Vyndaqel ®▼ (tafamidis) |Suspect | PfizerPro Ireland

This site is intended only for healthcare professionals resident in the Republic of Ireland

Log out

Our medicines

Therapy areas

Explore content

Materials

Videos

Podcasts

Let’s connect

Let's connect

About

About ATTR-CM

Urgency

Study Design

About ATTR-PN

Urgency

Awareness

Partnership

Efficacy & Safety

Pivotal efficacy

Long-term survival

Subgroup analysis

Key secondary endpoints

Early efficacy measures

Safety profile

ATTR-PN

Pivotal efficacy

Pivotal efficacy ALT

Long-term efficacy

Mutations efficacy

Long-term survival

Safety profile

Dosing

ATTR-CM dosing

ATTR-PN

Pivotal efficacy

Pivotal efficacy ALT

Long-term efficacy

Mutations efficacy

Long-term survival

Safety profile

MOD/MOA

Role of TTR

ATTR-CM MOD

ATTR-CM MOA

ATTR-PN

ATTR-PN MOD

ATTR-PN MOA

Support & Resources

Materials

Videos

ATTR-PN

ATTR-PN MOD

ATTR-PN MOA

Vyndaqel 61mg is indicated for the treatment of wild‑type or hereditary transthyretin amyloidosis in adult patients with cardiomyopathy
(ATTR-CM).¹

▼ This medicinal product is subject to additional monitoring. This will allow quick identification of new safety information. Healthcare professionals are asked to report any suspected adverse reactions. Refer to section 4.8 of the SmPC for how to report adverse reactions.

Suspect the signs of ATTR-CM with cardiac and noncardiac clues¹

Consider the following clinical clues, especially in combination, to raise suspicion for ATTR-CM and the need for further testing.

Heart failure with preserved ejection fraction (HFpEF)^2-4 or other cardiac conditions (e.g severe aortic stenosis³ or cardiac arrhythmias)¹ in patients typically over the age of 60^2-4

In a retrospective cohort study of 382 patients with ATTR-CM, 69% (265 patients) had atrial fibrillation⁵

ECG: Low QRS voltage relative to LV mass¹
Echo: LV wall thickness ≥12 mm¹

Behind the cardiac clues

In ATTR-CM, diastolic function is impaired due to amyloid fibril deposition in the myocardium, resulting in thicker and inelastic ventricles, thereby decreasing stroke volume. It is not until the later stages of ATTR-CM disease that ejection fraction drops^6,7
Prevalence among older HFpEF patients
- ~10% of HFpEF patients referred to a dedicated center had ATTR-CM confirmed by endomyocardial biopsy^8*
- ~13% of hospitalized patients with HFpEF and increased LV wall thickness had wtATTR-CM confirmed by scintigraphy^2†
- In patients undergoing transcatheter aortic valve replacement (TAVR) for severe calcific aortic stenosis, prevalence of ATTR-CM was 16% overall and 22% among men³

Reduced longitudinal strain with apical sparing should raise suspicion of ATTR-CM^7,9

Illustrative representation.

A prospective analysis in 108 patients (61% women, age range: 57-74 years) seen at the Johns Hopkins University HFpEF Clinic who underwent endomyocardial biopsy (EMB) to evaluate myocardial tissue histopathology. Approximately 10% had ATTR-CM confirmed.⁸

A prospective, cross-sectional, single-center study at a tertiary university hospital in Madrid, Spain. Included 120 patients ≥60 years of age (59% women, mean age: 82 ± 8 years) admitted for HFpEF, with LV ejection fraction ≥50% and LV hypertrophy ≥12 mm. ^99mtechnetium-labeled 3,3-diphosphono-1,2-propanodicarboxylic acid (^99mTc-OPD) scintigraphy was used to confirm ATTR-CM. wtATTR was confirmed in 13% (13.3%; 95% Cl: 7.2-19.5) of the patients.²

Carpal tunnel syndrome¹
Lumbar spinal stenosis¹
Peripheral and autonomic nervous system dysfunction¹
Biceps tendon rupture¹

In a study of patients with wATTR-CM, 46% had carpal tunnel syndrome¹⁰

Behind the noncardiac clues

Bilateral carpal tunnel syndrome and lumbar spinal stenosis

Often seen in ATTR-CM due to amyloid deposition in these areas; lumbar spinal stenosis is principally seen in wtATTR-CM^12,13
Bilateral carpal tunnel syndrome and lumbar spinal stenosis are known clinical predictors of ATTR-CM and may precede heart failure symptoms by several years^12,14
Among patients undergoing carpal tunnel release surgery, 10.2% had amyloid deposits¹⁵

