Updated: 6/15/2020

Restrictive / Obliterative Cardiomyopathy

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Snapshot 
  • A 40-year-old woman presents to her physician’s office for dyspnea on exertion and exercise intolerance. She states that her symptoms seem to be worsening over time. Her medical history includes breast cancer. A year prior to presentation, she had completed multiple rounds of chemotherapy and radiation therapy for her breast cancer. Echocardiogram shows diastolic dysfunction with preserved systolic function.
Introduction
  • Clinical definition
    • a cardiomyopathy characterized by a stiff myocardium and diastolic dysfunction
  • Epidemiology
    • incidence
      • least common type of cardiomyopathy
    • demographics
      • can occur in both children and adults
  • Etiology
    • post-radiation fibrosis
    • Loffler endocarditis
      • endomyocardial fibrosis with a prominent eosinophilic infiltrate
      • can be associated with large mural thrombi and peripheral hypereosinophilia
    • endocardial fibroelastosis in children and young adults
    • amyloidosis
    • sarcoidosis
    • scleroderma
    • neoplasia
    • hemochromatosis
      • more commonly causes dilated cardiomyopathy but can also cause restrictive cardiomyopathy
    • radiation therapy 
      • can result in pericarditis, myocarditis, coronary artery narrowing, and sclerotic valvular changes 
  • Pathogenesis
    • rigid noncompliant myocardium (e.g., from amyloid deposition, sarcoidosis, or radiation) impedes ventricular filling and causes diastolic dysfunction
    • typically preserved left ventricular systolic function
    • mimics constrictive pericarditis
  • Prognosis
    • poor prognosis without treatment
    • progressive over time
Presentation
  • Symptoms 
    • progressive heart failure
      • dyspnea on exertion
      • exercise intolerance
      • fatigue
    • sudden cardiac death
  • Physical exam
    • edema
    • hepatomegaly
    • ascites
    • pulmonary rales
    • S3 gallop
    • jugular venous distension
    • Kussmaul sign
      • increase in jugular venous pressure during inhalation
Imaging
  • Radiography
    • indication
      • for all patients
    • recommend views
      • chest
    • findings
      • cardiomegaly
      • pulmonary congestion
  • Echocardiogram
    • indication
      • for all patients as diagnostic test
    • findings
      • thickening of all structures
      • diastolic dysfunction
      • preserved systolic function
Studies
  • Electrocardiogram
    • findings
      • may have low-voltages
  • Endomyocardial biopsy
    • indication
      • the most accurate diagnostic test of etiology
      • tissue biopsy needed to diagnose amyloidosis (apple-green birefringence on Congo Red stain) or Loffler syndrome 
    • findings
      • eosinophilic infiltrate in Loffler syndrome
      • amyloid deposits in cardiac amyloidosis
  • Making the diagnosis
    • based on clinical presentation, echocardiogram, and biopsy
Differential
  • Constrictive pericarditis
    • distinguishing factors
      • patients with a history of acute pericarditis or cardiac surgery
      • pericardial knock on exam
      • chest radiography shows calcification
      • computed tomography shows thickened pericardium
  • Dilated cardiomyopathy
    • distinguishing factors
      • echocardiogram with reduced ejection fraction
      • progressive heart failure
Treatment
  • Management approach
    • treatments are aimed at managing symptoms and treating underlying etiology
  • Medical
    • diuretics
      • indication
        • symptomatic management of congestive heart failure
  • Non-operative
    • phlebotomy
      • indication
        • for patients with hemochromatosis
  • Operative
    • heart transplant
      • indication
        • the only definitive treatment
Complications
  • Heart failure
  • Arrhythmias
 

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(M2.CV.15.4041) A 57-year-old man presents to his primary care physician with swollen legs. He first noticed the swelling several months ago, but only decided to come in when he began having chest pain while climbing 2 flights of stairs. He also confirms shortness of breath when lying flat at night, and several months of tingling in his fingers. He denies a prior history of chest pain or hypertension. His vital signs are: BP 125/75, HR 97, T 98.6 F, and RR 14. An EKG is shown in Figure A, and an echocardiogram shows concentric thickening of the ventricular walls (Figure B). Which of the following is most likely to reveal the cause of this patient's symptoms? Tested Concept

QID: 107034
FIGURES:
1

Exercise stress test

16%

(5/32)

2

Dobutamine stress test

3%

(1/32)

3

Nuclear stress test

9%

(3/32)

4

Cardiac biopsy

62%

(20/32)

5

Pulmonary function tests

3%

(1/32)

M 6 D

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