Electrophysiologic-histologic correlation of the cardiac specialized conduction system in two cases of single ventricle and levotransposition of the great arteries

The specialized cardiac conduction system was delineated by electrophysiologic mapping during open heart surgery in two patients with single ventricle and levotransposition of the great arteries. In both patients the conduction system was found to lie superior and anterior to the bulboventricular foramen. The sites where specialized conduction system electrograms were obtained were subsequently shown to contain the His bundle and the right and left bundle branches. In one patient with congenital complete heart block, the histologic studies demonstrated the site of block to be due to complete interruption of the His bundle. The electrophysiologic-histologic correlation carried out in this study demonstrates the relationship between specialized conduction system electrograms obtained under direct vision during open heart surgery and the anatomic location of the specialized conduction system. The study further supports the value of the electrophysiologic technique for intraoperative identification of the specialized conduction system and its reliability in cases where electrograms were obtained showing the specialized conduction system to be not in its anticipated location.     

Electrophysiologic studies in Mobitz type II second degree heart block

The electrophysiologic basis for Mobitz type II second degree heart block was studied in a patient with documented episodes of complete heart block and Stokes-Adams seizures. Recording of His bundle electrograms demonstrated that all atrial impulses were conducted through the atrioventricular (A-V) node to the bundle of His. Nonconducted P waves were blocked distal to the bundle of His. The frequency of blocked beats increased when the rate was accelerated by atrial pacing. Administration of atropine caused more frequent blocked beats, reaffirming the concept that the block occurred beyond the node. It is concluded that Mobitz type II second degree heart block is a manifestation of bilateral bundle branch block. Testing with atropine in this case was useful in distinguishing block in the bundle branches from that in the A-V node.     

Accelerated A-V conduction associated with complete A-V block

HBE's were recorded in a 70-year-old female patient with complete heart block, narrow QRS, and past episodes of supraventricular tachycardia. The HBE revealed split His potentials with intra-His block. Atrial pacing at increased rates failed to increase the AH1 interval, suggesting an accelerated pathway between the atria and the proximal His bundle bypassing the A-V node. Below the site of block, the ventricles were paced by a distal His bundle (H2) with a resultant normal QRS interval.     

Mechanism and significance of widened QRS complexes during complete atrioventricular block in acute inferior myocardial infarction

Twelve of 35 consecutive patients admitted with complete, atrioventricular (A-V) block complicating acute inferior myocardial infarction manifested widened QRS complexes. The escape beats had the pattern of left bundle branch block in four patients, right bundle branch block in five patients and both left and right bundle branch block in three patients.

His bundle recordings in five patients with escape beats that had a left bundle branch block configuration revealed a His bundle potential preceding the widened QRS complex at His-V intervals of 45 to 60 msec. Bradycardia-dependent left bundle branch block was demonstrated in two patients by His bundle pacing. In three patients the conducted beats had a left bundle branch block configuration after critical lengthening of the R-R interval during second degree A-V block before or after the episode of complete A-V block. In six patients whose escape beats had a right bundle branch block configuration, His bundle recordings did not reveal a His bundle potential preceding these beats.

Our observations suggest that widened QRS complexes with a left bundle branch block configuration could be due to an A-V junctional escape rhythm with phase 4 left bundle branch block. Alternatively in association with a right bundle branch block configuration it is possible that the widened QRS complexes represent a ventricular or fascicular escape rhythm.

Two of 12 patients with widened QRS complexes died. There were no significant differences in immediate mortality, 6 month mortality or mean peak serum glutamic oxaloacetic transaminase (SGOT) values between patients with narrow and widened QRS complexes. This finding suggests that widened QRS complexes during complete A-V block in acute inferior myocardial infarction have no prognostic significance.     

