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Infiltrative Diseases
Published in Andreas P. Kalogeropoulos, Hal A. Skopicki, Javed Butler, Heart Failure, 2023
The characteristic appearance of cardiac amyloidosis is increased biventricular wall thickness and severe diastolic dysfunction. Common symptoms associated with cardiac amyloidosis include fatigue and dyspnea, along with other non-specific findings of HF. Physical examination findings are often consistent with right-sided HF, including elevated jugular venous distention, abdominal ascites, and lower extremity edema.
The QRS complex
Published in Andrew R Houghton, Making Sense of the ECG, 2019
Cardiac amyloidosis is an infiltrative cardiomyopathy caused by the deposition of an abnormal protein (amyloid) in the extracellular space of the myocardium. Most cases of cardiac amyloidosis are caused by one of two proteins: light chains (AL amyloidosis) or transthyretin (ATTR amyloidosis). The condition leads to myocardial thickening, which is clearly seen on echocardiography or cardiac magnetic resonance imaging.
Test Paper 1
Published in Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike, Get Through, 2017
Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike
A 76-year-old male patient with chronic inflammatory disease and known history of secondary generalised multisystem amyloidosis showed an abnormal appearance of the heart on echocardiography. Dynamic enhanced cardiac MR imaging was advised for further characterisation. All of the following are imaging findings seen with cardiac amyloidosis, except Left ventricular wall hypertrophySubendocardial delayed myocardial hyperenhancementSystolic dysfunctionGranular echogenic myocardiumInteratrial septal thickening
Cervical myelopathy due to amyloid deposition with accompanying cardiac abnormalities
Published in The Journal of Spinal Cord Medicine, 2023
Masashi Uehara, Shugo Kuraishi, Shota Ikegami, Hiroki Oba, Takashi Takizawa, Ryo Munakata, Terue Hatakenaka, Takayuki Kamanaka, Yoshinari Miyaoka, Yoshiki Sekijima, Jun Takahashi
Cardiac amyloidosis is present in many patients with amyloidosis, with approximately 50% of cases presenting with diastolic heart failure at the time of diagnosis.20 Amyloid heart disease is a major prognostic factor as it accounts for approximately 75% of deaths due to heart failure or arrhythmia.21,22 The deposition of amyloid fibrils within the myocardium causes restrictive heart failure with thickening of the ventricular and atrial walls and a gradual increase in asthenia, dyspnea, and lower extremity edema. Amyloid infiltration into the myocardium may also induce conduction defects and ventricular or supraventricular arrhythmias.21–23 In the present case, preoperative ECG indicated conduction disturbances, which led to a preoperative diagnosis of cardiac amyloidosis. Although the patient had significant cardiac dysfunction, we were able to identify it before surgery and manage the patient's perioperative treatment successfully.
The flutemetamol analogue cyano-flutemetamol detects myocardial AL and ATTR amyloid deposits: a post-mortem histofluorescence analysis
Published in Amyloid, 2023
Eric E. Abrahamson, Robert F. Padera, Julie Davies, Gill Farrar, Victor L. Villemagne, Sharmila Dorbala, Milos D. Ikonomovic
Advances in cardiovascular imaging over the past decade have resulted in the development of non-invasive methods for detection and diagnosis of cardiac amyloidosis. Echocardiography and cardiac magnetic resonance imaging (MRI) are the usual first tests that raise the suspicion of amyloidosis after the history and physical examination; however, they are not sufficiently specific [16]. Thus, when cardiac amyloidosis is suspected on echocardiography or cardiac MRI, it traditionally requires confirmation by endomyocardial biopsy as described above. Single photon emission computed tomography (SPECT) has recently emerged as a highly specific non-invasive method to diagnose ATTR cardiac amyloidosis. With nearly 100% specificity for amyloid deposits [17], technetium-99m-labeled bone avid tracer SPECT, [99mTc]pyrophosphate (PYP), 3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) or hydroxymethylene diphosphonate (HMDP) methods, combined with serum and urine based evaluation for a plasma cell dyscrasia, are replacing endomyocardial biopsy for the diagnosis of ATTR cardiac amyloidosis [18]. However, bone avid SPECT tracers have low sensitivity to image AL amyloidosis [19,20], and emerging data suggest a key role for amyloid PET tracers in imaging both ATTR and AL types of cardiac amyloidosis [21].
Association between spinal stenosis and wild-type ATTR amyloidosis
Published in Amyloid, 2021
Amandeep Godara, Ron I. Riesenburger, Diana X. Zhang, Cindy Varga, Teresa Fogaren, Nauman S. Siddiqui, Anthony Yu, Andy Wang, Michael Mastroianni, Richard Dowd, Tara J. Nail, Ellen D. McPhail, Paul J. Kurtin, Jason D. Theis, Denis Toskic, Knarik Arkun, Monika Pilichowska, James Kryzanski, Ayan R. Patel, Raymond Comenzo
Some limitations of our study require a mention. This is a cross-sectional study and without prospective long-term follow-up, the magnitude of risk of cardiac amyloidosis cannot be completely ascertained. Additionally, the prevalence of ATTR involvement in the ligamentum flavum was lower than the reported prevalence of ATTR deposits in Swedish and Japanese patients undergoing surgery for lumbar spinal stenosis (33–45%) [9–11,29]. This could be due to the differences in detection methods for amyloid identification and inclusion of all age groups and spinal regions (cervical, thoracic and lumbar). The type of amyloid could not be identified in several patients and it is possible that some of these amyloid deposits were from ATTR. Furthermore, processes leading to amyloid involvement of the spine may have a genetic basis that varies among ethnic groups, an observation that has been reported in other degenerative spinal disorders. For example, ossification of the posterior longitudinal ligament is seen predominantly in Asian patients; such differences could also contribute to differential amyloid involvement of the spine [30]. Non-invasive diagnoses via PYP scan is now an established method for diagnosis of ATTR cardiac amyloidosis and can obviate the need for endomyocardial biopsy but false-positive uptakes have been reported [31,32]. A large number of PYP results were equivocal and it is unknown if this represents an evolving process, further highlighting the challenges associated with the use of imaging modalities in diagnosing ATTR amyloidosis.