AMYLOIDOSIS MONTH


 

🏅5 Best Papers
💖Tips, Tricks & Thread
🔴Diagnostic delays➡️3 years from symptom onset
👫 may be evaluated > 5 specialists prior to receiving the correct diagnosis
✅vast majority of cardiac amyloidosis➡️transthyretin amyloidosis or light chain amyloidosis
✅“red flag”🚩suspicion for this diagnosis👇
💟ECG⬇️voltage QRS amplitude limb leads (≤0.5 mV), pseudo-infarct pattern & relative⬇️voltage QRS (S wave in V1 + Rwave in V5 or V6 ≤15 mm) in the setting of LVH on #EchoFirst AV block
💟Echo concentric LVH, biatrial⬆️& small pericardial💦, apical sparing of LV strain
💟#whyCMR Diffuse, subendocardial LGE. T1 mapping techniques & measurement of extracellular volumes
💟#CVnuc TTR-CA➡️91% sens & nearly 100% spec in the absence➡️monoclonal gammopathy
💟Majority of false➕ tests➡️LC cardiac amyloidosis, exclusion➡️LC amyloidosis is critical
✅Several types of amyloidosis have been described based on the specific protein comprising the deposited fibrils
✅Uncommon for secondary (AA) amyloidosis
to affect🫀& this is rarely seen in🗺️where severe chronic inflammatory processes are generally well managed


🔴Clinical cardiac amyloidosis➡️extracellular deposition of proteins which have folded & aggregated such that they form amyloid fibrils
✅Val122IIe mutation, an hATTR subtype, is highly aggressive & therefore necessitates unique therapeutic and follow up regimens
✅CyBorD ➕ Daratumumab works effectively
to produce responses in AL amyloidosis
🧬Genetic testing➡️critical component of the evaluation
of transthyretin amyloidosis, determining prognosis & treatment
🔵Val122Ile➡️1st common mutation🇺🇸
🔵Thr60Ala➡️2nd most common mutation🇺🇸
🔴Val30Met (pV50M)➡️most common🌏mutation outside of the🇺🇸. Endemic in 🇯🇵🇸🇪🇵🇹
✅TTR genetic testing is recommended for all patients
with an established diagnosis of ATTR-CA regardless
of age🧒👫👴🧓
✅Once a patient carrying a pathological TTR gene
variant is identified, the predicted age of disease
onset (PADO) should be determined
🔴Recognizing a constellation of ATTR amyloidosis symptoms➡️suspicion of amyloidosis early in its course
🔴cardiac, neurologic, nephropathy or musculoskeletal manifestations
🔴ATTR amyloidosis➡️musculoskeletal manifestations 5 to 15 years prior to other symptoms
🟡Carpal tunnel syndrome🟡Trigger finger🟡Lumbar spinal stenosis
🚩trigger finger➕carpal tunnel syndrome reported🇯🇵family w/ATTRv
🟡Rupture distal biceps tendon (Popeye sign)🚩>50y
🔴ATTR amyloidosis is now a treatable disease, recognizing the constellation signs & symptoms, including those that are neurologic & musculoskeletal
🔴Early treatment will make a meaningful impact on a patient’s quality of life, autonomy, and physical function
🔴Previously considered a rare disease, CA is increasingly recognized among patients:
✅misdiagnosed as undifferentiated HFPEF
✅paradoxical low-flow/low-gradient aortic stenosis
✅otherwise unexplained left ventricular hypertrophy
🔴Basis for effective diagnostic & therapeutic
management of CA patients➡️early recognition and
suspicion of possible cases based on clinical red flags,
cardiac imaging, and biomarkers

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