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Introduction: Recent studies have shown that His-bundle pacing could be an alternative in patients requiring cardiac resynchronization therapy as it is comparable or better in terms of amelioration of ventricular activation, narrowing of the QRS complex, or clinical outcomes. However, in case of high threshold at the level of His-bundle or inability to correct conduction through a diseased His-Purkinje system other option should be searched like left bundle pacing., Patient Concerns: A 77-year-old man presented to the Emergency Department for dizziness and dizziness and lightheadedness due to an intermittent 2:1 atrioventricular block with a QRS complex morphology of a major left branch block., Diagnosis: Given the documented symptomatic 2:1 AV block, according to the European Guideliness the patient was considered to have a class 1 indication of permanent double chamber cardiostimulation., Interventions: A lead delivery system with a C315 His catheter and a Select Secure 3830 69 cm pacing lead were placed at the His bundle area with important narrowing of the QRS complex but with an unacceptable high threshold. The delivery system was moved towards the apex 1,5 cm and the lead screwed deep into the septum until capture of the left bundle branch was achieved with complete normalization of the conduction troubles., Outcomes: At 3 month follow-up the patient was asymptomatic and the pacing and sensing thresholds remained at same values as during implantation: 0.75/0.4 ms and 14 mV respectively., Conclusion: Left bundle-pacing represents the next step of His-Purkinje system pacing to overcome all difficulties related to His-bundle pacing.

Hisian extrasystoles originate from the His bundle. They are rare and usually misdiagnosed. In fact, they manifest on the EKG, with a p' wave located before, in or after the QRS complex. More rarely, the extrasystole blocks the propagation of the influx to the ventricles simulating a Mobitz II atrioventricular (AV) block. We report the case of a 36-Year-old woman with no medical History, suffering from presyncope and palpitations at rest. Her physical examination and EKG were normal. The 24-hour Holter monitoring showed some long periods with unexpected blocked p waves but with no significant pause. Considering her young age and the absence of causes of AV block, we performed an intracardiac electrophysiological study which showed hisian extrasystoles with normal conduction tissue properties at baseline and under flecainide. The diagnosis of hisian extrasystoles simulating Mobitz II AV block was made. A simple monitoring with beta-blockers therapy was recommended. Hisian extrasystoles may simulate first or second degree AV block with different therapeutic and prognostic implications. Nevertheless, these hisian extrasystoles may be the marker of a vulnerable AV conduction, long-term follow up should be considered.

Background: Catheter cryoablation of supraventricular tachycardias involving the perinodal regions is considered to be a safer alternative compared to radiofrequency ablation. Limited information is available for efficacy, midterm outcomes, and complications regarding the ablation of parahissian accessory pathways (APs) in pediatric patients., Methods: A retrospective review of all pediatric patients who underwent cryoablation for treatment of a parahissian AP was performed., Results: Twenty-five patients (median age 13 years and weight 45.6 kg) underwent cryoablation of a parahissian AP. Median number of cryolesions applied was four (range: 3-6). Initial procedural success was achieved in 23 patients (23/25, 92%). Transient third-degree atrioventricular (AV) block was noted in two patients. There was no permanent AV block. Transient right bundle branch block (RBBB) was observed in one patient and permanent RBBB occurred in two patients. Of the patients successfully ablated with cryo, there was only one recurrence (1/23, 4.3%) over a follow-up of 17.5 months (range 6-34 months)., Conclusion: Cryoablation of parahissian APs is both safe and effective with a low risk of recurrence in pediatric patients., (©2013, The Authors. Journal compilation ©2013 Wiley Periodicals, Inc.)