Pathologic correlations in a case of complete heart block with split his potentials resulting from a stab wound of the heart

A 23 year old previously healthy man was stabbed in the anterior chest. This resulted in a ventricular septal defect and complete atrioventricular (A-V) block. The electrocardiogram revealed complete A-V block with a QRS pattern of right bundle branch block. His bundle recordings 26 days later revealed A-V dissociation with split His potentials (P-H₁ interval of 100 msec and H₂-V interval of 40 msec). During the study the escape QRS shifted from right to left bundle branch block with H₂ potentials still preceding each QRS interval with H₂-V intervals of 40 msec. A permanent pacemaker was implanted because of persistent congestive heart failure and bradycardia due to A-V block. The patient subsequently became asymptomatic. He died suddenly 3 $\text{1}\text{2}$ years later.Pathologically there were sizable openings in both the tricuspid and mitral valve substance and a ventricular septal defect involving the pars membranacea and part of the adjacent muscular septum. Serial sections of the conduction system revealed total destruction and fibrous replacement of the bifurcation and beginning of the right and left bundle branches and subtotal fibrous replacement of the branching bundle. Thus, the bifurcation of the bundle of His was totally absent at autopsy despite apparent electrophysiologic evidence of its existence 26 days after the stab wound. A possible explanation for this discrepancy is the subsequent fibrosis of the bifurcation produced by hemodynamic changes at the lower margin of the ventricular septal defect.     

Management of surgical complete atrioventricular block in children

Because there is disagreement concerning the efficacy of and indication for permanent pacemaker implantation in children with postoperative complete (third degree) atrioventricular (A-V) block, experience in the management of this problem at one institution was reviewed. Thirty-four patients with postoperative complete atrioventricular block were identified. They ranged In age from 4 months to 22 years and in weight from 4 to 60 kg and were evaluated from 1 month to 20 years postoperatively.

Complete A-V block developed within 24 hours of operation in 28 of the 34 patients. A permanent pacemaker was implanted in 13 of the 28. Death occurred in 4 of these 13 patients and in 5 of the remaining 15 patients who did not have an artificial permanent pacemaker. Complete A-V block developed later than 1 day (2 days to 4 months) postoperatlvely in 6 of the 34 patients; all 6 of these patients survived, and only 3 required permanent pacemaker implantation.

Intracardiac electrophysiologic studies were performed by 14 of the 34 patients. The site of complete block was above the His bundle in 5, within the His bundle in 2, and below the His bundle in 4; it was undetermined in 3. The results of intracardiac electrophysiologic studies are important in delineating the natural history of surgically induced complete A-V block and in the clinical management of this lesion. Permanent pacemaker implantation is indicated if complete A-V block persists longer than 2 weeks postoperatively and if the site of the block is within or below the bundle of His.     

Inexcitable right ventricle and bilateral bundle branch block in Uhl's disease

A 29-year-old female with Uhl's anomaly developed complete atrioventricular (A-V) block. His bundle studies revealed block distal to the His bundle recording site with narrow QRS complexes. Right ventricular capture could not be obtained and despite successful left ventricular epicardial pacing, the patient died. Autopsy revealed absence of myocardium in most areas of the right ventricle and the right side of the ventricular septum with a normal tricuspid valve. Conduction system examination revelaed total destruction of both bundle branches. This is the first case where bilateral bundle branch block is shown to be present in Uhl's anomaly. Narrow QRS complexes probably reflected the absence of right ventricular forces.     

Significance of A-H interval in patients with chronic bundle branch block. Clinical, electrophysiologic and follow-up observations.

His bundle electrograms were recorded in 308 adults with chronic bundle branch block. The A-H interval was normal in 249 patients and prolonged in 59. Comparison of patients with normal and prolonged A-H intervals revealed a greater incidence of demonstrable organic heart disease in the latter (P less than 0.01). Dyspnea, cardiomegaly and congestive heart failure were more frequent in patients with A-H prolongation. These patients also had longer P-R intervals and atrioventricular (A-V) nodal effective refractory periods, lower paced rates producing second degree A-V block proximal to the His bundle and a greater frequency of H-V prolongation. All patients were prospectively followed up in a conduction disease clinic with mean follow-up periods (+/- standard error of the mean) of 523 +/- 23 and 588 +/- 47 days in the patients with normal and prolonged A-H intervals, respectively. Seven (3 percent) of the patients with a normal A-H interval had A-V block with probable or definite site of block proximal to the His bundle in three and distal to the His bundle in four. In five of the six patients with a prolonged A-H interval who experienced A-V block (10 percent), the probable or definite site of block was proximal to the His bundle. Mortality (both sudden and nonsudden) was not significantly different in the patients with normal and prolonged A-H intervals. In summary, A-H prolongation was associated with increased incidence of organic heart disease and myocardial dysfunction. The risk of development of A-V nodal block was greater in patients with a prolonged A-H interval but appeared to be of minimal clinical significance.     

