Cardiac amyloidosis
Updates to Article Attributes
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.
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).
Radiographic features
MRI
MRI is the modality of choice for evaluating cardiac amyloidosis. Features include:
- diffuse heterogeneous pattern of increased signal on delayed contrast enhanced inversion recovery T1-weighted images
(this is a characteristic(characteristic feature) - diffuse left ventricular wall thickening (rarely even multiple chambers)
-
reduced(progressive) diastolic and subsequently systolicfunctionbiventricular dysfunction with decreased ejection fraction - restriction of diastolic filling
- disproportionate atrial enlargement
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 systemic amyloidosis, and is the most common cause of <a href="/articles/restrictive-cardiomyopathy" title="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" title="Amyloidosis (general)">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 multiple myeloma 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>-<li>diffuse heterogeneous pattern of increased signal on delayed contrast enhanced inversion recovery T1-weighted images (this is a characteristic feature)</li>-<li>left ventricular wall thickening</li>-<li>reduced systolic function with decreased ejection fraction</li>-<li>restriction of diastolic filling</li>-<li>disproportionate atrial enlargement</li>-</ul><h4>Differential diagnosis</h4><p>Cardiac amyloidosis needs to be distinguished from other forms of <a href="/articles/restrictive-cardiomyopathy" title="Restrictive Cardiomyopathy">restrictive cardiomyopathy</a>, including:</p><ul>-<li><a href="/articles/hypertrophic-cardiomyopathy" title="Hypertrophic cardiomyopathy">hypertrophic cardiomyopathy</a></li>-<li><a href="/articles/cardiac-manifestations-of-sarcoidosis" title="Cardiac sarcoidosis">cardiac sarcoidosis</a></li>-<li><a href="/articles/cardiac-lymphoma" title="cardiac lymphoma">cardiac lymphoma</a></li>-</ul><h4>See also</h4><ul><li>-<a href="/articles/amyloidosis" title="Amyloidosis (general)">amyloidosis</a> (general article)<ul>-<li><a href="/articles/missing" style="padding-top: 0px; padding-right: 0px; padding-bottom: 0px; padding-left: 0px; margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; color: rgb(127, 127, 127); text-decoration: none; ">renal amyloidosis</a></li>-<li>-<a href="/articles/missing" style="padding-top: 0px; padding-right: 0px; padding-bottom: 0px; padding-left: 0px; margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; color: rgb(127, 127, 127); text-decoration: none; "></a><a href="/articles/missing" style="padding-top: 0px; padding-right: 0px; padding-bottom: 0px; padding-left: 0px; margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; color: rgb(127, 127, 127); text-decoration: none; ">splenic amyloidosis</a>-</li>-<li>-<a href="/articles/missing" style="padding-top: 0px; padding-right: 0px; padding-bottom: 0px; padding-left: 0px; margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; color: rgb(127, 127, 127); text-decoration: none; "></a><a href="/articles/missing" style="padding-top: 0px; padding-right: 0px; padding-bottom: 0px; padding-left: 0px; margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; color: rgb(127, 127, 127); text-decoration: none; ">hepatic amyloidosis</a>-</li>-<li>-<a href="/articles/missing" style="padding-top: 0px; padding-right: 0px; padding-bottom: 0px; padding-left: 0px; margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; color: rgb(127, 127, 127); text-decoration: none; "></a><a href="/articles/missing" title="pulmonary amyloidosis" style="padding-top: 0px; padding-right: 0px; padding-bottom: 0px; padding-left: 0px; margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; color: rgb(127, 127, 127); text-decoration: none; ">pulmonary amyloidosis</a>-</li>-<li>-<a href="/articles/missing" title="pulmonary amyloidosis" style="padding-top: 0px; padding-right: 0px; padding-bottom: 0px; padding-left: 0px; margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; color: rgb(127, 127, 127); text-decoration: none; "></a><a href="/articles/missing" title="gastrointenstinal amyloidosis" style="padding-top: 0px; padding-right: 0px; padding-bottom: 0px; padding-left: 0px; margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; color: rgb(127, 127, 127); text-decoration: none; ">gastrointenstinal amyloidosis</a>-</li>-<li>-<a href="/articles/missing" title="gastrointenstinal amyloidosis" style="padding-top: 0px; padding-right: 0px; padding-bottom: 0px; padding-left: 0px; margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; color: rgb(127, 127, 127); text-decoration: none; "></a><a href="/articles/missing" title="skeletal amyloidosis" style="padding-top: 0px; padding-right: 0px; padding-bottom: 0px; padding-left: 0px; margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; color: rgb(127, 127, 127); text-decoration: none; ">skeletal amyloidosis</a><ul><li><a href="/articles/amyloid-arthropathy" title="Amyloid arthropathy" style="padding-top: 0px; padding-right: 0px; padding-bottom: 0px; padding-left: 0px; margin-top: 0px; margin-right: 0px; margin-bottom: 0px; margin-left: 0px; color: rgb(63, 117, 216); text-decoration: none; ">amyloid arthropathy</a></li></ul>-</li>- +<p><strong>Cardiac amyloidosis</strong> is a significant source of morbidity among patients with systemic amyloidosis, 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 multiple myeloma 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>
- +<li>diffuse heterogeneous pattern of increased signal on delayed contrast enhanced inversion recovery T1-weighted images (characteristic feature)</li>
- +<li>diffuse left ventricular wall thickening (rarely even multiple chambers)</li>
- +<li>(progressive) diastolic and subsequently systolic biventricular dysfunction with decreased ejection fraction</li>
- +<li>restriction of diastolic filling</li>
- +<li>disproportionate atrial enlargement</li>
- +</ul><h4>Differential diagnosis</h4><p>Cardiac amyloidosis needs to be distinguished from other forms of <a href="/articles/restrictive-cardiomyopathy">restrictive cardiomyopathy</a>, including:</p><ul>
- +<li><a href="/articles/hypertrophic-cardiomyopathy">hypertrophic cardiomyopathy</a></li>
- +<li><a href="/articles/cardiac-manifestations-of-sarcoidosis">cardiac sarcoidosis</a></li>
- +<li><a href="/articles/cardiac-lymphoma">cardiac lymphoma</a></li>
- +</ul><h4>See also</h4><ul><li>
- +<a href="/articles/amyloidosis">amyloidosis</a> (general article)<ul>
- +<li><a href="/articles/missing">renal amyloidosis</a></li>
- +<li><a href="/articles/missing">splenic amyloidosis</a></li>
- +<li><a href="/articles/missing">hepatic amyloidosis</a></li>
- +<li><a href="/articles/missing">pulmonary amyloidosis</a></li>
- +<li><a href="/articles/missing">gastrointenstinal amyloidosis</a></li>
- +<li>
- +<a href="/articles/missing">skeletal amyloidosis</a><ul><li><a href="/articles/amyloid-arthropathy">amyloid arthropathy</a></li></ul>
- +</li>