Key result
Cardiac amyloidosis exhibits heterogeneous myocardial stiffness and contractile performance within the same heart.
Why the study?
Functional assessment of cardiac amyloidosis usually relies on echocardiography, which does not assess preload-dependent contractile reserve (Frank-Starling mechanism).
Case Report (n=1)
This study demonstrates significant functional and histological heterogeneity of amyloid deposition and tissue mechanics within a single patient's heart.
Intra-myocardial heterogeneity may complicate uniform assessment in cardiac amyloidosis; leaves open questions on regional sampling and targeted therapies.
OBJECTIVE: The goal of this study was to examine in vitro tissue stiffness and contractile performance in myocardial amyloidosis. BACKGROUND: Primary systemic amyloidosis involves the deposition of amyloid protein in mesodermal tissues including the heart. Functional assessment of cardiac amyloidosis is usually performed using echocardiography. However, this technique does not involve assessment of preload-dependent contractile reserve (the Frank-Starling mechanism). METHODS: At the time of heart transplantation, isolated myocardial trabeculae were dissected from the right ventricle of a patient with primary systemic amyloidosis. In vitro length-tension experiments were performed and trabeculae were subsequently fixed, sectioned and stained with crystal violet to determine amyloid deposition. RESULTS: Among the nine trabeculae capable of generating force transients, various combinations of myocardial stiffness and contractile performance were observed including normal stiffness and contractility, severely increased stiffness with impaired contractility and hybrid patterns. Histological analysis demonstrated varying degrees of amyloid deposition among sampled trabeculae. CONCLUSIONS: Our findings extend previous reports of functional heterogeneity among patients by demonstrating functional heterogeneity within a single patient's heart. Our findings also highlight the functional interdependence of passive stiffness and systolic performance in the diseased myocardium and demonstrate the value of dynamic assessments of myocardial performance.
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Petre et al. (2005) conducted a case report in Primary systemic amyloidosis (n=1). In vitro length-tension experiments and histological analysis was evaluated on In vitro tissue stiffness, contractile performance, and amyloid deposition. In vitro analysis of 9 myocardial trabeculae from a single patient with cardiac amyloidosis revealed heterogeneous patterns of tissue stiffness, contractile performance, and amyloid deposition.
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