[ARVD-FORUM] Answers by Drs. Sandeep Joshi and Dr. Jonathan S. Steinberg.

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English - Spanish

Why in this disease, sotalol seems to be more efficient as an antiarrhythmic 
agent than amiodarone? 
 
Studies have shown that increased heart rate with shortening of the coupling 
intervals of the first cycles preceding the tachycardia may be due to a 
change in the autonomic balance, i.e. an increased sympathetic tone in ARVD.  
Some reports suggest therapeutic efficacy rates of sotalol in the range of 
68%-83%, at least as judged by noninducibility in the electrophysiology (EP) 
laboratory.  Required doses are often high and necessary to achieve beta 
blockade.  Greater success compared to amiodarone is not based on randomized 
comparisons, but may be due to better tolerance.

- Sen-Chowdhry S, Lowe MD, Sporton SC, et al.  Arrhythmogenic right 
ventricular cardiomyopathy: Clinical presentation, diagnosis, and management.  
Am J Med 2004; 117:685-695.
- Wichter T, Borggrefe M, Haverkamp W, et al.  Efficacy of antiarrhythmic 
drugs in patients with arrhythmogenic right ventricular disease.  Results in 
patients with inducible and noninducible ventricular tachycardia.  Circulation 
1992; 86:29-37.
- Marcus FI, Fontaine GH, Frank R, et al.  Long term follow up in patients 
with arrhythmogenic right ventricular disease.  Eur Heart J 1989; 10 (suppl 
D):68-73.

What criteria would you follow to consider an asymptomatic patient with ARVD 
as in high risk for developing sudden death (SCD) and therefore, for the 
implantation of an ICD? 
 
Risk factors for SCD in the asymptomatic patient with ARVD are poorly 
described.  Presentation with syncope and a severely depressed RV function 
have been reported as risk factors for SCD.  Complicating the challenges of 
risk stratification is the fact that ARVD is a progressive disease and the 
patient’s risk of SCD may increase with time.  Improved risk stratification 
will clarify the indications for ICD therapy in the asymptomatic patient.  

- Corrado D, Basso C, Nava A, et al.  Arrhythmogenic right ventricular 
cardiomyopathy: Current diagnostic and management strategies.  Cardiology In 
Review 2001; 9(5):259-265.
- Marcus FI, Fontaine G, Guiraudon G, et al.  Right ventricular dysplasia: A 
report of 24 adult cases.  Circulation 1982;65:384-398
- Corrado D, Basso C, Thiene G, et al.  Spectrum of clinicopathological 
manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: 
A multicenter study.  J Am Coll Cardiology 1997;30:1512-1520.
- Corrado D, Leoni L, Link MS, et al.  Implantable cardioverter-defibrillator 
therapy for prevention of sudden death in patients with arrhythmogenic right 
ventricular cardiomyopathy/dysplasia.  Circulation 2003;108:3084-3091.
- Link MS, Wang PJ, Haugh CJ, et al.  Arrhythmogenic right ventricular 
dysplasia: Clinical results with implantable cardioverter defibrillators.  J 
Intervent Card Electrophysiol 1997;1:41-48.

A cardioverter defibrillator should be implanted in patients with ARVD and 
sustained VT? 
 
ICD therapy confers protection against SCD and is indicated in cardiac arrest 
survivors, those who present with syncope thought to be due to VT, or VT 
refractory to drug therapy.  Some centers use pharmacological therapy as 
first line treatment in patients without any of the aforementioned conditions 
for sustained VT.  However depending on the clinical circumstances and prior 
history, ICD therapy may be warranted in the vast majority of high risk 
patients.  

- Sen-Chowdhry S, Lowe MD, Sporton SC, et al.  Arrhythmogenic right 
ventricular cardiomyopathy: Clinical presentation, diagnosis, and management.  
Am J Med 2004; 117:685-695.
- Corrado D, Basso C, Nava A, et al.  Arrhythmogenic right ventricular 
cardiomyopathy: Current diagnostic and management strategies. Cardiology In 
Review 2001; 9(5):259-265.
- R Tavernier, S Gevaert, J De Sutter, et al.  Long term results of 
cardioverter-defibrillator implantation in patients with right ventricular 
dysplasia and malignant ventricular tachyarrhythmias.  Heart 2001;85(1):53-6.
- Roguin A, Bomma CS, Nasir K, et al.  Implantable cardioverter-defibrillators 
in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy.  
J Am Coll Cardiol 2004;43:1843-52.

In the cases in which ICD is indicated, is it convenient to associate an 
antiarrhythmic drug? If so, which one? 
 
Among patients with an ICD for ARVD sustained VT is often terminated via 
antitachycardia pacing without the need for cardioversion, therefore allowing 
the reduction of pharmacological therapy in patients with debilitating 
adverse drug effects.  Insertion of an ICD may allow withdrawal or complete 
discontinuation of drug therapy.  However, when necessary after device 
placement to reduce the likelihood of discharge, pharmacological drug therapy 
may be instituted.  Sotalol has been effective in this patient population due 
to its combination of beta-adrenergic receptor blockade properties and class 
III antiarrhythmic activities.   Antiarrhythmic drugs are not routinely 
required. 

Sandeep Joshi and Dr. Jonathan S. Steinberg.

-------------------------

Por que en esta enfermedad, el sotalol parece ser mas eficiente como agente 
antiarritmico que la amiodarona? 

