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Transthyretin amyloid deposition is present in 17% of autopsies of patients with heart failure and a preserved ejection fraction (HFpEF). 99mTechnetium-pyrophosphate scintigraphy (99mTc-PYP) is sensitive and specific to diagnose cardiac transthyretin amyloid deposition (ATTR). The prevalence of ATTR by 99mTc-PYP was evaluated along with echocardiographic parameters in patients with HFpEF. One-hundred consecutive patients with HFpEF, who had 99mTc-PYP, were retrospectively evaluated. Echocardiographic variables were analyzed to compare patients with positive versus negative ATTR infiltration. Myocardial ATTR was present in 19% of patients. Individuals with ATTR were older with a mean age of 82 ± 7 versus 75 ± 13 years (P = 0.03), had increased left ventricular hypertrophy with the interventricular septum measuring 1.6 (IQR, 1.4–2.0) versus 1.4 (IQR, 1.3–1.6) cm (P = 0.002), had a greater mean left ventricular mass index of 160 ± 50 g/m2 versus 131 ± 44 g/m2 (P = 0.01), and a reduced global longitudinal strain measuring −11% (IQR, −9 to −12) versus −12% (IQR, −10 to −16), P = 0.04. The prevalence of ATTR myocardial deposition demonstrated by 99mTc-PYP in patients with HFpEF is comparable to that of autopsy studies. It is more common in older patients, with increased left ventricular hypertrophy and reduced global longitudinal strain.

Amyloidosis is a systemic disorder that results from abnormal protein metabolism, producing amyloid fibrils that are subsequently deposited within vital organs. Cardiac involvement is typically associated with the specific subtypes of immunoglobulin lightchain, transthyretin, secondary amyloidosis, and dialysis-related amyloidosis. The hallmark of cardiac amyloidosis is the development of restrictive cardiomyopathy and heart failure, usually with a preserved left ventricular ejection fraction. The diagnosis is based on the integration of clinical signs and symptoms, echocardiography, cardiac magnetic resonance imaging, nuclear scintigraphy, electrocardiography, and cardiac biomarkers. Traditionally, management of heart failure symptoms and prevention of heart failure exacerbations have been the cornerstones of therapy. However, various treatments are currently under investigation that aim to eliminate or neutralize the underlying amyloidogenic substrate. Herein, we provide a focused review and discussion of the cardiovascular manifestations, epidemiologic and clinical characteristics, diagnostic modalities, and treatment strategies of cardiac amyloidosis.