Unusual features of arrhythmogenic right ventricular dysplasia.
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Arrhythmia, Sinus↗
Biomedical subjects
F Morady
Publications and source records attributed to F Morady.
Programmed ventricular stimulation in mitral valve prolapse: analysis of 36 patients.
Programmed ventricular stimulation with 3 extrastimuli was performed in 36 patients with mitral valve prolapse (MVP). Among 11 patients without transient cerebral symptoms, none had inducible ventricular tachycardia (VT) or ventricular fibrillation (VF), whether or not nonsustained VT or ventricular premature complexes (VPC) were present during ambulatory electrocardiographic recordings. These patients remained well without antiarrhythmic drug therapy for 6 to 57 months (mean 23) of follow-up. Two patients with recurrent unexplained syncope and no documented ventricular arrhythmia during electrocardiographic monitoring also had no inducible VT or VF. Among 20 patients with syncope or presyncope and documented nonsustained VT or VPCs during electrocardiographic monitoring, polymorphic nonsustained VT was induced in 8, sustained unimorphic VT in 2, and VF in 3. In 1 patient who had inducible polymorphic nonsustained VT, electrocardiographic monitoring during syncope showed sinus rhythm. Among 3 patients with a history of sustained VT or VF, unimorphic VT was induced in each. Patients with MVP who have asymptomatic ventricular ectopic activity and no inducible VT may have a benign prognosis without treatment. In patients who have transient cerebral symptoms and documented nonsustained VT or VPCs, VT or VF is inducible in 65%, most often polymorphic VT. It is unclear in which patients this finding is clinically significant and in which it is a nonspecific response to programmed stimulation.
Adult↗
Programmed ventricular stimulation in patients without spontaneous ventricular tachycardia.
Programmed ventricular stimulation was performed in 52 patients who had not had a documented or suspected episode of spontaneous ventricular tachycardia (VT) or ventricular fibrillation (VF). Programmed stimulation with up to three extrastimuli was performed from the right ventricular (RV) apex in all patients and from the left ventricular (LV) apex in 14 patients. A maximum response of one to five intraventricular reentry beats was induced in 52% of patients. Nonsustained VT (six or more repetitive beats terminating spontaneously within 30 seconds) was never induced in the 16 patients without structural heart disease but was induced (usually with triple extrastimuli) in 45% of nine patients with mitral valve prolapse and in 37% of 27 patients with other types of heart disease. Sustained VT was never induced; however, sustained VF was induced in two patients. During programmed RV and LV stimulation with up to three extrastimuli (with 2 msec pulses, 5 mA in intensity), (1) a maximum response of one to five repetitive beats is a nonspecific finding of no predictive value; (2) nonsustained VT was not induced in patients without structural heart disease who had not had spontaneous VT; (3) nonsustained VT was frequently induced in patients with structural heart disease who had not previously been known to have had VT; (4) the induction of sustained VT appears to be a response specific to patients who have had spontaneous VT or VF; and (5) sustained VF can be induced infrequently in patients who have never had spontaneous VT or VF.
Aged↗
Evaluation of the patient with sudden cardiac death.
Sudden cardiac death is defined as death from natural causes that occurs within one hour of the patient's collapse. Public education programs and the proliferation of rapidly deployable community life-support teams have resulted in the ability to deliver emergency medical care to many patients who suffer a cardiac arrest and who otherwise would have died suddenly. This article reviews the diagnostic evaluation and therapeutic management of the patient who has survived a cardiac arrest.
Anti-Arrhythmia Agents↗
Catheter-induced His bundle ablation in a patient with reentrant tachycardia associated with a nodoventricular tract.
