OBJECTIVES This study investigated the incidence of appropriate implantable cardioverter defibrillator (ICD) interventions for ventricular tachycardia (VT) or ventricular fibrillation (VF) in patients with idiopathic dilated cardiomyopathy (IDC) and nonsustained VT in the presence of a left ventricular ejection fraction below 30%, versus in patients with syncope and patients with a history of VT or VF.BACKGROUND To date, only limited information is available about the prophylactic use of ICDs in patients with IDC. METHODS From January 1993 to July 2000, 101 patients with IDC underwent implantation of ICDs with electrogram storage capability at our institution. Patients were placed into one of three groups according to their clinical presentation: asymptomatic or mildly symptomatic nonsustained 7 in the presence of a left ventricular ejection fraction less than or equal to30% (49 patients, prophylactic group), unexplained syncope or near syncope (26 patients, syncope group) and a history of sustained VT or VT (26 patients, VT/VF group),RESULTS During 36 +/- 22 months follow-up, 18 of 49 patients (37%) in the prophylactic group received appropriate shocks for VT or VF, compared with 9 of 26 patients, (31%) in the syncope group and with 9 of 26 patients (35%) of the VT/VF group. Multivariate Cox analysis of baseline clinical variables identified left ventricular ejection fraction, atrial fibrillation and a history, of sustained VT or VF as predictors for appropriate lCD interventions during follow-up.CONCLUSION Patients with IDC and prophylactic ICD implantation for nonsustained 7 in the presence of a left ventricular ejection fraction less than or equal to30% had an incidence of appropriate ICD interventions similar to that of patients with a history of syncope or sustained VT or VF. These findings indicate that ICDs may have a role in not only secondary but also primary prevention of Sudden death in IDC. (C) 2002 by the American College of Cardiology Foundation.

Background/Aims: Recently, a decrease in heart rate variability measures was found in patients with carcinoid syndrome suffering from carcinoid heart disease compared to those without cardiac involvement of carcinoid syndrome. The prognostic relevance of this finding, however, was not clear. Patients and Methods: Therefore, 35 patients with carcinoid syndrome (21 men, age 56 ± 11 years), all of them suffering from metastatic carcinoid tumors, were followed prospectively at our institution. Digital 24-hour Holter monitoring, echocardiography, and serum serotonin and urine 5-hydroxyindole acetic acid (5-HIAA) samplings were performed in all study patients at baseline. Indices of time domain heart rate variability obtained from Holter recordings included the standard deviation of all normal RR intervals (SDNN) representing overall variability, the square root of the mean of the squared differences between adjacent normal RR intervals (rMSSD), and the percentage of the number of pairs of adjacent normal RR intervals differing by >50 ms (pNN50), both indices reflecting predominantly vagal influences on heart rate. Results: During a mean follow-up of 18 ± 7 months, 15 of 35 patients with carcinoid syndrome (43%) died. Patients with cardiac manifestation of the carcinoid syndrome showed a tendency towards an increased mortality in comparison to patients without cardiac involvement (p = 0.09). Patients with the combination of decreased heart rate variability (SDNN <100 ms) and presence of carcinoid heart disease had a significant worse prognosis (p = 0.04) compared to patients without carcinoid heart disease and preserved heart rate variability (SDNN ≧100 ms). Conclusions: The presence of carcinoid heart disease in combination with decreased heart rate variability is associated with the most adverse prognosis in the setting of carcinoid syndrome.

Radiofrequency catheter ablation was performed in four adults with myocardial dysfunction related to repetitive monomorphic ventricular tachycardia (RMVT) originating in the right ventricular outflow tract. Serial echocardiographic assessment of left ventricular function before and after radiofrequency catheter ablation of RMVT showed complete reversal of left ventricular dysfunction without arrhythmia recurrence during 31+/-28 months follow-up.

