Arrhythmogenic Right Ventricular Cardiomyopathy: Difference between revisions

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Arrhythmogenic Right Ventricular Cardiomyopathy (view source)

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One unique form of ARVD, called Naxos disease (after the Greek island where it was first diagnosed), has an autosomal recessive pattern of inheritance.  
One unique form of ARVD, called Naxos disease (after the Greek island where it was first diagnosed), has an autosomal recessive pattern of inheritance.  


'''Diagnosis''' ARVC is a difficult diagnosis to make. Therefore, the European Society of Cardiology has created a list of diagnositc criteria for the diagnosis of ARVC<cite>#McKenna1994</cite>
'''Diagnosis''' ARVC is a difficult diagnosis to make. Therefore, the European Society of Cardiology has created a list of diagnositc criteria for the diagnosis of ARVC<cite>#McKenna1994</cite> (see table).
 
 
===Treatment===
Treatment focusses on avoiding complications.<cite>ACC2006</cite>
*Medication:
**anti-arrithmics: Amiodarone, Sotalol
**ACE-inhibitors to prevent cardiac remodelling
*[[ICD]] implantation is recommended for the prevention of sudden cardiac death in patients with ARVC with documented sustained VT or VF who are receiving chronic optimal medical therapy.
*[[ICD]]] implantation can be considered for the prevention of sudden cardiac death in patients with ARVC with extensive disease, including those with left ventricular involvement, 1 or more affected family member with SCD, or undiagnosed syncope when [[Ventricular Tachycardia|ventricular tachycardia]] or [[Ventricular Fibrillation|ventricular Fibrillation]] has not been excluded as the cause of syncope, who are receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 y.
*Radiofrequency [[ablation]] can be useful as adjunctive therapy in management of patients with ARVC with recurrent [[Ventricular Tachycardia|ventricular tachycardia]], despite optimal antiarrhythmic drug therapy.
{| class="wikitable" align="left" width="400px"
{| class="wikitable" align="left" width="400px"
!Major diagnostic criteria for Aritmogenic Right Ventricular Cardiomyopathy<cite>McKenna1994</cite>
!Major diagnostic criteria for Aritmogenic Right Ventricular Cardiomyopathy<cite>McKenna1994</cite>
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|}
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[[Image:epsilon_wave.png|thumb|ECG with an epsilon wave in V1]]
[[Image:epsilon_wave.png|thumb|ECG with an epsilon wave in V1]]
'''Treatment''' focuses on avoiding complications.<cite>ACC2006</cite>
*Medication:
**anti-arrithmics: Amiodarone, Sotalol
**ACE-inhibitors to prevent cardiac remodelling
*[[ICD]] implantation is recommended for the prevention of sudden cardiac death in patients with ARVC with documented sustained VT or VF who are receiving chronic optimal medical therapy.
*[[ICD]]] implantation can be considered for the prevention of sudden cardiac death in patients with ARVC with extensive disease, including those with left ventricular involvement, 1 or more affected family member with SCD, or undiagnosed syncope when [[Ventricular Tachycardia|ventricular tachycardia]] or [[Ventricular Fibrillation|ventricular Fibrillation]] has not been excluded as the cause of syncope, who are receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 y.
*Radiofrequency [[ablation]] can be useful as adjunctive therapy in management of patients with ARVC with recurrent [[Ventricular Tachycardia|ventricular tachycardia]], despite optimal antiarrhythmic drug therapy.
==Referenties==
==Referenties==
<biblio>
<biblio>
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