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Special Issue "New Insights in Cardiac Amyloidosis and Its Complications"

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Guest Editor

Department of Cardiology and Vascular Medicine, West German Heart and Vascular Center, University Hospital Essen, Essen, Germany
Interests: heart failure; cardiomyopathies; cardiogenic shock; cardiac physiology and hemodynamics of cardiac disease; interventional cardiology

Dr. Maria Papathanasiou

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Guest Editor

Department of Cardiology and Vascular Medicine, West German Heart and Vascular Center, University Hospital Essen, Essen, Germany
Interests: heart failure; cardiomyopathies; mechanical circulatory support; heart transplantation

Special Issue Information

Dear Colleagues,

Cardiac amyloidosis is no longer a rare and exotic disease. Even if our understanding of the disease remains limited, tremendous progress has recently been made in diagnosing and treating this fatal disease. In this dedicated Special Issue we aim to provide new insights into the epidemiology, acute and chronic manifestations, as well as new and emerging therapies of cardiac amyloidosis and its complications. We welcome authors to submit their valuable work and contribute to demystifying this challenging clinical problem.

Prof. Dr. Peter Luedike
Dr. Maria Papathanasiou
Guest Editors

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Keywords

amyloidosis
light chains
tafamidis
transthyretin
restrictive cardiomyopathy
peripheral polyneuropathy
heart failure

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Research

Open AccessArticle

Risk Stratification in Transthyretin Cardiac Amyloidosis: The Added Value of Lung Spirometry

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J. Clin. Med. 2023, 12(11), 3684; https://doi.org/10.3390/jcm12113684 - 26 May 2023

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Abstract

Transthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized disease that often results in heart failure and death. Traditionally, biological staging systems are used to stratify disease severity. Reduced aerobic capacity has recently been described as useful in identifying higher risk of cardiovascular events and death. Assessment of lung volume via simple spirometry might also hold prognostic relevance. We aimed to assess the combined prognostic value of spirometry, cardiopulmonary exercise testing (CPET) and biomarker staging in ATTR-CA patients in a multi-parametric approach. We retrospectively reviewed patient records with pulmonary function and CPET testing. Patients were followed until study endpoint (MACE: composite of heart-failure-related hospitalization and all-cause death) or censure (1 April 2022). In total, 82 patients were enrolled. Median follow-up was 9 months with 31 (38%) MACE. Impaired peak VO₂ and forced vital capacity (FVC) were independent predictors of MACE-free survival, with peak VO₂ < 50% and FVC < 70% defining the highest risk group (HR 26, 95% CI: 5–142, mean survival: 15 months) compared to patients with the lowest risk (peak VO₂ ≥ 50% and FVC ≥ 70%). Combined peak VO₂, FVC and ATTR biomarker staging significantly improved MACE prediction by 35% compared to ATTR staging alone, with 67% patients reassigned a higher risk category (p < 0.01). In conclusion, combining functional and biological markers might synergistically improve risk stratification in ATTR-CA. Integrating simple, non-invasive and easily applicable CPET and spirometry in the routine management of ATTR-CA patients might prove useful for improved risk prediction, optimized monitoring and timely introduction of newer-generation therapies. Full article

(This article belongs to the Special Issue New Insights in Cardiac Amyloidosis and Its Complications)

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Special Issue "New Insights in Cardiac Amyloidosis and Its Complications"

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