Restrictive cardiomyopathy resident survival guide: Difference between revisions
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! style="font-size: 80%; padding: 0 5px; background: #DCDCDC" align=left | [[{{PAGENAME}}#Treatment|Treatment]] | ! style="font-size: 80%; padding: 0 5px; background: #DCDCDC" align=left | [[{{PAGENAME}}#Treatment|Treatment]] | ||
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==Overview== | ==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 | 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 [[pulmonary edema]]).<ref name="pmid8995091">{{cite journal| author=Kushwaha SS, Fallon JT, Fuster V| title=Restrictive cardiomyopathy. | journal=N Engl J Med | year= 1997 | volume= 336 | issue= 4 | pages= 267-76 | pmid=8995091 | doi=10.1056/NEJM199701233360407 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8995091 }} </ref> | ||
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==Complete Diagnostic Approach== | ==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. | A complete diagnostic approach should be carried out after a focused initial rapid evaluation is conducted and following initiation of any urgent intervention.<ref name="pmid8995091">{{cite journal| author=Kushwaha SS, Fallon JT, Fuster V| title=Restrictive cardiomyopathy. | journal=N Engl J Med | year= 1997 | volume= 336 | issue= 4 | pages= 267-76 | pmid=8995091 | doi=10.1056/NEJM199701233360407 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8995091 }} </ref> | ||
{{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> | ||
❑ Weight loss/weight gain ([[cachexia]]/ volume overload?)<br> | ❑ Weight loss/weight gain ([[cachexia]]/ volume overload?)<br> | ||
❑ <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> | ||
:❑ Prior hospitalizations | :❑ Prior hospitalizations? | ||
:❑ Medication<br> | :❑ Medication?<br> | ||
❑ Diet (restriction of sodium and fluid intake?)</div>}} | ❑ Diet (restriction of sodium and fluid intake?)</div>}} | ||
{{familytree | | | | | | | | G01 |G01=<div style="text-align: left;"><b><u>Physical examination:</u></b><br> | {{familytree | | | | | | | | G01 |G01=<div style="text-align: left;"><b><u>Physical examination:</u></b><br> | ||
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:❑ [[JVD]] may show severity of hemodynamic impairment, most prominent wave is the y descent/ jugular venous pulse doesn't fall during inspiration (<b>Kussmaul's sign</b>)<br> | :❑ [[JVD]] may show severity of hemodynamic impairment, most prominent wave is the y descent/ jugular venous pulse doesn't fall during inspiration (<b>Kussmaul's sign</b>)<br> | ||
❑ Heart: <br> | ❑ Heart: <br> | ||
:❑ S¹ sound is usually normal, and S² sound is split normally,S3 may be present, rarely S4 | :❑ S¹ sound is usually normal, and S² sound is split normally, S3 may be present, rarely S4 | ||
:❑ Regurgitant murmurs common | :❑ Regurgitant murmurs common | ||
:❑ Carotid and peripheral pulses may show evidence of a low output<br> | :❑ Carotid and peripheral pulses may show evidence of a low output<br> | ||
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❑ Lungs:<br> | ❑ Lungs:<br> | ||
:❑ Rales? | :❑ Rales? | ||
:❑ Pleural effusion?<br> | :❑ [[Pleural effusion]]?<br> | ||
❑ Abdomen:<br> | ❑ Abdomen:<br> | ||
:❑ [[Hepatomegaly]], pulsatile liver and/or [[ascites]] (volume overload) <br> | :❑ [[Hepatomegaly]], pulsatile liver and/or [[ascites]] (volume overload) <br> | ||
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::❑ Low voltage (especially in amyloidosis) | ::❑ Low voltage (especially in amyloidosis) | ||
::❑ Non-specific ST- and T-wave abnormalities | ::❑ Non-specific ST- and T-wave abnormalities | ||
::❑ 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]]) | |||
::❑ Biatrial enlargement | |||
:: | ::❑ Normal or small left ventricular size with generally preserved systolic left ventricular function, and abnormal diastolic function | ||
❑ <b>Invasive imaging and tests:</b> | :❑ <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 | ||
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::❑ Right ventricular systolic pressure may be elevated | ::❑ Right ventricular systolic pressure may be elevated | ||
:❑ <u>Endomyocardial biopsy</u> | :❑ <u>Endomyocardial biopsy</u> | ||
::❑ For patients in whom the diagnosis is not clear by other methods of evaluation</div>}} | ::❑ For patients in whom the diagnosis is not clear by other methods of evaluation</div>}} | ||
