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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Questions (5)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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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? Review Topic | Tested Concept

QID: 107034
FIGURES:
1

Exercise stress test

21%

(7/34)

2

Dobutamine stress test

3%

(1/34)

3

Nuclear stress test

9%

(3/34)

4

Cardiac biopsy

59%

(20/34)

5

Pulmonary function tests

3%

(1/34)

L 2 D

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