A patient with refractory tachycardia associated with a nodoventricular tract in whom tachycardia was successfully controlled with catheter-induced ablation of the His bundle is reported. Tachycardia was always initiated by ventricular impulses that blocked retrogradely in the nodoventricular tract and conducted by way of the His-Purkinje system. The His bundle ablation was successfully accomplished by delivering two direct current countershocks of 400 J each in the region of the His bundle. Postablation, the patient manifested stable 1:1 anterograde conduction via the atrioventricular (AV) node-nodoventricular fiber over a wide range of heart rates (50 to 180 beats/min). A permanent pacemaker was not implanted at the patient's request. During 16 months of follow-up, the patient has had stable sinus rhythm with no sustained tachycardia. Brief asymptomatic episodes of ectopic atrial tachycardia have been recorded on ambulatory electrocardiographic monitoring. This case 1) demonstrates the potential role of ablation of the His bundle in patients with refractory tachycardia associated with a nodoventricular tract provided that the His bundle is a critical component in the initiation of the tachycardia or a part of the tachycardia circuit; 2) reveals stable 1:1 AV conduction over a nodoventricular tract; and 3) emphasizes the utility of the phase image technique for diagnosis of a Mahaim tract.
Adult↗
Dissociation of atrial electrograms by right and left atrial pacing in patients with atrioventricular reciprocating tachycardia.
Seventeen patients had atrioventricular (AV) reciprocating tachycardia incorporating an AV bypass tract as the retrograde limb of the tachycardia circuit. High right atrial pacing during tachycardia dissociated the low septal right atrial electrogram in four of seven patients with a left free wall bypass tract, neither of two patients with a right free wall bypass tract, four of six patients with a posteroseptal bypass tract and both patients with an anteroseptal bypass tract. Pacing from the coronary sinus during tachycardia dissociated the atrial electrogram recorded at the os of the coronary sinus in no patient with a left free wall bypass tract, both patients with a right free wall bypass tract, two patients with a posteroseptal bypass tract and one patient with an anteroseptal bypass tract. These findings suggest two distinct inputs to the AV node, with the left-sided input being part of the tachycardia circuit in patients with a left free wall bypass tract and the right-sided input being part of the tachycardia circuit in patients with a right free wall bypass tract. However, in some patients with a septal bypass tract, neither the right- nor the left-sided atrial input appears to be a necessary link in the tachycardia circuit.
Adult↗
Electrophysiologic and hemodynamic effects of intravenous propafenone in patients with recurrent ventricular tachycardia.
Electrophysiologic and hemodynamic studies were performed before and after intravenous infusion of a new antiarrhythmic agent, propafenone, in 28 patients with recurrent ventricular tachycardia. Propafenone was given at a loading dose of 2 mg/kg in all patients. Subsequently, group A, the first 14 patients, received 1 mg/min and group B, the second 14 patients, received 2 mg/min continuous infusion. Propafenone exerted no effect on sinus nodal recovery time and sinoatrial conduction time, but significantly prolonged atrioventricular (AV) nodal and His-Purkinje conduction time and the QRS duration (respectively, 95 +/- 19, 48 +/- 10 and 120 +/- 23 ms before, and 110 +/- 28, 53 +/- 10 and 135 +/- 27 ms after; p less than 0.001). Propafenone did not change the mean arterial blood pressure but slightly increased right atrial, pulmonary artery and capillary wedge pressures resulting in mild depression of the cardiac index (2.6 +/- 0.8 liters/min per m2 before and 2.3 +/- 0.7 liters/min per m2 after; p less than 0.001). None of the patients were symptomatic from these changes. In group A, propafenone did not affect the inducibility of ventricular tachycardia except for one patient whose arrhythmia was sustained before and become nonsustained after propafenone. In group B, sustained ventricular tachycardia became noninducible in three patients and nonsustained in two patients, and nonsustained ventricular tachycardia became noninducible in one patient after propafenone. Therefore, an appropriate loading dose of intravenous propafenone such as 2 mg/kg followed by 2 mg/min infusion may be given safely and may suppress ventricular tachycardia. Propafenone may be a useful addition to currently available antiarrhythmic agents.
Adolescent↗
Phase image evaluation of patients with ventricular pre-excitation syndromes.
