In order to bring you the best possible user experience, this site uses Javascript. If you are seeing this message, it is likely that the Javascript option in your browser is disabled. For optimal viewing of this site, please ensure that Javascript is enabled for your browser.

The free consultation period for this content is over.

It is now only available year-round to HFA Silver & Gold Members, Fellows of the ESC and Young combined Members

Imaging phenotypes of left-dominant arrhytmogenic cardiomyopathy and dilated cardiomyopathy - a comparative magnetic resonance study

Session Comorbidities and cardiomyopathies - How to manage?

Speaker Rocio Eiros

Event : Heart Failure 2018

  • Topic : heart failure
  • Sub-topic : Chronic Heart Failure – Diagnostic Methods: Imaging
  • Session type : Rapid Fire Abstracts

Authors : R Eiros (Navarra,ES), JB Augusto (Lisboa,PT), T Treibel (London,GB), G Captur (London,GB), MM Akhtar (London,GB), A Protonotarios (London,GB), T Gkosios (London,GB), K Savvatis (London,GB), S Mohiddin (London,GB), J Moon (London,GB), LR Lopes (London,GB)

Authors:
R Eiros1 , JB Augusto2 , T Treibel3 , G Captur3 , MM Akhtar3 , A Protonotarios3 , T Gkosios3 , K Savvatis3 , S Mohiddin3 , J Moon3 , LR Lopes3 , 1University Clinic of Navarra, Cardiology - Navarra - Spain , 2Hospital Prof. Doutor Fernando Fonseca - Lisboa - Portugal , 3Barts Health NHS Trust - London - United Kingdom ,

Citation:

Background: Emerging data have illustrated the diversity of genetic causes of arrhythmogenic cardiomyopathy (AC) with dominant LV involvement (ALVC).The relation between genotype and clinical phenotype is scarce and there are no systematic studies examining imaging phenotypes using cardiac magnetic resonance (CMR).

Objective: To identify phenotypic traits that distinguish ALVC from other genetic causes of dilated cardiomyopathy(DCM). Methods:CMR data acquired for 30 patients (pts) with confirmed ALVC /DCM associated mutations in desmoplakin (DSP), filamin C (FLNC), lamin A/C (LMNA), titin (TTN) and BAG3 were retrospectively re-analysed and compared on the basis of different individual genes and when grouped as ALVC (DSP / FLNC) vs DCM (others).

Results: Causal genes included DSP (n=11), FLNC (n=4), lamin A/C (n=6), BAG3 (n=4), titin (n=5). A comparison of clinical and imaging features is presented in Table 1. Figure 1 shows a comparison of late gadolinium enhancement patterns between different genes.

Conclusions Patients with genetic causes of ALVC have a higher burden of myocardial scar, in a more often sub-epicardial and ring-shaped pattern, in contrast with a more discrete midwall septal late gadolinium enhancement in other genetic causes of DCM. Other imaging parameters, including LV and RV sizes and function were similar.

DSP-FLNC

(n=15)

Non-DSP-FLNC

(n=15)

P Value

Age, years

44.5± 17.2

44.2± 13.3

0.972

Male gender,n (%)

7 (46.7)

11 (73.3)

0.136

LV EDVi,ml/m2

105.1± 32.3

97.6 ± 20.6

0.463

LV SVi,ml/m2

49.5 ± 10.6

48.5 ± 13.3

0.816

LV Mass index, g/m2

59.7 ± 10.9

60.4 ± 16.3

0.883

LV EF, %

48.1 ± 13

49.2 ± 7.5

0.784

RV EDVi,ml/m2

90.1 ± 19.0

81.1 ± 25.7

0.284

TAPSE,mm

21 ± 4.6

21.6 ± 6.8

0.827

RV EF, %

52.6 ± 16.3

56.4 ± 5.7

0.405

LGE 5SD, g

14.2 (7.8-18.9)

1.12 (0-34)

<0.001

LGE over median 4.5g, n (%)

13 (86.7)

2 (13.3)

<0.001

LGE as % of mass

13.2 (9.9-19.5)

0.7 (0-2.3)

<0.001

LGE over median 5.2%,n (%)

13 (86.7)

2 (13.3%9

<0.001

Subepicardial LGE,n (%)

13 (86.7)

2 (13.3)

0.001

Ring-shaped LGE, n (%)*

11 (73.3)

0

<0.001

* > 3 contiguous subepicardial segments in the same slice.

The free consultation period for this content is over.

It is now only available year-round to HFA Silver & Gold Members, Fellows of the ESC and Young combined Members

Members get more

Join now
  • 1ESC Professional Members – access all resources from general ESC events 
  • 2ESC Association Members (Ivory, Silver, Gold) – access your Association’s resources
  • 3Under 40 or in training - with a Combined Membership, access all resources
Join now

Our sponsors

ESC 365 is supported by Bayer, Boehringer Ingelheim and Lilly Alliance, Bristol-Myers Squibb and Pfizer Alliance, Novartis Pharma AG and Vifor Pharma in the form of educational grants. The sponsors were not involved in the development of this platform and had no influence on its content.

logo esc

Our mission: To reduce the burden of cardiovascular disease

Who we are