Liver and Coagulation Disorders in Cardiac Transthyretin Amyloidosis

Summary

Transthyretin cardiac amyloidosis (ATTR-CA) is a progressive infiltrative cardiomyopathy caused by the deposition of misfolded transthyretin protein within the myocardium. Current disease staging and follow-up strategies mainly rely on cardiac biomarkers and renal function; however, the systemic nature of ATTR suggests that additional organ involvement may provide valuable prognostic information. The purpose of this prospective observational study is to investigate liver dysfunction and coagulation abnormalities in patients with wild-type or hereditary ATTR-CA and to evaluate their potential role as novel markers of disease severity and progression. Patients with ATTR-CA will be compared with an age-matched control population with non-amyloid hypertrophic cardiomyopathy. Clinical, laboratory, echocardiographic, hepatic ultrasound, liver stiffness, and coagulation parameters will be assessed at baseline and during follow-up. The study will also evaluate changes in these parameters after 6 and 12 months of treatment with tafamidis. The results may improve the understanding of cardio-hepatic interactions in ATTR-CA and identify new tools for disease staging and longitudinal monitoring.

Eligibility

Inclusion Criteria:

* Written informed consent obtained prior to study participation.
* Diagnosis of wild-type or hereditary transthyretin cardiac amyloidosis (ATTR-CA) according to current European recommendations.
* Ability to comply with study procedures and follow-up visits.

Exclusion Criteria:

* Age younger than 18 years.
* Severe liver dysfunction due to causes other than amyloidosis.
* Inability to comply with study procedures because of language barriers, cognitive impairment, or severe psychiatric disorders.
* Comorbidities associated with life expectancy less than 12 months.
* Active alcohol or substance abuse.
* For coagulation analyses: congenital coagulation disorders, thrombotic disorders, active malignancy, or sepsis.
* Pregnancy or breastfeeding.

Conditions7

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