To localize bypass pathways, left and right ventricular regions were analyzed at rest by phase image analysis in 18 patients with ventricular pre-excitation syndromes. These were compared with image findings in 18 normal subjects. In each of 17 patients with pre-excitation, the site localized on electrophysiologic study correlated closely with the region of earliest ventricular phase angle. This site could be objectively separated from that in normal subjects in each of eight patients with an active left-sided pathway and in both patients with a right-sided pathway. Those with a septal pathway revealed earliest septal phase angle, but could not be separated from normal subjects. In the eight patients with an active left bypass tract, the onset, upstroke and peak of the left ventricular phase histogram preceded those of the right ventricular histogram. Those with a left-sided pathway demonstrated a mean left ventricular phase angle, a difference between mean left and mean right ventricular phase angle and a difference between earliest left and right ventricular phase angles which was significantly less than that in normal subjects (p less than 0.05). These variables presented characteristic converse changes in those with a right-sided pathway. Sequential phase changes in 10 studies suggested "fusion" of normal septal with lateral bypass fronts. Electrocardiographic and electrophysiologic localization of the bypass pathway agreed in only 8 of 14 patients with a recognized delta wave. The phase image represents a new, noninvasive method of evaluating ventricular pre-excitation. The method may provide useful information complementary to that of electrocardiographic and electrophysiologic analysis.
Adolescent↗
Clinical features and prognosis of patients with out of hospital cardiac arrest and a normal electrophysiologic study.
Nineteen patients survived a cardiac arrest not associated with an acute myocardial infarction, and had a normal electrophysiologic study with no inducible ventricular tachycardia despite programmed stimulation with one to three extrastimuli at two or more ventricular sites. Among 14 patients who had obstructive coronary artery disease, cardiac arrest occurred during exertion or an episode of angina pectoris in 11; 24 hour ambulatory electrocardiographic recordings demonstrated infrequent or no premature ventricular complexes in 10 and an ischemic response occurred during stage I or II (Bruce protocol) in 6 of 9 patients who underwent exercise testing. Treatment of these patients consisted of myocardial revascularization (eight patients) or antianginal medications (six patients). Only three patients were also treated with an antiarrhythmic drug. Over a follow-up period of 26 +/- 15 months (mean +/- standard deviation), only one patient died suddenly. Two patients who had coronary artery spasm were treated with coronary vasodilator medications and had no recurrence of cardiac arrest over 7 and 36 months of follow-up, respectively. Three patients who had cardiomyopathy or no identifiable structural heart disease were treated with nadolol or amiodarone and had no recurrence of cardiac arrest over 3 to 27 months of follow-up. Among patients who survive a cardiac arrest and have a normal electrophysiologic study, those with obstructive coronary artery disease or coronary artery spasm generally have an excellent prognosis with treatment directed primarily at the underlying heart disease. The clinical features of these patients suggest that cardiac arrest was related to ischemia rather than a primary arrhythmia.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Invasive electrophysiologic testing: a critical appraisal.
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Arrhythmias, Cardiac↗
A prospective comparison of triple extrastimuli and left ventricular stimulation in studies of ventricular tachycardia induction.
One hundred and one patients with sustained unimorphic ventricular tachycardia underwent programmed ventricular stimulation with one of two protocols. Fifty patients underwent programmed stimulation with protocol A, which consisted of burst overdrive pacing, single, double, and triple extrastimuli at the right ventricular apex, right ventricular outflow tract, or septum, and then at the left ventricular apex. Fifty-one patients underwent programmed stimulation with protocol B, which consisted of burst overdrive pacing, single and double extrastimuli at the right ventricular apex, right ventricular outflow tract or septum, and at the left ventricular apex, followed by triple extrastimuli at these sites. The stimulation protocol was continued until sustained ventricular tachycardia or rapid, polymorphic ventricular tachycardia greater than 10 sec in duration was induced. With protocol A, clinical and nonclinical ventricular tachycardia was induced in 76% and 36% of patients, respectively; with protocol B, clinical and nonclinical ventricular tachycardia was induced in 85% and 38% of patients, respectively. Direct-current countershock for sustained polymorphic ventricular tachycardia was required in 10% of patients studied under protocol A, compared with in 2% of patients studied under protocol B. With protocol A, near-maximal yield of induced clinical (72%) and nonclinical ventricular tachycardia (30%) was attained after the use of triple extrastimuli at the first stimulation site. The yield of stimulation at a second right ventricular site and of left ventricular stimulation was only an additional 2% each. With protocol B, triple extrastimuli increased the yield of induced clinical ventricular tachycardia from 61% to 85%.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Efficacy of left cardiac sympathectomy in the treatment of patients with the long QT syndrome.