Introduction: In this case, electrophysiology and histology could be studied in the same heart. Clinical investigation, clinical electrophysiology, and postmortem serial histological sections of the septum were analyzed., Methods: A patient with repeated seizures and a short PR interval with narrow QRS complex underwent electrophysiologic studies., Results: The patient died while experiencing a very rapid supraventricular tachycardia and histologic examination showed a atrio-hisian bypass tract., Conclusion: In our study, the lack of lengthening of the PR interval in spite of progressively premature atrial stimulation connected with the presence of atrio-hisian bypass tract., (©2012, The Authors. Journal compilation ©2012 Wiley Periodicals, Inc.)

We present electrocardiograms of a 37 year-old male with frequent extrasystoles and second-degree atrioventricular block. Electrophysiological study confirmed the initial diagnosis of manifest and concealed His bundle ectopy as the cause of his brady- and tachyarrhythmia.

Ablation of parahissian accessory pathways (APs) is a challenging procedure because of the high risk to provoke "iatrogenic" atrioventricular (AV) nodal block. The feasibility and safety of cryoablation (CA) have been already demonstrated both in patients with AV nodal reentry tachycardia and in those with anteroseptal APs. However, dissociation between anterograde and retrograde conduction after CA has not yet been described. We report two cases of CA of parahissian AP associated with transient dissociation between anterograde and retrograde conduction.

Background: As the technique for radiofrequency catheter ablation for atrial fibrillation (AF) has progressed, so has our knowledge of both normal and abnormal anatomy of the left atrium and pulmonary veins (PV). We treated several AF patients with accessory conduction pathways (ACP) who were also found to have a common ostium of inferior PVs (CIPV), a relatively rare PV anomaly. No relation between ACP and PV anomalies has ever been reported, and the aim of our study was to study this association., Methods and Results: This study included 137 consecutive patients (104 men; mean age, 60±9 years) who underwent AF ablation for paroxysmal and persistent AF at our institution from March 2009 to August 2010. We analyzed coexisting supraventricular tachycardias and left atrium and PV morphology by multidetector row CT. Thirty-eight of 137 patients (27.7%) were found to have some PV anomaly, consisting of 13 with a common trunk of left PV, 19 with right additional PV, 3 with a common trunk of right PV, and 3 with CIPV. Thirty-one patients (22.6%) had supraventricular tachycardias. They were 26 cases of atrial flutter, 4 of Wolff-Parkinson-White syndrome, and 3 of atrioventricular nodal reentrant tachycardia. The prevalence of a coexisting ACP was significantly higher in patients with CIPV than in those without CIPV (3 of 3 [100%] versus 1 in 134 [0.7%]; P<0.0001). All ACPs with CIPV were located in the left side. The other supraventricular tachycardias were not associated with any PV anomalies., Conclusions: There is a possible association between CIPV and left-sided ACP in AF patients. This suggests that there is a likelihood of developmental association between them.

A 75-year-old woman with dextrocardia, situs inversus, and subpulmonic outflow obstruction presented with recurrent supraventricular tachycardia (SVT). This SVT was easily inducible during electrophysiology study, and pacing maneuvers during SVT were consistent with atypical, slow-slow atrioventricular nodal reentrant tachycardia (AVNRT). The His bundle was identified in the low postero-septal morphologic right atrium, at the typical anatomic site for slow pathway ablation of AVNRT. Mapping of the retrograde earliest atrial electrogram during AVNRT localized this site to the mid-septal morphologic left atrium, and cryoablation at this site terminated the AVNRT and rendered it noninducible., (©2010, The Authors. Journal compilation ©2010 Wiley Periodicals, Inc.)

We report a case showing 4 types of aberrant conduction and 2 types of gap phenomena produced by single atrial extrastimulus during sinus rhythm. One of the gap phenomena was associated with dual His bundle pathways, demonstrating a fast-to-slow jump-up phenomenon. Of note, a sharp potential considered as retrograde His bundle activations was recorded, which indicated that the activation wave front descended the slow His bundle pathway and turned up the fast His bundle pathway.

