Restrictive cardiomyopathy resident survival guide: Difference between revisions
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❑ 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]], [[ascites]] or [[anasarca]] (volume overload?)<br> | ❑ 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> |
Revision as of 12:24, 11 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 |
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)
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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:
Invasive imaging and tests:
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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.