History

Systemic amyloidosis (AL) is a complex multisystem disorder, and symptoms depend on the organ involvement.^[30]Symptoms occur because of infiltrative cardiomyopathy and diastolic dysfunction. Approximately half of the patients with cardiac involvement present with right-sided heart failure. Patients may present with the following signs and symptoms^[20]:

Fatigue and weakness (most common symptoms)
Dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea
Leg edema
Dizziness
Presyncope/syncope^[31]
Postural hypotension
Easy bruising
Angina^{[13, 32]}
Pleural effusion, pulmonary edema (rare)
Sudden cardiac death
Pericardial effusion, cardiac tamponade (rare)^[33]
Heart block^[34]
Ischemic stroke (in 30% cases, cardioembolic in the remaining 70%)^[35]
Painful polyneuropathy (10-20% cases)
Carpal tunnel syndrome (20%)^[36]
Pulmonary amyloid infiltration, pulmonary hypertension, and cor pulmonale
Abnormal voice quality

Physical Examination

Physical examination findings in primary amyloidosis are consistent with features of right-sided heart failure and includes elevated jugular venous pressure, right-sided third heart sound, and lower extremity edema. A prominent fourth heart sound is present in patients without atrial fibrillation.

Periorbital purpura and macroglossia are specific for the presence of disease but have poor sensitivity (10-20%). Abnormal phonation resulting in a change in speech quality is seen in most cases and may be an early feature of systemic amyloidosis.^[37]Visceral involvement may result in the enlargement of liver and kidneys. Ascites and pleural effusion may be present. The patient may present with bilateral carpel tunnel syndrome or painful peripheral neuropathy.

Hypotension may be from low cardiac output or autonomic neuropathy. Murmurs of tricuspid or mitral regurgitation may be present in patients with valvular infiltration of amyloid.

Differential Diagnoses

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Media Gallery

Low voltage complexes, atrial fibrillation.
Long axis parasternal view: Left ventricular hypertrophy (LVH) with sparkling appearance, pericardial effusion.
Short axis view: Left ventricular hypertrophy (LVH), pericardial effusion, normal RV size.
Short axis left ventricle: Global subendocardial gadolinium enhancement.

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Cardiac Amyloidosis Clinical Presentation

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