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We evaluated the incidence of atrial fibrillation in 189 patients (92 males, 97 females, mean age 75 +/- 12 yrs, range 41-100 yrs) with pacemaker, during a mean follow-up of 5.5 yrs (range 1-24 yrs). The indications for implant were: complete AV block (115 pts), second degree Möbitz 2 AV block (51 pts), bifascicular block (5 pts), sick sinus syndrome (14 pts), symptomatic bradycardia (4 pts). The mode of stimulation considered were VVI (105 pt), VVI rate responsive (21 pts), single lead VDD (43 pts), DDD (20 pts). The occurrence of retrograde VA conduction in patients with VVI or VVI rate responsive pacing was also evaluated. Atrial fibrillation occurred in 40 pts (21%). The highest incidence was evidenced in patients with sick sinus syndrome (9 pts, 64%), and in patients with VVI stimulation (28 pts, 27%). On the contrary, the lowest incidence was found in single lead VDD stimulation (4 pts, 9%). The patients with dual chamber pacing showed a relatively high incidence of the arrhythmia (5 pts, 25%). Atrial fibrillation occurred in 9 out of 32 patients with retrograde VA conduction, and in 22 out of 94 patients without retrograde conduction (28% versus 23%, p = NS). In conclusion, it is confirmed that patients with sick sinus syndrome are at high risk for atrial fibrillation. Single lead VDD stimulation seems to be the better mode of pacing in preventing atrial fibrillation, while dual chamber pacing showed minor efficacy. The presence of retrograde VA conduction could not predict the occurrence of the arrhythmia.

The purpose of this study was to define the history and prognosis of 12 patients (8 males, 4 females) with syncope of unknown origin (5 to 15 episodes), who developed prolonged asystole or complete AV block during the upright tilt test (UTT). The mean age (+/- SD) of the patients was 29 +/- 7.4 years, and all had normal neurological and cardiological findings on evaluation. These patients were selected from a larger group of 92 cases with positive UTT out of a total of 136 subjects who were referred for recurrence of syncope. Neither clinical nor autonomic nervous system evaluation distinguished these 12 patients from those with positive UTT. Following UTT, therapy was initiated and consisted of transdermal scopolamine in four, disopyramide in two, and beta-blockers in four patients. During follow-up (mean, 17 +/- 5.4 months), four patients had recurrences but none experienced episodes of life-threatening syncope. These patients do not show an enhanced risk of sudden death, and drug therapy seems to improve their clinical course. Only long-term follow-up would correctly identify a subgroup at higher risk.

A 43-year-old woman with progressive external ophthalmoplegia developed a bifascicular block and dilatation of the right ventricle during 4 years of follow-up. Histochemical and electron microscopy studies detected mitochondrial abnormalities in ocular, skeletal muscle and cardiac biopsies. This case registers disease progression from the external ocular to the skeletal and cardiac muscles. Mitochondrial DNA was deleted in relation to the morphological abnormality.

Twenty six patients (aged 46-80, mean age 64) with bifascicular block in the presence of prolonged H-V interval (trifascicular block), were followed for an average of 31 months after inserting an R-wave inhibited pacemaker (PM) because of syncope and/or dizzy attacks. The underlying rhythm was evaluated at 4-6-month intervals by three different techniques: 1) 12-lead ECG when intrinsic patient rate was faster than PM rate; 2) abrupt PM inhibition (APMI) by the rapid chest-wall stimulation technique, and 3) progressive PM inhibition (PPMI) using a programmed chest-wall stimulation technique capable of decreasing the PM rate gradually to 30 beats/min before complete PM inhibition. In addition, the PPMI allowed the underlying rhythm to be induced and sustained and properly evaluated without any discomfort to the patient. Following PM insertion, 4 patients (15%) developed complete heart block after a mean follow-up of 43 months, and one patient (4%) developed 2nd degree 2:1 A-V block (VX) after 83 months. The P-R interval increased in 5 patients (19%) and decreased in 2 (8%). No change of A-V conduction was found in 9 patients (34%). Three patients developed low atrial rhythm, atrial flutter and atrial fibrillation, respectively (12%). After PM insertion 2 patients still complained of dizziness. None reported syncope. Two patients died during follow-up, both of congestive heart failure (8%). By detection of intrinsic rhythm it was recognized that a long symptomatic paroxysmal phase may precede the development of chronic complete A-V block. Therefore, the insertion of a permanent PM is recommended in patients with unexplained neurologic symptoms and trifascicular disease, without waiting for documented episodes of complete A-V block.

We analyzed the VCGs of 100 young subjects without apparent cardiac disease showing an ECG superior Axis Deviation, i.e. AQRS greater than - 30 degrees, and a rS aspect in V1. Our findings demonstrated that, in these subjects, the superior axis deviation is due to a distal right bundle branch block with posterior displacement of the terminal forces in 78% of the cases. The other cases being left anterior hemiblocks, either isolated (12%) or associated with a block of the anterior subdivision of the right bundle branch (10%). The differential diagnosis, easy on VCG, is sometimes very difficult on ECG. The most reliable ECG criteria to discriminate this kind of right bundle branch block from left anterior hemiblock are: intrinsic deflection in a VL-V6 greater than or equal to 0.015" (sensitivity 100%; specificity 57%), RV6/Ra VL ratio greater than 1 (sensibility 100%; specificity 50%) and the presence of a notched R wave in L2, L3 and a VF (sensitivity 90%; specificity 100%).

Thirteen non-consecutive patients, aging 7 to 61 (average 27) years, underwent left ventricular myotomy-myectomy for a severely symptomatic idiopathic hypertrophic subaortic stenosis (IHSS). In all patients the resting ECG before surgery showed P-R less than 0.18 sec, QRS duration less than 0.11 sec, QRS axis ranging from +10 to +80 degrees. In the immediate post-surgical period 3 patients has complete heart block and 1 had 2nd degree type 2 atrio ventricular block. Lesion was infra-Hisian in 3 patients and intra-Hisian in 1 patient. In the remaining 9 patients an immediate post-surgical left bundle branch block appeared; in 3 out of these patients ECG and an electrophysiologic study documented severe infra-Hisian conduction impairments after an average period of 4 years from surgery. During follow-up 3 patients died suddenly.

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.

The rate of av conduction disturbances in corrected transposition of the great arteries is high, possibly as consequence of mechanical injury by ventricular output along with the mis-positioned specialized av junction. A sudden death occurred in a 54 year old female affected by a chronic "idiopathic" complete av block, who showed at autopsy a heart with av and ventricular arterial discordance in the absence of other anomalies (isolated corrected transposition). The cardiac arrest had the ecg features of ventricular fibrillation, preceded by sustained ventricular tachycardia. Histologic study of the conduction system by serial sections confirmed the anterior position of the connected av node, with a long subendocardial course of the bundle in the pulmonary outflow tract. The histologic basis of the block consisted of sclero-atrophy of the common bundle, quite similar to the substrate of acquired av block in heart with normal connections. Possible etiologies, responsible of the relation between the av block and onset of ventricular arrhythmias, are discussed.