Cerebrovascular Involvement in Transthyretin Amyloid Cardiomyopathy

Area of Science:

• Neurology
• Cardiology
• Radiology

Background:

• Intracardiac thrombosis is common in transthyretin amyloid cardiomyopathy (ATTR-CM), posing a risk for thromboembolic events.
• The prevalence of silent cerebral infarcts and cerebral small vessel disease in ATTR-CM patients is largely unknown.
• ATTR-CM is a progressive condition affecting the heart and potentially other organs, including the brain.

Purpose of the Study:

• To investigate the presence and extent of silent cerebral infarcts and cerebral small vessel disease in patients with ATTR-CM.
• To compare cerebral magnetic resonance imaging (cMRI) findings between ATTR-CM patients and matched controls.
• To evaluate the association between CHA2DS2-VASc score and cerebral small vessel disease in ATTR-CM.

Main Methods:

• Prospective study of 32 ATTR-CM patients and 43 CHA2DS2-VASc-matched controls using cMRI.
• Standard cMRI sequences were used to assess structural features and cerebral vessel involvement.
• Neuroradiologists, blinded to clinical status, quantified infarcts, microbleeds, Virchow-Robin spaces, and lacunar lesions.

Main Results:

• ATTR-CM patients had a higher number of territorial infarcts (4 vs. 0) and cerebral microbleeds (1.4 vs. 0.3) compared to controls.
• ATTR-CM patients also showed more Virchow-Robin spaces (43.8 vs. 20.6) and a trend towards higher lacunar lesion presence (6 vs. 2).
• CHA2DS2-VASc score, atrial fibrillation, or anticoagulation status did not significantly impact the likelihood of territorial or lacunar lesions.

Conclusions:

• Cerebral MRI reveals significant, previously unreported small vessel disease and territorial ischemia in ATTR-CM patients, even those without overt neurological symptoms.
• These findings suggest that ATTR-CM is associated with a higher burden of cerebrovascular disease.
• The results support considering lower thresholds for anticoagulation and cMRI screening in ATTR-CM patients.