An uncommon disorder in which proteins change conformation, aggregate, and form fibrils that infiltrate tissues, leading to organ dysfunction and increased risk of mortality. Deposit...
Four essential steps in diagnosing AL:
Considering the diagnosis based on presentation
Confirm diagnosis by tissue biopsy.
Determine the precursor protein.
Define the extent of involvemen...
Familial (ATTR): liver transplant to remove site of synthesis of the mutant protein
Hemodialysis and renal transplantation for renal amyloid
Hemodialysis-related amyloidosis: C...
Routine monitoring of renal function
Monitor adverse effects of medications.
AA: Maintenance of low SAA protein levels correlates to better outcom...
Gertz MA. Cardiac amyloidosis. Heart Fail Clin. 2022;18(3):479–488. doi: 10.1016/j.hfc.2022.02.005.
Khedraki R. Cardiac amyloidosis: an update on diag...
E85.9 Amyloidosis, unspecified
E85.3 Secondary systemic amyloidosis
E85.2 Heredofamilial amyloidosis, unspecified
E85.8 Other amyloidosis
E85.4 Organ-limited amyloidosis
E85.1 Neuropathic heredo...
The most commonly affected organs resulting in clinical symptoms are heart, kidneys, skin, peripheral and autonomic nerves, and liver.
Early detection is key to improved survival. Clini...
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Septal amyloidosis shows characteristic apple green birefringence of amyloid on Congo red stain.
<bold>FIG. 93.1.</bold> A: Electron micrograph of amyloid surrounding a cardiac myocyte (×20, 125). B: High-magnification image showing typical fi illar nature of amyloid deposit (×68,125).
<bold>FIG. 93.1.</bold> A: Electron micrograph of amyloid surrounding a cardiac myocyte (×20, 125). B: High-magnification imag...
<bold>FIG. 93.16.</bold> Two-dimensional echocardiogram for the same subject as depicted in Fig. 93.6. Classic features of amyloid cardiomyopathy are thickened intraventricular septum <italic>(IVS)</bold>, left ventricular posterior wall <italic>(PW)</bold>, and enlarged left atrium <italic>(LA)</bold>.
<bold>FIG. 93.16.</bold> Two-dimensional echocardiogram for the same subject as depicted in Fig. 93.6. Classic features of amy...
<bold>FIG. 93.17.</bold> Electrocardiogram of a patient with amyloid cardiomyopathy. Low voltage is a prominent feature, and the standard, but erroneous, interpretation as "septal infarct, age indeterminate" is common.
<bold>FIG. 93.17.</bold> Electrocardiogram of a patient with amyloid cardiomyopathy. Low voltage is a prominent feature, and t...
<bold>Figure 31.19</bold> Hemodynamics of amyloidosis, a restrictive cardiomyopathy. The elevated right atrial pressure, Kussmaul sign, and prominent Y descents <bold>(A)</bold> as well as the dip and plateau in the right ventricular tracing <bold>(B)</bold> are indicative of severe right ventricular dysfunction and not constriction. Prominent V waves <bold>(C)</bold> are present in the wedge tracing despite the lack of mitral regurgitation and reflect the volume overload in the stiff atrium, characteristic of amy...
<bold>Figure 31.19</bold> Hemodynamics of amyloidosis, a restrictive cardiomyopathy. The elevated right atrial pressure, Kussm...
<bold>Figure 31.20</bold> Electrocardiogram of a patient with amyloid. Classic findings include low limb lead voltage but preserved precordial voltage, nonspecific ST changes, P wave prominence, and pseudoinfarct pattern with precordial Q waves.
<bold>Figure 31.20</bold> Electrocardiogram of a patient with amyloid. Classic findings include low limb lead voltage but pres...
<bold>Figure 31.22</bold> Endomyocardial biopsy of patient with amyloid. A. Even without congo red staining, amyloid is suggested by separation of myofibrils and infiltration of blood vessels by pink amorphous proteinaceous material. Hypertrophy notably absent as well. <bold>B.</bold> At autopsy, left atrial walls appear leathery and waxy, consistent with endocardial deposition of amyloid protein.
<bold>Figure 31.22</bold> Endomyocardial biopsy of patient with amyloid. A. Even without congo red staining, amyloid is sugges...
Parasternal long (A) and apical four-chamber (B) and mitral inflow (C) in advanced cardiac amyloidosis. Note that the left ventricular size is normal with markedly thickened ventricular walls and has its characteristic granular sparkling appearance. DT, deceleration time; LA, left atrium; LV, left ventricle; PE, pericardial effusion; PL EFF, pleural effusion; pm, papillary muscle; RA, right atrium; RV, right ventricle.
Parasternal long (A) and apical four-chamber (B) and mitral inflow (C) in advanced cardiac amyloidosis. Note that the left ventricular siz...
<bold>FIGURE 11-44. Cardiac amyloidosis.</bold><bold>A.</bold> A section of myocardium stained with Congo red shows interstitial, pink-staining deposits of amyloid. <bold>B.</bold> Under polarized light, the same section displays the characteristic green birefringence of amyloid fibrils.
<bold>FIGURE 11-44. Cardiac amyloidosis.</bold><bold>A.</bold> A section of myocardium stained with Congo red show...