Why the study?
Cardiac transthyretin-derived amyloidosis is an underdiagnosed cause of heart failure with preserved ejection fraction.
Cardiac ATTR amyloidosis is an increasingly recognized and treatable cause of HFpEF, warranting screening in patients with unexplained left ventricular hypertrophy.
Raises suspicion for ATTR amyloidosis in HFpEF with unexplained LVH; leaves open need for prospective validation of scintigraphy.
Heart failure with preserved ejection fraction (HFpEF) comprises half of the heart failure population. A specific, but underdiagnosed, cause for HFpEF is transthyretin-derived (ATTR) amyloidosis. This article reviews the clinical characteristics of cardiac ATTR amyloidosis. The clinical suspicion of cardiac ATTR amyloidosis is strong if pronounced left ventricular hypertrophy is present in the absence of hypertension. Scintigraphy with a diphosphonate tracer is a diagnostic tool for the early detection of cardiac ATTR amyloidosis with high sensitivity and specificity. First treatment options for ATTR amyloidosis recently emerged, and showed a reduction in morbidity and mortality, especially if treatment was started in the early stages of disease. In light of these results, screening for ATTR amyloidosis in the general HFpEF population with left ventricular hypertrophy might be useful.
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Klaassen et al. (2020) studied this question.
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