Arrhythmogenic right ventricular cardiomyopathy

https://www.internationaljournalofcardiology.com/article/S0167-5273(99)00142-4/pdf

Risk stratification of sudden cardiac death and malignant ventricular arrhythmias in right ventricular dysplasia-cardiomyopathy

Twenty-four patients presenting with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ventricular tachycardia of right ventricular origin associated with structural abnormalities of the right ventricle) were divided into two groups with left ventricular ejection fraction (LVEF) above or below 45%. The distribution of LVEF in the group with LVEF below 45% was comparable with the distribution in 6 patients with idiopathic dilated cardiomyopathy who had ventricular tachycardia originating in the left ventricle (P = 0.2). They also had the same unfavorable long-term prognosis. Therefore, it is suggested that the term, arrhythmogenic right ventricular cardiomyopathy (ARVC), be restricted to patients with a LVEF below 45%. Histological data obtained in the ARVC group showed signs of acute or chronic myocarditis (in the right and left ventricles). It can be hypothesized that patients with arrhythmogenic right ventricular dysplasia (ARVD) may be prone to develop infectious myocarditis. In patients in whom an abnormal host immune response had been seen, progressive deterioration of right and left ventricular function could be observed. This pattern may be superimposed on the genetically determined background of ARVD. This could explain the wide spectrum of clinical presentation observed in patients with tachycardia originating in an abnormal right ventricle.

The arrhythmogenic right ventricle. Dysplasia versus cardiomyopathy

The natural history of arrhythmogenic right ventricular cardiomyopathy is determined by the electrical instability of the dystrophic myocardium, which can precipitate arrhythmic cardiac arrest any time during the course of the disease and by the progressive myocardial loss that results in ventricular dysfunction and heart failure. Sudden death accounts for the majority of the fatal events but its occurrence is mostly unpredictable. There are no prospective and controlled studies assessing clinical markers that can predict the occurrence of life-threatening ventricular arrhythmias. However, the noninvasive risk profile, which emerges from retrospective analysis of clinical and pathologic series, is characterized by history of syncope, physical exercise, spontaneous ventricular tachycardia or ventricular fibrillation, right ventricular dysfunction, left ventricular involvement, right precordial negative T wave, right bundle branch block, QT-QRS dispersion, right precordial ST-segment elevation and late potentials. At present only QRS dispersion, history of syncope and right and/or left ventricular abnormalities at radionuclide angiography proved to be independent noninvasive predictors of sudden death.

Noninvasive risk stratification in arrhythmogenic right ventricular cardiomyopathy.

https://www.internationaljournalofcardiology.com/article/S0167-5273(99)00143-6/pdf

Heart failure in arrhythmogenic right ventricular dysplasia-cardiomyopathy

The authors report the first case of arrhythmogenic right ventricular dysplasia presenting with a sudden death due to primary ventricular fibrillation (ventricular fibrillation not preceded by ventricular tachycardia) recorded by the Holter method. The patient was a 56 year old man whose only complaint was near syncopal case is the fact that it is the first documented case of ventricular fibrillation revealing arrhythmogenic right ventricular dysplasia, the diagnosis of which was made at autopsy. In addition, the Holter recording showed the factors which triggered the arrhythmia: the "trigger" of 4 monomorphic ventricular extrasystoles during the minute preceding the ventricular fibrillation; the arrhythmogenic substrate giving rise to late ventricular potentials and, finally, the analysis of the R-R intervals suggesting a role of the sympathetic and parasympathetic nervous systems. Holter recordings could help identify subjects at high risk of severe ventricular arrhythmias.

[Holter and sudden death: value in a case of arrhythmogenic right ventricular dysplasia].

The authors report 8 cases of regular tachycardia with wide QRS complexes during treatment with Vaughan-Williams class 1 antiarrhythmic drugs. These antiarrhythmics, prescribed to prevent atrial fibrillation (3 patients) and atrial flutter (5 patients), were flecainide in 4 cases, propafenone in 2 cases and cibenzoline and hydroquinidine respectively associated with digitoxine and propranolol. These wide complex tachycardias were regular atrial tachycardias with 1/1 conduction to the ventricle. The action of the drug, more pronounced on intra-atrial conduction velocities than on atrioventricular node refractoriness resulted in transformation of flutter at 300 cycles/min with 2/1 conduction and a ventricular rate of 150 cycles/min to atrial flutter at 210 cycles/min with 1/1 ventricular conduction. This acceleration of the ventricular rate was accompanied by widening of the QRS complex. Using the new ventricular tachycardia criteria recently published by Brugada resulted in a diagnostic error in 7 out of the 8 cases. The recording of a wide QRS complex tachycardia in a patient on class 1 antiarrhythmic therapy for an atrial arrhythmia should alert the physician to 1/1 atrial tachycardia despite morphological electrocardiographic criteria of ventricular tachycardia. The 1/1 atrial tachycardia may be poorly tolerated and require emergency treatment. The preventive association of a drug which slows conduction through the atrioventricular node is not always effective.

[Supraventricular tachycardia with wide QRS complexes during Vaughan-Williams class I anti-arrhythmic treatment. Diagnostic and therapeutic implications]

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[Holter and sudden death: value in a case of arrhythmogenic right ventricular dysplasia].

[Supraventricular tachycardia with wide QRS complexes during Vaughan-Williams class I anti-arrhythmic treatment. Diagnostic and therapeutic implications]