Ten patients with the long QT syndrome and recurrent syncope and/or cardiac arrest caused by ventricular arrhythmias underwent left stellate (one patient) or left cervicothoracic sympathectomy (nine patients) after failing to respond to high-dose beta-blocker therapy. The syndrome was familial in four and idiopathic in six. All patients had a prolonged resting QT interval (548 +/- 51 msec, mean +/- SD) and corrected QT interval (QTc) (556 +/- 43 msec). After sympathectomy the mean QTc shortened significantly from 556 +/- 43 to 508 +/- 65 msec (p less than .05) but the QTc remained abnormal in all but one patient. Over a mean follow-up period of 38.6 +/- 19 months, eight patients developed recurrent symptoms that included cardiac arrest in three (one fatal, two nonfatal), syncope in four, and presyncope in six. The addition of beta-blockers was ineffective in suppressing the recurrent symptoms. The control of symptoms required more extensive sympathectomy (three patients), chronic atrial pacing (three patients), and implantation of an automatic internal defibrillator (one patient). Only one patient has remained asymptomatic without drug or pacemaker therapy. In conclusion, left cervicothoracic sympathectomy proved inadequate for long-term control of symptoms in most patients with the long QT syndrome. These patients usually required concomitant drugs, more extensive surgery, or long-term cardiac pacing for symptomatic relief.
Adolescent↗
Frequent neurologic toxicity associated with amiodarone therapy.
Fifty-four consecutive patients were treated with amiodarone for symptomatic ventricular tachycardia or ventricular fibrillation refractory to treatment with conventional antiarrhythmic drugs. A reversible neurologic syndrome of tremor, ataxia, and occasionally peripheral neuropathy without nystagmus, dizziness, encephalopathy, or long-tract signs developed in 54% of the patients and was the most common reason for altering or discontinuing drug therapy. Neurologic side effects improved or resolved within 2 days to 4 weeks of decreasing or discontinuing amiodarone. Frequent neurologic toxicity is a hitherto undescribed complication of amiodarone therapy. Wider recognition of this syndrome will avoid unnecessary and costly diagnostic evaluation.
Amiodarone↗
Unusual features of bypass conduction in Wolff-Parkinson-White syndrome and atrial fibrillation.
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Adult↗
Long-term efficacy and toxicity of high-dose amiodarone therapy for ventricular tachycardia or ventricular fibrillation.
Amiodarone was administered to 154 patients who had sustained, symptomatic ventricular tachycardia (VT) (n = 118) or a cardiac arrest (n = 36) and who were refractory to conventional antiarrhythmic drugs. The loading dose was 800 mg/day for 6 weeks and the maintenance dose was 600 mg/day. Sixty-nine percent of patients continued treatment with amiodarone and had no recurrence of symptomatic VT or ventricular fibrillation (VF) over a follow-up of 6 to 52 months (mean +/- standard deviation 14.2 +/- 8.2). Six percent of the patients had a nonfatal recurrence of VT and were successfully managed by continuing amiodarone at a higher dose or by the addition of a conventional antiarrhythmic drug. One or more adverse drug reactions occurred in 51% of patients. Adverse effects forced a reduction in the dose of amiodarone in 41% and discontinuation of amiodarone in 10% of patients. The most common symptomatic adverse reactions were tremor or ataxia (35%), nausea and anorexia (8%), visual halos or blurring (6%), thyroid function abnormalities (6%) and pulmonary interstitial infiltrates (5%). Although large-dose amiodarone is highly effective in the long-term treatment of VT or VF refractory to conventional antiarrhythmic drugs, it causes significant toxicity in approximately 50% of patients. However, when the dose is adjusted based on clinical response or the development of adverse effects, 75% of patients with VT or VF can be successfully managed with amiodarone.
Aged↗
Electrophysiologic testing in the management of survivors of out-of-hospital cardiac arrest.
