Restrictive cardiomyopathy and cardiac amyloidosis
Stiff, non-dilated ventricles. Causes, restriction versus constriction, ATTR and AL amyloid red flags, the non-biopsy bone scan pathway, tafamidis and the PBS, and drugs that go badly in amyloid.
Tier 2Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- Restrictive cardiomyopathy: stiff, non-dilated ventricles with restrictive filling, biatrial enlargement and normal or near-normal LVEF early on.
- Presents as HFpEF with right-sided congestion, AF, conduction disease or low output.
- Cardiac amyloidosis is by far the commonest cause in adults, and wild-type ATTR in older men is the commonest amyloid type.
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Sources
- Arbelo E, Protonotarios A, Gimeno JR et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J 2023;44:3503-3626. doi:10.1093/eurheartj/ehad194European Society of Cardiology, 2023
- Køber L, Adamo M et al. 2026 ESC Guidelines for the management of heart failure. Eur Heart J 2026. doi:10.1093/eurheartj/ehag100European Society of Cardiology, 2026
- Kittleson MM, Ambardekar AV, Cheng RK et al. Transthyretin cardiac amyloidosis evaluation and management: 2025 ACC Concise Clinical Guidance. J Am Coll Cardiol 2026;87:549-565American College of Cardiology, 2025
- Gillmore JD, Maurer MS, Falk RH et al. Nonbiopsy diagnosis of cardiac transthyretin amyloidosis. Circulation 2016;133:2404-2412Circulation, 2016
- Maurer MS, Schwartz JH, Gundapaneni B et al. Tafamidis treatment for patients with transthyretin amyloid cardiomyopathy (ATTR-ACT). N Engl J Med 2018;379:1007-1016New England Journal of Medicine, 2018
- Gillmore JD, Judge DP, Cappelli F et al. Efficacy and safety of acoramidis in transthyretin amyloid cardiomyopathy (ATTRibute-CM). N Engl J Med 2024;390:132-142New England Journal of Medicine, 2024
- Fontana M, Berk JL, Gillmore JD et al. Vutrisiran in patients with transthyretin amyloidosis with cardiomyopathy (HELIOS-B). N Engl J Med 2025;392:33-44New England Journal of Medicine, 2025
- Government adds two new heart disease treatments on the PBS (tafamidis listing, 1 May 2024)AU/NZAustralian Government Minister for Health and Aged Care, 2024
- Vutrisiran for ATTR-CM, PBAC July 2026 outcome (medicine status)AU/NZPharmaceutical Benefits Scheme, 2026
- Last updated
- 14 November 2026
What changed
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