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Background: Previous studies proposed as one of the main mechanisms involved in neurally mediated syncope, the stimulation of ventricular mechanoreceptors as the final trigger for vagal discharge., Objectives: This study aimed to verify the presence of a sympathetic driven increase of cardiac contractility before vasovagal syncope., Methods: We studied 23 patients with recurrent syncope. All underwent a 60 ° tilt with pharmacologic challenge (sublingual spray nitrate). Two conditions were used to assess autonomic activity by heart rate variability analysis: in a supine position after 5 min of rest and after 15 min of tilt. Simultaneously, cardiac contractility was quantified by tissue-Doppler echocardiography at the base of the free walls of left ventricle. The peak myocardial velocities during systole (Sw) and late diastole (Aw) were considered., Results: Passive tilt induced a significant increase of the low frequency component (LF) as well as a decrease of the high frequency component (HF) in positive patients (LF: from 49 ± 18 to 65 ± 18 nu, p<0.05; HF: from 41 ± 21 to 26 ± 16 nu, p<0.05). Tissue-Doppler showed a similar increase in Sw in both positive and negative patients but showed a significant decrease of Aw in syncopal subjects (p<0.005)., Conclusions: Our results do not show an increase in ventricular contractility before tilt-induced syncope, or in presence of a valuable increase of sympathetic activity. Instead, we observe a reduction of atrial contractility, which may be a contributory component in the pathogenesis of vasovagal syncope., (Copyright © 2008 Elsevier Ireland Ltd. All rights reserved.)

Cardiovascular remodelling in the conditioned athlete is frequently associated with physiological ECG changes. Abnormalities, however, may be detected which represent expression of an underlying heart disease that puts the athlete at risk of arrhythmic cardiac arrest during sports. It is mandatory that ECG changes resulting from intensive physical training are distinguished from abnormalities which reflect a potential cardiac pathology. The present article represents the consensus statement of an international panel of cardiologists and sports medical physicians with expertise in the fields of electrocardiography, imaging, inherited cardiovascular disease, cardiovascular pathology, and management of young competitive athletes. The document provides cardiologists and sports medical physicians with a modern approach to correct interpretation of 12-lead ECG in the athlete and emerging understanding of incomplete penetrance of inherited cardiovascular disease. When the ECG of an athlete is examined, the main objective is to distinguish between physiological patterns that should cause no alarm and those that require action and/or additional testing to exclude (or confirm) the suspicion of an underlying cardiovascular condition carrying the risk of sudden death during sports. The aim of the present position paper is to provide a framework for this distinction. For every ECG abnormality, the document focuses on the ensuing clinical work-up required for differential diagnosis and clinical assessment. When appropriate the referral options for risk stratification and cardiovascular management of the athlete are briefly addressed.

A 63-year-old man presented with recurrent syncopal attacks associated with swallowing and right-sided throat pain. Immediately after admission, he presented a 16-s asystolia. The patient's clinical history was unremarkable except for previous postimplant periodontitis. Several episodes of severe bradycardia and sinus pauses, always associated with painful deglutition, were recorded subsequently. X-ray orthopanthomography and magnetic resonance imaging of the neck confirmed several areas of periodontitis around the previous dental implants and right mastoid inflammation. A barium swallow and fibre-optic endoscopy also revealed a small sliding hiatus hernia and distal chronic oesophageal inflammation. Despite complete dental curettage, antibiotics and antigastro-oesophageal reflux therapy, only partial relief of the pain and incomplete resolution of the arrhythmic disorder were obtained after 3 weeks, and the patient underwent pacemaker implantation. At 1-month follow-up, however, he reported the complete relief of the throat pain; subsequent Holter monitoring showed normal sinus rhythm, without pacemaker-induced electrical activity.

Objectives: We tested whether 3-dimensional electroanatomical voltage mapping (EVM) may help in the differential diagnosis between idiopathic right ventricular outflow tract (RVOT) tachycardia and arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D)., Background: Right ventricular EVM has been demonstrated to reliably identify low-voltage regions ("electroanatomical scar"), which in patients with ARVC/D correspond to areas of fibrofatty myocardial replacement., Methods: The study population comprised 27 patients (15 men and 12 women, age 33.9 +/- 8 years) with RVOT tachycardia and no echocardiographic/angiographic evidence of right ventricular (RV) dilation/dysfunction, who underwent EVM and endomyocardial biopsy (EMB) for characterization of ventricular tachycardia (VT) substrate before catheter ablation., Results: Electroanatomical voltage mapping was normal in 20 of 27 patients (74%, group A), with electrogram voltage >1.5 mV throughout the RV. The other 7 patients (26%, group B) showed >/=1 (1.4 +/- 07) RV electroanatomical scar area(s) (bipolar voltage <0.5 mV) that correlated with fibrofatty myocardial replacement at EMB (p < 0.001). Clinical predictors of RV scar were right precordial QRS prolongation (p < 0.001) and VT inducibility (p = 0.001). Catheter ablation successfully eliminated VT in 18 of 20 patients (90%). During a follow-up of 41 +/- 8 months, 3 of 7 patients (43%) from group B received an implantable defibrillator because of life-threatening ventricular arrhythmias, compared with no patients from group A (p = 0.016)., Conclusions: An early/minor form of ARVC/D may mimic idiopathic RVOT tachycardia. Electroanatomical voltage mapping is able to identify RVOT tachycardia due to concealed ARVC/D by detecting RVOT electroanatomical scars that correlate with fibrofatty myocardial replacement at EMB and predispose to sudden arrhythmic death.

Background: Vasovagal syncope (VVS) results from a complex interaction among afferent vagal and sympathetic signals, cortical modulation and bulbar integration. The aim of our study was to evaluate the modifications of autonomic activity during Upright Tilt Test (UTT) in patients with unexplained syncope, and to correlate these changes with the specific cardiovascular reactions induced., Methods and Results: We studied 90 patients with a mean age of 44+/-17 yrs. Frequency domain analysis of heart rate variability (HRV) (normalized units) was performed on 2 periods of 300 beats: at baseline and after 5 min of 60 degrees tilt. UTT was positive in 56 patients (62%). The responses were cardioinhibitory in 8, vasodepressive in 15, mixed in 33. Baseline LF and HF components did not show significant difference between subjects with positive or negative test (HF: 39+/-21 versus 41+/-22; LF: 50+/-22 versus 49+/-23). HRV during UTT showed similar changes in patients with positive or negative test. However, subjects with mixed or cardioinhibitory reactions were characterized by a relevant increase of LF during UTT (from 47+/-23 to 66+/-21), whereas the others by a non-significant decrease of the same component (from 57+/-19 to 51+/-31)., Conclusions: Patients developing a reflex cardioinhibitory reaction during UTT were characterized by an increase of sympathetic activity during the test, that might represent an essential factor to induce a stronger vagal reaction on the sinus node. On the contrary, in subjects with vasodepressive reactions an inadequate enhancement of the sympathetic drive, probably causing a failure of peripheral vasoconstriction, was evidenced.

