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Cardiac Amyloidosis 11/02/2020 Cardiac Amyloidosis General Medicine for Palliative Physicians Conference 2020 Dr Eleanor Wicks Clinical Lead of Inherited Cardiac Diseases Service Consultant Cardiologist and Honorary Senior Clinical Lecture in Heart Failure, Imaging, Inherited and Acquired Cardiac Diseases Oxford University Hospitals @eleanorwicks @HeartFailureOUH @OxfordICC @Cardiomyopathy 1 Disclosures • Consultancy and speaker fees • Novartis, Servier, Alnylam • Advisory services • James Lind Alliance Project Steering Partnership • Cardiomyopathy UK, BHF, Takeda • Membership and advisory committees • ESC myocardial and pericardial diseases working group • Association for Inherited Cardiac Conditions • Cardiomyopathy UK and BHF Charity involvement • Inherited cardiac conditions (ICC) curriculum and training working group 2 1 11/02/2020 Amyloidosis • Aims: – Improve awareness and update your knowledge & understanding of amyloidosis – Reinforce knowledge of epidemiology, red flags and challenges in the diagnosis, assessment (and staging) of cardiac amyloidosis – Overview new treatment options – Think about a coordinated, multi-disciplinary approach 3 Case 1 Kidney damage with proteinuria, low albumin, hyperlipidaemia, +++ oedema Complications: blood clots, HTN, infections • 46-year old lady presented with nephrotic syndrome in January 2017 • Renal biopsy – minimal change disease • Treated with prednisolone – no response • Cyclosporine A added – no response • June 2017, repeat renal biopsy – amyloid • Cr 143, eGFR 35ml/min, serum albumin 17g/L, 24hr UPT 13.2 g • NT-proBNP 465 ng/L, TnT 38 ng/L • κ FLC 72.5, λ FLC 172.4, κ/λ ratio 0.42, lambda BJP 0.4 g/24hr • Bone marrow biopsy – 7% lambda restricted plasma cells • Echocardiogram – no definite evidence of amyloid • Non-contrast CMR – likely early cardiac amyloid • SAP scan – moderate visceral amyloid load in the spleen and kidneys • 6MWT – 438 metres (82% of normal for age) 6MWT, 6-minute walk test; BJP, Bence Jones protein; CMR, cardiac magnetic resonance imaging; eGFR, estimated glomerular filtration rate; FLC, free light chain; NT-proBNP, N-terminal pro-B-type natriuretic peptide; SAP, serum amyloid P; TnT, troponin T; UPT, urine preservative transport 4 2 11/02/2020 Case 1 Stage 1: both markers low (NT-proBNP <332, TnT <0.035, TnI <0.1) median survival 26.4 months Stage 2: one or other marker high survival 10.5 months • Both renal biopsies stained Stage 3: both markers high survival 3.5 months – AL (lambda sub-type) amyloid in both samples (by IHC) Diagnosis: Systemic AL amyloidosis Mayo Stage II disease1 • Treated with CVD to clonal complete response (CR) • Progression to ESRD, commenced HD in February 2018 • Listed for kidney transplant AL, amyloidosis light chain; CVD, cyclophosphamide, Velcade® and dexamethasone; ESRD, end-stage renal disease; HD, hemodialysis; IHC, immunohistochemistry Dispenzieri A, et al. J Clin Oncol 2004;22(18):3751–3757 5 Case 2 • 77-year-old male • Admitted with congestive cardiac failure • Bilateral carpal tunnel decompressions 15 years ago • Hx of AF and longstanding HTN • No FH of note • CKD (creatinine 180), proteinuria ++ • Resistant to diuretics +++ 6 3 11/02/2020 CXR 7 ECG 8 4 11/02/2020 CMR 9 DPD Scintigraphy: Perugini stage Perugini et al, JACC 2005 Huff et al, EHJ 2014 Grade 2 – cardiac uptake with intensity similar or greater than bone signal 10 5 11/02/2020 Diagnostic workup Clinical Suspicion (Hx, ECG, echo +/- CMR) DPD scan + serum, urine IFE + FLCs +ve DPD, -ve screen +ve DPD, +ve screen -ve DPD, +ve screen -ve DPD, -ve screen TTR cardiac amyloid likely AL amyloid possible Consider alternative diagnosis 1. Offload 1. Offload 2. Refer ICC clinic/NAC 2. Liaise NAC and Haem as IP 3. Discharge 3. Consider endomyocardial biopsy 4. Look for other organ involvement 11 Treatment • Diuretics as needed -> offload acutely • Long term usual fluid balance advice • Little role for ACEi, ARBs, BBs, rate limiting CCBs • Digoxin controversial – generally avoid Novel therapies considered: • Transthyretin tetramer stabilising agents (diflusinal and tafamidis) • Antisense and interfering RNA therapeutics (reduce TTR production by 80-90%) • Monoclonal anti-SAP antibodies (clearance of established amyloid) Maurer et al, Circ Heart Fail 2015; Sekijima et al, Society of Amyloidosis 2006; Coelho et al, NEJM 2013; Ackermann et al, the International Society of Amyloidosis 2012; Richards et al, NEJM 2015. 