Forty-five patients survived a cardiac arrest due to ventricular tachycardia (VT) or ventricular fibrillation (VF). Programmed ventricular stimulation was performed with the patients taking no antiarrhythmic medications. Sustained VT was induced in 26 patients (58%) and nonsustained VT in 8 (18%). With treatment aimed at the underlying heart disease (plus empiric antiarrhythmic therapy in 2 patients), the 11 patients who had no inducible VT have had no recurrence of symptomatic VT or cardiac arrest over a follow-up period of 19 +/- 9 months (mean +/- standard deviation). Conventional antiarrhythmic drugs suppressed the induction of VT and were used for chronic treatment in 9 of 34 patients (26%) with inducible VT. Three of these 9 patients had recurrent VT or sudden death, whereas 6 have had no recurrence over follow-up of 20 +/- 7 months. In the 25 of 34 patients in whom the induction of VT was not suppressed by conventional antiarrhythmic drugs, 23 were treated with amiodarone (daily dose 550 +/- 120 mg), and 2 underwent coronary artery bypass grafting with either aneurysmectomy or map-directed endocardial resection. One of the latter 2 patients died suddenly 12 months after surgery. Among the 23 patients treated with amiodarone, 2 had fatal VT or sudden death and 21 (91%) did not, over 18 +/- 14 months of follow-up. In survivors of a cardiac arrest, the chief value of electrophysiologic testing is in identifying patients without inducible VT, who appear to have a low risk of recurrent sudden death with treatment directed at the underlying heart disease. Serial electropharmacologic testing with conventional antiarrhythmic drugs is disappointing, with a low incidence of arrhythmia suppression.
Adult↗
Intravenous amiodarone in the acute treatment of recurrent symptomatic ventricular tachycardia.
Fifteen patients aged 59.3 +/- 11.5 years (mean +/- standard deviation [SD]) had recurrent symptomatic ventricular tachycardia (VT) refractory to at least 2 conventional antiarrhythmic drugs. All patients had organic heart disease; 4 had an acute myocardial infarction. The mean ejection fraction was 0.30 +/- 0.09. TWelve patients had overt congestive heart failure. Five had bundle branch block. Before treatment with intravenous amiodarone, the patients had had 6 to 40 episodes of symptomatic VT over 1 to 8 days of hospitalization. All patients received an initial bolus of 5 mg of amiodarone/kg over 15 minutes. Seven patients also received a continuous infusion of 600 to 1,000 mg of amiodarone over 12 to 24 hours. Additional doses depended on the patients' clinical responses. In 11 of 15 patients, antiarrhythmic drugs that had failed to suppress VT were continued during administration of amiodarone. In 12 of 15 patients acute control of VT was obtained with intravenous administration of amiodarone either alone or in combination with previously ineffective drugs. Three patients continued to have frequent episodes of VT while being treated with intravenous amiodarone. Mobitz type I atrioventricular block developed in 1 patient. No patient had high degree atrioventricular block, symptomatic hypotension, or a clinically apparent worsening of congestive heart failure. The use of intravenous amiodarone represents a significant advance in the acute treatment of frequent life-threatening VT refractory to other drugs. With appropriate monitoring, it can be used safely in patients with congestive heart failure, bundle branch block, or acute myocardial infarction.
Adult↗
Hemodynamic effects of intravenous amiodarone in patients with depressed left ventricular function and recurrent ventricular tachycardia.
The systemic hemodynamic effects of intravenously administered amiodarone were evaluated in patients with depressed left ventricular function and recurrent sustained ventricular tachycardia. Heart rate decreased linearly up to 1 hour after amiodarone infusion (5 mg/kg). Cardiac index varied in a cubic fashion, diminishing at 10 minutes and returning to baseline by 60 minutes (p less than 0.05). Stroke work index also showed a similar decrease at 10 minutes, which was transient (p less than 0.005). These changes occurred without any significant change in systemic vascular resistance and with slight or no increase in pulmonary capillary wedge pressure, indicating a negative inotropic effect of amiodarone. The depression of left ventricular function in these patients, however, was mild and transient, and intravenously administered amiodarone was tolerated by the vast majority of patients. In two patients with overt heart failure and severely depressed left ventricular ejection fraction and marked hemodynamic abnormalities, profound hypotension occurred during amiodarone therapy; in such patients, therefore, hemodynamic monitoring is preferable. Limited data are available on the hemodynamic effects of orally administered amiodarone, but the determination of left ventricular ejection fraction by radionuclide ventriculography before and during long-term amiodarone administration has shown no reduction of function even in patients with severely reduced myocardial performance.
Administration, Oral↗