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Does my patient have amyloid cardiomyopathy?

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The diagnosis of amyloid cardiomyopathy is based on symptomatology, echocardiogram findings, and tissue biopsy.

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HPI: Dyspnea, fatigue, syncope.

PMH: AL (primary) amyloidosis.

FH: Familial amyloidosis.

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Peripheral edema, hepatomegaly, hypotension, elevated jugular venous pressure, periorbital purpura.

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Low voltage, atrial fibrillation.

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ECHO: Granular sparkling appearance of myocardium, thickened left ventricle, thickened and dilated right ventricle, mitral and aortic valve leaflet thickening.

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AC = Amyloid Cardiomyopathy.

SYMP = SYMPtoms of dyspnea, fatigue, and/or syncope.

ECHO = ECHOcardiogram findings of granular sparkling myocardium, thickened left and right ventricular, mitral and aortic valve leaflet thickening.

NCARD-BX = Non-CARDiac tissue and/or (abdominal fat pad, rectum, or kidney) Biopsy positive for amyloid deposits.

IN-NCARD-BX = INconclusive Non-CARDiac tissue Biopsy.

CARD-BX = EndomyoCARDial Biopsy positive for amyloid deposits.

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SYMP + ECHO + NCARD-BX = AC

SYMP + ECHO + IN-NCARD-BX + CARD-BX = AC

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Cardiac amyloidosis should be ruled out in any patient with unexplained heart failure and increased wall thickness on echocardiogram, especially if other clues are present, such as unexplained renal dysfunction or atrial fibrillation.

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  • – Endomyocardial biopsy is virtually 100% sensitive in amyloid cardiomyopathy as amyloid will be deposited all throughout the heart.

  • – In patients with known amyloid deposits in other organs and a history of hypertension, there may be uncertainty as to whether ventricular thickening represents amyloid infiltration or hypertensive heart disease. In such cases, a biopsy may be helpful to determine whether patient has AC.

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  1. Falk RH. Diagnosis and Management of the Cardiac Amyloidosis. Circulation. 2005;112:2047–2060.

How do I manage my patient with atypical angina (cardiac syndrome X)?

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The management of cardiac syndrome X is based upon pain relief with medication and risk factor reduction.

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HPI: Chest pain/discomfort precipitated by exercise or at rest lasting average of 10 minutes.

PMH: Hypertension, hyperlipidemia, diabetes.

SH: Alcohol, smoking.

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ECG: Normal findings; ST-segment depression.

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Coronary angiogram: Normal.

Cardiac MRI: Subendocardial perfusion defects.

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RF-RED = Risk Factor REDuction: smoking, hypertension, hyperlipidemia, cholesterol, obesity, physical inactivity.

ATEN = ATENolol 100 mg/day for 4 weeks.

AMLO = AMLOdipine 10 mg/day.

NITR = isosorbide monoNITRate 10 mg BID.

CSX = Cardiac Syndrome X.

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CSX = RF-RED +/– ATEN/AMLO/NITR

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Cardiac syndrome X is believed to be caused by microvascular disease, endothelial dysfunction, and enhanced pain perception. Risk factors are obesity, hypertension, dyslipidemia, glucose intolerance, and proinflammatory states. Syndrome x rarely causes myocardial infarction and it has a good prognosis.

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  • – 10–20% of patients with typical angina chest pain have normal coronary angiograms.

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  1. Fraker TD, et al. 2007 Chronic Angina Focused Update of the ACC/AHA 2002 Guidelines for the Management of Patients with Chronic Stable Angina. J Am Coll Cardiol. 2007;50:2264.

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