Context: Recently, the implantable cardioverter-defibrillator (ICD) has been promoted for prevention of sudden death in hypertrophic cardiomyopathy (HCM). However, the effectiveness and appropriate selection of patients for this therapy is incompletely resolved., Objective: To study the relationship between clinical risk profile and incidence and efficacy of ICD intervention in HCM., Design, Setting, and Patients: Multicenter registry study of ICDs implanted between 1986 and 2003 in 506 unrelated patients with HCM. Patients were judged to be at high risk for sudden death; had received ICDs; underwent evaluation at 42 referral and nonreferral institutions in the United States, Europe, and Australia; and had a mean follow-up of 3.7 (SD, 2.8) years. Measured risk factors for sudden death included family history of sudden death, massive left ventricular hypertrophy, nonsustained ventricular tachycardia on Holter monitoring, and unexplained prior syncope., Main Outcome Measure: Appropriate ICD intervention terminating ventricular tachycardia or fibrillation., Results: The 506 patients were predominately young (mean age, 42 [SD, 17] years) at implantation, and most (439 [87%]) had no or only mildly limiting symptoms. ICD interventions appropriately terminated ventricular tachycardia/fibrillation in 103 patients (20%). Intervention rates were 10.6% per year for secondary prevention after cardiac arrest (5-year cumulative probability, 39% [SD, 5%]), and 3.6% per year for primary prevention (5-year probability, 17% [SD, 2%]). Time to first appropriate discharge was up to 10 years, with a 27% (SD, 7%) probability 5 years or more after implantation. For primary prevention, 18 of the 51 patients with appropriate ICD interventions (35%) had undergone implantation for only a single risk factor; likelihood of appropriate discharge was similar in patients with 1, 2, or 3 or more risk markers (3.83, 2.65, and 4.82 per 100 person-years, respectively; P = .77). The single sudden death due to an arrhythmia (in the absence of advanced heart failure) resulted from ICD malfunction. ICD complications included inappropriate shocks in 136 patients (27%)., Conclusions: In a high-risk HCM cohort, ICD interventions for life-threatening ventricular tachyarrhythmias were frequent and highly effective in restoring normal rhythm. An important proportion of ICD discharges occurred in primary prevention patients who had undergone implantation for a single risk factor. Therefore, a single marker of high risk for sudden death may be sufficient to justify consideration for prophylactic defibrillator implantation in selected patients with HCM.

One of the most important mechanisms proposed in the pathogenesis of neurally mediated syncope considers the stimulation of myocardial mechanoreceptors as the final trigger leading to reflex hypotension and bradycardia. The aim of our study was to analyze the modifications of systolic and diastolic velocities of the left ventricle by tissue Doppler echocardiography, during the upright tilt test, to confirm the presence of an increased ventricular contractility before syncope. We evaluated 47 patients (mean age 43 years) with unexplained syncope and 13 healthy subjects. Three echocardiographic recordings were collected: at baseline, during the initial 5 minutes of the test, and after 15 minutes of tilt. The upright tilt test was positive in 28 patients. During the test, all of them had a similar, significant, progressive reduction of left ventricular end-diastolic volume, left atrial area, and left atrial volume. In contrast, the left ventricular ejection fraction showed an early, more pronounced decrease in those with positive tests. By tissue Doppler echocardiography, the systolic waves remained almost unchanged, and the early filling waves decreased similarly in those with positive or negative findings and the controls. In contrast, the atrial filling waves showed a significant decrease only in patients with positive tests. In conclusion, our study did not find increased ventricular contractility before syncope. In contrast, a decreased atrial contribution to ventricular filling characterized patients with positive upright tilt test results, which, we postulate, may be an important contributory factor to vasovagal syncope.

In patients affected by isolated atrial fibrillation, epicardial pulmonary veins ablation can be performed with minimally invasive robotic-guided cardiac surgery techniques. This approach might become a feasible alternative to percutaneous transcatheter procedures. In this case report, we present a totally endoscopic robotic-guided pulmonary veins microwave ablation, on beating heart. A 64-year old male patient affected by paroxysmal atrial fibrillation was scheduled for an epicardial ablation procedure. Through three 1 cm-length port accesses, the "da Vinci" robotic system's camera and arms were introduced in the patient's chest. The pericardial reflections along the superior and inferior vena cava, as well as the transverse sinus, were dissected. Through an additional 0.5 cm-length port, a guidewire was advanced gradually across the transverse sinus, the diaphragmatic surface of the heart and the oblique sinus, finally surfacing outside the thoracic wall through the same port. Once tied to the microwave probe, the guidewire was pulled out carrying the probe inside the chest up to encircle the pulmonary veins. Once in place, a box lesion of the pulmonary veins was produced by microwave. At the 3-month follow up the patient is in sinus rhythm and so far did not longer experienced paroxysmal arrhythmic episodes.

