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

Blood correction of native T1 increases detection of cardiac involvement in patients with fabry disease

Session Poster session 1

Speaker Jannike Nickander

Congress : EuroCMR 2019

  • Topic : imaging
  • Sub-topic : T1 and T2 Mapping, T2*
  • Session type : Poster Session
  • FP Number : P171

Authors : J Nickander (Stockholm,SE), BR Cole (Sydney,AU), S Nordin (London,GB), R Vijapurapu (Birmingham,GB), RP Steeds (Birmingham,GB), JC Moon (London,GB), P Kellman (Washington,US), M Ugander (Stockholm,SE), R Kozor (Sydney,AU)

Authors:
J Nickander1 , BR Cole2 , S Nordin3 , R Vijapurapu4 , RP Steeds4 , JC Moon3 , P Kellman5 , M Ugander1 , R Kozor2 , 1Karolinska Institute, Clinical Physiology - Stockholm - Sweden , 2University of Sydney, Sydney Medical School - Sydney - Australia , 3University College London, Institute of Cardiovascular Science - London - United Kingdom of Great Britain & Northern Ireland , 4University of Birmingham, Institute of Cardiovascular Sciences - Birmingham - United Kingdom of Great Britain & Northern Ireland , 5National Institute of Health (Home), National Heart, Lung, and Blood Institute - Washington - United States of America ,

Citation:
European Heart Journal - Cardiovascular Imaging ( 2019 ) 20 ( Supplement 2 ), ii128

Background:

Fabry disease (FD) is a rare, lysosomal storage disorder with myocardial sphingolipid accumulation detectable by CMR native T1 mapping. Myocardial native T1 is affected by intramyocardial blood, and as blood has a longer and more variable T1 compared to myocardial T1, blood correction of myocardial T1 has been proposed.

Purpose:

To investigate whether blood correction of native T1 values increases the detection of myocardial sphingolipid accumulation (low native T1) in FD patients.

Methods:

A retrospective multinational multicenter study, FD patients (n=218, age 47±16 years) and healthy volunteers (n=117, age 29±5 years) underwent CMR at 1.5T (Siemens) at three different sites with a comparison set of non-FD patients (n=200, age 51 ± 18 years) at a fourth site. Native T1 maps used MOLLI. T1 was measured with a single region of interest in the LV septum, and both LV and RV blood pools. A linear correction model using mean R1 (1/T1 average LV + RV blood) was developed at the fourth site as previously described (1), and applied to the FD population and healthy volunteers. Sphingolipid accumulation in FD patients was defined as a native myocardial T1 value below the site specific 95% limits of agreement in healthy volunteers, both for corrected and uncorrected measures.

Results:

Prior to blood correction, 134 FD patients (61%) had low myocardial native T1 indicating sphingolipid accumulation (84 (39%) normal myocardial native T1). Of the 84 patients with normal native T1 prior to blood correction, 25 (30%) were reclassified as having low myocardial native T1 following blood correction. Of these 25 reclassified patients, 14 (56%) did not have any CMR signs of cardiac involvement by late gadolinium enhancement or left ventricular hypertrophy. Thus, 14/84 (17%) were reclassified as having cardiac involvement following blood correction.  By comparison, 4/134 (3%) of patients with low native T1 prior to blood correction were reclassified to normal myocardial native T1 following blood correction.

Conclusion:

Blood correction of myocardial native T1 increases the detection of low myocardial native T1 in 17% of patients with Fabry disease that otherwise had no CMR signs of cardiac involvement. Blood correction of myocardial native T1 has the potential to impact the ability to detect and track cardiac involvement in Fabry disease, and merits prospective studies and histological validation.



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