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Combined cardiomyopathy and skeletal myopathy: a variant with atrial fibrillation and ventricular tachycardia.

This article describes a family characterized by combined cardiomyopathy and nonspecific skeletal myopathy who present in the third to fifth decades with cardiac manifestations but earlier have evidence of subtle skeletal muscle dysfunction. They differ from previously defined syndromes and potentially represent a different genetic expression or mutation. Cardiomyopathy presents with atrial arrhythmias including AF and atrial flutter. Life-threatening ventricular tachyarrhythmias occur next with onset of ventricular dysfunction. Electrophysiological study revealed sustained monomorphic VT. Affected family members benefitted from an ICD and progression to congestive heart failure (CHF) occurred late. Skeletal myopathy continues with marked progressive muscle weakness and inability to ambulate without assistance. Genetic analysis is currently ongoing. Neurological evaluation in all three family members revealed nonspecific myopathy affecting the psoas and iliopsoas muscles. Atrophy and wasting of the facial and temporalis muscles were common. Skeletal muscle biopsy revealed myofiber atrophy consistent with myopathy.

Atrial Fibrillation↗

Management of atrial flutter.

Atrial flutter is a macroreentrant arrhythmia that is associated with cardiovascular and pulmonary disease. In the United States, 200,000 new cases of atrial flutter can be expected to develop every year with a male to female ratio of over 2:1. This arrhythmia is associated with atrial fibrillation in over half the cases. It is also associated with an increased risk of thromboembolic complications, but less than that is seen with atrial fibrillation. The most common form of atrial flutter involves a large reentrant circuit within the right atrium, encircling the tricuspid annulus. Other, less common forms of atrial flutter may involve other anatomic barriers, atriotomy scars, and infarcted areas of the atria. Treatment of atrial flutter often involves electrical cardioversion and/or antiarrhythmic medications. Type I and Type III antiarrhythmic drugs are often used to terminate or prevent recurrent episodes and Type II (beta-blockers) and Type IV (calcium channel blockers) can be used to control the ventricular rate during atrial flutter. However, antiarrhythmic drugs alone control atrial flutter in only 50% to 60% of patients. Since the early 1990s, radiofrequency catheter ablation has been used to interrupt the reentrant circuit and prevent recurrences of atrial flutter. Radiofrequency ablation is acutely successful in over 90% of cases and avoids the long-term toxicity seen with antiarrhythmic drugs. Advanced mapping techniques and newer methods of delivering the radiofrequency lesions are being used to delineate unusual forms of atrial flutter and to minimize fluoroscopic exposure during the procedure.

Anti-Arrhythmia Agents↗

Mitral valve prolapse, atrial flutter, and syncope in a young female patient.

BACKGROUND: The syndrome of mitral valve prolapse (MVP) is the most common form of valvular heart disease. CASE REPORT: The case of a 16-year-old girl with mitral valve prolapse, atrial flutter and syncope is presented. The patient was admitted to the clinic complaining of atypical chest pain, palpitations, breathlessness at physical efforts, fatigue, and a feeling of fogginess. Electrocardiogram showed the presence of the common-type atrial flutter with 3:1 ventricular responses. Echocardiography showed mitral valve prolapse with mild mitral regurgitation. The patient reported to have had these symptoms for about nine months. She was not aware of heart disease before. CONCLUSIONS: A young female patient with combined mitral valve prolapse, atrial flutter, and syncope is presented.

Adolescent↗

Improvement of ventricular function after atrioventricular junction ablation in a patient with atrial flutter after Mustard's operation.

Arrhythmias are common problems following Mustard's operation for D-transposition of the great arteries. A 19-year-old male is presented who was diagnosed at birth with D-transposition of the great arteries and a right aortic arch and underwent a Mustard procedure at 12 months of age. He developed sinus node dysfunction and atrial flutter unresponsive to antiarrhythmic drugs and dual chamber pacing. Following complete heart block with radiofrequency catheter ablation of the atrioventricular junction, the patient's heart was paced in VVIR mode. Ventricular function improved after the ablation and persisted in two years' follow-up.

