Monomorphic ventricular tachycardia
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Arrhythmogenic right ventricular dysplasia

Arrhythmogenic right ventricular dysplasia (ARVD, also known as arrhythmogenic right ventricular cardiomyopathy or ARVC) is a type of nonischemic cardiomyopathy that involves primarily the right ventricle. It is characterized by hypokinetic areas involving the free wall of the right ventricle, with fibrofatty replacement of the right ventricular myocardium, with associated arrhythmias originating in the right ventricle. more...

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Overview

ARVD is an important cause of ventricular arrhythmias in children and young adults. It is seen predominantly in males, and 30-50% of cases have a familial distribution. It is usually inherited in an autosomal dominant pattern, with variable expression. The penetrance is 20-35% in general, but significantly higher in Italy. Seven gene loci have been implicated in ARVD. However, about 50% of families that express ARVD that undergo genetic screening do not show linkage with any of the known chromosomal loci. It is unclear whether the pathogenesis varies with the different loci involved. A standard genetic screening test is not available.

Naxos Disease

Naxos disease is an autosomal recessive variant of ARVD, described initially on the Greek island of Naxos. There, the penetrance is >90%. It involves the gene that codes for plakoglobin (a protein that is involved in cellular adhesion), on chromosome 17p. Naxos disease is described as a triad of ARVD, palmoplantar keratosis, and wooly hair. The signs of Naxos disease are more severe than with autosomal dominant ARVD.

Incidence

The incidence of ARVD is about 1/10,000 in the general population in the United States. It accounts for up to 17% of all sudden cardiac deaths in the young. In Italy, the incidence is 40/10,000, making it the most common cause of sudden cardiac death in the young in Italy.

Presentation

Up to 80% of individuals with ARVD present with syncope or sudden cardiac death. The remainder frequently present with palpitations or other symptoms due to right ventricular outflow tract (RVOT) tachycardia (a type of monomorphic ventricular tachycardia).

Symptoms are usually exercise-related. In populations where hypertrophic cardiomyopathy is screened out prior to involvement in competitive athletics, it is a common cause of sudden cardiac death.

The first clinical signs of ARVD are usually during adolescence. However, signs of ARVD have been demonstrated in infants.

Pathogenesis

The pathogenesis of ARVD is largely unknown. Apoptosis (programmed cell death) appears to play a large role. It is unclear why only the right ventricle is involved. The disease process starts in the subepicardial region and works its way towards the endocardial surface, leading to transmural involvement (possibly accounting for the aneurysmal dilatation of the RV). Residual myocardium is confined to the subendocardial region and the trabeculae of the RV. These trabeculae may become hypertrophied.

Aneurysmal dilatation is seen in 50% of cases at autopsy. It usually occurs in the diaphragmatic, apical, and infundibular regions (known as the triangle of dysplasia). The left ventricle is involved in 50-67% of individuals. If the left ventricle is involved, it is usually late in the course of disease, and confers a poor prognosis.

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Recurrent syncope for over a decade due to idiopathic ventricular fibrillation
From CHEST, 11/1/94 by Kiritkumar Masrani

A 35-year-old man had a history of recurrent syncope for more than a decade. During a witnessed episode, an ambulatory electrocardiographic recording showed ventricular flutter/fibrillation that lasted for 2 1/2 minutes and terminated spontaneously without adverse neurologic sequelae. No structural heart disease and no possible etiologic factor for the ventricular tachyarrhythmia was found. The patient received an automatic implantable cardioverter defibrillator. Review of the literature suggests that the automatic implantable cardioverter defibrillator is a valid option in idiopathic ventricular fibrillation in young individuals to avoid the potential risk of recurrent cardiac arrest.

(Chest 1994; 106:1601-03)

VF=ventricular fibrillation

Key words: automatic implantable cardioverter defibrillator; idiopathic ventricular fibrillation; programmed electrical stimulation; syncope

Syncopal episodes in young individuals without heart disease are rarely due to ventricular fibrillation (VF). Ambulatory monitoring in these cases is rarely helpful due to infrequency of the attacks. We report herein a case of recurrent syncope for over 10 years due to idiopathic ventricular flutter-fibrillation first documented by Holter monitoring, lasting 2 min and 30 s, and resolving spontaneously.

CASE REPORT

A 35-year-old black man was referred to the Brooklyn Veterans Administration Medical Center for syncopal attacks after repeated unrewarding neurologic workup that included multiple electroencephalograms and two head computed tomographic (CT) scans.

For 10 years the patient was treated with phenytoin (Dilantin) in therapeutic dosage that he took regularly except during the period between 1983 to 1986 while serving in the Army in his effort to avoid medical restrictions. The syncopal episodes were often preceded by numbness of legs, diaphoresis, dizziness, and shortness of breath and were always associated with palpitations. The episodes were not related to change in posture, exertion, or meal intake. Tonicoclonic movements were frequently reported by sporadic witnesses. The frequency of attacks varied from 3 to 20 per year except for an asymptomatic period while in the military service. However, several attacks occurred while the patient was not taking phenytoin. There was no history of alcoholic intake, smoking, or illicit drugs.

Results of physical examination and laboratory tests, including magnesium level, phenytoin level, and electrocardiogram, were within normal limits. A witnessed syncopal episode occurred at home while having a 24-h Holter recording. The recording revealed 16 episodes of very fast (up to 420 beats/min) polymorphic ventricular flutter/fibrillation with the longest episode lasting for 2 1/2 minutes before terminating spontaneously (Fig 1). Four of the 16 episodes followed a short-long-short cardiac cycle due to a preceding ventricular premature beat followed by a compensatory pause (Fig 2).

