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 EACVI Silver Members, Fellows of the ESC and Young combined Members

Quantitative perfusion mapping in Fabry disease

Session Rapid Fire Abstract 3: stress CMR: utility in coronary artery disease and beyond

Speaker Kristopher Knott

Congress : EuroCMR 2019

  • Topic : imaging
  • Sub-topic : Stress CMR
  • Session type : Rapid Fire Abstracts
  • FP Number : 304

Authors : K Knott (London,GB), JB Augusto (London,GB), S Nordin (London,GB), R Kozor (Sydney,AU), C Camaioni (London,GB), H Xue (Bethesda,US), RK Hughes (London,GB), C Manisty (London,GB), LAE Brown (Leeds,GB), U Ramaswami (London,GB), D Hughes (London,GB), P Kellman (Bethesda,US), S Plein (Leeds,GB), JC Moon (London,GB)

Authors:
K Knott1 , JB Augusto1 , S Nordin1 , R Kozor2 , C Camaioni3 , H Xue4 , RK Hughes1 , C Manisty1 , LAE Brown5 , U Ramaswami6 , D Hughes1 , P Kellman4 , S Plein5 , JC Moon1 , 1University College London - London - United Kingdom of Great Britain & Northern Ireland , 2University of Sydney - Sydney - Australia , 3Barts Health NHS Trust - London - United Kingdom of Great Britain & Northern Ireland , 4National Institutes of Health - Bethesda - United States of America , 5University of Leeds - Leeds - United Kingdom of Great Britain & Northern Ireland , 6Royal Free Hospital - London - United Kingdom of Great Britain & Northern Ireland ,

Citation:
European Heart Journal - Cardiovascular Imaging ( 2019 ) 20 ( Supplement 2 ), ii224

Background

Fabry disease (FD) is an X-linked lysosomal storage disease resulting in tissue sphingolipid accumulation. Although storage is important, other processes occur and can be measured including hypertrophy, inflammation and fibrosis. These can be quantified by multi-parametric CMR including cine imaging, T1 and T2 mapping and late gadolinium enhancement (LGE).

Purpose

Recent developments in CMR perfusion mapping allow rapid in-line perfusion quantification permitting the assessment of microvascular dysfunction. We hypothesized that there is microvascular dysfunction in FD which is associated with storage, fibrosis and oedema.

Methods

A prospective, observational study of 44 FD patients (49 years, 43% male, 24 (55%) with left ventricular hypertrophy (LVH)) and 27 healthy controls. Multi-parametric CMR included vasodilator stress perfusion mapping. Myocardial blood flow (MBF) was measured and its associations with other processes investigated.

ResultsĀ 

Compared to LVH- FD, LVH+FD had a higher LV ejection fraction (73% vs 67%, p=0.04), lower T1 (937 vs 985ms, p=0.007), more LGE (85% vs 15%, p<0.001 and a lower stress MBF (1.76 vs 2.36 ml/g/min, p<0.001). The reduction in stress MBF was more pronounce in the subendocardium than subepicardium. LVH-FD had lower stress MBF than controls (2.36 vs 3.00 ml/g/min, p=0.001). Across all FD, LGE and low native T1 were independently associated with reduced stress MBF. On a per-segment basis segment basis, stress MBF was independently associated with increasing wall thickness, decreasing native T1, increasing T2, increasing ECV and LGE.

Conclusions

FD patients have reduced perfusion, particularly in the subendocardium with greater reductions with LVH, storage, oedema and scar. Perfusion is reduced even without LVH suggesting it is an early disease marker.

The free consultation period for this content is over.

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



Based on your interests

Members get more

Join now
  • 1ESC Professional Members – access all resources from ESC Congress and ESC Asia with APSC & AFC
  • 2ESC Association Members (Ivory, Silver, Gold) – access your Association’s congress resources
  • 3Under 40 or in training - with a Combined Membership, access resources from all congresses
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