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Table 1 Summary of cases with extraventricular transthyretin amyloid deposits

From: Transthyretin derived amyloid deposits in the atrium and the aortic valve: insights from multimodality evaluations and mid-term follow up

 

Case 1

Case 2

Case 3

Patients’ characteristics

 Age and sex

79 yo male

73 yo female

78 yo female

 Clinical diagnosis

Severe AS

Atrial fibrillation

LV dysfunction

Functional MR

Transient ischemic attack

LA mobile mass

AS

LVOT obstruction

 History of carpal tunnel syndrome or lumber canal stenosis

No

No

No

 Surgical interventions

Aortic valve replacement

Mitral valve plasty

Cryo-Maze

LA appendage resection

LA mass and partial LA wall resection

Aortic valve replacement

Subaortic septal myectomy

Amyloid deposition and types

 Left atrium / left atrial appendage

AANP + ATTR

AANP + ATTR

n/a

 Aortic valve

ATTR

n/a

ATTR

 Ventricles

Unlikely by PYP scintigraphy and CMR

Unlikely by PYP scintigraphy and CMR

Non-significant (ATTR limited to vessel walls of both ventricles)

Post surgical course

 

• Normalized ejection fraction and significantly decreased BNP at 6 months

• Uneventful for 24 months with no echocardiogram findings suggesting ventricular cardiac amyloidosis

• Normal ventricular function

• Uneventful for 24 months, with normal troponin and no echocardiogram findings suggesting ventricular cardiac amyloidosis

• No heart failure events and no echocardiogram findings suggesting ventricular cardiac amyloidosis for 5 years

  1. AANP Atrial natriuretic peptide, AS Aortic stenosis, BNP B-type natriuretic peptide, CMR Cardiac magnetic resonance, LA Left atrium, LV Left ventricle, LVOT Left ventricular outflow tract, MR Mitral regurgitation, PYP Pyrophosphate, ATTR Transthyretin