scholarly journals Diagnosing Cardiac Amyloidosis: From Heart Failure to Electrical Storm

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Adithya T. Mathews ◽  
Abu-Sayeef Mirza ◽  
Chandrashekar Bohra ◽  
Akshay G. Mathews ◽  
Philip Ritucci-Chinni ◽  
...  

Cardiac amyloidosis is a condition when amyloid fibers are deposited in the extracellular space of the heart causing tachyarrhythmias, heart failure, or sudden cardiac death. We present a 71-year-old woman presenting with dyspnea on admission. Echocardiogram revealed diastolic heart failure and left ventricular hypertrophy with strain pattern concerning for an infiltrative process. She was discharged with diuretic therapy and scheduled for a cardiac magnetic resonance imaging. One week after discharge, she was readmitted with progressive shortness of breath and syncope. She was found to be in shock and had multiple episodes of cardiac arrest with both ventricular tachycardia and pulseless electrical activity. She developed electrical storm and eventually passed within 24 hours. Autopsy revealed gross cardiomegaly and left ventricular hypertrophy with Congo red staining revealing amyloid fibrils with apple-green birefringence. This case demonstrates the rapid progression of cardiac amyloidosis from acute-onset diastolic heart failure to uncontrollable ventricular tachycardia, and eventually death. We review the literature regarding multiple diagnostic modalities that facilitate the confirmation of cardiac amyloidosis.


2011 ◽  
Vol 2011 ◽  
pp. 1-10 ◽  
Author(s):  
Richard E. Katholi ◽  
Daniel M. Couri

Left ventricular hypertrophy is a maladaptive response to chronic pressure overload and an important risk factor for atrial fibrillation, diastolic heart failure, systolic heart failure, and sudden death in patients with hypertension. Since not all patients with hypertension develop left ventricular hypertrophy, there are clinical findings that should be kept in mind that may alert the physician to the presence of left ventricular hypertrophy so a more definitive evaluation can be performed using an echocardiogram or cardiovascular magnetic resonance. Controlling arterial pressure, sodium restriction, and weight loss independently facilitate the regression of left ventricular hypertrophy. Choice of antihypertensive agents may be important when treating a patient with hypertensive left ventricular hypertrophy. Angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers followed by calcium channel antagonists most rapidly facilitate the regression of left ventricular hypertrophy. With the regression of left ventricular hypertrophy, diastolic function and coronary flow reserve usually improve, and cardiovascular risk decreases.



2021 ◽  
Author(s):  
Jorge Melero Polo ◽  
Ana Roteta Unceta-Barrenechea ◽  
Pablo Revilla Martí ◽  
Raquel Pérez-Palacios ◽  
Anyuli Gracia Gutiérrez ◽  
...  


2011 ◽  
Vol 4 (3) ◽  
pp. 246-256 ◽  
Author(s):  
Michael R. Zile ◽  
Stacia M. DeSantis ◽  
Catalin F. Baicu ◽  
Robert E. Stroud ◽  
Sheila B. Thompson ◽  
...  




BMJ Open ◽  
2016 ◽  
Vol 6 (2) ◽  
pp. e010282 ◽  
Author(s):  
Ehsan Bahramali ◽  
Mona Rajabi ◽  
Javad Jamshidi ◽  
Seyyed Mohammad Mousavi ◽  
Mehrdad Zarghami ◽  
...  


Author(s):  
Andrew Chiou ◽  
Edris Aman ◽  
Manoj Kesarwani

Abstract Background  Transthyretin amyloid cardiomyopathy (ATTR-CM) is a commonly misdiagnosed cardiac condition due to low disease awareness and perceived rarity, which frequently results in incorrect management and poor outcomes. Early and prompt diagnosis has become critical with emerging therapies that improve patient survival. Case summary  A 68-year-old woman presented to a tertiary care centre with acute decompensated heart failure following recurrent hospitalizations for the same issue over the past several months. Transthoracic echocardiography revealed severe concentric left ventricular hypertrophy with grade III diastolic dysfunction. However, QRS voltage by 12-lead electrocardiogram (ECG) was discordant with the degree of left ventricular hypertrophy seen by echocardiography, and the patient had recurrent non-sustained ventricular tachycardia that necessitated implantable cardioverter-defibrillator implantation a few months prior. After aggressive diuresis, the patient completed cardiac magnetic resonance imaging that raised concern for cardiac amyloidosis. Subsequent serum and urine protein electrophoresis with associated immunofixation were within normal limits. Finally, ATTR-CM was confirmed by technetium-99m pyrophosphate scintigraphy with plans to initiate tafamidis after genetic testing. Discussion  Patients >60 years of age with diastolic heart failure phenotypically similar to hypertrophic cardiomyopathy and/or hypertensive heart disease should always be evaluated for ATTR-CM. Features that increase suspicion include discordance between left ventricular wall thickness and ECG voltage, and signs/symptoms of a primary peripheral and autonomic neuropathy. Useful non-invasive diagnostic testing has also made the diagnosis of ATTR-CM inexpensive and possible without the need for an endomyocardial biopsy. Unfortunately, this patient’s diagnosis of ATTR-CM came late in her disease course, which delayed the onset of definitive therapy.



Medicina ◽  
2021 ◽  
Vol 57 (7) ◽  
pp. 660
Author(s):  
Csilla-Andrea Eötvös ◽  
Roxana-Daiana Lazar ◽  
Iulia-Georgiana Zehan ◽  
Erna-Brigitta Lévay-Hail ◽  
Giorgia Pastiu ◽  
...  

Among the different types, immunoglobulin light chain (AL) cardiac amyloidosis is associated with the highest morbidity and mortality. The outcome, however, is significantly better when an early diagnosis is made and treatment initiated promptly. We present a case of cardiac amyloidosis with left ventricular hypertrophy criteria on the electrocardiogram. After 9 months of follow-up, the patient developed low voltage in the limb leads, while still maintaining the Cornell criteria for left ventricular hypertrophy as well. The relative apical sparing by the disease process, as well as decreased cancellation of the opposing left ventricular walls could be responsible for this phenomenon. The discordance between the voltage in the frontal leads and precordial leads, when present in conjunction with other findings, may be helpful in raising the clinical suspicion of cardiac amyloidosis.



2019 ◽  
Vol 4 (1) ◽  
pp. 51 ◽  
Author(s):  
Ambarish Pandey ◽  
Neil Keshvani ◽  
Colby Ayers ◽  
Adolfo Correa ◽  
Mark H. Drazner ◽  
...  


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