Electrophysiologic and pathologic correlations in two cases of chronic second degree atrioventricular block with left bundle branch block.

This study concerns two cases of chronic 2 degrees atrioventricular (A-V) block with left bundle branch block (LBBB). Pathological studies included serial section of the conduction systems. Case 1 had type I 2 degrees block with LBBB. Electrophysiological studies revealed type I 2 degrees block proximal to the His bundle recording site and a prolonged H-V interval (60 msec). Pathologically there was a moderate to marked fibrosis of the approaches to the A-V node and of the A-V node, marked fibrosis of the left bundle branch, and moderate involvement of the right bundle branch. The changes proximal to the His bundle were more marked than the changes distal to this bundle. Case 2 had type II and 2:1 2 degrees A-V block with LBBB. Electrophysiologically the site of block was distal to the His bundle recording site, and there was a prolonged A-V node and severe involvement of both bundle branches. The changes distal to the His bundle were more severe than the changes proximal to the His bundle. This study reveals that the electrophysiologic data more closely approximated the pathologic findings than did surface electrocardiographic data alone. It also emphasizes that there may be multiple sites of disease in chronic 2 degrees block with bundle branch block.     

Tachycardia-dependent and bradycardia-dependent mobitz type II atrioventricular block within the bundle of His

This report describes the occurrence in a young man of transient Mobitz type II and complete atrioventricular (A-V) block due to a lesion within the proximal portion of the His bundle probably caused by excessive alcohol intake. Initially A-V block was precipitated by tachycardia only but it subsequently became solely bradycardia-dependent before disappearing completely after a few days. Bundle of His electrograms demonstrated block above the site of recording of the His bundle potential.     

His bundle electrocardiography in manifest and concealed right bundle branch extrasystoles.

His bundle electrocardiography was helpful in the diagnosis of impulse formation in the right bundle branch. Ten patients with narrow QRS complexes had ectopic beats with an "incomplete" left bundle branch pattern and almost simultaneous activation of His bundle and ventricles. Both QRS morphology and H- - V intervals depended on the more proximal or distal location of the ectopic focus. In four patients with "complete" right bundle branch block the morphology of ectopic ventricular complexes and H- - V intervals also depeneded on the presence or absence of retrograde block and differential degrees of forward and/or retrograde conduction delays. Nine patients with "complete" right bundle branch block and four with "complete" left bundle branch block had premature beats which could have originated in the proximal right bundle branch, proximal left bundle branch, or distal His bundle. In one patient with "complete" left bundle branch block, "concealed" His bundle depolarizations (probably originating in an ectopic focus located in the right bundle branch) produced pseudo Type II (Mobitz) A-V block. Although lidocaine appeared to have been more effective in patients with bundle branch block than in those with narrow QRS complexes, further studies are necessary to corroborate this impression.     

His bundle electrocardiography in digitalis-induced "atrioventricular junctional" Wenckebach periods with irregular H-H intervals.

His bundle electrograms were recorded during catheter insertion for prophylactic demand pacing in two patients with accelerated or nonaccelerated "atrioventricular (A-V) junctional" rhythms associated with A-V junctional Wenckebach periods. This appears to be the first published report of so-called A-V junctional Wenckebach periods in which the characteristic irregularities of the H-H intervals were recorded. Patient 1 had an additional area of "complete" anterograde A-V nodal (A-H) block. In Patient 2 the rate of impulse formation was consistent with nonparoxysmal A-V junctional tachycardia. The His bundle recordings were obtained in patients with digitalis toxicity and should be interpreted in the context. The integration of clinical and intracardiac findings with extrapolations from microelectrode and pharmacolic studies and with deductions from the clinical electrocardiograms suggests that the conduction disturbances probably occurred within the A-V node itself (in its AN region). This hypothesis implies that automaticity also originated in the A-V node because the site of impulse formation must have been proximal to the site of the Wenckebach periods. However, conclusive proof of of these postulates will require further studies with refined techniques.     

