ajmaline and Syncope

A 31-year-old male patient presented with complaints of palpitations, dizziness, and recurrent episodes of syncope. A 12-lead electrocardiogram (ECG) revealed manifest ventricular preexcitation, which suggested Wolff Parkinson White syndrome. In addition, an incomplete right bundle branch block and a 3-mm ST segment elevation ending with inverted T-waves in V2 were consistent with coved-type (type 1) Brugada pattern. An electrophysiological study was performed, and during the mapping, the earliest ventricular activation with the shortest A-V interval was found on the mitral annulus posterolateral site. After successful radiofrequency catheter ablation of the accessory pathway, the Brugada pattern on the ECG changed, which prompted an ajmaline provocation test. A type 1 Brugada ECG pattern occurred following the administration of ajmaline. Considering the probable symptom combinations of these 2 coexisting syndromes and the presence of recurrent episodes of syncope, programmed ventricular stimulation was performed and subsequently, ventricular fibrillation was induced. An implantable cardioverter-defibrillator was implanted soon after.

Topics: Adult; Ajmaline; Animals; Anti-Arrhythmia Agents; Brugada Syndrome; Bundle-Branch Block; Catheter Ablation; Defibrillators, Implantable; Dizziness; Electrocardiography; Electrophysiologic Techniques, Cardiac; Humans; Male; Recurrence; Syncope; Ventricular Fibrillation; Wolff-Parkinson-White Syndrome

Topics: Aged; Ajmaline; Bundle-Branch Block; Clinical Trials as Topic; Electrocardiography; Female; Heart Conduction System; Humans; Injections, Intravenous; Male; Middle Aged; Syncope

Topics: Ajmaline; Brugada Syndrome; Flecainide; Humans; Sodium Channel Blockers; Syncope

We describe the case of a 52-year-old male patient with a recently diagnosed hereditary haemochromatosis who was referred to our electrophysiology laboratory due to presyncopal spells during physical exertion. The electrophysiological study unexpectedly revealed a sinus node dysfunction as well as a Brugada syndrome--both diagnosed on the grounds of an ajmaline test.

Topics: Ajmaline; Anti-Arrhythmia Agents; Brugada Syndrome; Electrocardiography; Heart Conduction System; Hemochromatosis; Humans; Male; Middle Aged; Sick Sinus Syndrome; Syncope

The aim of the present study was to elucidate the molecular mechanism underlying the concomitant occurrence of cardiac conduction disease and long QT syndrome (LQT3), two SCN5A channelopathies that are explained by loss-of-function and gain-of-function, respectively, in the cardiac Na+ channel.. A Caucasian family with prolonged QT interval, intermittent bundle-branch block, sudden cardiac death, and syncope was investigated. Lidocaine (1 mg/kg i.v.) normalized the prolonged QT interval and rescued bundle-branch block. An SCN5A mutation analysis was performed that revealed a C-to-A mutation at position 4859 (exon 28), predicted to change a highly conserved threonine for a lysine at position 1620. Mutant channels were characterized both in Xenopus oocytes and HEK293 cells. The T1620K mutation remarkably altered the properties of Nav1.5 channels. In particular, the voltage-dependence of the current decay time constants was largely lost. As a consequence, mutant channels inactivated faster than wild-type channels at potentials negative to -30 mV, resulting in less Na+ inward current (loss-of-function), but significantly slower at potentials positive to -30 mV, resulting in an increased Na+ inward current (gain-of-function). Moreover, we found a hyperpolarized shift of steady-state activation and an accelerated recovery from inactivation (gain-of-function). At the same time, channel availability was significantly reduced at the resting membrane potential (loss-of-function).. We conclude that lysine at position 1620 leads to both loss-of-function and gain-of-function properties in hNav1.5 channels, which may consequently cause in the same individuals impaired impulse propagation in the conduction system and prolonged QTc intervals, respectively.

Topics: Action Potentials; Adolescent; Adult; Ajmaline; Animals; Anti-Arrhythmia Agents; Bundle-Branch Block; Cell Line; Child; Death, Sudden, Cardiac; DNA Mutational Analysis; Electrocardiography; Female; Gene Transfer Techniques; Genetic Predisposition to Disease; Humans; Kinetics; Lidocaine; Long QT Syndrome; Lysine; Male; Muscle Proteins; Mutation; Myocardium; NAV1.5 Voltage-Gated Sodium Channel; Patch-Clamp Techniques; Pedigree; Sodium; Sodium Channels; Syncope; Threonine; Xenopus laevis

