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. 2020 Jul 29;43(11):1223–1231. doi: 10.1002/clc.23434

TABLE 2.

Treatment for transthyretin‐related amyloidosis and its comorbidities and complications

Medical treatment for ATTR Invasive treatment for end‐stage ATTR Treatment for comorbidities and complications
  • Inhibitors of TTR gene expression

  • Tetramer stabilizers

  • Inhibitors of oligomer aggregation and disruption

  • Inhibitors of degradation and reabsorption of amyloid fibers

  • Left ventricular assist device and heart transplant (only if no extracardiac involvement)

  • Liver transplantation

  • Combined liver‐heart transplantation

1. Heart failure

a) Medical treatment
  • Diuretics
  • Beta blockers
  • Angiotensin‐converting enzyme inhibitors/angiotensin receptor blockers

(Nondihydropyridine calcium channel blockers are contraindicated due to their negative inotropic effect.)

b) Left ventricular assist device: bridging for heart transplant

2. Atrial fibrillation
  • Role of anticoagulation is unclear
  • Cardioversion remains a reasonable treatment option

3. Life‐threatening arrhythmia

a) ICD
  • Role of ICD as primary prevention of sudden cardiac death unclear given lack of overall survival benefit
b) Cardiac pacing
  • Indication of PPM placement is the same as current guideline
  • Limited evidence of survival benefit

Abbreviations: ATTR, transthyretin‐related amyloidosis; ICD, implantable cardioverter‐defibrillator; PPM, permanent pacemaker; TTR, transthyretin.