History of Extracardiac/Cardiac Events in Cardiac Amyloidosis: Prevalence and Time from Initial Onset to Diagnosis

Figures

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  Figure 1. Representation of number of patients with initial amyloidosis extracardiac (light grey), cardiac (black), or both (dark grey) events depending on the type of amyloidosis.

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  Figure 2. Chord diagrams: (A) chord diagram depicting the link and proportion between the initial amyloidosis extracardiac (AECE) (light grey), cardiac events (ACE) (black), or both (dark grey) and type of amyloidosis (AL in green, ATTRv with cardiomyopathy in orange, and ATTRwt in blue). Regarding AECE and ACE, there is a greater proportion of integumentary and ears‐nose‐throat symptoms in ATTRwt, neuropathy in ATTRv regardless of neural status, and heart failure and general poor condition in AL. (B) Depiction of the link and proportion between the type of amyloidosis and the initial amyloidosis extracardiac events. Regarding AECE, there is a greater proportion of integumentary and ENT symptoms in ATTRwt, neuropathy in ATTRv, poor general condition, skin and mucosal symptoms, and renal failure in AL. (C). Depiction of the link and proportion between the different types of amyloidosis and the initial ACE. Regarding cardiac events, there is a greater proportion of rhythm disorders in ATTRwt, biomarkers or imaging abnormalities in ATTRv, heart failure in AL.

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  Figure 3. Chord diagrams describing the link between time frame [(0–2) years in blue, (2–5) years in purple and (5 and +) years in pink] of cardiac amyloidosis diagnosis and the occurrence of the first amyloidosis extracardiac (light grey), cardiac (black), or both (dark grey) events depending on type of amyloidosis. The diagnosis of AL is performed mainly in the 2 years after the first symptoms, especially cardiac symptoms. The diagnosis of ATTRv and ATTRwt is mainly performed later than 5 years after the onset of symptoms, especially integumentary symptoms.

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  Figure 4. Kaplan–Meier curves describing the link between death or heart transplantation and delayed diagnosis [(0–2) years in blue and (2 and +) years in purple] (A), or [(0–2) years in blue, (2–5) years in purple, and (5 and +) years in pink (B) and (C)] for each type of amyloidosis: (A) AL; (B) ATTRv with cardiomyopathy with or without neuropathy; and (C) ATTRwt.

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  Figure 5. Kaplan–Meier curves describing the link between death or heart transplant and first amyloidosis extracardiac or cardiac events (AECE or ACE) for the three main classes of symptoms: ears‐nose‐throat (ENT) symptoms (solid, dark grey line), heart failure (dotted, black line), and integumentary symptoms (dotted, light grey line).