II. Incidence

  1. Least common form of Primary Cardiomyopathy (2-5% of cases)

III. Definition

  1. Heart Muscle dysfunction secondary to stiff ventricles that interfere with contraction

IV. Pathophysiology

  1. Normal systolic function
  2. Decreased ventricular filling progresses to right-sided Heart Failure

V. Causes

  1. See Cardiomyopathy
  2. Infiltrative Cardiomyopathy
    1. Sarcoidosis
    2. Hemochromatosis
    3. Amyloidosis
    4. Scleroderma
    5. Radiation Therapy
  3. Structural and component abnormalities
    1. Desmin abnormality
    2. Troponin abnormality

VI. Signs

VII. Imaging

  1. Chest XRay
    1. Pulmonary vascular congestion

VIII. Diagnostics

  1. Electrocardiogram
    1. Decreased voltage
    2. Prolonged PR Interval (first degree AV Block)
  2. Echocardiogram
    1. Biatrial enlargement
    2. Diastolic Dysfunction
    3. Normal or reduced ventricular volume with impaired ventricular filling
    4. Normal left ventricular wall thickness and normal systolic function

IX. Management

  1. General Measures
    1. See Diastolic Dysfunction
    2. Salt Restriction
  2. Specific management based on underlying condition
    1. Chelation therapy or phlebotomy for infiltrative processes
  3. Devices
    1. Pacemaker (for higher grade AV Block)
    2. Implantable Defibrillator
  4. Cardiac Transplantation for children with recently diagnosed Restrictive Cardiomyopathy
    1. Fenton (2006) Heart 92(1): 85-9 [PubMed]

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Ontology: Restrictive cardiomyopathy (C0007196)

Definition (NCI) A type of heart disorder referring to the inability of the ventricles to fill with blood because the myocardium (heart muscle) stiffens and looses its flexibility. Causes include replacement of the myocardium with scar tissue, abnormal cellular infiltration of the myocardium, or deposition of a substance (e.g., amyloid) in the myocardium.
Definition (NCI_CTCAE) A disorder characterized by an inability of the ventricles to fill with blood because the myocardium (heart muscle) stiffens and loses its flexibility.
Definition (MSH) A form of CARDIAC MUSCLE disease in which the ventricular walls are excessively rigid, impeding ventricular filling. It is marked by reduced diastolic volume of either or both ventricles but normal or nearly normal systolic function. It may be idiopathic or associated with other diseases (ENDOMYOCARDIAL FIBROSIS or AMYLOIDOSIS) causing interstitial fibrosis.
Concepts Disease or Syndrome (T047)
MSH D002313
ICD10 I42.5
SnomedCT 389996009, 90828009, 415295002
English Cardiomyopathies, Restrictive, Cardiomyopathy, Restrictive, Restrictive Cardiomyopathies, RCM - Restrictive cardmyopathy, Restrictive Cardiomyopathy, Restrictive cardiomyopathy, cardiomyopathy restrictive, restrictive cardiomyopathy (diagnosis), restrictive cardiomyopathy, constrictive cardiomyopathy, constrictive cardiomyopathy (diagnosis), Cardiomyopathy, restrictive, Constrictive cardiomyopathy NOS, Cardiomyopathy, Restrictive [Disease/Finding], Cardiomyopathy;restrictive, Familial Restrictive Cardiomyopathy, Constrictive cardiomyopathy, Primary restrictive cardiomyopathy, RCM - Restrictive cardiomyopathy, Primary restrictive cardiomyopathy (disorder), cardiomyopathy; restrictive, restrictive; cardiomyopathy, Restrictive cardiomyopathy (disorder), Cardiomyopathy, constrictive
Italian Cardiomiopatia restrittiva, Miocardiopatia restrittiva
Portuguese Cardiomiopatia Restritiva, Miocardiopatia Restritiva, Miocardiopatia restritiva
Spanish Cardiomiopatía Restrictiva, Miocardiopatía Restrictiva, miocardiopatía constrictiva, miocardiopatía restrictiva primaria (trastorno), miocardiopatía restrictiva primaria, Cardiomiopatía restrictiva, miocardiopatía restrictiva, cardiomiopatía restrictiva, miocardiopatía restrictiva (trastorno)
Japanese 拘束性心筋症, コウソクセイシンキンショウ
Swedish Kardiomyopati, restriktiv
Finnish Restriktiivinen kardiomyopatia
Czech Restriktivní kardiomyopatie, restriktivní kardiomyopatie, kardiomyopatie restriktivní
Polish Kardiomiopatia restrykcyjna
Hungarian Restrictiv cardiomyopathia
Norwegian Restriktiv kardiomyopati
Dutch cardiomyopathie; restrictief, restrictief; cardiomyopathie, restrictieve cardiomyopathie, Cardiomyopathie, restrictieve, Restrictieve cardiomyopathie
German Kardiomyopathie restriktiv, Kardiomyopathie, obliterative, Kardiomyopathie, restriktive
French Cardiomyopathie restrictive, Myocardiopathie restrictive