Adult↗

Risk of developing life-threatening ventricular arrhythmia associated with tefenadine in comparison with over-the-counter antihistamines, ibuprofen and clemastine.

An observational, historical cohort evaluation was performed to examine the hypothesis that terfenadine (Seldane) exposure increases the risk of developing life-threatening ventricular arrhythmias. The study population consisted of Medicaid recipients from 4 states that were included in the Computerized On-Line Medical Pharmaceutical Analysis and Surveillance System (COMPASS). The drug exposure period was defined prospectively as 30 days in all treatment cohorts. The primary end point was the development of life-threatening ventricular arrhythmias (ventricular tachycardia, fibrillation and flutter, and cardiac arrest and sudden death). The comparison cohorts included terfenadine (n = 181,672), over-the-counter antihistamines (n = 150,689), ibuprofen (n = 181,672) and clemastine (Tavist; n = 83,156). Over the exposure period, a total of 317 life-threatening ventricular arrhythmic events occurred, 244 of which were cardiac arrests. The incidence of total life-threatening ventricular arrhythmic events and cardiac arrests were more frequent in patients receiving over-the-counter antihistamines (relative risk 0.36) than in those receiving terfenadine, a finding that was consistent across all subgroups. There was no increased risk of life-threatening ventricular arrhythmias in the terfenadine cohort as compared with the ibuprofen cohort (relative risk 0.62), and in some analyses, the ibuprofen cohort had a significantly higher arrhythmic event rate. In all comparisons with the clemastine cohort, the terfenadine cohort had a statistically indistinguishable relative risk (1.08). Age, race, sex and cardiovascular risk were all considered in the adjusted relative-risk analyses. No baseline historical characteristic or imbalance of baseline medications explained the differences between groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Inconsistent response to rapid atrial rhythms in a DDD pacemaker with the fallback feature.

Dual chamber pacing may be problematic in patients with paroxysmal atrial arrhythmias. The fallback feature has been incorporated into some devices to avoid tracking of rapid atrial rhythms. A case is reported in which the initiation of fallback was inconsistent during paroxysms of atrial flutter. Evaluation of the ventricular output of an isolated device in response to various applied atrial rates revealed that fallback occurred consistently only when 2xTARP (ms) < or = URL (ms).

Aged↗

Sotalol infusion in the treatment of supraventricular tachyarrhythmias.

Sotalol, a noncardioselective beta-adrenergic blocking agent without partial agonist activity, has class-3 antiarrhythmic effects. Twenty-nine patients with supraventricular tachyarrhythmias were treated on 31 occasions to determine the effective therapeutic dosage and optimal method of administration. Fifty percent of patients with paroxysmal supraventricular tachycardia, 33 percent of those with atrial flutter, and 20 percent with atrial fibrillation were restored to sinus rhythm by bolus administration; and when sotalol was given by continuous infusion, sinus rhythm was obtained in 100 percent, 86 percent, and 33 percent, respectively. Comparable dosages were given to both responders and nonresponders in the bolus-treated group and higher dosages to nonresponders than to responders in those treated by infusion. Furthermore, plasma levels in nonresponders tended to be higher than in responders. When responders alone were considered, the average dose required was 0.630 +/- 0.090 mg/kg of body weight (+/- SE), and the plasma level obtained was 0.526 +/- 0.088 microgram/ml. No relationship between plasma levels and therapeutic effectiveness was shown. No serious side effects were observed at the dosages used. These data suggest that while no concordance with plasma levels was observed, sotalol given by infusion is an effective therapy for atrial flutter and supra-ventricular tachycardia. It is less effective in atrial fibrillation, where less than half were responders, but a satisfactory reduction in ventricular rate response was obtained. A controlled trial to further substantiate these findings is indicated.

Adult↗

[Successful control of rapid heart rate with atrial flutter by intravenous administration of esmolol in a patient after total correction of the tetralogy of Fallot].

