycardia arising in the right ventricular free wall in 1979. These localized procedures evolved into the development of a technique for isolating the entire right ventricular free wall from the remainder of the heart to control ventricular tachyarrhythmias arising from multiple sites in the right ventricle. Case histories are reported of two patients who underwent localized isolation procedures in 1979 as well as two patients who underwent total disconnection of the right ventricle in 1982. The follow-up period in these four patients ranges from 2 to 5 years and the control of their tachyarrhythmias has been uniformly successful. However, surgical isolation of the entire right ventricular free wall has resulted in progressive dilatation of the right ventricle as documented by serial echocardiography. The pathophysiology of the progressive right ventricular dilatation postoperatively is discussed in terms of etiology and prevention, and the indications for application of localized and total isolation procedures for nonischemic right ventricular tachycardia are outlined.

A solution of ethyl alcohol, polyethylglycol, and distilled water that did (n = 5) or did not (n = 5) contain 250 micrograms/kg of the calcium channel blocker, nifedipine, was infused into the right atrium in 10 healthy mongrel dogs. Hydrochloric acid (pH = 1.8), 2 ml/kg, was then instilled into both lungs of all dogs via the tracheal tube. Hemodynamic data were collected before and 10 minutes after nifedipine was infused and 10, 45, 90 and 180 minutes after acid was instilled into the lungs. Gas exchange, including PaO2 and venous admixture, did not differ between the two groups. However, after aspiration, oxygen delivery significantly improved in the dogs given nifedipine compared to oxygen delivery in those not treated with nifedipine. We conclude that, despite significant decreases in PaO2 and venous admixture, nifedipine can restore oxygen delivery back to normal levels.

Persistence of preexcitation in sinus rhythm with procainamide infusion has been reported to occur in patients with a short anterograde accessory pathway effective refractory period (AERPAP) and this test has been proposed as a reliable noninvasive method to identify patients with the Wolff-Parkinson-White syndrome who are at risk of sudden death. However, sudden death correlates best with a shortest preexcited RR interval during atrial fibrillation (SRRPE) of 260 msec or less. We infused 10 to 12 mg/kg procainamide to 56 patients to determine whether persistence or loss of preexcitation in sinus rhythm identified patients with SRRPEs of 260 or less or greater than 260 msec, respectively. Atrial fibrillation was induced in 53 patients. Of these, 32 patients had persistence of preexcitation with procainamide infusion and SRRPE in this group of patients was shorter than that in patients in whom preexcitation was lost (194 +/- 44 vs 235 +/- 55 msec, p less than .05). However, preexcitation persisted after procainamide infusion in only 31 of 46 (67%) patients with SRRPEs of 260 msec or less. Furthermore, 15 of 21 patients who lost preexcitation had SRRPEs of 260 msec or less and two of these patients had a history of ventricular fibrillation. The correlation between AERPAP and SRRPE was studied in a separate group of 79 patients with single accessory pathways. There was a significant (p less than .001) but poor (r = .58) correlation between these two variables. Thus, the procainamide test regarding accessory pathway refractoriness often cannot be extrapolated to SRRPE.(ABSTRACT TRUNCATED AT 250 WORDS)