Wild-type (senile) amyloidosis echocardiography and ultrasound
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]
Overview
The most commonly encountered pathology on 2D echocardiography is increased left ventricular thickness (secondary to amyloid fibrils deposition in the extracellular matrix). Advanced echocardiographic techniques (strain and strain rate imaging) can differentiate cardiomyopathy secondary to amyloidosis from other causes of left ventricular hypertrophy. In regards to the evaluation of cardiac amyloidosis, EFSR has approximately 90% and 92% sensitivity and specificity, respectively.
Echocardiography/Ultrasound
Echocardiography
- Echocardiography is the most frequently used diagnostic modality for cardiomyopathy.
- The most commonly encountered pathology on 2D echocardiography is increased left ventricular thickness (secondary to amyloid fibrils deposition in the extracellular matrix).[1]
- Other echocardiographic findings related to cardiac amyloidosis include:
- Diastolic dysfunction with preserved ejection fraction (hallmark)[2][3]
- Moderate left ventricular systolic dysfunction[4]
- Non-specific findings, such as:
- Biatrial enlargement[5][6]
- Thickening of the mitral/aortic leaflets and intra-atrial septum
- Small pericardial effusion
- Moderate pulmonary hypertension
- Intracardiac thrombus
Advanced Echocardiographic Techniques
- Advanced echocardiographic techniques (strain and strain rate imaging) can differentiate cardiomyopathy secondary to amyloidosis from other causes of left ventricular hypertrophy.[7]
- These techniques use vendor specific proprietary software to track the longitudinal shortening of speckles within the 2D image of the left ventricular wall during systole.
- Cardiac amyloidosis is characterized by:
- Greater restriction of basal speckle longitudinal movement compared to apical movement resulting in a relative "apical sparing" pattern of longitudinal strain with bulls-eye plot.[8]
Ejection Fraction Global Longitudinal Strain Ratio (EFSR)
- EFSR is calculated by ratio of left ventricular ejection fraction and global longitudinal strain.[9]
- In regards to the evaluation of cardiac amyloidosis, EFSR has approximately 90% and 92% sensitivity and specificity, respectively.
References
- ↑ Ilia G. Halatchev, Jingsheng Zheng & Jiafu Ou (2018). "Wild-type transthyretin cardiac amyloidosis (ATTRwt-CA), previously known as senile cardiac amyloidosis: clinical presentation, diagnosis, management and emerging therapies". Journal of thoracic disease. 10 (3): 2034–2045. doi:10.21037/jtd.2018.03.134. PMID 29707360. Unknown parameter
|month=ignored (help) - ↑ Wendy Tsang & Roberto M. Lang (2010). "Echocardiographic evaluation of cardiac amyloid". Current cardiology reports. 12 (3): 272–276. doi:10.1007/s11886-010-0108-7. PMID 20424972. Unknown parameter
|month=ignored (help) - ↑ Diego Bellavia, Patricia A. Pellikka, Theodore P. Abraham, Ghormallah B. Al-Zahrani, Angela Dispenzieri, Jae K. Oh, Kent R. Bailey, Christina M. Wood, Martha Q. Lacy, Chinami Miyazaki & Fletcher A. Jr Miller (2008). "Evidence of impaired left ventricular systolic function by Doppler myocardial imaging in patients with systemic amyloidosis and no evidence of cardiac involvement by standard two-dimensional and Doppler echocardiography". The American journal of cardiology. 101 (7): 1039–1045. doi:10.1016/j.amjcard.2007.11.047. PMID 18359328. Unknown parameter
|month=ignored (help) - ↑ Claudio Rapezzi, Giampaolo Merlini, Candida C. Quarta, Letizia Riva, Simone Longhi, Ornella Leone, Fabrizio Salvi, Paolo Ciliberti, Francesca Pastorelli, Elena Biagini, Fabio Coccolo, Robin M. T. Cooke, Letizia Bacchi-Reggiani, Diego Sangiorgi, Alessandra Ferlini, Michele Cavo, Elena Zamagni, Maria Luisa Fonte, Giovanni Palladini, Francesco Salinaro, Francesco Musca, Laura Obici, Angelo Branzi & Stefano Perlini (2009). "Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types". Circulation. 120 (13): 1203–1212. doi:10.1161/CIRCULATIONAHA.108.843334. PMID 19752327. Unknown parameter
|month=ignored (help) - ↑ Wendy Tsang & Roberto M. Lang (2010). "Echocardiographic evaluation of cardiac amyloid". Current cardiology reports. 12 (3): 272–276. doi:10.1007/s11886-010-0108-7. PMID 20424972. Unknown parameter
|month=ignored (help) - ↑ DaLi Feng, Imran S. Syed, Matthew Martinez, Jae K. Oh, Allan S. Jaffe, Martha Grogan, William D. Edwards, Morie A. Gertz & Kyle W. Klarich (2009). "Intracardiac thrombosis and anticoagulation therapy in cardiac amyloidosis". Circulation. 119 (18): 2490–2497. doi:10.1161/CIRCULATIONAHA.108.785014. PMID 19414641. Unknown parameter
|month=ignored (help) - ↑ Ilia G. Halatchev, Jingsheng Zheng & Jiafu Ou (2018). "Wild-type transthyretin cardiac amyloidosis (ATTRwt-CA), previously known as senile cardiac amyloidosis: clinical presentation, diagnosis, management and emerging therapies". Journal of thoracic disease. 10 (3): 2034–2045. doi:10.21037/jtd.2018.03.134. PMID 29707360. Unknown parameter
|month=ignored (help) - ↑ Dermot Phelan, Patrick Collier, Paaladinesh Thavendiranathan, Zoran B. Popovic, Mazen Hanna, Juan Carlos Plana, Thomas H. Marwick & James D. Thomas (2012). "Relative apical sparing of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis". Heart (British Cardiac Society). 98 (19): 1442–1448. doi:10.1136/heartjnl-2012-302353. PMID 22865865. Unknown parameter
|month=ignored (help) - ↑ Efstathios D. Pagourelias, Oana Mirea, Jurgen Duchenne, Johan Van Cleemput, Michel Delforge, Jan Bogaert, Tatyana Kuznetsova & Jens-Uwe Voigt (2017). "Echo Parameters for Differential Diagnosis in Cardiac Amyloidosis: A Head-to-Head Comparison of Deformation and Nondeformation Parameters". Circulation. Cardiovascular imaging. 10 (3): e005588. doi:10.1161/CIRCIMAGING.116.005588. PMID 28298286. Unknown parameter
|month=ignored (help)