A 30-year-old woman with atrial flutter after surgical correction of tetralogy of Fallot, underwent gynecological procedure under general anesthesia. Because she had been noted to have atrial flutter and heart failure at 8 weeks' gestation, she was scheduled for dilatation and curettage. Chest X-ray film showed cardiomegaly and pulmonary congestive changes. ECG showed atrial flutter with 3:2 atrio-ventricular conduction rate and complete right branch block. She was anesthetized with propofol infused with target-controlled infusion system, fentanyl and 66% of nitrous oxide under close monitoring and appropriate respiratory management. The quantity of hemorrhage was about 850 ml, and hypovolemia was treated with volume infusion and the use of vasoactive drugs. Soon after emergence from anesthesia, atrial flutter with 1:1 A-V conduction (> 230 bpm) occurred suddenly. Esmolol hydrochloride, 30 mg, was administered. Despite the relatively low doses, rapid control of heart rate was possible in a few minutes and the atrial flutter returned to 2:1 conduction. Although atrial flutter had continued until the discharge, tachyarrhythmia was no longer observed and the heart resumed sinus rhythm 3 month after the operation. The present case suggests that esmolol can be used effectively and safely for controling atrial flutter with rapid ventricular response in a patient after surgical correction of tetralogy of Fallot.

Adrenergic beta-Antagonists↗

Improvement in left ventricular systolic function after successful radiofrequency His bundle ablation for drug refractory, chronic atrial fibrillation and recurrent atrial flutter.

Incessant supraventricular tachyarrhythmia may lead to a reversible impairment of left ventricular (LV) function. This issue was investigated in 10 patients (aged 64 +/- 13 years) who underwent radiofrequency His bundle ablation for control of drug refractory, chronic atrial fibrillation (n = 9) and recurrent atrial flutter (n = 1). LV function was assessed by 2-dimensional guided M-mode echocardiography within 24 hours (baseline) and 49 +/- 18 days (follow-up) after successful ablation, both during VVI pacing at 70 beats/min. Fractional shortening increased from 28 +/- 9% at baseline to 35 +/- 8% at follow-up (p = 0.006). This increase in fractional shortening was due to a significant reduction of end-systolic diameter from 41 +/- 10 to 36 +/- 10 mm (p = 0.02), whereas there was no appreciable change in end-diastolic diameter (56 +/- 7 to 55 +/- 10 mm; p = 0.5). These changes were substantially greater in patients with baseline impairment of LV function (fractional shortening less than 27%). Fractional shortening increased by 12% (p = 0.14) in patients with normal LV function (n = 5) and by 44% (p = 0.02) in those with impaired LV function at baseline (n = 5). The greater increase in fractional shortening in patients with preexisting LV impairment was due to a more pronounced decline in end-systolic dimensions (-11.9%; p = 0.08) compared with that of patients with normal LV function at baseline (-9.21%; p = 0.2). End-diastolic diameter showed no significant change in either group (-3.53% [p = 0.8] and -0.58% [p = 0.4]).(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Effects of intravenous adenosine on antegrade refractoriness of accessory atrioventricular connections.