The typical Brugada ECG pattern consists of a prominent J-wave associated with ST-segment elevation localized in the right precordial leads V1-V3. In many patients, the ECG presents periods of transient normalization and the Brugada-phenotype can be unmasked by the administration of class-I antiarrhythmics. Reports have documented the heterogeneity of the Brugada syndrome ECG-phenotype characterized by unusual localization of the ECG abnormalities in the inferior leads. Case report A 51-year-old man, without detectable structural heart disease, was referred to us because of a history of syncope, dizziness, and palpitations. The ECG showed a J-wave and ST-segment elevation in the right precordial leads, suggesting Brugada syndrome. As other causes of the ECG abnormalities were excluded, the patient underwent an electrophysiological study that documented easy induction of ventricular fibrillation. During infusion of ajmaline, new prominent J-waves and ST-segment elevation appeared in the inferior leads, whereas the basal ECG abnormalities in the right precordial leads normalized. After infusion of isoprenaline, the ECG-pattern resumed the typical Brugada pattern. An implantable cardioverter-defibrillator was recommended.. In our patient, the double localization of the typical Brugada-pattern and the paradoxical effect of ajmaline on the ECG abnormalities confirmed the possibility of a phenotype heterogeneity in the Brugada syndrome.

Topics: Ajmaline; Anti-Arrhythmia Agents; Arrhythmias, Cardiac; Dizziness; Electrocardiography; Humans; Male; Middle Aged; Syncope; Syndrome

Sodium channel-blocking agents are routinely used to unveil the Brugada syndrome in patients in whom the typical electrocardiographic pattern is absent or doubtful. In this article, the authors report a patient with syncopal episodes of unknown origin in whom the conventional electrocardiographic result was normal and a negligibly small "saddle back" type repolarization was present in lead V2 recorded 2 intercostal spaces above the conventional site. Intravenous ajmaline (50 mg) did not elicit the type 1 pattern of the Brugada syndrome in the precordial leads obtained at their usual level, but a clear-cut coved-type repolarization was apparent in high right precordial leads. These findings indicate that high precordial leads should be routinely recorded while assessing the ajmaline test in patients suspected of having the Brugada syndrome.

Topics: Adult; Ajmaline; Anti-Arrhythmia Agents; Arrhythmias, Cardiac; Defibrillators, Implantable; Electrocardiography; Female; Humans; Injections, Intravenous; Sodium Channel Blockers; Syncope; Syndrome; Vectorcardiography

An association between arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C) and Brugada syndrome can be supposed according to several case reports. In order to examine a possible link between ARVD/C and Brugada syndrome, systematic ajmaline testing with 1 mg/kg body weight intravenously, was done in 55 patients (32 males, mean age 46.7+/-12.3 years) with ISFC/ESC criteria of ARVD/C. In nine patients ajmaline testing could demonstrate coved ST segment elevation of at least 2 mm in at least two right precordial leads. Three of these patients had recurrent syncopes. Electrophysiological study revealed non-sustained ventricular tachycardia with left bundle branch block configuration and inferior axis in only one case. Systematic ajmaline testing could demonstrate a definite link between ARVD/C and Brugada syndrome.

Topics: Adult; Aged; Ajmaline; Anti-Arrhythmia Agents; Arrhythmogenic Right Ventricular Dysplasia; Bundle-Branch Block; Electrocardiography; Female; Germany; Humans; Male; Middle Aged; Prevalence; Syncope; Syndrome; Tachycardia, Ventricular

The diagnostic ECG pattern in Brugada syndrome (BS) can transiently normalize and may be unmasked by sodium channel blockers such as ajmaline. Proarrhythmic effects of the drug have been well documented in the literature. A detailed protocol for the ajmaline challenge in Brugada syndrome has not yet been described. Therefore, we prospectively studied the risks of a standardized ajmaline test.. During a period of 60 months, 158 patients underwent the ajmaline test in our institution. Ajmaline was given intravenously in fractions (10mg every two minutes) up to a target dose of 1mg/kg. In 37 patients (23%) the typical coved-type ECG pattern of BS was unmasked. During the test, symptomatic VT appeared in 2 patients (1.3%). In all other patients, the drug challenge did not induce VT if the target dose, QRS prolongation >30%, presence/appearance of the typical ECG, or the occurrence of premature ventricular ectopy were considered as end points of the test. A positive response to ajmaline was induced in 2 of 94 patients (2%) with a normal baseline ECG, who underwent evaluation solely for syncope of unknown origin.. The ajmaline challenge using a protocol with fractionated drug administration is a safe method to diagnose BS. Because of the potential induction of VT, it should be performed under continuous medical surveillance with advanced life-support facilities. Due to the prognostic importance all patients with aborted sudden death or unexplained syncope without demonstrable structural heart disease and family members of affected individuals should presently undergo drug testing for unmasking BS.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Ajmaline; Anti-Arrhythmia Agents; Bundle-Branch Block; Child; Clinical Protocols; Death, Sudden, Cardiac; Dose-Response Relationship, Drug; Electrocardiography; Humans; Infusions, Intravenous; Middle Aged; Syncope; Syndrome

