Tolerability and Efficacy of a Combination of Doxycycline and TUDCA in Patients With Transthyretin Amyloid Cardiomyopathy

Inclusion Criteria:

- Documented transthyretin cardiac amyloidosis by biopsy and staining using
immunohistochemistry or mass spectrometry

- Echocardiographic appearance of left ventricular wall thickness of 13mm or more in the
absence of hypertensive heart disease

- Confirmed ATTR or SSA by genetic testing

- Age 18-90

- Male or non-pregnant, non-lactating females

- Willingness to return to the treatment center for follow-up

Exclusion Criteria:

- Prior liver transplantation or liver transplantation anticipated in less than 6 months

- Alanine Transaminase and/or Aspartate Transaminase ≥2 x upper normal limit (UNL)

- Alkaline Phosphatase ≥2 x UNL

- Creatinine clearance <20 mL/min

- Any other lab values that in the opinion of the investigator might place the subject
at unacceptable risk for participation in the study

- History of poor compliance

- History of hypersensitivity to any of the ingredients of the study therapies

- Any investigational drug within 4 weeks prior to study entry or during the study

- Current use of diflunisal for therapy of amyloidosis