Difference between revisions of "Restrictive cardiomyopathy"

(Background)
 
(10 intermediate revisions by 6 users not shown)
Line 1: Line 1:
 
==Background==
 
==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 regurgitation|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]]
 
*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}}
  
==Diagnosis==
+
===Restrictive cardiomyopathy etiology DDX===
*Consider if CHF but no e/o cardiomegaly or systolic dysfunction
+
**Idiopathic
 +
**Familial non=infiltrative disease
 +
**Infiltrative disease ([[amyloidosis]], [[sarcoidosis]], Gaucher, Hurler)
 +
**Storage diseases ([[hemochromatosis]], Fabry, glycogen/lysosomal storage diseases)
 +
**[[Diabetes]]
 +
**[[Scleroderma]]
 +
**Endomyocardial fibrosis (hypereosinophilic syndrome, medication toxicity)
 +
**Radiation, chemotherapy (doxorubicin)
 +
**Metastatic disease, [[carcinoid syndrome]]
 +
 
 +
==Evaluation==
 +
*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==
 +
*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
  
==Treatment==
 
*Symptom directed (diuretics and ACEI)
 
  
 
==See Also==
 
==See Also==
 
*[[Cardiomyopathy (Main)]]
 
*[[Cardiomyopathy (Main)]]
  
==Source==
+
==References==
Tintinalli
+
<references/>
 +
https://www.merckmanuals.com/professional/cardiovascular-disorders/cardiomyopathies/restrictive-cardiomyopathy
  
[[Category:Cards]]
+
[[Category:Cardiology]]

Latest 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