What is the Median survival time in cardiac amyloidosis?
Correct Answer: 6-12months
Description: ANSWER: (C) 6-12 monthsREF: Harrison's 18th ed ch: 238Once heart failure develops, the median survival is 6-12 months in primary amyloidosisAmyloidosis is the major cause of restrictive cardiomyopathy, most often due to "primary amyloidosis". Amyloidosis secondary to other chronic diseases rarely involves the heart. Only 5% of patients of primary amyloidosis survive beyond 10 yearsPresentation: Amyloid fibrils infiltrate the myocardium, especially around the conduction system and coronary vessels. Typical clinical features are conduction block, autonomic neuropathy, renal involvement, and occasionally thickened skin lesions. Cardiac amyloid is suspected from thickened ventricular walls in conjunction with an electrocardiogram that shows low voltage. A characteristic refractile brightness in the septum on echocardiography is suggestive, but neither sensitive nor specific. Both atria are dilated, often dramatically so.Diagnosis: The diagnosis of primary or familial can be made from biopsies of an abdominal fat pad or the rectum, but cardiac amyloidosis is most reliably identified from the myocardium Therapy: Therapy is largely symptomaticDiuretics are used as needed to treat fluid retention, which often requires high doses.Digoxin bound to the amyloid fibrils can reach toxic levels, and should therefore be used only in very low doses, if at all. There is no evidence regarding use of neurohormonal antagonists in amyloid heart disease, where the possible theoretical benefit has to be balanced against their potential side effects in light of frequent autonomic neuropathy and dependence on heart rate reserve.The risk of intracardiac thrombi may warrant chronic anticoagulation. Once heart failure develops, the median survival is 6-12 months in primary amyloidosis. Multiple myeloma is treated with chemotherapy {prednisone, melphalan, bortezomib), the extent of which is usually limited by the potential of worsening cardiac dysfunction.Colchicine can be of some benefit in inflammation-associated (AA) amyloid. Transthyretin-associated cardiac amyloid requires heart and liver transplantation, while senile cardiac amyloid is treated with conventional heart failure regimens.Immunoglobulin associated amyloid has occasionally been treated with sequential heart transplantation and delayed bone marrow transplant, with frequent recurrence of amyloid in the transplanted heart.Poor prognostic factors in cardiac amyloidosis:Congestive heart failureSyncopeComplex arrhythmiaDegree of left ventricular hypertrophy (More=worse prognosis)Low left ventricular ejection fraction (LVEF)Restrictive hemodynamic.Right ventricular dilatationPulmonary hypertensionLow voltage on ECGHigh brain natriuretic peptide (BNP)High troponinT1 kinetics on gadolinium-enhanced MRI (to demonstrate extent of myocardial involvement)
Category:
Medicine
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now