The Brugada-Brugada syndrome is a rhythmologic disorder which can be diagnosed because of typical ECG criteria. A high-take off descending ST segment localized to the right chest leads, associated with right bundle branch block and ventricular fibrillation or syncopes are characteristic of the syndrome. ECG alterations in the right precordial leads were recorded in a 47 year old female patient who was admitted to hospital because of enteritis and associated syncope. The ECG alterations were initially not realized as Brugada-Brugada syndrome. Because of "recognizing" comparable ECG alterations during a congress lecture, the diagnosis was made. The patient was treated with an ICD.

Topics: Ajmaline; Anti-Arrhythmia Agents; Bundle-Branch Block; Defibrillators, Implantable; Diagnosis, Differential; Electrocardiography; Female; Humans; Middle Aged; Syncope; Syndrome; Ventricular Fibrillation

The aim of this retrospective study was to determine the mechanism of syncope in idiopathic hypertrophic cardiomyopathy (HCM). An electrocardiographic study was undertaken in 43 patients with HCM: 27 (Group I) had a history of syncope and 16 (Group II) had no history of syncope but were investigated for conduction defects (n = 7) or unsustained ventricular tachycardia (VT) (n = 9). The stimulation protocol used programmed atrial pacing with 1 and 2 extrastimuli and ventricular pacing using up to 3 extrastimuli delivered at 2 sites. The following results were obtained: sustained atrial fibrillation (AF) (> 1 min) was induced in 21 patients in Group I (78%), 4 in Group II (25%); VT was induced in 3 patients in Group I (11%), and 3 in Group II (19%); infra-Hisian block was detected in 1 patient in Group I. The mechanism of syncope was elucidated in 23 patients in Group I (85%): one atrioventricular block 1 sinus node dysfunction, 18 atrial fibrillations, 2 associations of AF-VT and 1 VT. The authors conclude that the prevalence of inducible AF was higher in patients with HCA and syncope than in controls and HCM without syncope: this was the only detectable difference in 67% of patients with unexplained syncope. Paroxysmal AF could therefore explain malaise or syncope in up to 2/3 of cases of HCM.

Topics: Adult; Aged; Ajmaline; Arrhythmias, Cardiac; Atrial Fibrillation; Cardiac Pacing, Artificial; Cardiomyopathy, Hypertrophic; Female; Humans; Isoproterenol; Male; Middle Aged; Retrospective Studies; Syncope

Thirty-five patients with bundle branch block (BBB) and unexplained syncope underwent electrophysiologic study (EPS) including programmed ventricular stimulation and ajmaline administration (1 mg/kg, IV) to induce infra-His block. A prolonged HV interval (greater than 55 ms) was present in 16 of the 35 patients. Ajmaline-induced HV block occurred in 12 patients (complete HV block in 10, and 2:1 HV block in two). Monomorphic ventricular tachycardia (VT) was inducible in nine (25.7%) and polymorphic VT in two patients (5.7%). Left ventricular ejection fraction (LVEF) was less than 40% in five patients (45.5%) with inducible VT. Two patients had an unexpected co-existence of inducible HV block and VT. The remaining 14 patients (40%) had no detectable abnormality. The incidence of inducible VT was higher (45% vs 13.3%), and the presence of negative studies was lower (30% vs 53.3%) in patients with structural heart disease (n = 20), when compared to those with no significant heart disease (n = 15) (differences not significant [NS]). During a mean follow-up period of 16.5 +/- 9.2 months, all the patients with inducible HV block have been asymptomatic after having received permanent pacemakers. Patients with inducible monomorphic VT (except one with poor left ventricular function who died suddenly) have also been asymptomatic on antiarrhythmic drugs. Of the remaining patients, seven with normal EPS, two with prolonged HV intervals but no inducible HV block (despite being given permanent pacemakers) and one patient with polymorphic VT on antiarrhythmic drugs continue to have recurrent syncope. Approximately 60% of patients with BBB and unexplained syncope have clinically significant electrophysiologic abnormalities.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Adult; Aged; Ajmaline; Bundle-Branch Block; Cardiac Pacing, Artificial; Electric Stimulation; Electrophysiology; Female; Heart Block; Heart Conduction System; Humans; Male; Middle Aged; Monitoring, Physiologic; Prospective Studies; Syncope; Tachycardia