BACKGROUND: Several groups have suggested the use of intravenous adenosine or adenosine triphosphate in the diagnosis of regular broad complex tachycardias. However, the short half-life of these agents has precluded assessment of their effects on refractoriness of accessory connections, and their safety in preexcited arrhythmias has not been demonstrated. METHODS AND RESULTS: We examined the effects of intravenous adenosine on accessory atrioventricular (AV) connections in 30 patients with the Wolff-Parkinson-White syndrome. Intravenous adenosine (12 mg, rapid bolus) was administered to 14 patients (group 1) during continuous atrial pacing at a cycle length 20 msec below that required to cause 2:1 conduction block in the accessory connection (mean pacing cycle length 261 +/- 41 msec). After adenosine, transient 1:1 conduction occurred via the accessory connection in 12 of 14 patients, indicating a shortening of antegrade refractoriness. In three of seven patients, this effect was abolished after intravenous propranolol (0.2 mg/kg). Nineteen patients (group 2) received adenosine (0.17 +/- 0.04 mg/kg) during induced, preexcited atrial arrhythmias. The minimum RR interval during preexcited atrial fibrillation transiently decreased (252 +/- 44 msec to 224 +/- 35 msec, p less than 0.01) after adenosine, but no change in average RR interval was observed (360 +/- 59 msec to 357 +/- 60 msec, NS). The preexcited ventricular response to atrial flutter was transiently accelerated in five of eight patients (415 +/- 21 msec to 360 +/- 49 msec, p less than 0.05) due to shortening of flutter cycle length (207 +/- 10 msec to 180 +/- 24 msec, p less than 0.05). However, 2:1 accessory connection conduction was maintained in all eight patients. All effects were short lived, with the decrease in RR interval during atrial fibrillation occurring for a maximum of two RR intervals only. No patient suffered ventricular arrhythmias or hemodynamic deterioration. CONCLUSIONS: Adenosine shortens antegrade refractoriness of accessory AV connections, and in some patients this action is mediated by beta-adrenergic stimulation. Adenosine may cause acceleration of preexcited atrial arrhythmias, but these effects are transient and should not discourage the use of adenosine as a diagnostic agent in broad complex, regular tachycardias of uncertain origin.

Adenosine↗

Electrophysiological profile of bepridil, a new anti-anginal drug with calcium blocking properties.

The electrophysiological effects of intravenously administered bepridil, a new anti-anginal drug with slow calcium current blocking properties, were studied in 20 patients undergoing an endocavitary cardiac exploration for various reasons. Classical techniques of programmed electrical stimulation were used. Bepridil markedly increased the refractory period of the atrium, atrioventricular node and the AH-interval. The effects on HR and ventricular refractory period were minimal, and sinus rate decreased slightly. A pronounced reduction in the heart rate at which the Wenckebach phenomenon was obtained in the anterograde direction (16%) and particularly in the retrograde direction (26%) was observed. During this study the drug appeared beneficial in suppressing atrial flutter, atrial extrasystoles, and ventricular extrasystoles. In two patients with intranodal reciprocal rhythm, the injection of bepridil terminated the tachycardia which could not be reinitiated later. No side-effects occurred and it appears that bundle branch block and prolonged HV interval are not contraindications to the use of bepridil.

Adult↗

[Drug therapy of cardiac arrhythmias 199].

Current antiarrhythmic drug therapy is employed in strict compliance with the proper indication, which is itself limited by the side effects of the available drugs, and modified by the increasing success of such non-drug options as electrical ablation and implantable cardioverters/defibrillators. Drug treatment is restricted to 3 major indications, regular paroxysmal supraventricular tachycardias, atrial flutter and fibrillation, and ventricular tachycardias. Class IA and IC agents are used only to treat supraventricular and ventricular arrhythmias with no structural heart disease. Class III drugs, e.g. sotalol and in particular amiodarone are used preferentially to treat the pre-damaged heart, in particular left-ventricular functional impairment and in coronary heart disease in consideration of their side effects. In the case of the most common treatment-requiring arrhythmia-atrial fibrillation-anticoagulation alone may be indicated. For the prevention of sudden death, beta-blockers continue to be the drugs of first choice.

Anti-Arrhythmia Agents↗

[Paroxysmal atrial arrhythmias in arrhythmogenic cardiomyopathy of the right ventricle with a familial character: the role of right atrial involvement].