The ventricular conduction system is responsible for rapid propagation of electrical activity to coordinate ventricular contraction. To investigate the role of the transcription factor Nkx2.5 in the morphogenesis of the ventricular conduction system, we crossed Nkx2.5(+/-) mice with Cx40(eGFP/+) mice in which eGFP expression permits visualization of the His-Purkinje conduction system. Major anatomical and functional disturbances were detected in the His-Purkinje system of adult Nkx2.5(+/-)/Cx40(eGFP/+) mice, including hypoplasia of eGFP-positive Purkinje fibers and the disorganization of the Purkinje fiber network in the ventricular apex. Although the action potential properties of the individual eGFP-positive cells were normal, the deficiency of Purkinje fibers in Nkx2.5 haploinsufficient mice was associated with abnormalities of ventricular electrical activation, including slowed and decremented conduction along the left bundle branch. During embryonic development, eGFP expression in the ventricular trabeculae of Nkx2.5(+/-) hearts was qualitatively normal, with a measurable deficiency in eGFP-positive cells being observed only after birth. Chimeric analyses showed that maximal Nkx2.5 levels are required cell-autonomously. Reduced Nkx2.5 levels are associated with a delay in cell cycle withdrawal in surrounding GFP-negative myocytes. Our results suggest that the formation of the peripheral conduction system is time- and dose-dependent on the transcription factor Nkx2.5 that is cell-autonomously required for the postnatal differentiation of Purkinje fibers.

There are very limited data on the effects of bundle branch block (BBB) in patients with atrioventricular nodal reentrant tachycardia (AVNRT). Studies in a total of 155 patients with 162 episodes of AVNRT were retrospectively analyzed. A total of 38 patients (25%) developed spontaneous right BBB, whereas 5 (3%) developed left BBB during tachycardia. Five of the 38 (13%) with right BBB showed near identical prolongation of both the ventriculoatrial (VA) (15 +/- 5 ms; 10 to 23) and His to atrial intervals (HA) (14 +/- 4 ms; 10 to 20) with an identical atrial activation sequence for both right BBB or normal QRS tachycardia complexes. In contrast, all 5 patients with left BBB showed a decrease in the VA (-18 +/- 11 ms; 10 to 36) with unchanged HA comparing left BBB to normal QRS patterns during AVNRT. The magnitude of prolongation of the His to ventricular interval (HV) during left BBB (19 +/- 12 ms; 10 to 40) was nearly identical to the decrease in the VA. In conclusion, prolongation of VA and HA with unchanged HV in patients with AVNRT and right BBB suggests that right BBB is due to a block in the fibers in close proximity to the His recording site. The data suggest that fibers in the His bundle are predestined to activate the right bundle branch, and in AVNRT the lower turnaround point may be within the His bundle.

The congenital form of His bundle tachycardia is an uncommon pediatric arrhythmia. We report the case of a 7-year-old child with tachycardiomyopathy. The incessant arrhythmia, detected in infancy, was resistant to amiodarone and beta-blockers. During electrophysiologic study, the tachycardia converted to sinus rhythm with intravenous adenosine and diltiazem. Subsequently, the child is maintaining sinus rhythm on oral verapamil. Calcium-channel blockers should be considered for the treatment of this arrhythmia, which is often resistant to multiple antiarrhythmic drugs.

Automatic junctional tachycardias are rare supraventricular tachyarrhythmias observed mainly in infants and children. This arrhythmia has a high mortality rate. We report the case of a 22-month-old infant in which the electrophysiological study disclosed an incessant junctional tachycardia originating from an automatic focus located in the His bundle region. Radiofrequency pulse delivery to the His bundle with controlled progressive heating of this region resulted in a definitive ablation of the ectopic focus without complications.

We examined the pathology findings of the cardiac conduction system in 42 human autopsies who were considered to have died an accidental death. Abnormalities in serial sections of the conduction system were found in seven and of these three had an accessory pathway in the conduction system. In one of these there was mild Ebstein's anomaly (ME), two had fibromuscular dysplasia of the atrioventricular node artery with mitral valve prolapse, and two others had abnormal routes showing fragmentation of the bundle of His with ME. A full investigation of the cardiac conduction system can therefore be useful for determining the precise cause in cases of accidental death.