Introduction: Intraoperative left atrial radiofrequency (RF) ablation recently has been suggested as an effective surgical treatment for atrial fibrillation (AF). The aim of this study was to verify the outcome of this technique in a controlled multicenter trial., Methods and Results: One hundred three consecutive patients (39 men and 65 women; age 62 +/- 11 years) affected by AF underwent cardiac surgery and RF ablation in the left atrium (RF group). The control group consisted of 27 patients (6 men and 21 women; age 64 +/- 7 years) with AF who underwent cardiac surgery during the same period and refused RF ablation. Mitral valve disease was present in 89 (86%) and 25 (92%) patients, respectively (P = NS). RF endocardial ablation was performed in order to obtain isolation of both right and left pulmonary veins, a lesion connecting the previous lines, and a lesion connecting the line encircling the left veins to the mitral annulus. Upon discharge from the hospital, sinus rhythm was present in 65 patients (63%) versus 5 patients (18%) in the control group (P < 0.0001). Mean time of cardiopulmonary bypass was longer in the RF group (148 +/- 50 min vs 117 +/- 30 min, P = 0.013). The complication rate was similar in both groups, but RF ablation-related complications occurred in 4 RF group patients (3.9%). After a mean follow-up of 12.5 +/- 5 months (range 4-24), 83 (81%) of 102 RF group patients were in stable sinus rhythm versus 3 (11%) of 27 in the control group (P < 0.0001). The success rate was similar among the four surgical centers. Atrial contraction was present in 66 (79.5%) of 83 patients in the RF group in sinus rhythm., Conclusion: Endocardial RF left atrial compartmentalization during cardiac surgery is effective in restoring sinus rhythm in many patients. This technique is easy to perform and reproducible. Rare RF ablation-related complications can occur. During follow-up, sinus rhythm persistence is good, and biatrial contraction is preserved in most patients.

The aim of the present study was to identify the way the occupational injuries with biological risk can happen in order to find the right measures of prevention and protection and to periodically verify their efficacy. The study started in January 1996 and ended in December 2001 and included 2750 subjects working in the sanitary field. A module (module A), was created to collect all the accident data. For every occupational injury with biological risk, a specific procedure is activated, including: Emergency Room, Sanitary Direction, Infective Diseases Division, Competent Physician. The injuries were classified, according to: sex, age, work qualify, division, object causing injury, modality, anatomical location, type of injury, working turn, and time phase. Most of the division underlined a higher risk the procedures that expose the subjects to accidental punctures with infected needle, such as injection therapy, blood tests, butterfly insertion and removal, etc. Old and bad attitudes, such as needle recapping are still used, even if the law is clear. None occupational injury showed serum conversion. It could be a consequence of the post exposition prophylaxis performed by the infectivologists and of the HBV prophylaxis.

The identification of subjects with arrhythmogenic right ventricular cardiomyopathy (ARVC) at higher risk for sudden death is an unresolved issue. An influence of the autonomic activity on the genesis of ventricular arrhythmias was postulated. Heart rate variability (HRV) analysis provides a useful method to measure autonomic activity, and is a predictor of increased risk of death after myocardial infarction. For these reasons, the aim of the study was to evaluate HRV and its correlations with ventricular arrhythmias, heart function, and prognostic outcome in patients with ARVC. The study included 46 patients with ARVC who were not taking antiarrhythmic medications. The diagnosis was made by ECG, echocardiography, angiography, and endomyocardial biopsy. Exercise stress test and Holter monitoring were obtained in all patients. Time-domain analysis of HRV was expressed as the standard deviation of all normal to normal NN intervals (SDNN) detected during 24-hour Holter monitoring. Thirty healthy subjects represented a control group for HRV analysis. The mean follow-up was 10.8 +/- 1.86 years. SDNN was reduced in patients with ARVC in comparison with the control group (151 +/- 36 vs 176 +/- 34, P = 0.00042). Moreover, there was a significant correlation of this index with the age of the patients (r = - 0.59, P < 0.001), with the left (r = 0.44, P = 0.002) and right (r = 0.47, P = 0.001) ventricle ejection fraction, with the right ventricular end diastolic volume (r = - 0.62, P < 0.001), and with the ventricular arrhythmias, detected during the same Holter record used for HRV analysis (patients with isolated ventricular ectopic beats < 1,000/24 hours, 184 +/- 34; patients with isolated ventricular ectopic beats > 1,000/24 hours and/or couplets, 156 +/- 25; patients with repetitive ventricular ectopic beats (> or = 3) and/or ventricular tachycardia, 129 +/- 25; P < 0.001). During follow-up two patients showed a transient but significant reduction of SDNN and a concomitant increase of the arrhythmic events. In eight patients an episode of sustained ventricular tachycardia occurred, but the mean SDNN of this subgroup did not differ from the mean value of the remaining patients (152 +/- 15 vs 150 +/- 39; P = NS). Only one subject died after heart transplantation during follow-up (case censored). Time-domain analysis of HRV seems to be a useful method to assess the autonomic influences in ARVC. A reduction of vagal influences correlates with the extent of the disease. The significant correlation between SDNN and ventricular arrhythmias confirmed the influences of autonomic activity in the modulation of the electrical instability in ARVC patients. However, SDNN was not predictive of spontaneous episodes of sustained ventricular tachycardia.

Objectives: We sought to define the clinical picture and natural history of familial arrhythmogenic right ventricular cardiomyopathy (ARVC)., Background: Arrhythmogenic right ventricular cardiomyopathy is a myocardial disease, often familial, clinically characterized by the impending risk of ventricular arrhythmias and sudden death., Methods: Thirty-seven ARVC families of northeast Italy were studied. Probands had a histologic diagnosis of ARVC, either at autopsy (19 families) or endomyocardial biopsy (18 families). Protocol of the investigation included basal electrocardiogram (ECG), 24-hour ECG, signal-averaged ECG, stress test and two-dimensional Doppler echocardiography. Invasive evaluation was performed when deemed necessary., Results: Of the 365 subjects, 151 (41%) were affected, 157 (43%) were unaffected, 17 (5%) were healthy carriers, and 40 (11%) were uncertain. Mean age at diagnosis was 31+/-13 years. By echocardiography, 64% had mild, 30% had moderate, and 6% had severe form. Forty percent had ventricular arrhythmias, 49 were treated with antiarrhythmic drugs, and two were treated with implantable cardioverter defibrillators. Sport activity was restricted in all. Of the 28 families who underwent linkage analysis, 6 mapped to chromosome 14q23-q24, 4 to 1q42-q43, and 4 to 2q32.1-q32.3. No linkage with known loci was found in four families and 10 had uninformative results. During a follow-up of 8.5+/-4.6 years, one patient died (0.08 patient/year mortality), and 15 developed an overt form of ARVC., Conclusions: Arrhythmogenic right ventricular cardiomyopathy is a progressive disease appearing during adolescence and early adulthood. Systematic evaluation of family members leads to early identification of ARVC, characterized by a broad clinical spectrum with a favorable outcome. In the setting of positive family history, even minor ECG and echocardiographic abnormalities are diagnostic.

