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Restrictive cardiomyopathy

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Cardiovascular system

Pathology

Vascular disorders
Congenital heart defects
Cardiac arrhythmias
Valvular disorders
Cardiomyopathies
Heart failure
Cardiac infections
Pericardial disorders
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Cardiovascular system pathology review

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Restrictive cardiomyopathy

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High Yield Notes
8 pages
Flashcards

Restrictive cardiomyopathy

6 flashcards
Questions

USMLE® Step 1 style questions USMLE

1 questions
Preview

A 68-year-old woman comes to the clinic complaining of dyspnea on exertion, fatigue, and chest pain. She also states that she has to use 3 pillows at night when she sleeps. Past medical history includes carcinoma of the breast, for which she received surgery and adjuvant radiation therapy but no chemotherapy. Temperature is 37.2°C (98.9°F), pulse is 80/min, respirations are 20/min, and blood pressure is 100/68. Physical exam shows jugular venous distension and bilateral leg edema. Cardiac auscultation shows S3 and S4 heart sounds. Electrocardiogram (ECG) shows low amplitude signals. Which of the following is the most likely cause of this patient’s disease?  


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Transcript

Content Reviewers:

Rishi Desai, MD, MPH

Contributors:

Tanner Marshall, MS

Cardiomyopathy translates to “heart muscle disease.” Cardiomyopathy is a broad term used to describe a variety of issues that result from disease of the myocardium, or heart muscle.

When cardiomyopathy develops as a way to compensate for some other underlying disease, such as hypertension or valve diseases, it’s called secondary cardiomyopathy.

When it develops all by itself, it’s called primary cardiomyopathy.

Restrictive cardiomyopathy describes when the heart muscle is restricted, meaning that it becomes stiffer and less compliant. However, the muscles and size of the ventricles stay about the same size, or only get slightly enlarged.

Normally, when blood fills the ventricles, they’re compliant; this means that they stretch out and allow more blood to fill in. When blood fills restricted ventricles, however, they can’t expand as much as they’re supposed to. Because stiffer, less compliant ventricles prevent the ventricles from stretching, less blood fills into the ventricle, which means the heart starts to fail to pump out enough blood to the body. Thus, restrictive cardiomyopathy causes heart failure; since filling happens during diastole, we call this a type of diastolic heart failure.

Now, several mechanisms can lead to stiffer heart muscles and restrictive cardiomyopathies. One of mechanisms is amyloidosis. Amyloids are proteins that have been misfolded; once misfolded, they become insoluble, and can deposit in various tissues and organs, making these tissues less compliant.

Summary
Restrictive cardiomyopathy is a form of cardiac disease in which the ventricles are too stiff to contract adequately. Major causes include sarcoidosis, amyloidosis, postradiation fibrosis, and endocardial fibroelastosis. Diastolic dysfunction ensues with decreased filling of the heart. The ECG might show low voltage complexes despite a thick myocardium. Loffler syndrome is a related disorder that presents with endomyocardial fibrosis with a prominent eosinophilic infiltrate and hemochromatosis with restrictive or dilated cardiomyopathy
Sources
  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "Restrictive Cardiomyopathy" Circulation Research (2017)
  5. "Restrictive Cardiomyopathy" Pacing and Clinical Electrophysiology (2009)
  6. "Idiopathic Restrictive Cardiomyopathy in Children and Young Adults" The American Journal of Cardiology (2018)