Biceps tendon rupture

Among patients with wtATTR-CM, biceps tendon rupture has been observed in 33% of patients, occurring in the dominant arm in 95% and bilaterally in 24% of patients¹⁶

Hip and knee arthroplasty

In a study of 313 patients (172 with ATTR-CM), hip and knee arthroplasty surgeries were more frequent than in the general population, and on average, arthroplasty occurred 7.2 years before ATTR-CM diagnosis¹⁷

Nervous system dysfunction

Nervous system dysfunction, including polyneuropathy and autonomic dysfunction, including gastrointestinal complaints and/or unexplained weight loss¹⁸
Gastrointestinal complaints due to autonomic dysfunction include chronic diarrhoea, constipation, or both¹⁸
Orthostatic hypotension due to autonomic dysfunction is another symptom that may occur with ATTR-CM¹⁸

European Society of Cardiology (ESC) recommendations

The ESC Working Group recommends screening for ATTR-CM if LV wall thickness is ≥12 mm + the presence of ≥1 red flag or clinical scenario¹¹

Download resources to
help suspect ATTR-CM

Clinical findings that should further raise suspicion of ATTR-CM^1,19

ECG^1,19

Discrepancy between LV thickness and QRS voltage
Atrioventricular block, in the presence of increased wall thickness

CMR^1,19

Marked ECV expansion, abnormal nulling time for the myocardium or diffuse late gadolinium enhancement on CMR

Echo^1,19,20

Increased LV wall thickness
Reduction in longitudinal strain with apical sparing
Hypertrophic phenotype with associated infiltrative features

Biomarkers^1,11,19

Mild increase in troponin levels on repeated occasions
Disproportionately elevated N-terminal pro-B-type natriuretic peptide (NT-proBNP) to degree of heart failure

ATTR-CM=transthyretin amyloid cardiomyopathy; CMR=cardiac magnetic resonance; ECG=electrocardiography; Echo=echocardiography; hATTR-CM=hereditary transthyretin amyloid cardiomyopathy; HFpEF=heart failure with preserved ejection fraction; LV=left ventricular; QRS=Q wave, R wave, S wave; wtATTR-CM=wild-type transthyretin amyloid cardiomyopathy.

Explore More

Pivotal and 5-year data for ATTR-CM

View the Body of Evidence

ATTR-CM MOD

Learn the Mechanism of Disease

NEXT: ATTR-CM safety profile

References:

Witteles RM, Bokhari S, Damy T, et al. Screening for transthyretin amyloid cardiomyopathy in everyday practice. JACC Heart Fail. 2019;7(8):709-716. doi:10.1016/j.jchf.2019.04.010

Gonzalez-Lopez E, Gallego-Delgado M, Guzzo-Merello G, et al. Wild-type transthyretin amyloidosis as a cause of heart failure with preserved ejection fraction. Eur Heart J. 2015;36(38):2585-2594. doi:10.1093/eurheartj/ehv338

Castano A, Drachman BM, Judge D, Maurer MS. Natural history and therapy of TTR-cardiac amyloidosis: emerging disease-modifying therapies from organ transplantation to stabilizer and silencer drugs. Heart Fail Rev. 2015;20(2):163-178. doi:10.1007/s10741-014-9462-7

Ruberg FL, Berk JL. Transthyretin (TTR) cardiac amyloidosis. Circulation. 2012;126(10):1286-1300. doi:10.1161/CIRCULATIONAHA.111.078915

Donnellan E, Wazni OM, Hanna M, et al. Atrial fibrillation in transthyretin cardiac amyloidosis: predictors, prevalence, and efficacy of rhythm control strategies.JACC Clin Electrophysiol. 2020;6(9):1118-1127. doi:10.1016/j.jacep.2020.04.019

Borlaug BA, Paulus WJ. Heart failure with preserved ejection fraction: pathophysiology, diagnosis, and treatment. Eur Heart J. 2011;32(6):670-679. doi:10.1093/eurheartj/ehq426

Siddiqi OK, Ruberg FL. Cardiac amyloidosis: an update on pathophysiology, diagnosis, and treatment. Trends Cardiovasc Med. 2018;28(1):10-21. doi:10.1016/j.tcm.2017.07.004

Hahn VS, Yanek LR, Vaishnav J, et al. Endomyocardial biopsy characterization of heart failure with preserved ejection fraction and prevalence of cardiac amyloidosis. JACC Heart Fail. 2020;8(9):712-724. doi:10.1016/j.jchf.2020.04.007