The mechanism underlying cardiac arrest in patients with hypertrophic cardiomyopathy (HC) is intriguing. In the clinical setting, myocardial ischemia has long been incriminated, particularly in the young. Among 274 cardiovascular sudden deaths in the young (< or = 35 years), 19 (7.0%), 14 males and 5 females, median age 23 years, had HC. Familial occurrence of HC was ascertained in 3 (16%). SD occurred on effort in 6 (31%). Previous syncope occurred in 5 and palpitations in 3. Basal electrocardiogram (ECG) was abnormal in 7 of 8 available cases. Hypertrophy was septal asymmetric in 14. Gross examination showed large isolated or multiple septal scars in 11 (58%); at histomorphometry, the mean percent area of fibrosis of the septal myocardium was 18.6 +/- 6. Four showed a deep intramyocardial course of the left anterior descending coronary artery. At histology, myocardial disarray involved 30 +/- 16% of the septal myocardium; evidence of acute-subacute myocardial necrosis was present in 14 (74%), 1 of them with a regional acute myocardial infarction. By comparing hearts with (n = 11) and without (n = 8) areas of scar-type fibrosis, we found a statistically significant difference in terms of age (25.5 +/- 5.4 v 15.5 +/- 12.4 years, P = .04), septal thickness (25.4 +/- 5.4 v 15.4 +/- 4.9 mm, P < .001), percent increase of septal thickness versus normal value for age and sex (46.2 +/- 15 v 25.2 +/- 13.6%, P < .01) and mean score of small vessel disease (1.7 +/- 0.4 v 1.2 +/- 0.4, P = .04). Linear regression analysis showed a positive correlation of percent area of replacement fibrosis with septal thickness (P = .01) and with mean score of small vessel disease (P < .01). In conclusion, our pathologic findings of ischemic damage, either acute-subacute or in the form of fibrotic scars, support the clinical evidence that ischemia occurs in the natural history of HC and may contribute to life-threatening electrical instability.

Objective: The aim of the study was to assess the prevalence, sensitivity, specificity and predictive value of the signal-averaged ECG in patients with arrhythmogenic right ventricular cardiomyopathy and different forms of ventricular arrhythmias., Methods: The signal averaged ECG in 138 patients and 146 healthy subjects (control group), using a three bandpass filter system (25-250, 40-250, 80-250 Hz), was considered abnormal when at least two parameters were abnormal at each filter setting. Patients were divided into three groups according to the extent of the right ventricular enlargement (mild, moderate, extensive), and into five groups according to the type of ventricular arrhythmia., Results: The signal averaged ECG was abnormal in 57% of the patients and in 4% of the healthy subjects. The sensitivity was 57%, specificity 95% and positive predictive value 92%. The signal averaged ECG was abnormal in 94.4% of patients with the extensive form of the disease, in 77.7% of patients with the moderate form and in 31.8% of patients with the minor form, demonstrating good correlation with the extent of the disease. According to the type of ventricular arrhythmia, a higher correlation was found between signal averaged ECG abnormality and sustained ventricular tachycardia with superior axis (94.4%, P<0. 02); the correlation for the other arrhythmias varied from 16.6% to 55.8%., Conclusion: There is a closer correlation between the signal averaged ECG and extent of disease than with the presence of ventricular arrhythmias. The signal averaged ECG is not helpful in diagnosing minor forms of the disease, but since it is a non-invasive method, it may be useful in evaluating progression of the disease., (Copyright 2000 The European Society of Cardiology.)

In 1992, Brugada and Brugada reported a distinct subgroup of patients with episodes of "idiopathic"polymorphic ventricular tachycardia or ventricular fibrillation characterized by a unique electrocardiographic (ECG) pattern, which consisted of right bundle branch block and ST-segment elevation from V1 to V2-V3. As in patients with long QT syndrome, the ECG changes and the ventricular electrical instability could not be explained by structural heart disease, myocardial ischemia, or electrolyte disturbances. The syndrome can be inherited and predominantly affects males. Clinical presentation includes cardiac arrest or syncope caused by rapid ventricular tachycardia or fibrillation characteristically occurring at rest or during sleep. The clinical outcome of affected patients is poor unless they receive an implantable cardioverter defibrillator. The ECG pattern and the electrical ventricular instability have been explained by the dispersion of repolarization between the right ventricular epicardium and endocardium, which predisposes to local reexcitation of myocytes with different action potential durations. A disease-causing missense mutation in the cardiac sodium channel gene SCN5A has been recently reported in patients with Brugada syndrome. It is mandatory for the clinician to carefully rule out any organic heart disease before suggesting a diagnosis of Brugada syndrome, because the typical ECG pattern with the risk of sudden arrhythmic death is also observed in patients with structural heart diseases in the setting of arrhythmogenic right ventricular cardiomyopathy.

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 aim of our study was to analyze how the clinical history and the main clinical characteristics of patients suffering from loss of consciousness may influence the results of the upright tilt test. A series of 745 patients (333 males, 412 females; mean age 44 +/- 18 years) with recurrent episodes of syncope or presyncope underwent complete clinical and noninvasive laboratory examination, including vagal maneuvers and upright tilt test (60 degrees for 45 min). Cardiological and neurological findings were normal in every case. Upright tilt test was positive in 462 patients (62%). Patients with presyncope showed a lower positivity compared to patients with syncope (70.2% vs 42.9%, P < 0.001). Younger patients (< 25 years) displayed highest upright tilt test positivity (68.5%), while familial occurrence of syncope or presyncope, results of vagal maneuvers, and different gender did not correlate with the results of the test. The time interval between the last syncopal episode and the day of upright tilt test negatively influenced the proportion of positive tests (> 30 days = 45.1% vs < 30 days = 77.2%; P < 0.001). Patients with more than three syncopal episodes in the 2 months preceding the test showed a higher upright tilt test positivity (83.9% vs 64.5%, P < 0.001). In conclusion, upright tilt test seems to be more sensitive in young patients with syncopal episodes during symptomatic periods. These findings suggest both an individual and temporal variability in autonomic nervous system activity, the implication of which are relevant to the indications for testing as well as the analysis of results.

