Gateway and Journey of Patients with Cardiac Amyloidosis

In this retrospective cross-sectional study, Oliver Lairez, MD, PhD, Department Head, Cardiac Imaging Center and Cardiac Exploration Unit, Rangueil University Hospital, Toulouse, France, set out to describe the journey to cardiac amyloidosis diagnosis from initial clinical presentation and to analyze time to diagnosis.

Between January 2001 and May 2019, 270 consecutive patients diagnosed with cardiac amyloidosis were included in this study: 111 (41%) had light chain amyloidosis, 122 (45%) had wild-type transthyretin amyloidosis (ATTR), and 37 (14%) had hereditary ATTR. The journey to diagnosis including symptoms, first-line specialist, number of consulted specialists, and tests performed before diagnosis were investigated.

Cardiac amyloidosis onset occurred mostly with dyspnea (50%) or systematic follow-up (10%). The most common first-line specialist to see a patient was a cardiologist (68%), followed by nephrologist (9%), and neurologist (8%). Patients encountered a median number of 2 (range, 1-7) physician specialists and received 3 (range, 1-8) tests before diagnosis. Median delay between symptom onset and cardiac amyloidosis diagnosis was 8 months (interquartile range [IQR], 5-14), 10 months (IQR 3-34), and 18 months (IQR 4-49), respectively in light chain, wild-type ATTR, and hereditary ATTR subgroups (P = .060). Having performed an electromyography or a spirometry was associated with a longer delay in diagnosis in the overall population, likely due to nonspecific initial symptoms.

“Cardiac amyloidosis is a protean disease with various first-line specialists causing a diagnostic wandering despite increasing medical community awareness. It requires educating a multidisciplinary specialist care network to manage symptoms and therapies,” concluded Dr Lairez.

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