A 60-year-old female comes to your office for eval - highsky

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A 60-year-old female comes to your office for evaluation of worsening breathlessness, ankle swelling and back pain. She has a 10 pack-year history of smoking and she drinks 2-3 glasses of wine each weekend. Her past medical history is insignificant. Her PR: 80/min; BP: 130/70 mmHg; Temperature: 37.1C(98.8F); RR: 14/min. Her examination is significant for jugular venous distension and bilateral ankle edema. There is an inspiratory increase in her jugular venous pulse. Chest auscultation shows basilar crepitations in both lungs. Heart sounds are distant and there is no murmur. Important laboratory findings are, serum calcium of 11.5 mg/dl, BUN of 40 and Cr of 2.0. Chest x-ray shows mild cardiomegaly. Echocardiography shows symmetrical thickening of the ventricular walls with a ˜speckled pattern™, normal ventricular dimensions and a slightly reduced systolic function. Based on these findings, which of the following is the most likely diagnosis in this patient?


A. Dilated cardiomyopathy secondary to alcoholism
B. Restrictive cardiomyopathy secondary to amyloidosis
C. Constrictive pericarditis
D. Dilated cardiomyopathy secondary to sarcoidosis
E. Dilated cardiomyopathy secondary to amyloidosis
F. Restrictive cardiomyopathy secondary to sarcoidosis

[ArchivalUser]

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[ArchivalUser]

BB."Speckled pattern" in Echo seen in Amyloidosis.

[ArchivalUser]

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This patient is most likely suffering from restrictive cardiomyopathy. In cases of restrictive cardiomyopathy, chest x-ray shows only mild or no enlargement of the cardiac silhouette. Echocardiography shows a symmetrically thickened ventricular wall, a normal or slightly reduced ventricular cavity size, and a normal or near normal systolic function. Kussmaul™s sign may also be present. Regarding the cause of restrictive cardiomyopathy in this patient, the appearance of ˜speckled pattern™ suggests the diagnosis of cardiac amyloidosis. This patient is most likely suffering from a combination of primary amyloidosis and myeloma suggested by the combination of bone pain, hypercalcemia, and renal failure.

Chest x-rays, in cases of dilated cardiomyopathy, show marked or moderate enlargement of cardiac silhouette. Echocardiography shows systolic dysfunction, left ventricular dilation, and normal thickening of the ventricle wall. Alcoholism is an important cause of dilated cardiomyopathy. However, this patient's alcohol intake is moderate so it is unlikely to cause cardiomyopathy. Echocardiographic findings are not compatible with dilated cardiomyopathy either.

With constrictive pericarditis, chest x-rays may show calcified pericardium and CT scan usually shows increased thickness of the pericardium. Kussmaul™s sign may be positive in both conditions. With constrictive pericarditis, thickness of myocardium will be normal.

Educational Objective:
Know how to diagnose restrictive cardiomyopathy in the setting of amyloidosis. Speckled pattern on echocardiogram is very specific for amyloidosis.

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