Objectives: We sought to assess whether structural heart disease underlies the syndrome of right bundle branch block, persistent ST segment elevation and sudden death., Background: Ventricular fibrillation and sudden death may occur in patients with a distinctive electrocardiographic (ECG) pattern of right bundle branch block and persistent ST segment elevation in the right precordial leads., Methods: Sixteen members of a family affected by this syndrome underwent noninvasive cardiac evaluation, including electrocardiography, Holter ambulatory ECG monitoring, stress testing, echocardiography and signal-averaged electrocardiography; two patients had electrophysiologic and angiographic study. Endomyocardial biopsy was performed in one living patient, and postmortem examination, including study of the specialized conduction system, was performed in one victim of sudden death., Results: Five years before a fatal cardiac arrest, the proband had been resuscitated from sudden cardiac arrest due to recorded ventricular fibrillation. Serial ECGs showed a prolonged PR interval, right bundle branch block, left-axis deviation and persistent ST segment elevation in the right precordial leads, in the absence of clinical heart disease. Postmortem investigation disclosed right ventricular dilation and myocardial atrophy with adipose replacement of the right ventricular free wall as well as sclerotic interruption of the right bundle branch. A variable degree of right bundle branch block and upsloping right precordial ST segment was observed in seven family members; four of the seven had structural right ventricular abnormalities on echocardiography and late potentials on signal-averaged electrocardiography. A sib of the proband also had a prolonged HV interval, inducible ventricular tachycardia and fibrofatty replacement on endomyocardial biopsy., Conclusions: An autosomal dominant familial cardiomyopathy, mainly involving the right ventricle and the conduction system, accounted for the ECG changes and the electrical instability of the syndrome.

Since 1988 to 1992 we analyzed 116,452 consecutive 12-lead electrocardiograms belonging to the entire cohort of 18-year old young boys resident in Padova, Treviso, Rovigo, Venezia, Belluno area (2,834,000 inhabitants). We identified 173 cases of overt WPW pattern (short PR interval, delta wave, anomalous configuration of QRS complex) with a calculated incidence of 1.48/1000. Accessory pathway location was: left free wall (70 patients), right free wall (39 patients), postero-septal (37 patients), antero-septal (15 patients) and undetermined (12 patients). Sixty patients (34.6%) complained of different symptoms as palpitations, near syncope and dizziness. Fifty-three patients (30.6%) went in a regular sport activity. Twenty-four hour Holter monitoring (41 patients) and exercise stress test (43 patients) did not show sustained tachyarrhythmias; intermittent preexcitation was recorded in 23 and 32 patients, respectively. Two-dimensional echocardiogram (68 out of 173) was normal in 44 patients, while 24 showed minor cardiac abnormalities with two major disease; mitral valve prolapse was diagnosed in 8 patients. On the basis of transesophageal (24 patients) or intracardiac (5 patients) electrophysiologic study, 11 patients were considered at high risk for sudden death. Eight of them suffered from spontaneous symptoms.

In diabetic patients, the pathophysiologic mechanisms of exercise-induced left ventricular (LV) dysfunction remain controversial. In this study, the role of myocardial contractility recruitment in determining an abnormal LV response to isometric or dynamic exercise has been investigated in 14 diabetic patients with autonomic dysfunction. Ischemic heart disease was excluded by the absence of LV wall motion abnormalities induced by isotonic and isometric exercise and by coronary angiography. Left ventricular and myocardial function were studied at rest, and during isometric and isotonic exercise, by two-dimensional echocardiography; moreover, recruitment of an inotropic reserve was assessed by postextrasystolic potentiation at rest and at peak handgrip. An abnormal response of LV ejection fraction to isometric (9/14) or to dynamic (8/14) exercise was frequent in study patients. In these patients, baseline myocardial contractility was normal, and the significant increase in ejection fraction by postextrasystolic potentiation indicated a normal contractile reserve (65 +/- 7% vs. 74 +/- 6%, p = 0.001). Nevertheless, the downward displacement of LV ejection fraction-systolic wall stress relationships during exercise suggests an inadequate increase in myocardial contractility. However, the abnormal ejection fraction at peak handgrip was completely reversed by postextrasystolic potentiation (67 +/- 6% vs. 58.1 +/- 10%, p = 0.008), a potent inotropic stimulation independent of the integrity of adrenergic cardiac receptors. A defective inotropic recruitment, despite the presence of a normal LV contractile reserve, plays an important role in deexercise LV dysfunction in diabetic patients with autonomic neuropathy.

Objectives: This study was designed to evaluate and compare the patterns of arrhythmogenic right ventricular cardiomyopathy in young people and adults., Background: Few data are available on this cardiomyopathy in young people because clinical and morphologic findings considered pathognomonic are normally based on observations in adults. However, a familial occurrence with a probable genetic transmission led to the study of children and to early detection of this disease, in which sudden death has been reported even in young people., Methods: Seventeen young patients with arrhythmogenic right ventricular cardiomyopathy diagnosed at a mean age +/- SD of 14.9 +/- 4.9 years were studied. Clinical, electrocardiographic, echocardiographic, cineangiographic and biopsy findings were compared with those of 19 adult patients whose condition was diagnosed at a mean age of 38.1 +/- 13.4 years., Results: Syncope occurred in 23.5% of the young patients but in none of the adults (odds ratio of familial sudden death 5.54, p = 0.1). Ventricular couplets (odds ratio 16.0, p = 0.004) and subtricuspid bulging on echocardiography (odds ratio 5.95, p = 0.036) were prevalent in the young group. Cineangiographic data in the two groups were similar, except that more hypokinetic areas were found in adults (odds ratio 4.44, p = 0.05). Morphometric analysis of biopsy sections showed a greater amount of fibrous tissue in the young patients (p = 0.04) and a prevalence of fatty tissue in the adults (odds ratio 12, p = 0.005). During an equivalent follow-up time (mean 7 years), two young patients died suddenly, and two had ventricular fibrillation in the absence of antiarrhythmic therapy., Conclusions: The pathognomonic criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy in adults are also valid for young people. Sudden or aborted death occurred frequently in young untreated patients.