We prospectively evaluated and followed-up 45 patients with syncope in whom conventional cardiovascular and neurological investigations did not reveal the cause. All patients underwent electrophysiologic studies to assess the function of the sinus node and the integrity of atrioventricular conduction. These included the ajmaline test and the inducibility of supraventricular or ventricular tachycardia. Seven patients (15.5%) had evidence of sinus node dysfunction, 8 patients (17.7%) had evidence of infra-His atrioventricular block after ajmaline administration and 5 patients (11.1%) had inducible ventricular tachycardia. The remaining 25 patients (55.5%) had non-diagnostic studies. All patients with sinus node dysfunction and inducible infra-His atrioventricular block were asymptomatic during a mean follow-up period of 14.3 +/- 9.5 months after implantation of a permanent pacemaker. Patients with inducible ventricular tachycardia (except 1 with poor left ventricular function who died) were likewise asymptomatic while receiving laboratory guided anti-arrhythmic drug therapy. Twenty-five patients with non-diagnostic studies who were treated empirically are alive but the symptoms persist in 14 (56%). Provocative electrophysiological studies are of diagnostic and therapeutic utility in a significant number of patients with recurrent syncope of "unknown cause".

Topics: Adult; Aged; Ajmaline; Cardiac Catheterization; Electrocardiography; Female; Heart Block; Heart Conduction System; Humans; Male; Middle Aged; Pacemaker, Artificial; Sinoatrial Node; Syncope; Tachycardia

Topics: Adult; Aged; Ajmaline; Anti-Arrhythmia Agents; Arrhythmias, Cardiac; Benzeneacetamides; Electrocardiography; Female; Humans; Male; Middle Aged; Piperidines; Syncope; Time Factors

Topics: Aged; Ajmaline; Electrocardiography; Female; Heart Block; Humans; Male; Middle Aged; Syncope

An ajmaline test was conducted in 120 patients with a history of disorders of consciousness : Adams-Stokes syndrome (n = 49), loss of consciousness (n = 42), or lipothymia (n = 29). Four types of response were observed after ajmaline : VH less than 80 ms (n = 63); VH between 80 and 100 ms (n = 19); VH greater than 100 ms (n = 17); distal block (n = 21). One hundred and fifteen of these patients were followed-up for from three to six years (mean 56 months). Pacemakers had been fitted in 46 of them. Atrioventricular block was eventually detected in 37 patients but was excluded in the other 78 cases, either because the syncope attacks did not recur or because another cause was demonstrated. The predictive value of the ajmaline test was confirmed by the subsequent course of the disorders. Based on only clinical findings, diagnosis was confirmed in 42 p. cent, excluded in 12 p. cent, and impossible to define in 46 p.cent of cases. After the ajmaline test, diagnosis was confirmed in 79 p.cent, excluded in 6 p.cent, and impossible to define in 15 p.cent. The risk of atrioventricular block can be evaluated as 1-6 p.cent when the increase in VH is less than 80 ms, 35.3 p.cent when the increase is between 80 and 100 ms, 62.5 p.cent when it is greater than 100 ms, and 100 p.cent when there is a distal block. The indications for fitting a pacemaker depend upon the results of this test. If contra-indications are respected (recent history of an infarct, cardiac failure, marked enlargement of the heart), complications are rare, being observed in less than 3 p.cent of cases.

Topics: Aged; Ajmaline; Electrocardiography; Female; Follow-Up Studies; Heart Block; Humans; Male; Syncope; Time Factors

For the provocation of trifascicular blockings in patients with fascicular block a rapid ventricular stimulation after an application of ajmalin of maximally 100 mg performed. Of 80 patients with clinically and electrocardiographically certain intermitting total atrioventricular blockings (group A) and 24 patients with typical anamnesis without ECG-proof of the atrioventricular block of higher degree (group B) altogether 88 patients had an atrioventricular block of 2nd or 3rd degree after provocation. On the other hand, only 2 of 20 patients with bifascicular block without syncopes (group C) had a positive result of the test. The method is regarded as suitable for the detection of trifascicular latent blockings.

Topics: Aged; Ajmaline; Bundle-Branch Block; Cardiac Pacing, Artificial; Humans; Syncope

Topics: Adolescent; Adult; Aged; Ajmaline; Atropine; Bundle of His; Electrocardiography; Female; Heart Atria; Heart Block; Humans; Male; Methods; Middle Aged; Pacemaker, Artificial; Stimulation, Chemical; Syncope

Topics: Adenosine Triphosphatases; Adult; Ajmaline; Female; Heart Block; Humans; Male; Muscular Dystrophies; Pacemaker, Artificial; Syncope