2) Follow this 🧵to earn 0.5hr CE/#CME. This program is intended for #healthcare providers. Statement of accreditation & faculty disclosures at https://t.co/gvXca4G9Xm. This program supported by an independent educational grant from AstraZeneca.#MedEd #CardioTwitter #FOAMed
— cardio-met (@cardiomet_CE) November 15, 2023
4) You can see here that #amyloid deposition contributes to other diseases such as #Alzheimer’s. We will focus on two types of systemic #amyloidosis that affect the heart and nervous system: immunoglobulin #light_chain (#AL) and #transthyretin (#ATTR) amyloidosis. pic.twitter.com/YMGxNR7d91
— cardio-met (@cardiomet_CE) November 15, 2023
6) #AL #amyloid is caused by overproduction of clonal #immunoglobulin light chains originating in the bone marrow. About 20% of patients with AL #amyloidosis will also meet criteria for multiple myeloma #MM. pic.twitter.com/cP8BXVuM8E
— cardio-met (@cardiomet_CE) November 15, 2023
8) #ATTR #amyloidosis may occur as a result of the native (wild-type) protein or from a hereditary variant in the protein structure. This fantastic figure from Dr. Rapezzi depicts some common mutations on a spectrum from #cardiomyopathy to #neuropathy.
— cardio-met (@cardiomet_CE) November 15, 2023
🔓 https://t.co/dcWLXSXyt8 pic.twitter.com/LK55dYav0Y
10) #ROS should focus on polyneuropathy and/or entrapment #neuropathy like carpal tunnel syndrome #CTS or spinal stenosis which are common (ATTR > AL).#Macroglossia or periorbital purpura should 👉AL.
— cardio-met (@cardiomet_CE) November 15, 2023
Think #ATTR if rupture of the long head of the biceps tendon & Popeye sign. pic.twitter.com/Bn80j0mLD7
12a) Typical echo characteristics are increased #LV wall thickness and #diastolic dysfunction. “Sparkling” #myocardiumcan be seen, but is not sensitive or specific.
— cardio-met (@cardiomet_CE) November 15, 2023
13a) Longitudinal strain analysis reveals the classic “apical sparing” pattern with severely abnormal strains in the basal and mid segments. A relative ratio can be calculated as avg apical / (avg base + avg mid) strains, with values >1 being concerning for #cardiac #amyloid. pic.twitter.com/BZJtK8nHs5
— cardio-met (@cardiomet_CE) November 15, 2023
14) We know #strain can be helpful to reclassify patients with “LVH”, particularly into the #amyloidosis category.https://t.co/xoLgPUU4Ac pic.twitter.com/Y0QELoIS5j
— cardio-met (@cardiomet_CE) November 15, 2023
16a) STEP 1⃣: check free kappa/lambda light chains and serum/urine #immunofixation which rule out >95% of AL. #SPEP/#UPEP is not enough. Both kappa and lambda may be elevated (with K>L) in renal disease and inflammation.
— cardio-met (@cardiomet_CE) November 15, 2023
17a) Step 2⃣ : If light chain testing is abnormal, stat phone call & referral to #hematology. Bone marrow biopsy & fat biopsy are typical next steps. Incisional fat biopsy has highest yield to pick up #amyloidosis.
— cardio-met (@cardiomet_CE) November 15, 2023
18a) Step 3⃣ : Perform and interpret #TcPYP properly
— cardio-met (@cardiomet_CE) November 15, 2023
The TcPYP agent is infused; imaging with #SPECT or SPECT/CT is done 1 hr later. A 3-hour delay can be done by site preference. We rarely use 3-hour imaging–only if 1-hour interpretation is unclear.https://t.co/YbgYL0dry4 pic.twitter.com/751RtyhdA6
18c) Uptake on #SPECT typically starts in the septum at the base, progresses to include the entire myocardium. A less diffuse, apical sparing pattern of #LV uptake may ➡️better prognosis. Diffuse uptake & #RV uptake probably ➡️more advanced disease.https://t.co/rcUXdjVCVW pic.twitter.com/Ci8ABUpszw
— cardio-met (@cardiomet_CE) November 15, 2023
19a) Cardiac #MRI is used frequently as a diagnostic test for various cardiomyopathies, & is highly sensitive & specific for #ATTR_CA. But does not reliably differentiate AL vs ATTR, so not included in diagnostic algorithms.
— cardio-met (@cardiomet_CE) November 15, 2023
20a) SPECIAL POPULATIONS#AorticStenosis: #ATTR & #AS coexist in the elderly. Higher prevalence in the low-flow low-gradient AS population.
— cardio-met (@cardiomet_CE) November 15, 2023
20c) Carpal tunnel syndrome #CTS: ~ 10% of pts will have amyloid deposits in tenosynovial tissue at time of surgery and some will have #cardiac involvement. Recognition presents opportunity for early diagnosis, monitoring, genetic screening, & treatment.https://t.co/dzJYcmbCES pic.twitter.com/kQTnJyDDfQ
— cardio-met (@cardiomet_CE) November 15, 2023
21) PROGNOSIS
— cardio-met (@cardiomet_CE) November 15, 2023
See ⤵️ for staging systems for #AL and #ATTR.
Treatment significantly improves prognosis!
🔓 https://t.co/Rc1SzIQtt8
🔓https://t.co/Ii490zu0EC
🔓 https://t.co/fFrPrSdgzO pic.twitter.com/iyFqOxFtXl
22b) Autologous stem cell transplantation #ASCT can be considered in lower cardiac risk patients.https://t.co/4ZYF9NxMeI
— cardio-met (@cardiomet_CE) November 15, 2023
24a) Also,
— cardio-met (@cardiomet_CE) November 15, 2023
Clinical trials abound in #ATTR #amyloidosis.
👉 Improving basic science on understanding mechanism of #fibril formation and clearance.
👉Several ongoing trials on novel non-invasive diagnostic tools including #PET.
25a) So what have we learned? #Transthyretin, aka #prealbumin, is made in the liver and transports what proteins?
— cardio-met (@cardiomet_CE) November 15, 2023
a. thyroxine
b. steroid hormones
c. retinol binding protein (Vitamin A)
d. a & c
26a) If you are concerned about cardiac amyloidosis, what tests are needed to non-invasively diagnose ATTR_CA?
— cardio-met (@cardiomet_CE) November 15, 2023
a. Free light chains, SIFE/UIFE
b. TcPYP
c. Genetic testing
d. All of the above
27) There's 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! 😎
— cardio-met (@cardiomet_CE) November 15, 2023
28) YOUR future is bright, too, because you just earned 0.5hr 🆓CE/#CME! Claim your certificate now at https://t.co/YwNJ0yMOJH, and then one more click of the 🖱️ means you FOLLOW US for more expert-led #MedEd! @BrettSperryMD thanks you for joining us!
— cardio-met (@cardiomet_CE) November 15, 2023