OBJECTIVE To examine the relation between cardiac autonomic tone, assessed by baroreflex sensitivity and heart rate variability, and left ventricular function, arrhythmias on Holter monitoring, and clinical variables in patients with idiopathic dilated cardiomyopathy. DESIGN A prospective observational study. PATIENTS 160 patients with idiopathic dilated cardiomyopathy and preserved sinus rhythm in the absence of antiarrhythmic drug treatment. Measures of heart rate variability obtained by digital 24 hour Holter recordings included the mean of all coupling intervals between normal beats (RRm), the standard deviation of the mean of normal RR intervals (SDNN), and the square root of the mean of the squared differences between adjacent normal RR intervals (rMSSD). Baroreflex sensitivity testing was performed using the phenylephrine method. RESULTS Mean SDNN (SEM) was 112 (46) ms, and baroreflex sensitivity was 7.5 (5.0) ms/mm Hg. SDNN showed a weak correlation with baroreflex sensitivity (r = 0.19, p < 0.05) and with left ventricular ejection fraction (r = 0.29, p < 0.05). SDNN showed no significant correlation with age (r = −0.07), the presence of non-sustained ventricular tachycardia (r = −0.13), or left ventricular end diastolic diameter (r = −0.07). In addition, baroreflex sensitivity showed no significant correlation with age (r = −0.13), non-sustained ventricular tachycardia (r = −0.08), left ventricular end diastolic diameter (r = 0.09), or ejection fraction (r = 0.14). CONCLUSIONS The weak correlation between baroreflex sensitivity and heart rate variability suggests that these two indices explore different aspects of cardiac autonomic control in patients with idiopathic dilated cardiomyopathy. The weak or absent correlation between baroreflex sensitivity, heart rate variability, and other potential non-invasive risk predictors, including left ventricular ejection fraction, left ventricular end diastolic diameter, and non-sustained ventricular tachycardia on Holter monitoring, indicate that these variables may have independent prognostic value in idiopathic dilated cardiomyopathy.

The relation between microvolt level T wave alternans (TWA) and other noninvasive arrhythmia risk predictors was analyzed in 221 consecutive patients with idiopathic dilated cardiomyopathy (IDC) and sinus rhythm enrolled in the Marburg Cardiomyopathy Study between March 1996 and May 2000. TWA analysis was also performed in 110 healthy controls of similar age and sex. TWA during symptom‐limited exercise was positive, negative and indeterminate in, respectively, 108 (49%), 65 (29%) and 48 (22%) patients with IDC versus, respectively, 5 (5%), 98 (89%) and 7 (6%) healthy controls (P < 0.05). Patients with IDC and positive TWA had a lower left ventricular (LV) ejection fraction (29 ± 9% vs 34 ± 10%, P < 0.05) and greater LV end‐diastolic diameter (69 ± 8 mm versus 64 ± 6 mm, P<0.05) than patients with negative TWA. Other variables, including age, gender, New York Heart Association functional class, presence of bundle branch block, arrhythmias on 24‐hour ambulatory electrocardiogram, heart rate variability and baroreflex sensitivity, were not significantly different between patients with positive vs negative TWA. The prognostic significance of TWA in IDC with regard to arrhythmic events and total mortality will be determined by multivariate Cox analysis at the end of a 5‐year follow‐up in this ongoing study.

Holter monitoring was performed in 202 patients with idiopathic dilated cardiomyopathy, which revealed accelerated idioventricular rhythm in 16 patients (8%) and nonsustained ventricular tachycardia in 70 patients (35%). During 32 +/- 15-month prospective follow-up, no significant difference was observed for major arrhythmic events and transplant-free survival between patients with and without accelerated idioventricular rhythm, whereas patients with nonsustained ventricular tachycardia had a significantly higher incidence of major arrhythmic events and a lower transplant-free survival rate.

In recent years, evaluation of cardiac autonomic activity by means of heart rate variability (HRV) determination and baroreflex sensitivity (BRS) testing has become readily available. The results of the ATRAMI (Autonomic Tone and Reflexes After Myocardial Infarction) study showed that both diminished HRV and baro-reflex sensitivity are associated with poor outcome in patients after myocardial infarction. In contrast to patients with coronary disease little information is available concerning cardiac autonomic activity in idiopathic dilated cardiomyopathy (IDC). Therefore, HRV and BRS were assessed in 160 patients with IDC and preserved sinus rhythm in order to investigate the relationship between HRV, BRS, and left ventricular ejection fraction. Time domain indices of HRV were computed from 24-hour digital Holter recordings. BRS testing was performed using the noninvasive phenylephrine method. Mean standard deviation of all normal RR intervals (SDNN) of the whole study population was 112 +/- 46 ms. A well preserved HRV (SDNN > 105 ms) was found in 74 patients (46%), a moderately decreased HRV (SDNN 70-105 ms) in 59 patients (37%), and a severely decreased HRV (SDNN < 70 ms) in 27 patients (17%). Mean BRS was 7.5 +/- 5.0 ms/mm Hg. A well preserved BRS (> 6 ms/mm Hg) was present in 78 patients (57%), a moderately decreased BRS (3-6 ms/mm Hg) was present in 38 patients (28%), and a severely decreased BRS (< 3 ms/mm Hg) in 21 patients (15%). There was only a weak correlation between SDNN and BRS (r = 0.19; p < 0.05). A weak correlation was found for SDNN and left ventricular ejection fraction (r = 0.29; p < 0.05). There was no significant correlation between BRS and left ventricular ejection fraction (r = 0.14). In summary, there was only a weak correlation between the HRV, BRS, and left ventricular ejection fraction in patients with IDC suggesting that these 3 variables may be independent predictors of sudden death in IDC. The relative prognostic value of these variables and other potential risk predictors including the presence of arrhythmias on Holter, microvolt T wave alternans, QTc dispersion, and signal-averaged ECG is currently under investigation in a large prospective observational study (Marburg Cardiomyopathy Study (MACAS)) during 5-year follow-up at our institution.