Arrhythmogenic right ventricular cardiomyopathy presents a rather frequent familial occurrence. In the present study the cases of two brothers (25 and 30 years old, respectively) presenting with the peculiar finding of early involvement of right atrium, are described. Both subjects were investigated because of recurrent complex arrhythmias, including supraventricular (sustained atrial flutter, atrial tachycardia) and ventricular tachyarrhythmias (very frequent premature beats, nonsustained ventricular tachycardia). The associated echo-angiographic aspects of the right atrium and right ventricle were those typically described in arrhythmogenic right ventricular cardiomyopathy. The familial occurrence of simultaneous right atrial and ventricular involvement, to the best of our knowledge not previously described, can likely be due to the similar phenotypic expression of the same genotype. The prognostic significance of atrial involvement in right ventricular arrhythmogenic dysplasia is still to be defined and the clinical follow-up of these two cases may contribute to it.

Adult↗

Double tachycardia following surgery for recurrent ventricular arrhythmias.

Simultaneous accelerated "junctional" rhythm and atrial flutter were observed postoperatively in a patient who had undergone electrophysiologic surgery for recurrent ventricular fibrillation. Digitalis toxicity was excluded, based on the low serum level of the drug and on the recurrence of the tachycardia after withdrawal of digitalis. While atrial flutter probably represented a postoperative recurrence of a clinically occurring arrhythmia, the accelerated "junctional" rhythm at unusually fast rates most likely developed as a consequence of a cryolesion applied to the ventricular septum as part of the surgical treatment. Phenytoin proved effective in suppressing this "junctional" tachycardia.

Aged↗

Malignant cardiac involvement in a family with myotonic dystrophy.

BACKGROUND: In patients with myotonic dystrophy, histopathological and electrophysiologic abnormalities of cardiac conduction system may lead to sudden cardiac death due to atrioventricular block or to ventricular electrical instability. METHODS: Four members of a family affected by myotonic dystrophy are reported, which underwent a cardiological examination including invasive electrophysiological study and prolonged follow-up. Other 3 members of the same family had died suddenly. No clinical data are available for 2 of these patients, while paroxysmal atrial flutter and non sustained ventricular tachycardia had been detected at Holter in the third one. RESULTS: Signs of atrioventricular conduction impairment, poorly predictable with non invasive electrocardiography, were found in the 4 patients undergoing intracardiac electrophysiologic study. In 2/4 patients, both having dizzy spells and the most impaired atrioventricular conduction, a pace-maker was implanted. Polymorphic, non sustained ventricular response was induced in 2/4 patients, 1 of them with spontaneous high grade ventricular arrhythmias. CONCLUSIONS: The respective role of atrioventricular conduction impairment and ventricular vulnerability in determining sudden death has not been stated so far in these patients. The observed polymorphic non sustained response should not be "a priori" disregarded as aspecific, since it could be the electrophysiological counterpart of a peculiar anatomic arrhythmogenic substrate. A comprehensive study, including invasive electrophysiology, is advisable in all patients with myotonic dystrophy whenever a member of their family presents with cardiac involvement, to assess the most probable life-threatening arrhythmogenic mechanism.

Adolescent↗

Electrophysiological actions of amrinone in dogs with cardiac lesions.

We examined the actions of amrinone in five models using dogs to determine under what circumstances intravenous amrinone might exert arrhythmogenic or antiarrhythmic properties. In dogs with 24-h post-coronary artery ligation arrhythmias, amrinone, given at incrementally increasing doses of 1.5, 3.0, and 6.0 mg/kg at 30-min intervals, produced significant increases of cardiac contractility without altering the severity of the arrhythmia. In dogs with 2- to 6-day-old ischemic lesions and 90-100% sinus beats, a bolus dose of 3.0 mg/kg amrinone was followed by an increased incidence of abnormal beats (p = 0.013); neither 1.5 nor 6.0 mg/kg caused a significant incidence of arrhythmias. Acute occlusion of the left anterior descending coronary artery followed by reperfusion caused fibrillation in nine of 15 control dogs and two of 14 dogs treated with 2.3 mg/kg amrinone. This difference was significant at the level p less than 0.05. In ouabain-intoxicated dogs, amrinone at 1.0 and 3.0 mg/kg neither worsened nor improved the arrhythmias. In the atrial circus flutter arrhythmia, amrinone increased ventricular heart rate by a significantly greater amount than it increased atrial rate, suggesting that amrinone facilitates atrioventricular conduction.

