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Cardiac amyloidosis: An emerging target in HFpEF?
Peter van der Meer, MD Groningen, The Netherlands
May, 2019 - Athens, Greece
Amyloidosis
University Medical Center Groningen
Peter van der Meer
Universitair Medisch Centrum Groningen
Cardiac amyloidosis: An emerging target in HFpEF? Peter van der - - PowerPoint PPT Presentation
Cardiac amyloidosis: An emerging target in HFpEF? Peter van der - - PowerPoint PPT Presentation
Cardiac amyloidosis: An emerging target in HFpEF? Peter van der Meer, MD Groningen, The Netherlands May, 2019 - Athens, Greece Amyloidosis Cardiac amyloidosis: An emerging target in HFpEF? Peter van der Meer Universitair Medisch Centrum
SLIDE 2
SLIDE 3
Amyloidosis
University Medical Center Groningen
- Male patient, 74 years old.
- Medical history: hypertension + depression
- Home medications:
- Metoprolol succ 50mg once daily
- Paroxetine 20mg once daily
History + Medication
SLIDE 4
Amyloidosis
University Medical Center Groningen
- Walking with friends
- Suddenly shortness of breath
- Denies chest pain and palpitations
- Can normally bike 70-80km without any problems
- Ambulance brings patient to ER
What happened
SLIDE 5
Amyloidosis
University Medical Center Groningen
Physical Examination
Weight: 104kg Height: 182cm BMI: 31.4 BP: 150/82 mmHg, Heart: 55 bpm, no murmers Lungs: crepitus basal both lungs Extremities: Trace peripheral edema, pulses & neuro intact
Lab values:
Hemoglobin: 8.1 mmol/L NTproBNP: 2.058 ng/L eGFR: 48 ml/mn/1.73 hsTroponin T: 38 ng/L
SLIDE 6
Amyloidosis
University Medical Center Groningen
ECG
SR 65 bpm, 1st degree AV block qs-pattern V1-3 and + lateral QRS: 131ms Rightward axis
SLIDE 7
Amyloidosis
University Medical Center Groningen
Chest X-ray
SLIDE 8
Amyloidosis
University Medical Center Groningen
Trans Thoracic Echocardiogram
SLIDE 9
Amyloidosis
University Medical Center Groningen
Trans Thoracic Echocardiogram E' - lat 4.6 cm/s E' - sept 3.5 cm/s E/e' 19.8
SLIDE 10
Amyloidosis
University Medical Center Groningen
Summary of echocardiography findings
- Hypertrophic LV (septal:29mm), (pw: 25mm)
- LVEF: 57%. Severely impaired diastolic LV function
- Hypertrophic RV; RV free wall: 10mm.
- No significant valvular abnormalies.
- Enlarged left and right atrium.
SLIDE 11
Amyloidosis
University Medical Center Groningen
Bone Scintigraphy Tc-99m HDP Patient 1 2 3 Visual grading score
SLIDE 12
Amyloidosis
University Medical Center Groningen
Coronary Angiogram: No Coronary Artery Disease Fat biopsy Genetics Congo Red positive staining. WES TTR gene: no mutations
Additional investigations
SLIDE 13
Amyloidosis
University Medical Center Groningen
Diagnosis
Heart Failure with Preserved Ejection fraction based on wild-type aTTR amyloidosis
SLIDE 14
Amyloidosis
University Medical Center Groningen
Search for Specific etiologies of HFpEF
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Amyloidosis
University Medical Center Groningen
Treatment options for ATTR-cardiomyopathy
Jono et al. Exp. Opinion on Orphan drugs 2017
SLIDE 16
Amyloidosis
University Medical Center Groningen
ATTR-ACT Study Design
NEJM 2018
SLIDE 17
Amyloidosis
University Medical Center Groningen
All-Cause Mortality
^
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Amyloidosis
University Medical Center Groningen
Effects of tafamidis on 6 MWT and different subgroups
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Amyloidosis
University Medical Center Groningen
Other novel treatment option: RNA therapeutics
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Amyloidosis
University Medical Center Groningen
APOLLO trial – Patisiran; RNA based treatment
Solomon et al. Circulation 2019
Hereditary TTR Substudy on cardiac effects Decrease = LVWT < 2mm
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Amyloidosis
University Medical Center Groningen
- Search for the etiology of HFpEF (aTTR / Fabry / atypical HCM / etc)
- Bone scintigrafy has a high sensitivity and specificity for aTTR
- AL needs to be excluded
- Genetic testing to exclude heriditary form
- aTTR = first steps in personalized medicine in HFpEF (tafamidis – patisiran)