{{familytree/end}} | {{familytree/end}} | ||
==Treatment== | ==Treatment== | ||
< | ====Symptomatic therapy==== | ||
*<b>Loop [[diuretics]]</b> (low to medium dose): to relieve venous congestion in the pulmonary and systemic circulation, may cause signs of [[hypotension]] and [[hypoperfusion]]. Monitor systemic perfusion with physical examination, blood urea nitrogen and serum creatinine concentration | |||
*<b>[[Calcium channel blockers]]</b>: may increase diastolic function | |||
*<b>[[Beta blockers]]</b>: may have benefit by improving ventricular relaxation | |||
< | *<b>[[Angiotensin-converting enzyme inhibitors]] and/or [[angiotensin II receptor blockers]]</b>: may improve diastolic filling | ||
*<b>[[Digoxin]]:</b> increases intracellular calcium and therefore should be used with caution | |||
* <b>Treat [[atrial fibrillation]]:</b> atrial fibrillation with the removal of the atrial contribution to ventricular filling may worsen existing diastolic dysfunction, patients with [[atrial fibrillation]] should be anticoagulated | |||
*<b>Advanced conduction-system disease</b> (i.e. advanced [[AV-block]]): Treat by the implantation of a pacemaker | |||
</ | *<b>Malignant [[ventricular arrhythmias]]</b>:May require treatment with an automatic implantable defibrillator or an antitachycardia device | ||
*<b>Cardiac transplantation</b> should be performed in eligible patients with intractable heart failure<ref name="pmid23079066">{{cite journal| author=DePasquale EC, Nasir K, Jacoby DL| title=Outcomes of adults with restrictive cardiomyopathy after heart transplantation. | journal=J Heart Lung Transplant | year= 2012 | volume= 31 | issue= 12 | pages= 1269-75 | pmid=23079066 | doi=10.1016/j.healun.2012.09.018 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23079066 }} </ref> | |||
== | |||
== | |||
==References== | ==References== | ||
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[[Category:Cardiology]] | [[Category:Cardiology]] | ||
[[Category:Emergency medicine]] | [[Category:Emergency medicine]] | ||
Latest revision as of 23:58, 29 July 2020
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Steven Bellm, M.D. [2]
Restrictive cardiomyopathy resident survival guide Microchapters |
---|
Overview |
Classification |
Causes |
Diagnosis |
Treatment |
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 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.[1]
History and symptoms: ❑ Hints for etiology (history of pericarditis/ tuberculosis/ trauma DD:contrictive pericarditis or amyloidosis/ sarcoidosis)
| |||||||||||||||||||
Physical examination: ❑ Vital signs:
❑ General appearance:
❑ Heart:
❑ Lungs:
❑ Abdomen:
❑ Extremities:
| |||||||||||||||||||
Laboratory findings: ❑ Complete blood count
| |||||||||||||||||||
Imaging and additional tests: ❑ Noninvasive imaging and tests:
Invasive imaging and tests:
| |||||||||||||||||||
Treatment
Symptomatic therapy
- Loop diuretics (low to medium dose): to relieve venous congestion in the pulmonary and systemic circulation, may cause signs of hypotension and hypoperfusion. Monitor systemic perfusion with physical examination, blood urea nitrogen and serum creatinine concentration
- Calcium channel blockers: may increase diastolic function
- Beta blockers: may have benefit by improving ventricular relaxation
- Angiotensin-converting enzyme inhibitors and/or angiotensin II receptor blockers: may improve diastolic filling
- Digoxin: increases intracellular calcium and therefore should be used with caution
- Treat atrial fibrillation: atrial fibrillation with the removal of the atrial contribution to ventricular filling may worsen existing diastolic dysfunction, patients with atrial fibrillation should be anticoagulated
- Advanced conduction-system disease (i.e. advanced AV-block): Treat by the implantation of a pacemaker
- Malignant ventricular arrhythmias:May require treatment with an automatic implantable defibrillator or an antitachycardia device
- Cardiac transplantation should be performed in eligible patients with intractable heart failure[2]
References
- ↑ 1.0 1.1 Kushwaha SS, Fallon JT, Fuster V (1997). "Restrictive cardiomyopathy". N Engl J Med. 336 (4): 267–76. doi:10.1056/NEJM199701233360407. PMID 8995091.
- ↑ DePasquale EC, Nasir K, Jacoby DL (2012). "Outcomes of adults with restrictive cardiomyopathy after heart transplantation". J Heart Lung Transplant. 31 (12): 1269–75. doi:10.1016/j.healun.2012.09.018. PMID 23079066.