Transforming ATTR cardiac amyloidosis into a chronic disease: The enormous potential of quantitative SPECT to improve diagnosis, prognosis, and monitoring of disease progression

Excerpt

A handful of slides…. that was the extent of my medical school education on cardiac amyloidosis. An accumulation of misfolded precursor proteins deposited in extracellular tissue, amyloid comprised a group of systemic illnesses with myriad presentations.1 In the past, diagnosis of cardiac amyloidosis required a biopsy, or often multiple biopsies, and was usually recognized late in the course of illness. Once diagnosed, the disease itself proved difficult to manage, with significant morbidity and mortality. Worse still, there were no disease modifying therapies, with only supportive care to offer.2 Fast-forward a decade plus, and the tables are turning. Disease modifying agents target the very biochemistry of the pathogenic proteins.3,4 Our newfound ability to alter the course of this highly morbid disease and change a patient’s course of illness provided a moral imperative to improve the rate of diagnosis, strive for earlier recognition of disease, and identify mechanisms to surveil treatment progress. To fill this void, radionuclide imaging has stepped in, revolutionizing accurate non-invasive approaches for a patient population in need. As obtaining a diagnosis became less invasive, and treatments offered genuine promise, it became increasingly apparent that amyloidosis may not be as rare as once thought.5 Some recent studies have estimated that upwards of 10% of patients with heart failure with preserved ejection fraction may have a form of amyloidosis.6 …