Objectives: Because sudden death due to complete atrioventricular (AV) block or ventricular arrhythmias is the most dramatic event in myotonic dystrophy, we assessed the relation of cardiac disease to cytosine-thymine-guanine (CTG) triplet mutation in adults affected with myotonic dystrophy., Background: The myotonic dystrophy mutation, identified as an unstable deoxyribonucleic acid (DNA) sequence (CTG) prone to increase the number of trinucleotide repeats, produces clinical manifestations of the disease in skeletal muscle, the heart and many organ systems., Methods: Forty-two adult patients underwent electrocardiography and echocardiography; in addition, signal-averaging electrocardiography was performed in 22, and 24-h Holter monitoring was recorded in 32. The diagnosis was established by neurologic examination, electromyography, muscle biopsy and DNA analysis. The patients were then classified into three subgroups on the basis of the number of CTG trinucleotide repeat expansions: E1 = 18 patients with 0 to 500 CTG repeats; E2 = 12 patients with up to 1,000 repeats; E3 + E4 = 10 patients with up to 1,500 repeats and 2 patients with > 1,500 repeats., Results: The incidence of normal electrocardiographic (ECG) results was found to be significantly different in the three subgroups (55%, 50%, 17% in E1, E2, E3, + E4, respectively, p = 0.04), with the highest values in the group with fewer repeat expansions. The incidence of complete left bundle branch block was also significantly different among the groups (5% in E1, 0% in E2, 42% in E3 + E4 p = 0.01) and was directly correlated with the size of the expansion. A time-domain analysis of the signal-averaged ECG obtained in 12 patients in E1, 4 in E2, 5 in E3 and 1 in E4 showed that abnormal ventricular late potentials were directly correlated with CTG expansion (33% in E1, 75% in E2, 83% in E3 + E4, p = 0.05). Moreover, the incidence of ventricular couplets or triplets showed a positive correlation with size of CTG expansion (0 in E1, 0 in E2, 29% in E3 + E4, chi square 0.02)., Conclusions: Our findings suggest that the involvement of specialized cardiac tissue, accounting for severe AV and intraventricular conduction defects, is related to CTG repeat length. In addition, the presence of abnormal late potentials directly correlates to CTG expansion. Abnormal late potentials, caused by slowed and fragmented conduction through damaged areas of myocardium, represent a substrate for malignant reentrant ventricular arrhythmias. In the future, therefore, molecular analysis of DNA should identify patients with cardiac disease at high risk for development of AV block or lethal ventricular arrhythmias.

The purpose of this study was to assess the long-term results of a single lead VDD pacing system (Phymos MPS) in 85 patients (48 males, 37 females, mean age 74 +/- 9 years). The system, which incorporates two atrial floating electrodes (Phymos 830-S), was implanted for second- or third-degree Mobitz II AV block. The percentage of AV synchronized pacing, the presence of fusion beats due to short AV delay, and the occurrence of supraventricular arrhythmias was evaluated by Holter monitoring at 6-month intervals. Over a mean period of follow-up of 44 months (range 2-56), over 90% of AV synchronized pacing events were noted in 74 patients (87%). The presence of persistent fusion beats was detected in 12 patients (14%). In ten patients the pacemaker was reprogrammed to VVI mode for supraventricular arrhythmias (7 patients, 8.2%) or unsatisfactory atrial sensing (3 patients, 3.5%). One pacemaker was removed for pocket infection and two for battery depletion. These observations support the use of VDD single lead pacing to combine a physiological mode of stimulation with simple implantation techniques.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVD) is a dominantly inherited disorder progressively affecting the myocardium and it is one of the major causes of juvenile sudden death. The chromosomal localization of the disease gene is reported here for the first time. A maximum lod score of 6.04 was obtained at theta = 0 for linkage with the polymorphic marker D14S42 (14q23-q24) in two families, one of which has 82 subjects (19 affected) in four generations. The pre-symptomatic identification of ARVD carriers by linkage analysis in the affected families strongly increases the possibility of prevention of life-threatening complications.

Objectives: The purpose of this study was to assess the incidence of myocardial involvement and the relation of cardiac disease to the molecular defect at the deoxyribonucleic acid (DNA) or protein level in Becker muscular dystrophy., Background: Dystrophin gene mutations produce clinical manifestations of disease in the heart and skeletal muscle of patients with Becker muscular dystrophy., Methods: Thirty-one patients underwent electrocardiographic and echocardiographic examination and 24-h Holter monitoring. The diagnosis was established by neurologic examination, dystrophin immunohistochemical assays or Western blot on muscle biopsy, or both, and DNA analysis., Results: Electrocardiographic and echocardiographic findings were abnormal in 68% and 62% of the patients, respectively. Right ventricular involvement was detected in 52%. Left ventricular impairment was observed either as an isolated phenomenon (10%) or in association with right ventricular dysfunction (29%). Right ventricular disease was manifested in the teenagers, and an impairment of the left ventricle was observed in older patients. Right ventricular end-diastolic volumes were significantly increased compared with those in a control group. The left ventricular ejection fraction was significantly lower in older patients than in control subjects or younger patients. Life-threatening ventricular arrhythmias were detected in four patients. No correlations were found between skeletal muscle disease, cardiac involvement and dystrophin abnormalities. In our patients, exon 49 deletion was invariably associated with cardiac involvement. Exon 48 deletion was associated with cardiac disease in all but two patients., Conclusions: The cardiac manifestation of Becker muscular dystrophy is characterized by early right ventricular involvement associated or not with left ventricular impairment. Exon 49 deletion is associated with cardiac disease.

We describe the first sets of identical and nonidentical twins with right ventricular cardiomyopathy (RVC). Pair A: A 12-year-old boy was referred because of palpitation and syncope. Clinical and instrument examinations revealed an enlarged and depressed right ventricle (end-diastolic volume = 110 ml/m2; ejection fraction = 44%), spontaneous ventricular tachycardia, and fatty-fibrous infiltrates in the biopsy specimens. His asymptomatic, monozygotic twin showed localized involvement of the right ventricle with isolated, ventricular extrasystoles. Pair B: These 18-year-old nonidentical twin boys showed diffuse right ventricular involvement (end-diastolic volume = 110 ml/m2 and 114 ml/m2; ejection fraction = 30% and 24%, respectively), induction of sustained and nonsustained ventricular tachycardia, respectively, and fibrosis on endomyocardial biopsy. One of the boys died suddenly at rest after documented ventricular fibrillation. These cases support the hypothesis of a genetic etiology with a minor role for genotype and point to the important influence of environmental factors in determining the clinical features of the disease.

