Cardiac amyloidosis
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Cardiac amyloidosis is a significant source of morbidity among patients with systemic amyloidosis, and is the most common cause of restrictive cardiomyopathy outside the tropics.
Pathology
Amyloidosis represents the extra-cellular deposition of insoluble fibrillar proteinaceous material in various organs and tissues in a variety of clinical settings. For a general discussion of the condition please refer to amyloidosis.
Cardiac involvement is seen with most forms of amyloidosis, although it is most common and most often clinically significant with primary amyloidosis (i.e. associated with multiple myeloma or other monoclonal gammopathies).
There are 2 main forms of amyloidosis that significantly affect the heart.
- AL amyloidosis: acquired disease
-
transthyretin-related (TTR) amyloidosis: there are 2 types of TTR-related amyloidosis:
- genetic form: hereditary transthyretin-related amyloidosis (ATTR)
- non-hereditary form: senile systemic amyloidosis (SSA)
Radiographic features
Echocardiography
Described features include
- atrial septal thickening: considered a characteristic feature of cardiac amyloidosis
- characteristic granular / sparkling appearance of the left ventricular (LV) myocardium: not specific and need to differentiate from other infiltrative diseases
- increased LV wall thickness: results from amyloid infiltration of interstitial space and may relate to amyloid burden
- decreased LV end-diastolic volume: leads to reduced stroke volume despite near normal LVEF
- typically preserved or mildly reduced LV ejection fraction: LV ejection fraction may decrease in end stage disease
- high E/A ratio: due to restrictive pathophysiology; but, a reduced amplitude A wave may suggest poor atrial function and higher risk of thrombus formation
- shortened mitral E deceleration time (restrictive filling pattern): high E/e’ ratioHigh E/e’ suggests increased left atrial pressures
- increased left and right atrial volumes and reduced atrial function
- atrial strain: can be significantly reduced
- longitudinal strain (LS) in the left ventricle is impaired and worse at the base and mid ventricular regions compared to the apex: specific patterns of left ventricular LS may differentiate amyloid from aortic stenosis and hypertrophic cardiomyopathy
- right ventricular thickening: reduced RV myocardial velocities on tissue Doppler imaging and reduced RV LS.TAPSE and RV LS are early indicators of cardiac involvement in patients with systemic AL amyloidosis
- reduced tricuspid annular plane excursion despite normal RV end-diastolic dimension: RV LS may be an independent predictor of cardiac death
- dynamic LV outflow tract obstruction: less common feature, but, need to distinguish from hypertrophic cardiomyopathy
MRI
MRI is the modality of choice for evaluating cardiac amyloidosis. Features include:
- diffuse decrease in the T1 and T2 signal intensity of myocardium
- diffuse
sub-endocardial heterogeneous increased signal on delayed contrast-enhanced inversion recovery T1-weighted images (a characteristic feature) -
diffuseleft ventricular wall thickening (usually multiple chambers are involved). Can; can be associated with thickening of both atrial walls in which case,a thickening of > 6mm of inter-atrial septum or posterior right atrial wall is specific to amyloidosis - biventricular diastolic dysfunction with normal systolic function and ejection fraction
- subsequently (later in the course of disease) may show systolic biventricular dysfunction with decreased ejection fraction
- restriction of diastolic filling
- disproportionate atrial enlargement
- pericardial and pleural effusions
- T1 mapping: subendocardial T1 relaxation time may be shortened in cardiac amyloidosis; considered an early feature of cardiac amyloid involvement
-
C+ Gd:
- diffuse sub-endocardial heterogeneous increased signal on delayed contrast-enhanced inversion recovery T1-weighted images (a characteristic feature)
- transmural enhancement may be more prevalent in patients with ATTR compared to patients with AL 6
Differential diagnosis
Cardiac amyloidosis needs to be distinguished from other forms of restrictive cardiomyopathy, including:
See also
-<p><strong>Cardiac amyloidosis</strong> is a significant source of morbidity among patients with <a href="/articles/amyloidosis">systemic amyloidosis</a>, and is the most common cause of <a href="/articles/restrictive-cardiomyopathy">restrictive cardiomyopathy</a> outside the tropics.</p><p>Amyloidosis represents the extra-cellular deposition of insoluble fibrillar proteinaceous material in various organs and tissues in a variety of clinical settings. For a general discussion of the condition please refer to <a href="/articles/amyloidosis">amyloidosis</a>. </p><p>Cardiac involvement is seen with most forms of amyloidosis, although it is most common and most often clinically significant with primary amyloidosis (i.e. associated with <a href="/articles/multiple-myeloma-1">multiple myeloma</a> or other monoclonal gammopathies).</p><h4>Radiographic features</h4><h5>MRI</h5><p>MRI is the modality of choice for evaluating cardiac amyloidosis. Features include:</p><ul>- +<p><strong>Cardiac amyloidosis</strong> is a significant source of morbidity among patients with <a href="/articles/amyloidosis">systemic amyloidosis</a>, and is the most common cause of <a href="/articles/restrictive-cardiomyopathy">restrictive cardiomyopathy</a> outside the tropics.</p><h4>Pathology</h4><p>Amyloidosis represents the extra-cellular deposition of insoluble fibrillar proteinaceous material in various organs and tissues in a variety of clinical settings. For a general discussion of the condition please refer to <a href="/articles/amyloidosis">amyloidosis</a>. </p><p>Cardiac involvement is seen with most forms of amyloidosis, although it is most common and most often clinically significant with primary amyloidosis (i.e. associated with <a href="/articles/multiple-myeloma-1">multiple myeloma</a> or other monoclonal gammopathies).</p><p>There are 2 main forms of amyloidosis that significantly affect the heart.</p><ul>
