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]]) | ||
*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 etiology DDX=== | |||
**Idiopathic | **Idiopathic | ||
**Familial | **Familial non=infiltrative disease | ||
**Infiltrative disease (amyloidosis, sarcoidosis, Gaucher, Hurler) | **Infiltrative disease ([[amyloidosis]], [[sarcoidosis]], Gaucher, Hurler) | ||
**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) | ||
**Metastatic disease, carcinoid syndrome | **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 | |||
==See Also== | ==See Also== | ||
Line 29: | Line 57: | ||
==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
- 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
Cardiomyopathy
- Dilated cardiomyopathy
- Hypertrophic cardiomyopathy
- Restrictive cardiomyopathy
- Peripartum cardiomyopathy
- Takotsubo cardiomyopathy
- Arrhythmogenic right ventricular dysplasia
Restrictive cardiomyopathy etiology DDX
- 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, 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
- Diuretics for edema or pulmonary vascular congestion