To evaluate heart rate variability (expressed as the standard deviation of RR intervals) within 5 years of follow-up, we studied 20 patients (14 males, 6 females, mean age 44 +/- 12 years) who underwent orthotopic heart transplantation. Six measurements were taken: one in the first 3 weeks after transplantation, and the others once annually, for 5 years. Twenty healthy subjects (mean age 44 +/- 7 years) constituted the control group. Heart rate variability increased significantly in the first 3 years of follow-up (7.2 +/- 1 vs. 11.1 +/- 4, p < 0.001; 11.1 +/- 4 vs. 15.2 +/- 4, p < 0.01; 15.2 +/- 4 vs. 18.9 +/- 5, p < 0.05); in the following years this trend slackened and values did not reach a statistically significant difference (18.9 +/- 5 vs. 21.4 +/- 5; 21.4 +/- 5 vs. 22.5 +/- 5). The mean standard deviation was invariably greater in the control group (63.6 +/- 12). These findings show that sinus rhythm variability in the denervated heart progressively increased over 5 years of follow-up. The absence of presynaptic uptake, which is responsible for adrenergic hypersensitivity to circulating catecholamines and intrinsic cardiac reflexes, does not appear to cause this phenomenon, since these mechanisms are not able to evolve in time after cardiac transplantation. Therefore, an enhanced beta-adrenergic receptors density or affinity to circulating catecholamines or a limited sympathetic reinnervation may be the more probable underlying mechanism.

In 24 subjects presenting with apparently idiopathic ventricular arrhythmias, a final diagnosis of arrhythmogenic right ventricular cardiomyopathy was formulated following global evaluation of the clinical, cross-sectional echocardiography and angiographic findings, and the observation of myocardial atrophy with fibrous-fatty substitution in right ventricular endomyocardial biopsy. All patients had good effort tolerance, and a normal cardiac silhouette. Ventricular arrhythmias with a left bundle branch block pattern were present in 23 cases (sustained ventricular tachycardia, nonsustained ventricular tachycardia, ventricular couplets, and ventricular premature complexes); 1 patient experienced an episode of ventricular fibrillation. A nearly constant electrocardiographic feature was T wave negativity in the right precordial leads. Cross-sectional echocardiography and hemodynamic studies showed that right ventricular impairment consisted only of localized structural and dynamic abnormalities; in a few cases the left ventricle was segmentally involved. Familial occurrence was present in 29% of the cases. No case of sudden death was observed during follow-up. These findings confirm that the concealed form of arrhythmogenic right ventricular cardiomyopathy is a cause of so-called "idiopathic" ventricular arrhythmias in subjects with apparently "normal hearts". Echocardiographic and angiographic investigations may lead to the correct diagnosis.

We describe a 47-year-old man affected with hypertrophic cardiomyopathy and frequent episodes of syncope. During ambulatory Holter monitoring and head-up tilt test, a syncopal attack was associated with sinus arrest and hypotension. This case suggests an additional mechanism of syncope in hypertrophic cardiomyopathy.

In right ventricular cardiomyopathy the relationship between the progression of structural abnormalities and arrhythmia development is not yet well known. This report describes a case in which severe ventricular arrhythmias appeared 3 years after the demonstration of right ventricular (RV) structural and dynamic abnormalities. In this interval of time structural changes were not detectable with the commonly used diagnostic methods, but endocavitary RV late fractionated QRS potentials appeared suggesting the development of an arrhythmic component of the disease.

An asymptomatic 19-year-old top-level athlete had electrocardiographic evidence of intermittent cardiac preexcitation and intermittent left bundle branch block. The electrophysiologic study demonstrated the presence of a direct accessory pathway and enhanced atrioventricular node conduction that resulted in infrahisian and intraventricular conduction disturbances. The echocardiogram disclosed tricuspid valve dysplasia.

Postoperative 24-hour Holter monitoring was performed in 106 patients with atrioventricular septal defect in order to identify the incidence of atrial and ventricular arrhythmias. Of the patients, 72 had separate atrioventricular orifices, including 13 with a small ventricular component to the defect, and 34 patients had a common atrioventricular orifice. Two groups of abnormal patients were found. First, patients with good electrical stability characterized by isolated atrial (9 patients) and ventricular (25 patients) extrasystoles falling in classes I and II of Lown. Second, patients with marked electrical instability characterized in one patient by repetitive atrial extrasystoles, in another by atrial flutter, in 2 by polymorphic ventricular extrasystole and in 8 by couplets or triplets. Electrical instability in individual patients was then compared, by means of logistic regression analysis, with operative, surgical and postoperative variables. There was no incidence of sudden death in our series. After surgical repair, ventricular arrhythmias were more frequent than atrial arrhythmias (33% vs. 10%) and were unrelated to the type of atrioventricular septal defect. Cardiac electrical instability after operation was significantly related to larger operative body size, higher postoperative end diastolic diameter of the right ventricle, larger size of the ventricular septal defect, coexistence of postoperative right bundle branch block and left anterior hemiblock. Conversely, the risk of arrhythmias was reduced by more recent operative data and by greater shortening fraction of the left ventricle.

B.P., asymptomatic until her fifties, was admitted to hospital because of precordial pain. Her ECG and chest X rays were normal. When 52 she was again admitted, because of congestive heart failure. ECG showed repolarization abnormalities and chest X rays showed marked cardiomegaly. 2d echocardiogram was consistent with right atrial dilatation, dislodged posterior leaflet of the tricuspid valve and marked tricuspid regurgitation; these features were consistent with Ebstein anomaly. The patient was successfully treated by digitalis and diuretics. This case shows that in very rare cases this congenital heart disease can produce its clinical manifestations only late in lifetime.

The morphology of ventricular extrasystole (VES) in 46 cases of arrhythmogenic dysplasia of the right ventricle (ADRV) was correlated with the point of origin located by intracavitary mapping. The cases concerned 41 of left bundle-branch block (LBB) with various axes on the frontal plane (FP), 4 of right bundle-branch block (RBB), and 5 of atypical morphology (frontal plane shifted inferiorly and increased R from V1 to V6; on the horizontal plane, clockwise rotation of the loop oriented anteriorly and leftward). There is a good correlation with the site of origin: VESs which were LBB in appearance originated in the right ventricle (apex, septum, infundibulum); VESs which were RBB in appearance originated in the apex of the left ventricle, while the atypical VESs started in the upper posterior septum. A study of morphology may therefore also give an indication of the location of the disease.