Junctional ectopy or tachycardia have been mapped and ablated in the atrioventricular node or its posterior extensions. There is no report of Hisian ectopy convincingly demonstrated and then successfully treated by ablation. A 39-year-old man presented with a 9-year history of recurrent supraventricular ectopy refractory to medical treatment. Intracardiac mapping revealed that the ectopy originated from the His bundle, which was indicated by a reversed His bundle activation sequence during ectopy compared to that during sinus rhythm, while the right bundle branch potential was anterogradely activated. Low power RF energy delivered to the site of earliest activation completely abolished the ectopic activity without modification of Hisian or atrioventricular conduction. After a 12-month followup, the patient remains free of recurrence.

We report the case of a patient with bundle branch reentrant ventricular tachycardia having two distinct components of the QRS complex due to simultaneous dual transseptal conduction. The macroreentrant circuit consisted of anterograde conduction down the left bundle branch and retrograde conduction up the right bundle branch. Extensively injured myocardium in the interventricular septum isolated the two separate transseptal passages, which exhibited a "fast" and "slow" conduction property, respectively. The QRS configuration resulted from a wavefront propagating from the left bundle branch via "fast" transseptal conduction, followed by another more delayed wavefront propagating via "slow" transseptal conduction, which played an important role in determining this unique QRS morphology.

Changes in the retrograde conduction time (ventriculoatrial [VA]) interval during functional bundle branch block (BBB) have been used to separate septal from free wall accessory pathways (APs), but different values of the VA interval prolongation (deltaVA) have been described in different reports. A total of 95 patients with single nondecremental APs who developed BBB during atrioventricular reentrant tachycardia were studied. Free wall APs were found in 60 patients, and 35 had septal APs. For patients with free wall APs, complete and incomplete BBB ipsilateral to the atrial insertion site of APs were observed in 39 of 60 patients (65%) and 31 of 60 patients (52%), respectively. For patients who had both complete (QRS > or = 120 ms) and incomplete (QRS <120 ms) BBB during atrioventricular reentrant tachycardia, deltaVA for patients with complete BBB was significantly greater than in those with incomplete BBB, 59 +/- 19 ms versus 30 +/- 11 ms, p <0.001. For patients with septal APs and complete and incomplete BBB during tachycardia, the mean deltaVA for those with complete BBB was 31 +/- 20 ms and was significantly longer than in patients with incomplete BBB (14 +/- 6 ms), p <0.001. There was no significant difference in deltaVA between those with free wall APs and incomplete BBB compared with those with septal APs and complete BBB. The criteria of QRS > or = 120 ms associated with deltaVA > or =40 ms served to best separate free wall from septal APs with a sensitivity of 88% and a specificity of 89%. Left anterior fascicular block was associated with marked lengthening of deltaVA for those with left free wall APs, whereas a left posterior fascicular block pattern resulted in a marked increase in the deltaVA for patients with posteroseptal APs. In the absence of fascicular block patterns, a deltaVA > or =40 ms provides strong evidence of a free wall AP, with a sensitivity of 95% and a specificity of 100%. The left posterior fascicle appears to provide predominant innervation of the posterior septum.