Los estudios han demostrado que una frecuencia cardiaca aumentada con 
acortamiento de los intervalos de acoplamiento de los primeros ciclos que 
preceden la taquicardia pueden deberse a un cambio en el balance autonomico, 
es decir, un tono simpatico aumentado en la DAVD. 
Algunos informes sugieren frecuencias de eficacia terapeutica de sotalol en el 
rango del 68%-83%, por lo menos considerando la no inducibilidad en el 
laboratorio electrofisiologico. Las dosis requeridas a menudo son altas y 
necesarias para alcanzar el beta-bloqueo. El mayor exito comparado con la 
amiodarona no se basa en comparaciones randomizadas, pero pueden deberse a 
una mejor tolerancia.

- Sen-Chowdhry S, Lowe MD, Sporton SC, et al.  Arrhythmogenic right 
ventricular cardiomyopathy: Clinical presentation, diagnosis, and management.  
Am J Med 2004; 117:685-695.
- Wichter T, Borggrefe M, Haverkamp W, et al.  Efficacy of antiarrhythmic 
drugs in patients with arrhythmogenic right ventricular disease.  Results in 
patients with inducible and noninducible ventricular tachycardia.  
Circulation 1992; 86:29-37.
- Marcus FI, Fontaine GH, Frank R, et al.  Long term follow up in patients 
with arrhythmogenic right ventricular disease.  Eur Heart J 1989; 10 (suppl 
D):68-73.

Que criterios seguiria para considerar que un paciente asintomatico con DAVD 
esta en alto riesgo de desarrollar muerte subita (MS) y por lo tanto, para el 
implante de un CDI? 

Los factores de riesgo para la MS en el paciente asintomatico con DAVD se 
describen pobremente. Se ha informado sobre la presentacion con sincope y 
funcion del VD gravemente deprimida como factores de riesgo para la MS. El 
hecho de que la DAVD es una enfermedad progresiva y que el riesgo del 
paciente de tener MS puede aumentar con el tiempo, complica los desafios de 
la estratificacion de riesgo. Una estratificacion de riesgo mejorada aclarara 
las indicaciones para la terapia con CDI en el paciente asintomatico. 

- Corrado D, Basso C, Nava A, et al.  Arrhythmogenic right ventricular 
cardiomyopathy: Current diagnostic and management strategies.  Cardiology In 
Review 2001; 9(5):259-265.
- Marcus FI, Fontaine G, Guiraudon G, et al.  Right ventricular dysplasia: A 
report of 24 adult cases.  Circulation 1982;65:384-398 
- Corrado D, Basso C, Thiene G, et al.  Spectrum of clinicopathological 
manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: 
A multicenter study.  J Am Coll Cardiology 1997;30:1512-1520. 
- Corrado D, Leoni L, Link MS, et al.  Implantable cardioverter-defibrillator 
therapy for prevention of sudden death in patients with arrhythmogenic right 
ventricular cardiomyopathy/dysplasia.  Circulation 2003;108:3084-3091.
- Link MS, Wang PJ, Haugh CJ, et al.  Arrhythmogenic right ventricular 
dysplasia: Clinical results with implantable cardioverter defibrillators.  J 
Intervent Card Electrophysiol 1997;1:41-48.

Un cardiodesfibrilador deberia ser implantado en pacientes con DAVD y TV 
sostenida? 

La terapia con CDI confiere proteccion contra la MS y se indica en los 
sobrevivientes de paro cardiaco, aquellos que se presentan con sincope que se 
piensa que se debe a TV o TV refractaria a la terapia con drogas. Algunos 
centros usan terapia farmacologica como tratamiento de primera linea en 
pacientes sin ninguna de las afecciones antes mencionadas para la TV 
sostenida. Sin embargo, dependiendo de las circunstancias clinicas y la 
historia previa, la terapia con CDI puede ser la unica opcion en la vasta 
mayoria de los pacientes con alto riesgo. 

- Sen-Chowdhry S, Lowe MD, Sporton SC, et al.  Arrhythmogenic right 
ventricular cardiomyopathy: Clinical presentation, diagnosis, and management.  
Am J Med 2004; 117:685-695. 
- Corrado D, Basso C, Nava A, et al.  Arrhythmogenic right ventricular 
cardiomyopathy: Current diagnostic and management strategies. Cardiology In 
Review 2001; 9(5):259-265. 
- R Tavernier, S Gevaert, J De Sutter, et al.  Long term results of  
cardioverter-defibrillator implantation in patients with right ventricular 
dysplasia and malignant ventricular tachyarrhythmias.  Heart 2001;85(1):53-6.
- Roguin A, Bomma CS, Nasir K, et al.  Implantable cardioverter-defibrillators 
in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. J 
Am Coll Cardiol 2004;43:1843-52.

En los casos en que se indique CDI, es conveniente asociar una droga 
antiarritmica? Si es asi, cual?

Entre los pacientes con CDI por DAVD, la TV sostenida con frecuencia es 
terminada mediante estimulacion antitaquicardica sin necesidad de 
cardioversion, permitiendo por lo tanto, la reduccion de la terapia 
farmacologica en pacientes con efectos adversos y debilitantes por las 
drogas. El implante de un CDI puede permitir la interrupcion o la 
discontinuacion completa de la terapia con drogas. Sin embargo, se puede 
iniciar terapia con drogas cuando sea necesario despues de la colocacion del 
dispositivo para reducir la posibilidad de descarga. El sotalol ha sido 
efectivo en esta poblacion de pacientes por su combinacion de propiedades de 
bloqueo de receptores beta-adrenergicos y actividades antiarritmicas clase 
III. Las drogas antiarritmicas no son necesarias en forma rutinaria. 

Sandeep Joshi and Dr. Jonathan S. Steinberg.




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