Transthyretin Amyloid Cardiomyopathy (ATTR-CM) falls under the disease group cardiac amyloidosis.¹ The most common types of cardiac amyloidosis, accounting for >98% of cases, are ATTR and immunoglobulin light chain amyloidosis (AL).² It is important to differentiate between ATTR and AL, as they have distinct clinical courses and require different approaches to management.^1,2

There are two types of ATTR-CM – Wild-type ATTR-CM and Hereditary ATTR-CM^3,4

The most common types of cardiac amyloidosis  are shown here:^5,6

VYNDAQEL® 61 mg (tafamidis) is indicated for the treatment of wild‑type or hereditary transthyretin amyloidosis in adult patients with cardiomyopathy (ATTR-CM).

Amyloid light chain (AL) amyloidosis must be excluded before starting treament with VYNDAQEL.

Wild-type ATTR-CM (wtATTR-CM)

Wild-type ATTR-CM is idiopathic and is not considered to be a hereditary disease.⁶ It is thought to account for the majority of all ATTR-CM cases.⁶

wtATTR-CM is a type of amyloidosis caused by age-related changes in the stability of TTR proteins.

• Found mostly in men^6,8
• Most common in older adults, and the average age at diagnosis is >60 years⁹
• The mean diagnostic delay for wtATTR-CM has been shown to be 6.1 years from onset of symptoms¹⁶
• Timely diagnosis and treatment of wtATTR-CM have been predicted to extend mean life expectancy by 5.46 years compared with delayed diagnosis¹⁷
• Median survival, from diagnosis: ~3.5 years^6,9,14

• Heart failure with preserved ejection fraction^6,8,10
• Cardiac arrhythmias, particularly atrial fibrillation^5,6,8,10
• A history of bilateral carpal tunnel syndrome^6,9,11–13
• Ruptured distal biceps tendon¹⁸

Hereditary ATTR-CM (hATTR-CM)

hATTR-CM is a type of amyloidosis caused by a mutation in the TTR gene.⁷ In the UK, there are thought to be around 40–50 new cases each year.¹⁵

• Found in men and women⁸
• Age of symptom onset depends on the mutation and may range from 44 to 97 years¹¹
• Mean age of symptom onset depends on the mutation and is 69.8 ± 9.0 years¹¹

Hereditary ATTR-CM frequently presents with:

• Heart failure with preserved ejection fraction⁸
• A range of neurological symptoms (peripheral and autonomic)⁸
• Gastrointestinal symptoms⁸

Advanced hATTR-CM in untreated patients is associated with rapid progression, serious cardiac complications, and increased mortality, regardless of phenotype.^5,19,20

ATTR-CM= transthyretin amyloid cardiomyopathy; hATTR-CM=hereditary ATTR-CM; NT-proBNP=N-terminal pro-B-type natriuretic peptide; TTR=transthyretin; wtATTR-CM=wild-type ATTR-CM.