Guideline-Directed Medical Therapy May Not Be Harmful in Patients with TTR-CA and Heart Failure with Reduced Ejection Fraction

No guidelines exist on the use of guideline-directed medical therapy (GDMT) in the treatment of patients with transthyretin cardiac amyloidosis (TTR-CA) and heart failure with reduced ejection fraction (HFrEF), and its use remains controversial. There are no survival data demonstrating the effects of beta-blockers in patients with TTR-CA, and the efficacy of angiotensin-converting enzyme inhibitors (ACEIs), angiotensin receptor blockers (ARBs), and angiotensin receptor-neprilysin inhibitors (ARNIs) is uncertain. This observational study describes the outcomes of patients with TTR-CA and HFrEF treated with GDMT.

Charlene L. Rohm, MD, Cardiologist, Wellstar Kennestone Regional Medical Center, Marietta, GA, and colleagues retrospectively reviewed the medical records of 8 consecutive patients with TTR-CA and HFrEF treated with GDMT at a single institution in Marietta, GA, in this observational case series.

Patients’ average age was 76.9 ± 5.7 years, and 75% were male. All-cause mortality was 50%. All patients received treatment with a beta-blocker; 75% were treated with either an ACEI, ARB, or ARNI; all were treated with a mineralocorticoid receptor antagonist; and all received diuretics. Fifty percent of patients (survivors) responded favorably to GDMT with improved ejection fraction (change in value, +26.25 vs –1.25%; P <.0001), decreased creatinine (change in value, –0.58 vs +1.18 mg/dL; P = .038), improved functional capacity, and had few heart failure–related hospitalizations over the study period. Fifty percent of patients remained in decompensated heart failure and were admitted to hospice care.

“While the natural history of TTR-CA is poor, our study suggests that GDMT may be used cautiously and may not be harmful in patients with TTR-CA and HFrEF; GDMT may be beneficial in select patients,” stated Dr Rohm and colleagues.