A-V conduction disturbances in Reiter's syndrome

High grade atrioventricular (A-V) block is a rarely described complication of Reiter's syndrome. This 65 year old man had recurrent episodes of arthritis, conjunctivitis and urethritis beginning at age 16. A prolonged P-R interval was first noted at age 32. The conduction disturbance progressed to intermittent episodes of high grade and complete heart block by age 65. His bundle electrograms located the site of block above the level of the bundle of His. Atrial pacing to rates of 150/min produced 5:1 A-V block, whereas exercise and atropine administration resulted in 1:1 A-V conduction. In view of these results, artificial pacemaker therapy is not indicated. The association of conduction disorders and Reiter's syndrome is reviewed.     

Failure to recognize a His bundle potential in complete atrioventricular block.

This report concerns a patient with complete heart block, in whom electrophysiological studies showed at times an escape rhythm with narrow QRS complexes preceded by His potentials with normal HV intervals (35--40 msec) and at other times an escape rhythm of similar rate, having wide QRS complexes of left bundle branch block configuration with no preceding His bundle activity. Complexes intermediate in width and configuration and preceded by His potentials with an HV interval inversely proportional to QRS width were also recorded. These observations are explained by a site of block proximal to the His bundle and competition between two pacemaker foci having similar discharge rates, one situated in the junctional region below the site of block and the other more distally in the right bundle branch or right ventricle. It is proposed that the combination of a proximal site of block and a distally situated dominant pacemaker may be a common reason for failure to record a His potential in patients with complete heart block.     

Effect of lidocaine on escape rate in patients with complete atrioventricular block: B. Proximal his bundle block

Lidocaine was administered intravenously (a loading dose of 1.5 mg/kg body weight followed by a 3 mg/min infusion) to 10 patients with complete atrioventricular (A-V) block proximal to the His bundle and A-V Junctional escape rhythm. A-V block was not due to an acute myocardial infarction in seven patients (group I) and was due to an acute inferior wall infarction in three patients (group II). Lidocaine had either no or only a slight depressant effect on the rate of the escape pacemaker in patients in group I but caused severe bradycardia or asystole in two of three patients in group II. Lidocaine had no consistent effect on the atrial rate and did not change the QRS duration and H-V intervals in any patient. These observations are consistent with the results of animal studies that showed that lidocaine selectively depressed conduction in Ischemic or depolarized myocardium. The findings also suggest that the use of lidocaine without prior insertion of a pacemaker is unsafe in patients with acute myocardial infarction and complete A-V block proximal to the His bundle.     

Bedside evaluation of atrioventricular block with narrow QRS complexes: Usefulness of carotid sinus massage and atropine administration

Second-degree intra-His bundle block is frequently of type I (Wenckebach periods) or 2:1. In this situation, the surface electrocardiogram does not permit distinction between intranodal (atrioventricular [A-V]) and subnodal (intra-His) block. This study examined the value of bedside carotid sinus massage and atropine administration in diagnosing the site of block from the standard electrocardiogram in subjects with chronic A-V block and narrow QRS complexes. Fifteen patients had intra-His bundle block and 10 had intranodal block. The combination of two tests correctly located the site of block in 22 subjects, and was noncontributory in 3. Thirteen of the 15 intra-His bundle blocks and 9 of the 10 intranodal blocks were properly identified; in three cases the results were nondiagnostic, but no wrong diagnoses were made.The noninvasive bedside method of carotid sinus massage and the use of atropine permit both the localization and the determination of the type of block in the majority of cases of second degree A-V block and narrow QRS complexes. In a proper clinical context they can obviate the need for invasive electrophysiologic studies.     

Atrioventricular block after reciprocating atrioventricular junctional tachycardia.

