Restrictive cardiomyopathy: Difference between revisions

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==Background==
==Background==
*Muscle is stiff from fibrosis or infiltrating process
*Muscle is stiff from fibrosis or infiltrating process (e.g. [[amyloidosis]], [[hemochromatosis]])
*EF is usually normal
*Form of diastolic dysfunction (difficulty filling); ventricular endocardial thickening or myocardial infiltration-->high filling pressures, impaired diastolic filling-->[[mitral regurgitation|mitral]] and/or tricuspid regurgitation--> venous [[pulmonary hypertension]]
*Form of diastolic dysfunction (difficulty filling)
**Ventricular EF typically normal, though systolic function may deteriorate in absence of compensatory hypertrophy
*If nodal/conduction tissues affected by infiltrative/fibrotic process, may cause SA or [[AV block]]
*Must distinguish from constrictive [[pericarditis]]
*Must distinguish from constrictive [[pericarditis]]
==Clinical features==
*Exertional [[dyspnea]], orthopnea, PND
*Peripheral edema
*[[Fatigue]], +/- [[syncope]], [[angina]] from poor cardiac output
*+/- [[Arrhythmias]], [[AV block]]
*Quiet precordium
*Crackles
*JVD
*+/- [[murmur]] from mitral/tricuspid regurgitation


==Differential Diagnosis==
==Differential Diagnosis==
{{Cardiomyopathy DDX}}
{{Cardiomyopathy DDX}}
*Restrictive cardiomyopathy ddx
 
===Restrictive cardiomyopathy etiology DDX===
**Idiopathic
**Idiopathic
**Familial noninfiltrative disease
**Familial non=infiltrative disease
**Infiltrative disease (amyloidosis, sarcoidosis, Gaucher, Hurler)
**Infiltrative disease ([[amyloidosis]], [[sarcoidosis]], Gaucher, Hurler)
**Storage diseases (hemachromatosis, Fabry, glycogen/lysosomal storage diseases)
**Storage diseases ([[hemochromatosis]], Fabry, glycogen/lysosomal storage diseases)
**Diabetes
**[[Diabetes]]
**Scleroderma
**[[Scleroderma]]
**Endomyocardial fibrosis (hypereosinophilic syndrome, medication toxicity)
**Endomyocardial fibrosis (hypereosinophilic syndrome, medication toxicity)
**Radiation, chemotherapy (doxorubicin)
**Radiation, chemotherapy (doxorubicin)
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==Evaluation==
==Evaluation==
*Consider if CHF but no evidence of cardiomegaly or systolic dysfunction
*Consider if [[CHF]] but no evidence of cardiomegaly or systolic dysfunction
*[[ECG]]
**Typically nonspecific ST/TW abnormalities
**Other features may include [[low voltage ECG|low voltage]], [[pathologic Q waves]], [[LVH]], [[AV block]]
showing ST-segment and T-wave abnormalities
*[[CXR]]- heart typically normally sized, though can be enlarged in advanced stages of some underlying disease processes
*[[Echocardiography]]
**Normal LVEF
**Elevated LV filling pressures, impaired longitudinal contraction
**+/- dilated atria, myocardial hypertrophy
*Definitive diagnosis may require MRI, catheterization, biopsy


==Management==
==Management==
*Symptom directed (diuretics and ACEI)
*Symptom-directed
**[[Diuretics]] for edema or pulmonary vascular congestion
***Caution as cardiac output preload dependant
**Caution with afterload reduction as may cause profound [[hypotension]]
**Treat [[arrhythmias]]
***Avoid [[digoxin]] in amyloidosis as sensitivity to dig-induced arrhythmias common
 


==See Also==
==See Also==
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==References==
==References==
<references/>
<references/>
 
https://www.merckmanuals.com/professional/cardiovascular-disorders/cardiomyopathies/restrictive-cardiomyopathy


[[Category:Cardiology]]
[[Category:Cardiology]]

Revision as of 16:02, 26 September 2019

Background

  • Muscle is stiff from fibrosis or infiltrating process (e.g. amyloidosis, hemochromatosis)
  • Form of diastolic dysfunction (difficulty filling); ventricular endocardial thickening or myocardial infiltration-->high filling pressures, impaired diastolic filling-->mitral and/or tricuspid regurgitation--> venous pulmonary hypertension
    • Ventricular EF typically normal, though systolic function may deteriorate in absence of compensatory hypertrophy
  • If nodal/conduction tissues affected by infiltrative/fibrotic process, may cause SA or AV block
  • Must distinguish from constrictive pericarditis

Clinical features

Differential Diagnosis

Cardiomyopathy

Restrictive cardiomyopathy etiology DDX

Evaluation

showing ST-segment and T-wave abnormalities

  • CXR- heart typically normally sized, though can be enlarged in advanced stages of some underlying disease processes
  • Echocardiography
    • Normal LVEF
    • Elevated LV filling pressures, impaired longitudinal contraction
    • +/- dilated atria, myocardial hypertrophy
  • Definitive diagnosis may require MRI, catheterization, biopsy

Management

  • Symptom-directed
    • Diuretics for edema or pulmonary vascular congestion
      • Caution as cardiac output preload dependant
    • Caution with afterload reduction as may cause profound hypotension
    • Treat arrhythmias
      • Avoid digoxin in amyloidosis as sensitivity to dig-induced arrhythmias common


See Also

References

https://www.merckmanuals.com/professional/cardiovascular-disorders/cardiomyopathies/restrictive-cardiomyopathy