This report describes a post-infarct patient with recurrent ventricular fibrillation in the absence of acute ischaemia, in whom arrhythmia recurrences could be prevented by ventricular rate stabilization of a third-generation cardioverter defibrillator. Review of the literature and clinical implications are discussed.

Background: In contrast to postinfarct patients, little is known about cardiac autonomic tone and its relation to spontaneous ventricular tachyarrhythmias in idiopathic dilated cardiomyopathy (IDC). Both heart rate variability (HRV) and baroreflex sensitivity (BRS) are indices of autonomic innervation of the heart.Hypothesis: The aim of the present study was to determine the relation between cardiac autonomic tone assessed by HRV and BRS and spontaneous nonsustained ventricular tachycardia (NSVT) on Holter in a large patient population with IDC.Methods: 24-h digital Kelter recordings including HRV analysis and BRS testing were prospectively performed in 137 patients with IDC and preserved sinus rhythm Mean age was 48 +/- 12 years, and mean left ventricular (LV) ejection fraction was 32 +/- 9%. The HRV analysis on Holter included the mean RR interval (RRm), the standard deviation of all normal RR intervals (SDNN), the square root of the mean of the squared differences between adjacent normal RR intervals (rMSSD), and the proportion of adjacent normal RR intervals differing more than 50 ms (pNN50). Testing for BRS was performed noninvasively using the phenylephrine method.Results: Of 137 study patients, 32 (31%) had spontaneous NSVT on 24-h Kelter. compared with patients without NSVT, patients with NSVT on Holter had a higher New York Heart Association (NYHA) functional class (HYHA III: 40 vs. 18%, p<0.01), a lower ejection fraction (29 +/- 9 vs. 34 +/- 9%, p = 0.01), and an increased LV end-diastolic diameter (69 +/- 8 mm vs. 66 +/- 7 mm, p = 0.03). The HRV variables rMSSD, pNN50, RRm, and BRS did not differ significantly between patients with and without spontaneous NSVT. Only SDNN on Holter was slightly lower in patients with versus without NSVT(106 +/- 45 vs. 121 +/- 46 ms, p = 0.08).Conclusions: Patients with IDC and spontaneous NSVT on Holter are characterized by a higher NYHA functional class, a lower LV ejection fraction, an increased LV end-diastolic diameter, and a tendency toward a lower SDNN value compared with patients without NSVT. The remaining measures of HRV including rMSSD and pNN50 reflecting primarily tonic vagal activity, as well as BRS reflecting predominantly reflex vagal activity, were similar in patients with and without NSVT The prognostic significance of these findings in patients with IDC is currently under investigation in the Marburg Cardiomyopathy Study (MACAS) at our institution.

Bei Patienten nach Myokardinfarkt wurde in der kürzlich veröffentlichten ATRAMI-Studie (Autonomic Tone and Reflexes After Myocardial Infarction) eine erhöhte Mortalität bei erniedrigter Herzfrequenzvariabilität (HRV) und eingeschränkter Barorezeptor-Sensitivität nachgewiesen. Im Gegensatz zur koronaren Herzerkrankung gibt es derzeit wenige Daten zur kardialen autonomen Aktivität bei dilatativer Kardiomyopathie (DCM). Im Rahmen einer 1996 begonnenen, prospektiven Verlaufsbeobachtung untersuchten wir deshalb den Zusammenhang zwischen HRV, Baroreflex-Sensitivität und linksventrikulärer Ejektionsfraktion bei 160 Patienten mit DCM und erhaltenem Sinusrhythmus. Die HRV wurde aus digitalen 24-h-Langzeit-EKG Registrierungen analysiert. Die Barorezeptor-Sensitivität wurde nichtinvasiv mit der Phenylephrin-Methode bestimmt.