Short runs of symptomatic atrioventricular (A-V) block occurred after spontaneous cessation of reciprocating A-V junctional tachycardia in a patient with right bundle branch block, normal H-V interval and sinus nodal dysfunction. These episodes were characterized by long (more than 1 sec) P-P intervals during which the A deflections were not followed by His bundle electrograms. Three possible explanations are: (1) a posttachycardia-induced period of abnormally prolonged A-V nodal refractoriness; (2) pseudo-A-V block produced by concealed A-V junctional tachycardia, or (3) bradycardia-dependent (phase 4) A-V block at the "upper" His bundle, above the site from which the H deflection was recorded.     

His bundle recordings in a case of complete atrioventricular block combined with pre-excitation syndrome.

In a patient with complete A-V block suffering from attacks of dizziness an intermittent A-V conduction with a short P-R interval and a delta wave of the conducted ventricular complex were observed. After accelerating the sinus rate by atropine and by exercise, one-to-one conduction was established with QRS complexes of WPW type A configuration. His bundle recordings revealed a complete block within the normal conduction system at the level of the A-V node. A slow junctional rhythm with a normal H-V interval was activating the ventricle. During atrial pacing a one-to-one conduction through an accessory pathway could be documented at cycle lengths between 800 and 380 msec. sandwiched in between zones of complete block at smaller or longer cycle lengths. During ventricular stimulation no retrograde V-A conduction could be observed. The findings support the thesis of at least two functionally different A-V pathways in patients with pre-excitation syndrome.     

Familial atrioventricular heart block; an autosomal dominant trait.

A family of 28 individuals spanning four generations was investigated because of a finding of complete heart block in five members and the existence of a low degree of atrioventricular (A-V) heart block in a sixth member. The disorder was characterized by 1) adult onset in all, 2) complete A-V heart block in five and first degree A-V heart block in one, 3) sinus bradycardia in three, 4) atrial fibrillation in five, 5) abnormal QRS complex in five, 6) ventricular tachycardia in three, 7) left ventricular enlargement in all, and 8) mitral insufficiency in five. Proximal location of the A-V heart block was suggested by the fact that atropine caused acceleration of the ventricular rate and by the presence of a His bundle potential preceding the QRS complexes. Involvement of the distal conducting system was indicated by the widened QRS complex and a prolonged H-V interval. Pathologic examination in one case showed extensive sinus node fibrosis and interruption of the A-V node-His bundle connection. This disorder is probably due to an autosomal dominant trait.     

Rhythm and conduction disturbances in isolated, congenitally corrected transposition of the great arteries

The incidence and types of rhythm and conduction disturbances in 11 male and 6 female patients with congenitally corrected transposition of the great arteries and no other intrinsic complicating anomalies were studied. Patient age ranged from 5 to 54 years; follow-up ranged from 5 to 37 years. Surface electrocardiograms were recorded in each patient; 15 also underwent 24-hour Holter monitoring and 10 underwent electrophysiologic study. The conduction system of a 54-year-old woman who died suddenly, with complete atrioventricular (AV) block since age 30 years, was studied by serial histologic sections. Fifteen patients were asymptomatic and 2 reported repeated episodes of palpitation since childhood. Ten patients presented with a normal PR interval and 2 with first-degree AV block (12%). Five patients had complete AV block (29%), but none had had it at birth; first- and second-degree AV block preceded complete AV block in 2 patients. The morphologic pattern and duration of QRS suggested a junctional rhythm in 4 patients and an idioventricular pacemaker in 1 patient. Electrophysiologic studies confirmed that the complete AV block site was supra-Hisian in 2 patients and proximal to the His bundle bifurcation in 1 patient. However, histologic investigation disclosed fibrosis and disruption of the proximal nonbifurcating His bundle in the patient who died suddenly. In 2 patients with recurrent supraventricular tachycardia, electrophysiologic studies suggested reentry through James fibers (or dual AV nodal pathway) in 1 and the presence of a left lateral accessory AV pathway in the other. Holter monitoring showed a high incidence of ventricular arrhythmias.