12 6 11/02/2020 Case 3 • 68-year old lady, previously fit and well • Gradual worsening of SOB • She had several investigations, including an ECG and echo → significant LVH and voltage criteria for LVH on ECG • Aortic stenosis and was planned for aortic valve surgery • Before surgery she was scheduled for a CMR scan – she had pulmonary oedema during cardiac MRI → scan interrupted before LGE • Emergency surgery and had mechanical aortic valve replacement • Struggled thereafter with SOB 13 Case 3 14 7 11/02/2020 Echo 5 years later Features of HCM: In an adult, HCM is defined by a wall thickness ≥15 mm in one or more LV myocardial segments – as measured by any imaging technique (echo, CMR or CT), that is not explained solely by loading conditions 15 CMR 5 years later 16 8 11/02/2020 CMR 5 years later: typical features of amyloid Classic extensive ‘zebra’ scar pattern on late enhancement Suspected cardiac amyloidosis -> Immunofixation serum and urine FL and electrophoresis -> negative 17 DPD 18 9 11/02/2020 Confirming ATTR amyloidosis AApoA1, apolipoprotein A-I; AL, amyloidosis light chain; ATTR, TTR amyloidosis; CMR, cardiac magnetic resonance imaging; DPD, 3,3-diphosphono-1,2-propanodicarboxylic acid; HDMP, hydroxymethylenediphosphonate; PYP, pyrophosphate; TTR, transthyretin Gillmore JD, et al. Circulation 2016;133(24):2404–2412 19 Amyloid in AS • Present in up to 15% of patients with AS and up to 30% of low-flow, low gradient AS • In AS, CA is associated with increased risk of HF, mortality, treatment futility with AVR • Look for specific red flags and confirm the diagnosis • Transcatheter rather than surgical AVR preferred • Start treatment for TTR amyloid as soon as diagnosis confirmed 20 10 11/02/2020 CHAD-STOPmnemonic for initial steps in CA: C: conduction and rhythm disorder prevention (amiodarone = ok, pacemaker) H: high heart rate maintenance A: anticoagulation D: diuretics STOP: STOP betablockers and calcium channel blockers (to optimize HR, CO and preload), STOP digoxin and STOP renin-angiotensin-aldosterone inhibitors (to minimize severe hypotension, esp in autonomic dysfunction) 21 WHAT IS AMYLOIDOSIS? 22 11 11/02/2020 Amyloidosis • Extracellular deposition of abnormally folded protein • Over 30 different proteins • Accumulate in tissues as amyloid fibrils which leads to progressive dysfunction Bind Congo red and SAP Unusual stability Damage tissue structure and organ function Serum Amyloid P (SAP) scintigraphy 23 Types of amyloidosis (differential diagnoses) • Cardiac AL (light chain) amyloidosis • Cardiac transthyretin (ATTR) amyloidosis – Wild-type ATTR (Senile Systemic/Senile Cardiac) – Hereditary transthyretin amyloidosis (hATTR) mutation of TTR gene e.g. V122I, T60A • AA amyloidosis • Rare (genetic) • Apo A1/A2 • Lysozyme Gonzalez-Lopez E, et al. Eur Heart J 2015;36:2585-94 24 12 11/02/2020 Amyloid fibril proteins Amyloid type Amyloid fibril protein (circulating) • AL • Light chain of immunoglobulin • AA • Amyloid A protein • ATTRwt • ‘Normal’ TTR • hATTR • ‘Mutated’ TTR There are more than 30 known amyloid fibril proteins! AA, amyloid A amyloidosis; AL, amyloidosis light chain; hATTR, hereditary; TTR amyloidosis; TTR, transthyretin; wt, wild- type. Data courtesy of National Amyloidosis Centre data 2018 (unpublished) 25 Terminology and classification 26 13 11/02/2020 Key facts 1: Cardiac Amyloidosis • Rare form of cardiomyopathy, challenging to diagnose, often associated with poor prognosis • Amyloidosis = a collection of disorders of protein mis- folding • Mis-folded protein forms aggregates within a cell which are potentially toxic • Excess of protein or impairment of clearance of abnormal protein can lead to amyloidosis Knowles TP, et al. Nat Rev Mol Cell Biol. 2014;15:384–96. 27 Key facts 2 • Two types account for 95% of all cardiac amyloidosis: • AL (light chain) fibril deposits – Clonal plasma cell dyscrasia (disorder) that results in overproduction and misfolding of light chain antibody fragments • TTR (transthyretin) deposits – Due to misfolding of the liver- derived precursor protein transthyretin (TTR, previously called prealbumin) AL, light chain; TTR, transthyretin. Donnelly J, Hanna M. Cleve Clin J Med 2017;84(12 Suppl 3):12–26. 28 14 11/02/2020 CARDIAC AL AMYLOIDOSIS 29 Cardiac AL amyloidosis • Commonest diagnosed form of cardiac amyloidosis • Cardiac involvement in 50-90% of systemic AL amyloidosis, often HF • Derived from monoclonal immunoglobulin light chain • Poor prognosis - median survival 12 months • Median age at diagnosis is 50-60yrs • May co-exist with multiple myeloma (10-15%) or any B-cell dyscrasia but >80% due to subtle or ‘benign’ gammopathies • Full clinical evaluation and investigations (ECG, Echo, CMR, biomarkers, biopsy, SAP ± DPD scan, immunofixation of urine and serum, serum immunoglobulin FLC, urine electrophoresis, bone marrow biopsy 30 15 11/02/2020 Cardiac AL amyloidosis: management Manage cardiac disease: – Diuresis, anticoagulate in AF (thromboembolism) – BB, vasoactive drugs poorly tolerated – hypotension – Avoid digoxin + CCBs as bind amyloid -> toxicity – PPM but avoid ICDs – SCD common Treat underlying disease:
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