Narotsky DL, Castaño A, Weinsaft JW, Bokhari S, Maurer MS. Wild-type transthyretin cardiac amyloidosis: novel insights from advanced imaging. Can J Cardiol. 2016;32(9):1166.e1-1166.e10. doi:10.1016/j.cjca.2016.05.008

Connors LH, Sam F, Skinner M, et al. Heart failure resulting from age-related cardiac amyloid disease associated with wild-type transthyretin: a prospective, observational cohort study. Circulation. 2016;133(3):282-290. doi:10.1161/CIRCULATIONAHA.115.018852

Garcia-Pavia P, Rapezzi C, Adler Y, et al. Diagnosis and treatment of cardiac amyloidosis. A position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur J Heart Fail. 2021;23(4):512-526. Doi:10.1002/ejhf.2140

Nakagawa M, Sekijima Y, Yazaki M, et al. Carpal tunnel syndrome: a common initial symptom of systemic wild-type ATTR (ATTRwt) amyloidosis. Amyloid. 2016;23(1):58-63. doi:10.3109/13506129.2015.1135792

Westermark P, Westermark GT, Suhr OB, Berg S. Transthyretin-derived amyloidosis: probably a common cause of lumbar spinal stenosis. Ups J Med Sci. 2014;119(3):223-228. doi:10.3109/03009734.2014.895786

Papoutsidakis N, Miller EJ, Rodonski A, Jacoby D. Time course of common clinical manifestations in patients with transthyretin cardiac amyloidosis: delay from symptom onset to diagnosis. J Card Fail. 2018;24(2):131-133. doi:10.1016/ j.cardfail.2017.12.005

Sperry BW, Reyes BA, lkram A, et al. Tenosynovial and cardiac amyloidosis in patients undergoing carpal tunnel release. J Am Coll Cardiol. 2018;72(17):2040-2050. doi:10.1016/j.jacc.2018.07.092

Geller HI, Singh A, Alexander KM, Mirto TM, Falk RH. Association between ruptured distal biceps tendon and wild-type transthyretin cardiac amyloidosis. JAMA. 2017;318(10):962-963. doi:10.1001/jama.2017.9236

Rubin J, Alvarez J, Teruya S, et al. Hip and knee arthroplasty are common among patients with transthyretin cardiac amyloidosis, occurring years before cardiac amyloid diagnosis: can we identify affected patients earlier? Amyloid. 2017;24(4):226-230. doi:10.1080/13506129.2017.1375908

Coelho T, Maurer MS, Suhr OB. THAOS - The Transthyretin Amyloidosis Outcomes Survey: initial report on clinical manifestations in patients with hereditary and wild-type transthyretin amyloidosis. Curr Med Res Opin. 2013;29(1):63-76. doi:10.1185/03007995.2012.754348

Maurer MS, Bokhari S, Damy T, et al. Expert consensus recommendations for the suspicion and diagnosis of transthyretin cardiac amyloidosis. Circ Heart Fail. 2019;12(9):1-11. doi:10.1161/CIRCHEARTFAILURE.119.006075

Dorbala S, Ando Y, Bokhari S, et al. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: part 1 of 2-evidence base and standardized methods of imaging. J Nucl Cardiol. 2019;26(6):2065-2123doi:10.1007/s12350-019-01760-6. Addendum: J Nucl Cardiol. Published online July 1, 2021. doi:10.1007/s12350-020-02455-z

VYNDAQEL Prescribing Information

About ATTR-CM

Legal Category: S1A.Further information available upon request

PP-VYN-IRL-0177. December 2022

Adverse events should be reported. Report an adverse event to your national reporting system (HPRA Pharmacovigilance)

Adverse events should also be reported to Pfizer Medical Information on 1800 633 363

PfizerPro Account

Please sign in or register to gain access to information relating to Pfizer medicines and vaccines, medical conditions, patient materials and services.

Account

Log out

This site is intended only for healthcare professionals resident in the Republic of Ireland. If you are a member of the public wishing to access information on a specific medicine, please visit https://www.medicines.ie

This website is brought to you by Pfizer Healthcare Ireland, 9 Riverwalk, National Digital Park, Citywest Business Campus, Dublin 24, Ireland. Registered in the Republic of Ireland No. 127002.

PP-UNP-IRL-0733. March 2024

You are now leaving PfizerPro

You are now leaving PfizerPro Ireland. Links to external websites are provided as a resource to the viewer. This website is neither owned nor controlled by Pfizer. Pfizer accepts no responsibility for the content or services of the linked site.

PP-UNP-IRL-0733. March 2024