Amyloidosis Month

03 Mar, 2021

Dr. Rajesh Bollam

5 Best Papers

Tips, Tricks & Thread

Diagnostic delays3 years from symptom onset

may be evaluated > 5 specialists prior to receiving the correct diagnosis

vast majority of cardiac amyloidosistransthyretin amyloidosis or light chain amyloidosis

â€œred flagâ€suspicion for this diagnosis

ECGvoltage QRS amplitude limb leads (â‰¤0.5 mV), pseudo-infarct pattern & relativevoltage QRS (S wave in V1 + Rwave in V5 or V6 â‰¤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-CA91% sens & nearly 100% spec in the absencemonoclonal gammopathy

Majority of false testsLC cardiac amyloidosis, exclusionLC 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 inwhere severe chronic inflammatory processes are generally well managed

Clinical cardiac amyloidosisextracellular 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 testingcritical component of the evaluation

of transthyretin amyloidosis, determining prognosis & treatment

Val122Ile1st common mutation

Thr60Ala2nd most common mutation

Val30Met (pV50M)most commonmutation 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 symptomssuspicion of amyloidosis early in its course

cardiac, neurologic, nephropathy or musculoskeletal manifestations

ATTR amyloidosismusculoskeletal manifestations 5 to 15 years prior to other symptoms

Carpal tunnel syndromeTrigger fingerLumbar spinal stenosis

trigger fingercarpal tunnel syndrome reportedfamily 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 patientâ€™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 patientsearly recognition and

suspicion of possible cases based on clinical red flags,

cardiac imaging, and biomarkers

#DrRajeshBollam #hematologistinhyderabad #clinicalpearls #oncologistinyashoda

#amyloidosis #BMT #Telangana

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