Restrictive cardiomyopathy resident survival guide: Difference between revisions
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{{familytree/start |summary=Sample 1}} | {{familytree/start |summary=Sample 1}} | ||
{{familytree | | | | | | | | A01 |A01=<div style="text-align: left;"><b><u>History and symptoms:</u></b><br> | {{familytree | | | | | | | | A01 |A01=<div style="text-align: left;"><b><u>History and symptoms:</u></b><br> | ||
❑ Hints for etiology (history of pericarditis/ tuberculosis/ trauma DD:contrictive pericarditis)<br> | ❑ Hints for etiology (history of pericarditis/ tuberculosis/ trauma DD:contrictive pericarditis or amyloidosis/ sarcoidosis))<br> | ||
❑ Duration and onset of illness/ symptoms<br> | ❑ Duration and onset of illness/ symptoms<br> | ||
❑ Severity and triggers of <b>[[dyspnea]]/ [[orthopnea]]</b> and <b> [[fatigue]]/ weakness</b>, presence of <b>chest pain</b>, exercise capacity, physical activity, sexual activity (NYHA?)<br> | ❑ Severity and triggers of <b>[[dyspnea]]/ [[orthopnea]]</b> and <b> [[fatigue]]/ weakness</b>, presence of <b>chest pain</b>, exercise capacity, physical activity, sexual activity (NYHA?)<br> | ||
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❑ <b>Palpitations/ (pre)[[syncope]]</b>/ [[ventricular tachycardia]]s/ [[cardiac arrest]] or [[fibrillation]]<br> | ❑ <b>Palpitations/ (pre)[[syncope]]</b>/ [[ventricular tachycardia]]s/ [[cardiac arrest]] or [[fibrillation]]<br> | ||
❑ Symptoms of [[transient ischemic attack]] or [[thromboembolism]] (anticoagulation necessary?)<br> | ❑ Symptoms of [[transient ischemic attack]] or [[thromboembolism]] (anticoagulation necessary?)<br> | ||
❑ Presence of peripheral [[edema]] or [[ | ❑ Presence of peripheral [[edema]], [[ascites]] or [[anasarca]] (volume overload?)<br> | ||
❑ Problems with breathing at night/ sleep<br> | ❑ Problems with breathing at night/ sleep<br> | ||
❑ Medical history<br> | ❑ Medical history<br> | ||
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::❑ Eventually Depolarization abnormalities: Such as bundle-branch or ventricular hypertrophy, or abnormalities of conduction, including atrioventricular block, atrial fibrillation, pseudoinfarction, | ::❑ Eventually Depolarization abnormalities: Such as bundle-branch or ventricular hypertrophy, or abnormalities of conduction, including atrioventricular block, atrial fibrillation, pseudoinfarction, | ||
::❑ Left-axis deviation | ::❑ Left-axis deviation | ||
:❑ <u>Chest x-ray:</u> Cardiac size | :❑ <u>Chest x-ray:</u> Cardiac size (normal or cardiomegaly), atrial enlargement, pulmonary congestion, interstitial edema with Kerley B lines, pleural effusions? | ||
:❑ <u>2D echocardiography with Doppler:</u> | :❑ <u>2D echocardiography with Doppler:</u> | ||
::❑ Pattern of mitral-inflow velocity: increased early diastolic filling velocity (>1.0 m per second)/ decreased atrial filling velocity (<0.5 m per second)/ increased ratio of early diastolic filling to atrial filling (>2)/ decreased deceleration time (<150 msec)/ decreased isovolumic relaxation time (<70 msec) | ::❑ Pattern of mitral-inflow velocity: increased early diastolic filling velocity (>1.0 m per second)/ decreased atrial filling velocity (<0.5 m per second)/ increased ratio of early diastolic filling to atrial filling (>2)/ decreased deceleration time (<150 msec)/ decreased isovolumic relaxation time (<70 msec) | ||
::❑ Pulmonary-vein or hepatic-vein flow: systolic forward flow is less than diastolic forward flow/ increased reversal of diastolic flow after atrial contraction with inspiration in the hepatic and pulmonary veins | ::❑ Pulmonary-vein or hepatic-vein flow: systolic forward flow is less than diastolic forward flow/ increased reversal of diastolic flow after atrial contraction with inspiration in the hepatic and pulmonary veins | ||
