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. 2024 Apr 12;13(8):2257.
doi: 10.3390/jcm13082257.

Prognostic Value of Standard Heart Failure Medication in Patients with Cardiac Transthyretin Amyloidosis

Fabian Aus dem Siepen  1 , Selina Hein  1 , Eva Hofmann  1 , Christian Nagel  1 , Stéphanie K Schwarting  1 , Ute Hegenbart  2 , Stefan O Schönland  2 , Markus Weiler  3 , Norbert Frey  1 , Arnt V Kristen  1
Affiliations

Prognostic Value of Standard Heart Failure Medication in Patients with Cardiac Transthyretin Amyloidosis

Fabian Aus dem Siepen et al. J Clin Med. .

Abstract

Introduction: Cardiac transthyretin amyloidosis (ATTR) is a progressive, fatal disease leading to heart failure due to accumulation of amyloid fibrils in the interstitial space and may occur as a hereditary (ATTRv) or wild-type (ATTRwt) form. Guidelines recommend the use of ACE inhibitors (ACEis) and beta-blockers (BBs) as heart failure therapy (HFT) in all patients with symptomatic heart failure and reduced ejection fraction, independent of the underlying etiology. However, the prognostic benefit of ACEis and BBs in ATTR has not been elucidated in detail yet. We thus sought to retrospectively investigate the outcome of patients with ATTRwt or ATTRv under HFT. Methods: Medical records of 403 patients with cardiac ATTR (ATTRwt: n = 268, ATTRv: n = 135) were screened for long-term medication as well as clinical, laboratory, electrocardiographic and echocardiographic data. Patients were assessed between 2005 and 2020 at the University Hospital Heidelberg. Kaplan-Meier analysis was used to analyze potential differences in survival among different subgroups. Results: The mean follow-up was 28 months. In total, 43 patients (32%) with ATTRv and 140 patients (52%) with ATTRwt received HFT. Survival was significantly shorter in patients receiving HFT in ATTRv (46 vs. 83 months, p = 0.0007) vs. non-HFT. A significantly better survival was observed in patients with comorbidities (coronary artery disease, arterial hypertension) and HFT among ATTRwt patients (p = 0.004). No significant differences in survival were observed in the other subgroups. Conclusions: Survival analysis revealed a potential benefit of HFT in patients with ATTRwt and cardiac comorbidities such as coronary artery disease and/or arterial hypertension. In contrast, HFT should be used with caution in patients with ATTRv.

Keywords: ATTR; amyloidosis; heart failure therapy; transthyretin.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Kaplan–Meier analysis of overall survival. HFT = heart failure therapy.
Figure 2
Figure 2
Kaplan–Meier analysis of survival in ATTRwt. HFT = heart failure therapy.
Figure 3
Figure 3
Kaplan–Meier analysis of survival in ATTRv. HFT = heart failure therapy.
Figure 4
Figure 4
Kaplan–Meier analysis of survival in ATTRv subgroup, mixed phenotype. HFT = heart failure therapy.
Figure 5
Figure 5
Kaplan–Meier analysis of survival in ATTRv subgroup, cardiac phenotype. HFT = heart failure therapy.
Figure 6
Figure 6
Kaplan–Meier analysis of survival in the subgroup ATTRwt with or without HFT. HFT = heart failure therapy.
See this image and copyright information in PMC

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