Background To date, considerable controversy exists regarding noninvasive arrhythmia risk stratification in idiopathic dilated cardiomyopathy (IDC). Methods and Results Between 1992 and 1997, 202 patients with IDC without a history of sustained ventricular tachycardia (VT) underwent echocardiography, signal-averaged electrocardiogram (ECG), and 24-hour Holter ECG in the absence of antiarrhythmic drugs. During 32 ± 15 months of prospective follow-up, major arrhythmic events, including sustained VT, ventricular fibrillation, or sudden death, occurred in 32 (16%) of 202 patients. After adjusting for baseline medical therapy and antiarrhythmic therapy during follow-up, multivariate Cox regression analysis identified a left ventricular (LV) end-diastolic diameter ≥70 mm and nonsustained VT on Holter as the only independent arrhythmia risk predictors. The combination of an LV end-diastolic diameter ≥70 mm and nonsustained VT was associated with a 14.3-fold risk for future arrhythmic events (95% confidence interval 2.3-90). To further elucidate the prognostic value of LV ejection fraction, multivariate Cox analysis was repeated with ejection fraction forced to remain in the model. In the latter model, an ejection fraction ≤30% combined with nonsustained VT on Holter was found to be a significant arrhythmia risk predictor with a relative risk of 14.6 (95% confidence interval 2.2-97). Conclusions The combination of an LV end-diastolic diameter ≥70 mm and nonsustained VT on Holter, and the combination of LV ejection fraction ≤30% and nonsustained VT on Holter, identify a subgroup of patients with IDC with a 14-fold risk for subsequent arrhythmic events. These findings have important implications for the design of future studies evaluating the role of prophylactic defibrillator therapy in patients with IDC. (Am Heart J 2000;140:43-51.)

Twenty-nine patients in whom severe bradyarrhythmias occurred exclusively during obstructive sleep apnea and in whom advanced sinus node disease or atrioventricular conduction system dysfunction had been excluded by invasive electrophysiologic evaluation were prospectively followed on nasal continuous positive airway pressure. During 54 +/- 10 months follow-up, no syncope and no sudden deaths were observed, suggesting that patients with sleep apnea-associated bradyarrhythmias and a normal electrophysiologic study appear to have a favorable prognosis with continuous positive airway pressure.

The efficacy of RF energy versus the neodymium:yittrium aluminum‐garnet laser to create linear lesions was compared in fresh ex vivo swine hearts. A total of 598 lesions were created in four locations: ostium of the pulmonary veins, trabeculated lateral left atrium, smooth posterior part of the right atrium, and the isthmus between the inferior vena cava and tricuspid valve. A 400‐μm bare quartz fiber with CO2 cooling (distance to the tissue 5, 10, and 15 mm) and an RF ablation catheter (4‐mm tip) were mechanically dragged over the tissue at speeds 0.5, 1.0, and 1.5 mm/s. A continuous and transmural ablation line was recorded as successful. A 100% success rate was achieved at the pulmonary veins and the isthmus at some settings of energy delivery by the laser and RF. In the thick posterior right atrium, RF resulted in transmural lesions only when associated with carbonization, while the laser produced successful ablation lines in 100% of the attempts. In the left atrium, because of the presence of prominent trabeculations, RF was unsuccessful at all settings of energy delivery. In contrast, deep photocoagulation by laser resulted in successful ablations in the left atrium in 100% of attempts. Lesion formation was faster by laser ablation and mean lesion width was at least 25% smaller with the laser than with RF. In conclusion, the formation of linear lesions at the isthmus and at the pulmonary veins was successful with the laser and RF. In the trabeculated left atrium and the thick posterior right atrium, only laser ablation was successful.

