66 | Letters to the Editor
International Cardiovascular Forum Journal 6 (2016)
DOI: 10.17987/icfj.v6i0.174
Amyloid Cardiomyopathy: All that Sparkles
is Amyloid
Abhinav Saxena, Sameer Chadha, On Chen, Adnan Sadiq, Jacob Shani
Address for correspondence:
Abhinav Saxena
Maimonides Medical Center, Brooklyn, NY
1016 50th street
#1E Brooklyn, NY, 11219, USA
Email: abhinavsaxenamd@gmail.com
A 73 year old female with no past medical history presented to our
emergency department (ED) with gradually worsening shortness
of breath for a few weeks. She denied any associated complaints
of chest pain, palpitations or dizziness. On physical exam, the
patient had bilateral rales at the lung bases. The electrocardiogram
showed low voltage complexes with right bundle branch block and
left anterior fascicular block (Figure 1). The laboratory work came
back signifcant for an elevated BNP (1452 pg/ml) with a normal
complete blood count, serum chemistry and negative cardiac
biomarkers. The patient was treated with intravenous diuretics in
the ED and admitted to a telemetry foor.
The echocardiogram performed on admission revealed
concentric bi-ventricular hypertrophy resulting in a restrictive
pattern and highly echogenic myocardium with ‘sparkling
appearance’, suggestive of Cardiac Amyloidosis (fgure 2, Video
1, 2, see supplementary material on website). The LV systolic
function was moderately decreased with global hypokinesis and
there was bilateral atrial enlargement along with thickening of
valve leafets and inter-atrial septum. A Cardiac MRI also showed
delayed nodular enhancement of the myocardium indicative of an
infltrative cardiomyopathy. Patient underwent an abdominal wall
fat pad biopsy which came positive for light-chain Amyloidosis.
Amyloid Cardiomyopathy is characterized by ventricular
thickening due to amyloid deposition leading to diastolic
dysfunction. It most commonly manifests with heart failure,
conduction abnormalities or exertional syncope due to inability to
augment cardiac output. The combination of increased ventricular
mass on echocardiogram and reduced electrocardiographic
voltages, along with granular appearance of the myocardium is
highly suggestive of Amyloid Cardiomyopathy
1
. The diagnosis
can be confrmed by demonstrating amyloid deposits on
histologic examination of tissues from abdominal fat pad or
kidney in patients with appropriate cardiac fndings.Cardiac
amyloidosis should be considered in any adult with unexplained
heart failure and an echocardiogram showing increased wall
thickness with a non-dilated left ventricular cavity, particularly
when associated with low voltage on electrocardiogram.
Declarations of Interest
The authors declare no conficts of interest
Acknowledgements
The authors agree to abide by the requirements of the statement of
publishing ethics of the International Cardiovascular Forum Journal
2
.
References
1. Falk RH. Diagnosis and management of the cardiac
amyloidoses. Circulation 2005;112(13):2047-60.
2. Shewan LG, Coats AJS, Henein M. Requirements for ethical
publishing in biomedical journals. International Cardiovascular
Forum Journal 2015;2:2 DOI: 10.17987/icfj.v2i1.4
* Corresponding author. abhinavsaxenamd@gmail.com
Keywords: Cardiac amyloidosis, amyloid cardiomyopathy
Citation: Saxena A, Chadha S, Chen O, Sadiq A, Shani J. Amyloid Cardiomyopathy: All that sparkles is Amyloid. International
Cardiovascular Forum Journal. 2016;6:66 DOI: 10.17987/icfj.v6i0.174
Figure 2. Add The echocardiogram.
Figure 1. The ECG
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