Brett Sperry, MD Profile picture
Aug 24, 2018 9 tweets 6 min read Twitter logo Read on Twitter
Sorry @venkmurthy @onco_cardiology @IbrahimMSaeed1 and others but #WhyCMR is not on this algorithm. Though, it is used frequently as a “catch all” diagnostic test for various cardiomyopathies.
I use cardiac MRI frequently for non-invasive evaluation in AL. Still, you will need biopsy proof of amyloid somewhere in the body before most hematologists will treat.

Amazing summary image by NAC group in London:

Aortic stenosis: ATTR and AS coexist in the elderly. Common cause of LFLG AS. We have described this @maz_hanna @ClevelandClinic as well as @JoaoLCavalcante, NAC, Columbia, +more. 16% of TAVRs and 30% of LFLG AS with EF<50% may have ATTR.
Carpal tunnel syndrome: about 10% of patients will have Congo red in tenosynovial tissue at time of surgery and some will have cardiac involvement. Opportunity for early diagnosis, monitoring, treatment. 2 abstracts at ISA meeting and publication from us in the coming weeks.
Hypertensive patients with renal disease, particularly African Americans, will have an echo that mimics amyloidosis including apical sparing strain pattern. Don’t be fooled!
Staging systems from @MarthaGrogan1 @ADispenzieri @MorieGertz at @MayoClinic involve Troponin T, NTproBNP, and free light chain difference (for AL). Diagnosing subtype is important due to difference in prognosis!

Mainstay of therapy in AL with symptomatic HF is CyBorD (cyclophosphamide, bortezomib, and dexamethasone). Daratumumab if +myeloma. High dose melphalan + autologous stem cell transplant in those with lower Mayo stage and lower # organs involved. (evolving)
Treatment in ATTR is very exciting. Onpattro (patisiran) just approved by FDA from @Alnylam. Results of ATTR-ACT for tafamidis from @Pfizer expected within the week at #ESCCongress. Inotersen @Akceatx coming soon.
Lots of nuance to many of these points, but I hope this was a helpful general overview and primer on #amyloidosis. The future is bright in this “rare” disease!

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More from @BrettSperryMD

Aug 24, 2018
As #ESCCongress nears, I thought I would do a #tweetorial on amyloidosis. Exciting times for the field and new data/treatments expected next week.

#FITSurvivalGuide #CardioTwitter @tony_breu @rodney_falk @marthagrogan1 @amyloidosisfdn @AmyloidosisSupp @Amyloidosis_ARC
What is amyloidosis?

A protein misfolding disorder in which one of thirty-five distinct proteins pathologically misfolds and aggregates extracellularly as insoluble amyloid fibrils, ultimately leading to organ dysfunction.
You can see that other diseases like Alzheimer’s involve amyloid deposition. We will focus on two types of amyloidosis that affect the heart and nervous system: immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis.
Read 16 tweets

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