Incessant supraventricular tachyarrhythmias are known to result in myocardial dysfunction indistinguishable from idiopathic dilated cardiomyopathy by current testing methods. This tachycardia-induced cardiomyopathy (TICM), however, is uniquely reversible with adequate rhythm control. Two dogs were presented to The Ohio State University for incessant supraventricular tachycardia (SVT) and echocardiographic signs of dilated cardiomyopathy, later proven to be TICM. A 3rd dog presented for frequent paroxysms of SVT and syncope had echocardiographic signs of mild myocardial systolic dysfunction. All 3 dogs had inadequate rhythm control with multiple antiarrhythmic agents, and 1 dog suffered from recurrent left-sided congestive heart failure. Generalized cardiomegaly was found in 1 dog and left-sided dilatation without concurrent right-sided enlargement in 1 dog. Mild-to-severe left ventricular systolic dysfunction was confirmed echocardiographically in all dogs. A total of 4 atrioventricular accessory pathways (APs) were found during invasive electrophysiologic studies in these 3 dogs. All APs were successfully ablated with radiofrequency energy delivered through a thermistor-tipped catheter. Elimination of AP conduction, and thus orthodromic atrioventricular reciprocating tachycardia, resulted in resolution of all clinical and echocardiographic evidence of TICM in these dogs. This result confirms that the cardiomyopathy was, in fact, reversible TICM. All cardiovascular medications were discontinued, and no complications occurred during a 15-25-month follow-up period.

Introduction: Despite the ability to cure atrioventricular nodal reentrant tachycardia (AVNRT) by radiofrequency catheter ablation with a high success rate, the exact localization of the tachycardia circuit is still not well established. The presence of AV nodal tissue between the typical AVNRT circuit and the His bundle, constituting a lower common pathway (LCP), remains controversial., Methods and Results: Entrainment of AVNRT during para-Hisian stimulation allows accurate measurement of the His- to- atrial (HA) interval which is part of the same circuit as that of the tachycardia. With an LCP, during tachycardia, there is simultaneous conduction from the low turnaround of the circuit to the atrium (via the fast pathway) and to the His bundle (via the LCP). However, during entrainment by para-Hisian pacing, the impulse has to retrogradely depolarize sequentially the LCP and the fast pathway. Therefore, in the presence of an LCP, the HA interval duration during tachycardia (HAt) should be shorter than that of during entrainment by para-Hisian stimulation (HAe). We considered an LCP present when Hae - HAt was > or = 10 msec. Entrainment of typical AVNRT with para-Hisian stimulation was performed in 23 consecutive patients (21 females) with a mean age of 45+/-17 years. LCP was considered to be present in 18 of 23 patients (78%). In addition, transient His-bundle dissociation from the ongoing tachycardia occurred in seven patients (30%)., Conclusion: These results support the presence of a LCP during typical AVNRT.

This article reports a case of sudden infant death syndrome in a 1-month-old male. Some important abnormalities of the cardiac conduction system were found, consisting of persistent fetal dispersion of the atrioventricular node and His bundle, accessory atrioventricular pathways of Mahaim type and resorptive degeneration. These changes could be considered as a morphological substrate for cardiac arrhythmias.

We report an unusual case of a 17-year-old girl who died in an filled bathtub. There were at least two attacks of unconsciousness in her premortem history. In April 1994, electrocardiography had shown an incomplete right bundle branch block. At autopsy, unspecific signs of drowning and hypoplasia of the terminal part of the atrioventricular bundle of His and the right bundle branch within the cardiac conduction system were found. There were no other obvious autopsy findings related to the sudden death of this girl. In addition, toxicologic examination for illegal drugs and alcohol was completely negative. We conclude that this might be a rare case of death because of acute decompensation of a partial hypoplastic cardiac conduction system with loss of consciousness and consequent drowning in the bathtub. This case illustrates the value of an extensive pathologic examination of the cardiac conduction system in unexpected death of young people.

The aim of this study was to search for a cardiac cause of death after an otherwise negative medico-legal autopsy in 1,000 adults under 65 years of age with no previous history, who suffered a sudden death. Four hundred and seven coronary causes were established with 152 negative autopsies and 441 other cardiac lesions. These 441 cases comprised 193 cardiomyopathies, 106 His bundle lesions, 50 partial forms of Uhl's anomaly (or arrhythmogenic right ventricular dysplasia), 14 isolated mitral valve prolapses, 22 chronic valvular lesions, 8 myocarditis, 16 pulmonary embolisms, 11 chronic cor pulmonale, 11 aortic dissections and 10 atrophic fibrous plaques of the left ventricle, sequellae of thoracic trauma. His bundle lesions (isolated and associated) were observed 299 times out of 1,000 cases and mitral valve prolapse 125 times. Age and gender, circumstances of sudden death, the weight of the heart, are reported for each group of conditions. The frequency of associations, especially of His bundle lesions, suggests that they may have a potentialising effect on the genesis of the fatal arrhythmia.