In recent years, evaluation of cardiac autonomic activity by means of heart rate variability (HRV) determination and baroreflex sensitivity (BRS) testing has become readily available. The results of the ATRAMI (Autonomic Tone and Reflexes After Myocardial Infarction) study showed that both diminished HRV and baroreflex sensitivity are associated with poor outcome in patients after myocardial infarction. In contrast to patients with coronary disease little information is available concerning cardiac autonomic activity in idiopathic dilated cardiomyopathy (IDC). Therefore, HRV and BRS were assessed in 160 patients with IDC and preserved sinus rhythm in order to investigate the relationship between HRV, BRS, and left ventricular ejection fraction. Time domain indices of HRV were computed from 24-hour digital Holter recordings. BRS testing was performed using the noninvasive phenylephrine: method. Mean standard deviation of all normal RR intervals (SDNN) of the whole study population was 112 +/- 46 ms, A well preserved HRV (SDNN > 105 ms) was found in 74 patients (46%), a moderately decreased HRV (SDNN 70-105 ms) in 59 patients (37%), and a severely decreased HRV (SDNN < 70 ms) in 27 patients (17%). Mean BRS was 7.5 +/- 5.0 ms/mm Hg. A well preserved BRS (> 6 ms/mm Hg) was present in 78 patients (57%), a moderately decreased BRS (3-6 ms/mm Hg) was present iu. 38 patients (28%), and a severely decreased BRS (< 3ms/mm Hg) in 21 patients (15%). There was only a weak correlation between SDNN and BRS (r = 0.19; p < 0.05). A weak correlation was Found for SDNN and left Ventricular ejection fraction (r = 0,29: p < 0.05). There was no significant correlation between BRS and left ventricular ejection fraction (r = 0,14). In summary, there was only a weak correlation between the HRV, BRS, and left ventricular ejection fraction in patients with IDC suggesting that these 3 variables may be independent predictors of sudden death in IDC. The relative prognostic value of these variables and other potential risk predictors including the presence of arrhythmias on Holter, microvolt T wave alternans, QTc dispersion, and signal-averaged ECG is currently under investigation in a large prospective observational study (Marburg Cardiomyopathy Study (MACAS)) during 5-year follow-up at our institution.

Time domain heart rate variability measurements and echocardiographic studies were performed in 35 patients with carcinoid syndrome. Carcinoid heart disease was present in 18 patients (51%). Heart rate variability parameters (standard deviation of all normal RR intervals, percentage of the number of pairs of adjacent normal RR intervals differing by >50 ms) were significantly reduced in patients with than in those without carcinoid heart disease.

Torsades de Pointes after PJRT Ablation. Introduction: Catheter ablation with radiofrequency energy is a curative therapy in patients with permanent junctional reciprocating tachycardia (PJRT). Methods and Results: For the first time, we report a case of transient QT prolongation with torsades de pointes tachycardia 18 hours after successful radiofrequency energy ablation of PJRT in a 25‐year‐old woman with tachycardia‐induced cardiomyopathy. Of note, the torsades de pointes occurred in the absence of bradycardia, electrolyte disturbances, or QT‐prolonging drugs. This patient initially was thought to have a hereditary long QT syndrome that was unmasked by PJRT ablation. Therefore, the patient received an implantable defibrillator in addition to beta‐blocker therapy, which was discontinued 6 months later. Surprisingly, the QT interval completely normalized within 1 week after PJRT ablation, and the patient remained free of arrhythmias during a follow‐up period of 4.5 years. Conclusion: Patients with incessant tachyarrhythmias should undergo ECG monitoring for at least 24 hours following successful radiofrequency catheter ablation because transient QT prolongation with torsades de pointes may occur even in the absence of bradycardia, QT‐prolonging drugs, or electrolyte disturbances.

To determine the incidence of complications of third-generation implantable cardioverter defibrillator (ICD) therapy, 144 patients were prospectively studied who underwent first implant of third-generation devices (i.e., ICD systems with biphasic shocks, ECG storage capability, and nonthoracotomy lead systems). During 21 +/- 15 months of follow-up, 41 (28%) patients had one or more complications. No patient died perioperatively (30 days) and no ICD infection was observed during follow-up. Complications included bleeding or pocket hematoma (hemoglobin drop > 2 g/dL) in 5 (3%) patients, prolonged reversible ischemic neurological deficit in 1 (1%) patient, postoperative deep venous thrombosis of leg in 1 (1%) patient, pneumothorax in 2 (1%) patients, difficulty to defibrillate ventricular fibrillation intraoperatively in 2 (1%) patients, generator malfunction in 1 (1%) patient, arthritis of the shoulder in 3 (2%) patients, and allergic reaction to prophylactic antibiotics in 2 (1%) patients. A total of seven lead related complications were observed in six (4%) patients including endocardial lead migration in four (3%) patients. Twenty-three (16%) patients received inappropriate shocks for supraventricular tachyarrhythmias (n = 13), non-sustained ventricular tachycardia (VT) (n = 7), or myopotential oversensing (n = 3). We conclude that serious complications such as perioperative death or ICD infection are rare in patients with third-generation ICDs. Lead-related problems and inappropriate shocks during follow-up are the most frequent complications of third-generation ICD therapy. Recognition of these complications should promote advances in ICD technology and management strategies to avoid their recurrence.