We studied in detail 17 patients presenting with monomorphic repetitive ventricular rhythms having left bundle branch block morphology and right axis deviation. All had an apparently normal heart at physical examination. At chest radiography, three patients had mild cardiomegaly, and at electrocardiography, five patients had inverted T waves beyond V2. Five patients had syncope or near syncope. In seven patients the tachycardia occurred on effort. One patient died suddenly. The patients were extensively investigated, using cross-sectional echocardiography, complete haemodynamic and angiographic studies, electrophysiology and histology, to search for any structural basis of the arrhythmias. Tachycardia was sustained in 8 patients, nonsustained in 3, and consistent with accelerated idioventricular rhythm and repetitive paroxysmal ventricular tachycardia in 5 and 1 patients, respectively. Despite the differences in clinical and arrhythmologic features, similar abnormalities of right ventricular structure and/or wall motion were detected in all patients, consistent with localized forms of right ventricular cardiomyopathy. Different antiarrhythmic drugs were successfully used in twelve patients (the four patients with accelerated idioventricular rhythm were not treated). The patient who died suddenly had previously had a sustained ventricular tachycardia and was being treated by beta-blockade. Postmortem study revealed massive fibro-adipose substitution of the right ventricular free wall and pulmonary infundibulum.

The object of the study was to define spreading and quantitative criteria of left ventricular hypertrophy in echocardiography by using a "score"--for this, the left ventricle has been divided into 11 regions and a "score" attributed to each one of them--and to find the correlation with the vectocardiogram (VCG) in 42 patients with hypertrophic myocardiopathy (HM). The results obtained show the following: 1) the left ventricular hypertrophy aspect on the ECG and the VCG is very sensitive for the identification of a diffuse HM; 2) the necrosis, hemiblock or septal hypertrophy indicate a hypertrophy located in the forepart septum or the whole of the septum; 3) the giant T waves indicate a hypertrophy of the apex; 4) a left ventricular hypertrophy associated with a necrosis or a hemiblock indicate a global myocardiopathy, with the basal region of the septum largely affected.

Clinico-pathological findings are described in two patients with typical variant angina who died suddenly during an ischemic attack. In both cases, detailed pathologic examination of the coronary arteries disclosed severe focal atherosclerosis of the anterior descending coronary artery. The only distinctive histological finding was new intimal proliferation of smooth muscle cells enmeshed within mucoid substance, superimposed on the old fibrous cap of the plaque. These findings agree with experimental and clinical data which suggest that coronary vasospasm may be related to growth of atherosclerotic plaques. This study provides histological evidence that progression of an atherosclerotic plaque may underlie variant angina and sudden death.

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.

Three types of atrioventricular nodal conduction curves, relating A1A2 to H1H2, generated with atrial extrastimulus technique, are known. The first type is smooth, suggesting the homogeneous structure of A-V node. The second type, with abrupt increase in H1H2 response over a critical range of A1A2 coupling intervals, suggests the presence of fast and slow A-V nodal pathways. We have found in five patients the third tipe of A-V conduction curve, giving evidence of an intranodal final common pathway, distal to the fast and slow A-V nodal pathways. The thyrd type of curve enables us to know also some alectrophysiological properties of final common pathway. Indeed we have defined effective and functional refractory periods of fast, slow and final common pathways as far as we can in this type of curve. Paced heart rate variations and atropine medications have led the third type of A-V conduction curve to change into the second type in three cases, into first type in one case. These changes of A-V nodal conduction curves are related to different influence of cardiac cycle lenghts and autonomic nervous system on refractory periods and conduction velocity of the outlined intranodal structures.

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.

In this paper we report the arrhythmias recorded on basal ECG, on Holter monitoring or on exercise test, in 32 pts affected by arrhythmogenic right ventricle (ARV). A sustained ventricular tachycardia (VT) was present in 11 pts a non sustained VT in 15 pts, a slow VT in 2 pts, a ventricular fibrillation (VF) in 3 pts and both sustained VT and VF in 1 pt. All but 1 case of sustained VT showed a LBBB like pattern. The heart rate during VT ranged between 170 and 280 beats/min. The frontal axis of the VT showed a wide range of deviation. Among non sustained VT, 9 cases had LBBB like pattern and 6 cases had polymorphic configuration. The 2 cases of slow VT showed LBBB like pattern with right axis deviation. A comparison between ventricular arrhythmias and RV impairment was made. The data obtained suggest that the effort plays an important role in the induction of VT in pts with localized RV impairment. In conclusion a wide spectrum of ventricular tachyarrhythmias is present in the ARV. Probably the RV "arrhythmogenic" zones and the electrophysiological mechanism causing the arrhythmias are various.

Smooth and interrupted curves of AV conduction, generated with an atrial extrastimulus technique, are seen in man, suggesting the involvement of two physiological AV pathways named "alpha" and "beta" and leading to a final common pathway (FCP). In view of this knowledge, the effects of some representative antiarrhytmic agents (Atropine, Verapamil, Ajmaline) were studied in eight patients in order to elucidate the physiological role of the various components of AV junction. Verapamil decreased conduction velocity of both "alpha" and "beta" pathways and increased their refractoriness; Atropine decreased "alpha" and "beta" pathways' refractory periods and increased their conduction velocity; further studies are required to clarify the effects of Ajmaline on AV conduction.

Electrophysiologic and histopathologic correlation has been carried out in a patient with scleroderma heart disease, affected by syncopal seizures, who died of recorded ventricular fibrillation. The electrophysiological investigation disclosed dysfunction of sinoatrial conduction, revealed by sinoatrial blocks and by an abnormal return cycle pattern after premature atrial beats. Atrial effective and functional refractory periods were increased and an unusual 'pseudo-Wenckebach' phenomenon between artificial stimulus and atrium was observed during atrial pacing. Intra-AV nodal conduction time was at normal upper limits and Wenckebach-type AV block was obtained on pacing the atrium at 100 beats/min. HV conduction was moderately prolonged in the presence of left anterior hemiblock. The histopathologic substrates of these electrophysiologic disturbances were fibrosis of the sinus node, disrupted internodal pathways and atrio-AV nodal connections, and left bundle branch atrophy. As far as fatal tachyarrhythmia is concerned, myofibrillar degeneration may have contributed to its pathogenesis. It is suggested that both lesions of the ordinary myocardium and specialized conduction system account for the electrical instability of sclerodermic patients.