A 24-year-old black man suddenly collapsed while playing basketball. He died despite prompt cardiopulmonary resuscitation. The only abnormality found at autopsy was a vascular anomaly confined to the atrioventricular bundle of His within the cardiac conduction system. This arborizing collection of abnormal and increased number of blood vessels lacked both elastic fibers and smooth muscle, both of which were replaced by collagen. The number of vessels in the decedent's bundle of His was quantitatively compared with 117 normal controls by using a point-counting method. The percentage of points landing on vessels per total points counted (% VP/TP) in this man was 31% compared with 5.6 +/- 2.7% SD in the controls. No vascular malformations were seen elsewhere. This lesion has not been previously reported in the medical literature. This case confirmed the occasional usefulness of sampling the cardiac conduction system in sudden death with no obvious autopsy findings.

Two patients are described with antidromic reentry tachycardia successfully treated by interruption of an anterior septal accessory atrioventricular node and bundle. This anomalous connection resembles an atrioventricular conduction sling seen in complex congenital heart malformations. It has atrioventricular node-like properties, is located in the anterior septal area, will only conduct antegrade, and has an insulated connection to the right bundle branch. Rather than nodoventricular, nodofascicular, atriofascicular, or Mahaim, a more appropriate label for the connection is accessory atrioventricular node and bundle.

We report the findings in a patient with Klippel-Feil syndrome and associated agenesis of right upper and middle lobes, hypoplasia of the right lower lobe of the lung, and Lown-Ganong-Levine syndrome. To our knowledge, such an association has not been previously described.

The introduction of transcatheter ablation techniques has opened a new era of the management of tachyarrhythmias. The rationale of these therapeutic procedures lies in the induction of an irreversible and limited damage to cardiac tissue involved in arrhythmias. In its original form, the technique involves the delivery of high energy DC discharges (fulguration) to the target area through a temporary catheter electrode. A primary drawback of fulguration, when used for ablation of AV node-His bundle, is the induction of a pacemaker-dependent state; the barotraumatic effect that results from high energy impulses constitutes the major limitation in the case of ablation of accessory pathways or arrhythmogenic areas. Recently, the efficiency of the procedure has been substantially improved by the use of low-power, high-frequency alternating current (radiofrequency), that allows to deliver graded amounts of disrupting energy to selected areas while avoiding undesirable biophysical effects to the surrounding tissue. Remarkable results, with a success rate close to 100%, are reported with radiofrequency ablation of AV node reentry tachycardia, as well as reciprocating tachycardia associated with the preexcitation syndrome, so that this technique can be considered the procedure of choice for both categories of patients. Catheter modification of AV conduction provides a therapeutic tool for treating atrial tachyarrhythmias with rapid ventricular responses. In such cases, however, the risk of AV block with the need for pacemaker implantation must be taken into account. Results of catheter ablation of ventricular tachycardia have been inconstant and generally disappointing, except for ventricular tachycardias due to reentry in the His-Purkinje system, for which a high success rate is reported. Experience with radiofrequency ablation in ventricular tachycardia is very limited, but localization of a critical segment of reentry could improve the efficiency of this technique.

A heart is described with congenitally corrected transposition of the great arteries, normally positioned atria, and a straddling mitral valve. Instead of an anteriorly positioned atrioventricular node, a regularly positioned posterior node and bundle were observed. This observation has important clinical and surgical implications and underlines that close inspection of the alignment of atrial and ventricular septa during operations is mandatory.