::❑ shortened deceleration time across the mitral and tricuspid valves, mitral and tricuspid regurgitation common | ::❑ shortened deceleration time across the mitral and tricuspid valves, mitral and tricuspid regurgitation common | ||
::Increased wall thickness, thickened cardiac valves, granular sparkling texture (in amyloidosis) | ::❑ Increased wall thickness, thickened cardiac valves, granular sparkling texture (in amyloidosis)::❑ | ||
❑ <b>Invasive imaging and tests:</b> | ❑ Biatrial enlargement, a normal or small left ventricular size with generally preserved systolic left ventricular function, and abnormal diastolic function | ||
:❑ <u>Cardiac magnetic resonance:</u> | |||
::❑ Increasingly information in the identification or exclusion of various causes, Late gadolinium enhancement (LGE) for identification of myocardial fibrosis and characteristic patterns of certain diseases | |||
<b>Invasive imaging and tests:</b> | |||
:❑ <u>Cardiac catheterization</u> | :❑ <u>Cardiac catheterization</u> | ||
::❑ Deep and rapid early decline in ventricular pressure at the onset of diastole and rapid rise to a plateau in the diastole (<b>dip and plateau/ square-root sign</b>), LVEDP often >5 mm Hg greater than RVEDP | ::❑ Deep and rapid early decline in ventricular pressure at the onset of diastole and rapid rise to a plateau in the diastole (<b>dip and plateau/ square-root sign</b>), LVEDP often >5 mm Hg greater than RVEDP |
Revision as of 13:32, 10 March 2015
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Steven Bellm, M.D. [2]
Restrictive cardiomyopathy resident survival guide Microchapters |
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Overview |
Classification |
Causes |
Diagnosis |
Treatment |
Do's |
Dont's |
Overview
Restrictive cardiomyopathy is defined as heart-muscle disease with impaired ventricular filling usually due to increased stiffness. The diastolic volume of either or both ventricles is normal or decreased,the systolic function usually remains normal and wall thickness may be normal or increased. The symptoms and signs may consist of right (jugular venous pressure, peripheral edema, and ascites) or left ventricular failure (breathlessness and evidence of pulmonary edema).[1]
Classification
Restrictive cardiomyopathy | |||||||||||||||||||||||||||||||||||||
Myocardial | Endomyocardial | ||||||||||||||||||||||||||||||||||||
Nininfiltrative | Infiltrative | Storage Disease | |||||||||||||||||||||||||||||||||||
Causes
Common Causes
- Idiopathic cardiomyopathy
- Amyloisosis
- Sarcoidosis
- Endomyocardial fibrosis
- Radiation
- Toxic effects of anthracycline
Complete Diagnostic Approach
A complete diagnostic approach should be carried out after a focused initial rapid evaluation is conducted and following initiation of any urgent intervention.
History and symptoms: ❑ Hints for etiology (history of pericarditis/ tuberculosis/ trauma DD:contrictive pericarditis or amyloidosis/ sarcoidosis))
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Physical examination: ❑ Vital signs:
❑ General appearance:
❑ Heart:
❑ Lungs:
❑ Abdomen:
❑ Extremities:
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Laboratory findings: ❑ Complete blood count
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Imaging and additional tests: ❑ Noninvasive imaging and tests:
❑ Biatrial enlargement, a normal or small left ventricular size with generally preserved systolic left ventricular function, and abnormal diastolic function
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Treatment
Symptomatic therapy
- Diuretics: for venous congestion in the pulmonary and systemic circulation, may cause signs of hypotension and hypoperfusion
Do's
Dont's
References
- ↑ Kushwaha SS, Fallon JT, Fuster V (1997). "Restrictive cardiomyopathy". N Engl J Med. 336 (4): 267–76. doi:10.1056/NEJM199701233360407. PMID 8995091.