More%Than%Just%Coronary%Artery Disease
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1 More%Than%Just%Coronary%Artery Disease Thomas%D.%Gossios Cardiomyopathies%Laboratory AUTH%1st%Cardiology%Department,%AHEPA%Hospital
2 First&presentation Male,&67&years&old Present&condition Typical&angina&at&rest. Progressive&dyspnea&on&exertion. No&history&of&syncope&or&palpitations. Medical&history Arterial&hypertension&(well&controlled& &5&yrs). Bilateral&carpal&tunnel&syndrome,&surgically&treated. Benign&prostate&hypertrophy. Previous&echo:&LVH,&likely&attributed&to&arterial hypertension
3 Electrocardiogram
4 Coronary'Angiography
5
6
7
8 Discharge*recommendations * Re1evaluation*of*symptoms*in*1*month. 24h*Holter*recording No*conduction*abnormalities*or*tachyarrhythmias CMR*study*pending Patient*lost*to*follow1up*for*1*year.
9 Re#admission Progressively1increasing1dyspnea,1NYHA1III No1angina NT1pro#BNP1levels13301pg/mL
10 CMR
11 Hematology*work.up ESR*10*mm Normal*Hct Normal*eGFR Serum*Albumin/Globulin*ratio*2.5/1 Serum*protein*electrophoresis:*no monoclonal Serum*immunoglobulin*quantification: normal Urine*protein*(.),*Bence*Jones*(.) Bone*marrow*aspiration*normal
12 L/R$catheterization$ Myocardial$biopsy 150$mmHg 100$mmHg 50$mmHg
13 Histology)study
14 Histology)study
15 Immunohistology-study - Severe-diffuse-amyloid-fibril-infiltration Severe-myocyte-damage Immunolabeling Anti-TTR-positive Anti;Kappa,-anti;Lamda,-anti;SAA, anti;apolipoprotein-a1-negative TTR-exon-and-flanking-sequence-analysis, compatible-with-wild;type-ttr-amyloid.
16 Cardiac'amyloidosis Type Protein Cardiac involvement Extracardiac involvement Median5survival AL#amyloidosis Light#chain amyloid 40350% Renal,#liver,#soft tissue,#neuropathy 8348#months Hereditary#TTR amyloidosis Variant#TTR (depending#on mutation) Rare#to#90% (depending#on mutation) Neuropathy Variable (dependence#from liver#tx) Wild3type#TTR amyloidosis#(ssa) Wild3type transthyretin Almost#invariable Carpal#tunnel syndrome 738#years Apolipoprotein#A1 amyloidosis SAA#(secondary) amyloidosis ApoA1#variant Rare Renal Slowly#progressive Serum#amyloid#A Rare Renal,#liver Good#prognosis Atrial#amyloidosis Atrial#natriuretic peptide Invariable None Unknown significance Banypersad#SM,#et#al.#J#Am#Heart#Assoc.#2012;1(2):e
17 Red$flags$for$amyloid cardiomyopathy Any$type$of$amyloid Conduction$disease Low$voltage$QRS$(50%$AL$vs$<25%$other$forms) Echocardiographic$findings Neuropathy $ Wild$type$TTR Age$>60$years Male$predominance Sporadic$disease Carpal$tunnel$syndrome$(34%$in$excised$tissue$wildMtype$TTR+) Hematologic$profile$incompatible$with$amyloid$(MGUS$5%$at$>$60 years) 99mTcMDPD$scintigraphy$uptake$ $100%$in$TTR$amyloid/25%$in$AL.
18 Falk%R.%Circulation%2005;112: % Flow%diagram%for%the%evaluation%of%a%patient%with suspected%cardiac%amyloidosis.
19 Treatment(options All(types(of(amyloid(cardiomyopathy Heart(failure(treatment Potential(PPM/ICD(implantation AL(amyloidosis Chemotherapeutic(agents Familial(TTR(amyloidosis Orthotopic/domino(liver(transplantation((favorable(for(neuropathy, FAP(not(cardiomyopathy) Diflunisal( (NSAID(related(adverse(effects Tafamidis( (currently(indicated(for(fap ALNITTR01/02( (RNA(silencing((phase(1(trial) ISISITTRRx( (oligonucleotide,(receptor(blockade((phase(1(trial) Senile(systemic(amyloidosis? ( Dungu(JN,(et(al.(Cardiac(transthyretin(amyloidosis.(Heart.(2012;98(21):1546I54. (
20 Epigallocatechin-3-gallate Kristen(AV,(et(al.(Green(tea(halts(progression(of(cardiac(transthyretin(amyloidosis:(an(observational(report.(Clin(Res(Cardiol.(2012;101(10):805F13
21
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