A histological study by the method of seriated sections in one case of "double-outlet left ventricle" made it possible to confirm and to describe the embryology of the nodal-His junction: not only the atrio-ventricular node (AV N) but also the His bundle (H) have a double origin, atrial and ventricular. Fusion of these two buds, inexistant in the "lone ventricle" is certainly imperfect. Two macroscopically normal hearts provided examples of nodal buds in one case, of a persisting cleft between the two His buds in the other.

A stillborn baby girl was found to have an anomalous muscle bundle of the right ventricle, associated with a doubly committed subarterial ventricular septal defect. The latter was separated from the area of the atrioventricular conduction bundle by muscle. Serial histologic sectioning of the specialized atrioventricular junctional area revealed that the final bifurcation of the branching bundle came astride the septum in the inferior angle of the defect. The right bundle branch bifurcated and one of its branches continued along the anomalous muscle bundle in subendocardial position. This anatomy supports the concept that the bundle, in this heart, represented an early takeoff of the moderator band. The surgical anatomy suggests that resection of the anomalous muscle bundle and closure of the ventricular septal defect might well have produced heart block. The generally accepted rule that resection of anomalous muscle bundles in the right ventricle is surgically safe should be reconsidered.

A patient is presented who had two paroxysmal supraventricular tachycardias, one slow and incessant and the other fast. Both paroxysmal tachycardias appeared to be atrioventricular (A-V) reentrant, with anterograde conduction by way of a normal A-V pathway. Two pathways conducting in retrograde manner were demonstrated, characterized by different conduction times (fast and slow), identical abnormal atrial activation sequence and A-V nodal-like properties (retrograde Wenckebach periodicity with rapid ventricular pacing, and depression with ouabain and propranolol). Thus, there appeared to be two anomalous A-V bundles with nodal-like properties conducting in retrograde fashion. Whether the paroxysmal tachycardia was fast or slow depended on which of these pathways was utilized. Spontaneous cure of incessant paroxysmal tachycardia was observed and coincided with unexplained total loss of ability for ventriculoatrial conduction.

A familial survey demonstrated mendelian inheritance in three large kindreds with conduction abnormalities and heart block. The trait was autosomal dominant, with varying expressivity and penetrance, apparent male preponderance, and congenital onset. Manifestations included right bundle branch block, left axis deviation, and right bundle branch block associated with left axis deviation. Complete heart block proved almost always to be a late event, and developed in all documented cases from bilateral bundle branch block. An r' pattern, most likely representing a right ventricular conduction delay, is discussed.

Syncope and sudden death occurs in certain purebred Pug dogs which have been found to have intermittent sinus pauses and paroxysmal second degree heart block on electrocardiographic (ECG) study. We have established a colony of such dogs to study this problem and here report the results of histological examination of the cardiac conduction system in twenty-one of them. These include a dam which may be considered the proband, three of her offspring (two littermates) and three fetal pups in an unborn litter of one of these; three of the four adult dogs died suddenly and unexpectedly. Two groups of puppies descendants of these lines were also studied after they all died within three days of birth. The first group of puppies (8) did not have suitable ECG studies but the second group (6) did and showed ECG changes similar to those in the adult dogs. In all 21 dogs the sinus node was anatomically normal, but in every one there was significant stenosis of the midportion of the His bundle. There was no abnormality of the atrioventricular (A-V) node or of the bundle branches, and the cardiac valves and coronary arteries were normal. Two pups had ventricular septal defects and one of these had an atrial defect as well. All the dogs were of the normal fawn color, had a normal physical appearance and no visible evidence of associated extracardiac abnormalities. The adult dogs were able to hear normally but the puppies died too early to test their hearing. The paroxysmal heart block may in part be attributable to the abnormality in the His bundle, but other factors possibly leading to heart block and long sinus pauses are discussed. Stenosis of the midportion of the His bundle appears to be a heritable trait in these purebred Pug dogs.