Prevalence and type of first amyloid extracardiac symptoms and cardiac disorders history and time from their occurrence to diagnosis between Al and transthyretin cardiac amyloidosis

Abstract

Cardiac amyloidosis (CA) have a bad prognosis aggravated by diagnostic delay. Cardiologists should be aware that Amyloidosis Extracardiac Disorders (AECD) and cardiac symptoms and history (ACD) may help to improve CA diagnosis and typing. The aim of this study was to compare AECD and ACD history between different CA types and their relationship with survival. AECD and ACD, from 983 CA patients with a mean age ± SD of 73 ± 12 years included from June 2008 to May 2019, were studied. 321 were AL, 434 wild type transthyretin (ATTRwt), and the other were hereditary transthyretin (ATTRv; 16 neurologic and 212 cardio or mixed). Median delay between declaration of symptoms/history and diagnosis varied from 45[54–72] months for ATTRv with neuropathy to 72[63–78] months for ATTRwt. Nature of first AECD or ACD depended on amyloidosis type, with heart failure symptoms for AL (26%), neuropathy (45%) for neurologic ATTRv and integumentary symptoms for ATTRv with cardiopathy or mixed and ATTRwt respectively 39%, and 42%. In AL and ATTRwt, the shorter delay between the first history and the diagnosis was associated with a shorter survival rate ( Fig. 1 ). Extra-cardiac history, such as integumentary symptoms, present early in the development of CA and increase awareness of physicians for these signs may improve diagnosis. The progressive nature of AECD seems indicating a slower progression of the cardiac involvement.