Aminopyridines↗

[A-V conduction in atrial fibrillation and flutter].

The assessment of A-V conduction in the presence of atrial fibrillation is based upon analysis of the R-R intervals. This is because in atrial fibrillation it is impossible both to identify the impulse that has been conducted to the ventricles, and to measure the A-V conduction time. The first step is, therefore, to evaluate whether the QRS complexes are the expression of conducted atrial impulses, or they are A-V junctional or ventricular in origin. In other words, it is necessary to distinguish between A-V conduction and A-V dissociation. Conduction in atrial fibrillation commonly results in irregular R-R cycles, whereas in the presence of dissociation the R-R cycles are mainly regular. This differentiation can be difficult in the presence of: aberrant conduction; A-V conduction disturbances; or A-V junctional tachycardia with anterograde 2nd degree exit block. The problem occurs both with tachycardia-dependent (or phase 3), and with bradycardia-dependent (or phase 4) aberrant conduction. Distinction between aberration and ectopy is helped by: the sequence long cycle-short cycle; the pause that follows the wide QRS complex; the configuration of the wide QRS complex. Since aberrant conduction may be sustained, due to the linking phenomenon, the pattern may mimick ventricular tachycardia. In atrial flutter the atrial electrical activity is far less chaotic than in atrial fibrillation, so that assessment of A-V conduction is less difficult. Nevertheless, it is impossible to determine exactly which out of the atrial impulses has been conducted, due to the extremely fast atrial rate: the conducted impulse, indeed, is not always the one that immediately precedes the QRS complex. Furthermore, it is also difficult to measure the A-V conduction time, because the F waves follow to each other without any interruption, so that it is impossible to define exactly the beginning of atrial activation. In atrial flutter, thus, as well as in atrial fibrillation, A-V conduction may be assessed by analysis of the R-R intervals, apart from measurement of F-R intervals. In the absence of drugs, atrial flutter is usually associated with 2:1 (or, less frequently, 4:1) conduction ratio, being the odd ratios (3:1, 5:1) far more rare. Due to concealed penetration of non-conducted impulses, A-V conduction intervals are often variable, so that the R-R cycles are irregular even in the presence of a constant A-V conduction ratio. The most common mechanisms leading to irregularity are the alternation of A-V conduction times, and the alternating Wenckebach phenomenon.(ABSTRACT TRUNCATED AT 400 WORDS)

Atrial Fibrillation↗

Transcatheter ablation of cardiac tissue: advantages and disadvantages of different ablative techniques.

Transcatheter ablation techniques are emerging as an alternative therapeutical tool in the management of cardiac arrhythmias. Catheter ablation was initially introduced as the last resort to ablate the atrioventricular nodal conduction in patients with atrial fibrillation and uncontrolled ventricular response and in patients with drug refractory ventricular tachycardias. Direct current energy was used as the sole source of energy, but because of potential significant complications and early and late mortality, presumably mostly due to ventricular tachyarrhythmias, other sources of energy were sought. Radiofrequency current which does not produce barotrauma and does not require general anesthesia rapidly replaced direct current ablation in many centers. Early results with radiofrequency current ablation of the atrioventricular node and accessory atrioventricular pathways are very encouraging. The results of radiofrequency as well as direct current ablation for atrial flutter, atrial tachycardia and ventricular tachycardia, where the components of reentry circuit are less defined, are not as favorable as those of AV junctional tachycardias. However, improvement of catheter and generatory technology and better understanding of the mechanisms of ventricular tachycardias and characteristics of the target site will enhance the results of catheter ablation in ventricular tachcardias. The procedures are still considered investigational, and mostly done by very experienced groups at tertiary referral hospitals with surgical teams available in case of serious complications. Larger patient populations and longer follow-up periods are required before these techniques expand to community hospitals and to patients with minimal symptoms or asymptomatic individuals as a prophylaxis therapy.

Cardiac Catheterization↗