- +<li>AL amyloidosis: acquired disease</li>
- +<li>transthyretin-related (TTR) amyloidosis: there are 2 types of TTR-related amyloidosis:<ul>
- +<li>genetic form: hereditary transthyretin-related amyloidosis (ATTR)</li>
- +<li>non-hereditary form: senile systemic amyloidosis (SSA)</li>
- +</ul>
- +</li>
- +</ul><h4>Radiographic features</h4><h5>Echocardiography</h5><p>Described features include</p><ul>
- +<li>
- +<a title="atrial septal thickening" href="/articles/atrial-septal-thickening">atrial septal thickening</a>: considered a characteristic feature of cardiac amyloidosis</li>
- +<li>characteristic granular / sparkling appearance of the left ventricular (LV) myocardium: not specific and need to differentiate from other infiltrative diseases</li>
- +<li>increased LV wall thickness: results from amyloid infiltration of interstitial space and may relate to amyloid burden</li>
- +<li>decreased LV end-diastolic volume: leads to reduced stroke volume despite near normal LVEF</li>
- +<li>typically preserved or mildly reduced LV ejection fraction: LV ejection fraction may decrease in end stage disease</li>
- +<li>high E/A ratio: due to restrictive pathophysiology; but, a reduced amplitude A wave may suggest poor atrial function and higher risk of thrombus formation</li>
- +<li>shortened mitral E deceleration time (restrictive filling pattern): high E/e’ ratioHigh E/e’ suggests increased left atrial pressures</li>
- +<li>increased left and right atrial volumes and reduced atrial function</li>
- +<li>atrial strain: can be significantly reduced</li>
- +<li>longitudinal strain (LS) in the left ventricle is impaired and worse at the base and mid ventricular regions compared to the apex: specific patterns of left ventricular LS may differentiate amyloid from aortic stenosis and hypertrophic cardiomyopathy</li>
- +<li>right ventricular thickening: reduced RV myocardial velocities on tissue Doppler imaging and reduced RV LS.TAPSE and RV LS are early indicators of cardiac involvement in patients with systemic AL amyloidosis</li>
- +<li>reduced tricuspid annular plane excursion despite normal RV end-diastolic dimension: RV LS may be an independent predictor of cardiac death</li>
- +<li>dynamic LV outflow tract obstruction: less common feature, but, need to distinguish from hypertrophic cardiomyopathy</li>
- +</ul><h5>MRI</h5><p>MRI is the modality of choice for evaluating cardiac amyloidosis. Features include:</p><ul>
-<li>diffuse sub-endocardial heterogeneous increased signal on delayed contrast-enhanced inversion recovery T1-weighted images (a characteristic feature)</li>-<li>diffuse left ventricular wall thickening (usually multiple chambers are involved). Can be associated with thickening of both atrial walls in which case,a thickening of > 6mm of inter-atrial septum or posterior right atrial wall is specific to amyloidosis</li>- +<li>diffuse left ventricular wall thickening (usually multiple chambers are involved); can be associated with thickening of both atrial walls in which case,a thickening of > 6mm of inter-atrial septum or posterior right atrial wall is specific to amyloidosis</li>
- +<li>
- +<strong>T1 mapping</strong>: subendocardial T1 relaxation time may be shortened in cardiac amyloidosis; considered an early feature of cardiac amyloid involvement</li>
- +<li>
- +<strong>C+ Gd</strong>: <ul>
- +<li>diffuse sub-endocardial heterogeneous increased signal on delayed contrast-enhanced inversion recovery T1-weighted images (a characteristic feature)</li>
- +<li>transmural enhancement may be more prevalent in patients with ATTR compared to patients with AL <sup>6</sup>
- +</li>
- +</ul>
- +</li>
References changed:
- 5. Quarta C, Kruger J, Falk R. Cardiac Amyloidosis. Circulation. 2012;126(12):e178-82. <a href="https://doi.org/10.1161/CIRCULATIONAHA.111.069195">doi:10.1161/CIRCULATIONAHA.111.069195</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/22988049">Pubmed</a>
- 6. Bhogal S, Ladia V, Sitwala P et al. Cardiac Amyloidosis: An Updated Review With Emphasis on Diagnosis and Future Directions. Curr Probl Cardiol. 2018;43(1):10-34. <a href="https://doi.org/10.1016/j.cpcardiol.2017.04.003">doi:10.1016/j.cpcardiol.2017.04.003</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29173805">Pubmed</a>
- 7. Donnelly J & Hanna M. Cardiac Amyloidosis: An Update on Diagnosis and Treatment. Cleve Clin J Med. 2017;84(12 Suppl 3):12-26. <a href="https://doi.org/10.3949/ccjm.84.s3.02">doi:10.3949/ccjm.84.s3.02</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29257735">Pubmed</a>
- 8. Alkhawam H, Patel D, Nguyen J et al. Cardiac Amyloidosis: Pathogenesis, Clinical Context, Diagnosis and Management Options. Acta Cardiol. 2017;72(4):380-9. <a href="https://doi.org/10.1080/00015385.2017.1335034">doi:10.1080/00015385.2017.1335034</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/28705053">Pubmed</a>
- 9. Falk R, Quarta C, Dorbala S. How to Image Cardiac Amyloidosis. Circ Cardiovasc Imaging. 2014;7(3):552-62. <a href="https://doi.org/10.1161/CIRCIMAGING.113.001396">doi:10.1161/CIRCIMAGING.113.001396</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/24847009">Pubmed</a>
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