[CHART OMITTED]

The patient was admitted to the Coronary Care Unit on the same day. The electrocardiogram and the QTc interval were within normal limits. The two-dimensional echocardiogram and Doppler studies were normal except for minimal interventricular septal hypertorphy (1.3 cm) with similar findings by follow-up transesophageal echocardiogram. Myocardial infarction was excluded at the time of hospital admission. The signal-averaged ECG did not reveal any late potentials. Cardiac catheterization revealed normal pressures, normal coronaries, and normal right and left ventriculograms. The ejection fraction was 73 percent and there was no abnormal gradient across the valves or left ventricular outflow tract. Tilt table did not reveal any abnormality. Electrophysiologic studies conducted while not receiving phenytoin showed a normal sinus and atrioventricular nodal function, normal effective refractory periods at the apex, and outflow tract of the right ventricle. There was no evidence of accessory pathways. A polymorphic ventricular tachycardia at a rate of 360 beats/min was induced by stimulation of the right ventricular outflow tract with three extrastimuli [S.sub.1]/[S.sub.2]/[S.sub.3]/[S.sub.4]=400/190/190/180 ms). The tachycardia terminated spontaneously after 10 s and was associated with near syncopal attack. A monophasic action potential recording from different right ventricular sites did not reveal any abnormality. Follow-up CT scan of the heart did not show any abnormal findings, including pericardial fat distribution in support of right ventricular dysplasia.

The patient was referred for implantation of an automatic implantable cardioverter defibrillator. During surgery, neither morphologic abnormalities nor abnormal fat distribution was noticed. The patient had been followed-up for 24 months after surgery and no discharge has occurred so far.

DISCUSSION

Syncope is a symptom caused by a wide variety of diseases ranging from physiologic derangements with few consequences to diseases that may be life-threatening. Causes of syncope include neurocardiogenic syncope, orthostatic hypotension, drug-induced syncope, cerebrovascular diseases, hypersensitive carotid sinus, and cardiac diseases.(1) The latter could be classified into mechanical or arrythmogenic causes. Cardiac mechanical causes of syncope include the following: obstruction to left ventricular flow like aortic stenosis; hypertrophic cardiomyopathy, and mitral stenosis; obstruction to pulmonary flow like pulmonic stenosis, tetralogy of Fallot, pulmonary hypertension, and pulmonary embolism; pump failure as secondary to myocardial infarction; and cardiac tamponade. Arrhythmogenic causes of syncope include both bradyarrhythmias like sick sinus syndrome and atrioventricular block and tachyarrhythmias like supraventricular or ventricular tachycardia. Cardiac causes of syncope have been identified in 8 percent to 39 percent of patients in various studies.(1) Most studies have shown that in a large proportion of patients with syncope (38 percent to 47 percent), a cause is not established after standard diagnostic evaluation.(1)

The most interesting aspect of the present case is the recurrent spontaneous conversion of sustained VF. Although this has been reported in six other cases in the literature, to our knowledge, such prolonged and frequent episodes without neurologic sequelae are unique. There are few reports of VF in the presence of minimal or structural heart diseases.(2)(3)(4)(5) In a review of 54 cases of idiopathic VF, 50 patients required resuscitation while 4 had nonsustained VF with syncope.(2) Ventricular fibrillation was initiated by a ventricular premature beat with a very short coupling interval in many of the patients, including the present patient. Induction of polymorphic ventricular tachycardia and VF has been considered a nonspecific response to programmed stimulation and its incidence varied from 39 percent(5) to 69 percent(2) in two large study groups. Sustained monomorphic ventricular tachycardia is rarely induced.(4) Thus, programmed stimulation may not be useful in guiding therapy in survivors of idiopathic VF.

There is no recommended strategy for the management of idiopathic VF at present. Pharmacologic therapy has been tried often. Failure of amiodarone as a single-drug therapy was seen in three of four cases reviewed by Viskin and Belhassen(2) who found class IA to be associated with better prognosis in the short term. However, the value of pharmacologic therapy in idiopathic VF has yet to be established. In a recent report, a ten-center retrospective study provided information on 28 survivors of VF with minimal or no structural abnormalities who were treated with an implantable cardioverter-defibrillator.(5) The 3-year survival rate with the device was excellent and superior to that reported in survivors of cardiac arrest with structural heart disease similarly treated. Only a small number of patients (4 of 28) received shocks considered to be appropriate. However, the implanted device had no arrhythmia disclosure capability to be able to provide an accurate estimate of the incidence of appropriate shocks. The report suggests that an implantable cardioverter-defibrillator is a valid option in patients with idiopathic VF to avoid the potential risk of recurrent cardiac arrest. However, to our knowledge, there are no good data as yet to support its superiority to other modes of treatment.

REFERENCES

(1)Kapoor WN. Diagnostic evaluation of syncope. Am J Med 1991; 90:91-106

(2)Viskin S, Belhassen B. Idiopathic ventricular fibrillation. Am Heart J 1990; 120:661-67

(3)Lemery R, Brugada P, Della Bella P, Dugemier T, Wellens HJJ. Ventricular fibrillation in six adults without overt heart disease. J Am Coll Cardiol 1989; 13:911-16

(4)Belhassen B, Shapira I, Shoshani D, Paredes A, Miller H, Laniado S. Idiopathic ventricular fibrillation: inducibility and beneficial effects of class I antiarrhythmic agents. Circulation 1987; 75:809-16

(5)Meissner MD, Lehmann MH, Steinman RT, Mosteller ED, Akhtar M, Calkins H, et al. Ventricular fibrillation in patients without significant structural heart diseases: a multicenter experience with implantable cardioverter-defibrillator therapy. J Am Coll Cardiol 1993; 21:1406-12

COPYRIGHT 1994 American College of Chest Physicians
COPYRIGHT 2004 Gale Group

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