Difference between revisions of "Arrhythmogenic Right Ventricular Cardiomyopathy"

From ECGpedia
Jump to navigation Jump to search
Line 58: Line 58:
 
</gallery>
 
</gallery>
  
 +
==Table 1==
 
{| class="wikitable"
 
{| class="wikitable"
 
|+ Table 1. Comparison of Original and Revised Task Force Criteria
 
|+ Table 1. Comparison of Original and Revised Task Force Criteria
Line 104: Line 105:
 
** Ratio of RV end-diastolic volume to BSA &ge;100 to &lt;110 mL/m<sup>2</sup> (male) or &ge;90 to &lt;100 mL/m<sup>2</sup> (female)
 
** Ratio of RV end-diastolic volume to BSA &ge;100 to &lt;110 mL/m<sup>2</sup> (male) or &ge;90 to &lt;100 mL/m<sup>2</sup> (female)
 
** <em>or</em> RV ejection fraction &gt;40% to &le;45%
 
** <em>or</em> RV ejection fraction &gt;40% to &le;45%
 +
|-
 +
| colspan="2" | II. Tissue characterization of wall
 +
|-
 +
| colspan="2" | Major
 +
|-
 +
| valign="top" |
 +
* Fibrofatty replacement of myocardium on endomyocardial biopsy
 +
|
 +
* Residual myocytes &lt;60% by morphometric analysis (or &lt;50% if estimated), with fibrous replacement of the RV free wall myocardium in &ge;1 sample, with or without fatty replacement of tissue on endomyocardial biopsy
 +
|-
 +
| colspan="2" | Minor
 +
|-
 +
|
 +
| valign="top" |
 +
* Residual myocytes 60% to 75% by morphometric analysis (or 50% to 65% if estimated), with fibrous replacement of the RV free wall myocardium in &ge;1 sample, with or without fatty replacement of tissue on endomyocardial biopsy
 
|}
 
|}
  

Revision as of 04:27, 13 April 2010

Author(s) J.S.S.G. de Jong, MD
Moderator J.S.S.G. de Jong, MD
Supervisor
some notes about authorship
ECG with an epsilon wave in V1
A section throughout the heart of an ARVC patient. (A) Transmural fatty replacement of the right ventricular free wall. (B) Myocardial atrophy is confined to the right ventricle and substantially spares the interventricular septum as well as the left ventricular free wall. [1] Reproduced with permission from BMJ Publishing Group Ltd.

Arrhythmogenic Right Ventricular Cardiomyopathy, (ARVC, or ARVD: Arrhythmogenic Right Ventricular Disease) is characterized by fatty replacement and fibrosis of the heart. Most commonly the right ventricle apex and outflow tract are involved. However the left ventricle can be affected to.[2]

As a result of the fatty replacement and fibrosis, ventricular arrhythmias are common in this disease and can lead to palpitations, syncope and sudden death. At more advanced ages right ventricular pump failure can occur.

The diagnosis is based on major and minor criteria, as published by the European Society of Cardiology.[3]

ARVC is a progressive disease. The incidence is estimated to be 1:3.000-1:10.000. Manifestations are usually seen in teenagers. Although the diagnosis is more often made in athletes, sports are not thought to have a causative relationship with the disease. ARVD can occur in families; more than 9 different chromosomal defects have been described, most often with autosomal dominant 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 diagnostic criteria for the diagnosis of ARVC[3] (see table).

Major diagnostic criteria for Arrhythmogenic Right Ventricular Cardiomyopathy[3]
  • Familial disease confirmed at necroscopy or surgery
  • Severe dilatation and reduction of right ventricular ejection fraction with no (or only mild) LV impairment
  • Localized right ventricular aneurysms (akinetic or diskinetic areas with diastolic bulging)
  • Severe segmental dilataion of the right ventricle
  • Fibrofatty replacement of myocardium on endomyocardial biopsy
Diagnostic criteria that can be diagnosed on the ECG

In 2009 three more criteria were added[4]:

  • Terminal activation duration of QRS≥55ms
  • ventricular tachycardia with left bundle branch block morphology and superior axis, and
  • genetic criteria.

Treatment focuses on avoiding complications.[5]

  • Medication:
    • Anti-arrhythmics: Sotalol better than Amiodarone.
    • 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 (Sudden Cardiac Death), or undiagnosed syncope when ventricular tachycardia or 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, despite optimal antiarrhythmic drug therapy.

Table 1

Table 1. Comparison of Original and Revised Task Force Criteria
Original Task Force Criteria Revised Task Force Criteria
I. Global or regional dysfunction and structural alterations
Major
  • Severe dilatation and reduction of RV ejection fraction with no (or only mild) LV impairment
  • Localized RV aneurysms (akinetic or dyskinetic areas with diastolic bulging)
  • Severe segmental dilatation of the RV

By 2D echo:

  • Regional RV akinesia, dyskinesia, or aneurysm
  • and 1 of the following (end diastole):
    • PLAX RVOT ≥32 mm (corrected for body size [PLAX/BSA] ≥19 mm/m2)
    • PSAX RVOT ≥36 mm (corrected for body size [PSAX/BSA] ≥21 mm/m2)
    • or fractional area change ≤33%

By MRI:

  • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction
  • and 1 of the following:
    • Ratio of RV end-diastolic volume to BSA ≥110 mL/m2 (male) or ≥100 mL/m2 (female)
    • or RV ejection fraction ≤40%

By RV angiography:

  • Regional RV akinesia, dyskinesia, or aneurysm
Major
  • Mild global RV dilatation and/or ejection fraction reduction with normal LV
  • Mild segmental dilatation of the RV
  • Regional RV hypokinesia

By 2D echo:

  • Regional RV akinesia or dyskinesia
  • and 1 of the following (end diastole):
    • PLAX RVOT ≥29 to <32 mm (corrected for body size [PLAX/BSA] ≥16 to <19 mm/m2)
    • PSAX RVOT ≥32 to <36 mm (corrected for body size [PSAX/BSA] ≥18 to <21 mm/m2)
    • or fractional area change >33% to ≤40%

By MRI:

  • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction
  • and 1 of the following:
    • Ratio of RV end-diastolic volume to BSA ≥100 to <110 mL/m2 (male) or ≥90 to <100 mL/m2 (female)
    • or RV ejection fraction >40% to ≤45%
II. Tissue characterization of wall
Major
  • Fibrofatty replacement of myocardium on endomyocardial biopsy
  • Residual myocytes <60% by morphometric analysis (or <50% if estimated), with fibrous replacement of the RV free wall myocardium in ≥1 sample, with or without fatty replacement of tissue on endomyocardial biopsy
Minor
  • Residual myocytes 60% to 75% by morphometric analysis (or 50% to 65% if estimated), with fibrous replacement of the RV free wall myocardium in ≥1 sample, with or without fatty replacement of tissue on endomyocardial biopsy

References

Error fetching PMID 8142187:
Error fetching PMID 10768917:
Error fetching PMID 16949478:
Error fetching PMID 19366719:
Error fetching PMID 20215590:
  1. Error fetching PMID 10768917: [Corrado]
  2. Error fetching PMID 19366719: [Corr]
  3. Error fetching PMID 8142187: [McKenna1994]
  4. Error fetching PMID 16949478: [ACC2006]
  5. Error fetching PMID 20215590: [Cox]

All Medline abstracts: PubMed | HubMed