What is it about?
The editorial focuses on particular and often overlooked characteristics of cardiac amyloidosis.
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Why is it important?
It is important because it gives a correct interpretation of the publication by Pozo et al. Furthermore, this editorial summarizes the correct steps to suspect and reach a final diagnosis of cardiac amyloidosis. It underlines the role of 99mTc-DPD scintigraphy (bone tracers scintigraphy) for the early diagnosis of TTR-related amyloidosis and for the differential diagnosis of cardiac amyloidosis. This concept is pioneering, expecially considering the following papers such as: Nonbiopsy Diagnosis of Cardiac Transthyretin Amyloidosis. Gillmore JD, Maurer MS, Falk R et al. Circulation 2016 Finally a little insights is provided related to hypothesis that amyloid deposition (in TTR amyloidosis) might occur preferentially in the interventricular septum in early disease stages and as disease progresses concentric LVH develops. This observation was recently confirmed by a paper published on JACC. Magnetic Resonance in Transthyretin Cardiac Amyloidosis. Martinez-Naharro A, Treibel TA, Abdel-Gadir A et al. JACC 2017 Volume 70, Issue 4.
Perspectives
Patients who experiences heart failure often have a cardiomyopathy as underlying disease but their cardiologists are not able to make a prompt diagnosis. Therefore, often when a final diagnosis of cardiomyopathy - and in particular for infiltrative cardiomyopathies - is reached is too late, and the patient has already developed an advanced heart failure (Stage D). This represents an unmet clinical need. We need to improve international collaborations both in diagnosis (echocardiography, CMR, nuclear medicine and biopsies) and in therapy (beyond TTR-stabilizers, the most promising therapies are Monoclonal antibodies ant-SAP).
Dr GIUSEPPE G GALATI
IRCCS Ospedale San Raffaele
Read the Original
This page is a summary of: Myocardial amyloid infiltration: a less than expected homogeneous process, Heart, July 2014, BMJ,
DOI: 10.1136/heartjnl-2014-306221.
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