Cardiopatia aritmogena destra, sinistra o biventricolare

1. Pasquale Vergara, MD, Ph-D
Arrhythmology Unit - San Raffaele Hospital - Milano - Italy
Cardiomiopatia aritmogena:
ventricolare destra, biventricolare o ventricolare sinistra?
Attualità in tema di Imaging integrato, criteri di classificazione ed
impatto prognostico
IMAGING ED ARITMIE

2. Arrhythmogenic Cardiomyopathy

3. Left dominant Arrhythmogenic Cardiomyopathy
Norman Circulation 2005
Desmoplakin

4. Ventricular involvement in ACM
17%
13%
Miles Circulation 2019

6. Left-Dominant Arrhythmogenic Cardiomyopathy Sen-Chowdhry JACC 2008

7. Left-Dominant Arrhythmogenic Cardiomyopathy Sen-Chowdhry JACC 2008
Five subjects fulfilled the Chin andJenni
criteria for LVNC
confluent
patchy circumferential

8. ARVC, BiV-ACM & LDAC continuum
<1
⇣ with age
≥ 1.4
⇡ with age
∽ 1
stable
A
R
V
C
B
i
v
L
D
A
C
RV/LV volume ratio
RV/LV changes

9. ARVC & LDAC: 1 or 2 diseases?
In LDAC, >30% had LV dilation and/or impairment in the
presence of preserved right-sided volumes and function.
Septum is generally spared even in late-stage ARVC with LV
involvement.
In contrast, >50% of LDAC had septal LGE
Isolated global RV dysfunction precedes LV involvement in
ARVC
A
R
V
C
L
D
A
C
A
R
V
C
L
D
A
C

10. ⇝ Sudden death from ACM:
» Of 5205 cases of SCD, 202 (4%) were diagnosed with ACM
» 82% male; 90% white.
» mean age at death 35.4±13.2 years (median 34.5 years).
Arrhythmogenic Cardiomyopathy & Sudden death
Miles Circulation 2019
none
93%
ACM
3%
DCM
4%
Ante-mortem diagnosis
⇝ Overlap between DCM and ACM
⇝ The great majority of cases (93%) did not have an ante-
mortem diagnosis of cardiac disease

11. How to distinguish between LDAC and DCM ?
aneurisms
D C M
L D A C
Subepicardial LGE
Mid-wall LGE Mid-wall LGE
VA exceeds the degree
of morphological
abnormality and
systolic impairment
VA & SCD in overt
systolic disfunction
VA with EF∽ 55% heart failure
pathognomonic
MRI
VA
Mode of
presentation:

12. ⇝ 2011 SD during sport activity
⇝ Coronary angio: normal
⇝ Echo: LVEF 43%; MRI: fibro-adipous
infiltration of LV
⇝ ICD implant
Left arrhythmogenic cardiomyopathy case
I
II
III
aVR
aVL
aVF
V1
V2
V3
V4
V5
V6

13. …put things in the right perspective

14. ACM task force criteria Marcus EHJ 2010;31: 806–814

15. Stratifying prognosis in ARVC Aquaro AmJCardiol 2018; 122:1745−1753
» Sudden cardiac death
» Resuscitated cardiac arrest
» Appropriate ICD shock

16. Stratifying prognosis in ARVC #MRI Aquaro AmJCardiol 2018; 122:1745−1753
⇝ Abnormal CMR defined as ≥1 criteria:
» RV wall motion abnormalities (akinesia, dyskinesia)
» LV wall motion abnormalities (hypokinesia,akinesia, dyskinesia)
» RV EF≤40%;
» LVEF<50%
» RV &/or LV fat infiltration
» LGE in RV &/or LV myocardium
⇝ Survival from Hard cardiac events:
» Sudden cardiac death
» Resuscitated cardiac arrest
» Appropriate ICD shock
1 pt with CMR- had event
NPV: 96.9%
No events
Definitive ARVC diagnosis
whole ARVC population

17. Stratifying prognosis in ARVC #MRI Aquaro AmJCardiol 2018; 122:1745−1753
Fat infiltration
&/or LGE
Survival from Hard Cardiac Events in whole population

18. ⇝ New tools for early
diagnosis?
⇝ Non invasive LV
electrophysiologic
evaluation?

19. Electric substrate @ ECGI in a healthy adult Andrews CircAE 2017;10:e005105
Activation Recovery intervals
~ Repolarization
Abnormal conduction
~ reentry

20. ACM: Advanced RV substrate Andrews CircAE 2017;10:e005105
Activation Recovery intervals
» earliest activation originating from the basal inferior LV (*)
» Normal unipolar electrograms
» activation-recovery interval (ARI) values were prolonged

21. » earliest epicardial activation originating from the basal lateral left ventricle
» RV & LV fractionated unipolar electrograms
» activation-recovery interval (ARI) values were prolonged
ACM: Biventricular substrate Andrews CircAE 2017;10:e005105
Activation Recovery intervals

22. LV dysfunction & ACM diagnosis Vives-Gilabert Int J Cardiol 2019;274:237–244
⇝ 35 ACM patients with LV involvement (LGE): 22 LV only + 13 biventricular
⇝ 23 non affected family members
30% of AC patients previously classified as
‘borderline’ or ‘possible’ categories according to 2010 TFC
moved to a ‘definite’ AC diagnosis without affecting
specificity

23. CONCLUSIONS
⇝ A significant number of patients with suspect of DCM,
may be affected by ACM
⇝ Most (70%) ACM patients have biventricular involvement
⇝ Current ARVC criteria do not allow L-ACM diagnosis
⇝ Best efforts should be payed to identification of LV
involvement