Hypertrophic cardiomyopathy resident survival guide: Difference between revisions
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==Overview== | ==Overview== | ||
[[Hypertrophic Cardiomyopathy]] is defined by LV hypertrophy associated with nondilated ventricular chambers without any other cardiac or systemic disease that itself would be capable of producing the magnitude of hypertrophy evident. HCM patients can also develop a LV outflow obstruction, diastolic dysfunction, [[myocardial ischemia]] and [[mitral regurgitation]]. [[HCM]] is usually recognized by maximal LV wall thickness ≥15 mm. Wall thickness of 13 to 14 mm is considered borderline if there are other compelling information (eg, family history of HCM), based on echocardiography. The diagnostic imaging mainly focused on echocardiography, however cardiovascular magnetic resonance (CMR) is used with increasing frequency.<ref name="pmid22093723">{{cite journal| author=American College of Cardiology Foundation/American Heart Association Task Force on Practice. American Association for Thoracic Surgery. American Society of Echocardiography. American Society of Nuclear Cardiology. Heart Failure Society of America. Heart Rhythm Society et al.| title=2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. | journal=J Thorac Cardiovasc Surg | year= 2011 | volume= 142 | issue= 6 | pages= e153-203 | pmid=22093723 | doi=10.1016/j.jtcvs.2011.10.020 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22093723 }} </ref> | [[Hypertrophic Cardiomyopathy]] is defined by LV hypertrophy associated with nondilated ventricular chambers without any other cardiac or systemic disease that itself would be capable of producing the magnitude of hypertrophy evident. HCM patients can also develop a LV outflow obstruction, diastolic dysfunction, [[myocardial ischemia]] and [[mitral regurgitation]]. [[HCM]] is usually recognized by maximal LV wall thickness ≥15 mm. Wall thickness of 13 to 14 mm is considered borderline if there are other compelling information (eg, family history of HCM), based on echocardiography. The diagnostic imaging mainly focused on echocardiography, however cardiovascular magnetic resonance (CMR) is used with increasing frequency.The risk of supraventricular and ventricular arrhythmias and for sudden cardiac death is increased.<ref name="pmid22093723">{{cite journal| author=American College of Cardiology Foundation/American Heart Association Task Force on Practice. American Association for Thoracic Surgery. American Society of Echocardiography. American Society of Nuclear Cardiology. Heart Failure Society of America. Heart Rhythm Society et al.| title=2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. | journal=J Thorac Cardiovasc Surg | year= 2011 | volume= 142 | issue= 6 | pages= e153-203 | pmid=22093723 | doi=10.1016/j.jtcvs.2011.10.020 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22093723 }} </ref> | ||
==Classification== | ==Classification== | ||
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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. | |||
{{familytree/start |summary=Sample 1}} | {{familytree/start |summary=Sample 1}} | ||
{{familytree | | | | | | | | A01 |A01= | {{familytree | | | | | | | | A01 |A01=<div style="text-align: left;"><b><u>History and symptoms:</u></b><br> | ||
❑ Hints for etiology (at least 3 generations of family history, and others)<br> | |||
❑ Duration and onset of illness<br> | |||
❑ Severity and triggers of <b>[[dyspnea]]</b> and <b> [[fatigue]]</b>, presence of <b>chest pain</b>, exercise capacity, physical activity, sexual activity (NYHA?)<br> | |||
❑ Weight loss/weight gain ([[cachexia]]/volume overload?)<br> | |||
❑ <b>Palpitations/(pre)[[syncope]]</b>/ICD shocks(adverse prognosis)<br> | |||
❑ Symptoms of [[transient ischemic attack]] or [[thromboembolism]] (anticoagulation necessary?)<br> | |||
❑ Presence of peripheral [[edema]] or [[ascites]] (volume overload?)<br> | |||
❑ Problems with breathing at night/ sleep<br> | |||
❑ Medical history: | |||
:❑ Prior hospitalizations for [[HF]] (adverse prognosis?) | |||
:❑ Discontinuation of medications (reasons?) | |||
:❑ Medications that may exacerbate [[HF]]<br> | |||
❑ Diet (restriction of sodium and fluid intake?)</div>}} | |||
{{familytree | | | | | | | | G01 |G01=<div style="text-align: left;"><b><u>Physical examination:</u></b><br> | |||
❑ Vital signs: | |||
:❑ Pulse (strength and regularity/may be brisk in upstroke and bifid through midsystolic obstruction) | |||
:❑ Blood pressure | |||
:❑ Respiratory rate | |||
❑ General appearance: | |||
:❑ BMI(weight loss/weight gain) | |||
:❑ Peripheral edema | |||
:❑ [[JVD]] may show a prominent "a" wave | |||
❑ Heart: | |||
:❑ <b>Systolic murmur</b> | |||
::❑ LVOT obstruction:harsh crescendo-decrescendo systolic murmur, may radiate to the axilla | |||
::❑ mitral regurgitation:mid-late systolic murmur at the apex | |||
::❑ LVOT obstruction murmur is similar to valvular aortic stenosis and subaortic stenosis, Valsalva increases systolic murmur in LVOT | |||
::❑ eventually have paradoxic split of S² | |||
::❑ eventually forceful LV apical impulse, presystolic apical impulse, systolic thrill at apex | |||
❑ Lungs: | |||
:❑ Rales | |||
:❑ Pleural effusion | |||
❑ Abdomen: | |||
:❑ [[Hepatomegaly]] and/or [[ascites]] (volume overload) | |||
❑ Extremities: | |||
:❑ Temperature of lower extremities</div>}} | |||
{{familytree | | | | | | | | H01 |H01=<div style="text-align: left;"><b><u>Laboratory findings:</u></b><br> | |||
❑ Complete blood count<br> | |||
❑ Chemistry:<br> | |||
:❑ Troponin, BNP or NT-proBNP<br> | |||
:❑ Serum electrolytes (including calcium and magnesium)<br> | |||
:❑ Blood urea nitrogen<br> | |||
:❑ Serum creatinine<br> | |||
:❑ Glucose<br> | |||
:❑ Fasting lipid profile<br> | |||
:❑ Liver function tests<br> | |||
:❑ Thyroid-stimulating hormone<br> | |||
:❑ Consider Screening for [[hemochromatosis]], [[HIV]], [[rheumatologic diseases]], [[amyloidosis]], or [[pheochromocytoma]]<br> | |||
:❑ Urinalysis</div>}} | |||
{{familytree | | | | | | | | I01 |I01=<div style="text-align: left;"><b><u>Imaging and additional tests:</u></b><br> | |||
❑ <b>Noninvasive imaging:</b>}} | |||
{{familytree | | | | |,|-|-|-|^|-|-|-|-|.| | | }} | {{familytree | | | | |,|-|-|-|^|-|-|-|-|.| | | }} | ||
{{familytree | | | B01 | | | | | | | | B02 | | |B01=B01|B02=B02}} | {{familytree | | | B01 | | | | | | | | B02 | | |B01=B01|B02=B02}} |
Revision as of 21:02, 6 March 2015
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Steven Bellm, M.D. [2]
Hypertrophic cardiomyopathy resident survival guide Microchapters |
---|
Overview |
Classification |
Causes |
Diagnosis |
Treatment |
Do's |
Dont's |
Overview
Hypertrophic Cardiomyopathy is defined by LV hypertrophy associated with nondilated ventricular chambers without any other cardiac or systemic disease that itself would be capable of producing the magnitude of hypertrophy evident. HCM patients can also develop a LV outflow obstruction, diastolic dysfunction, myocardial ischemia and mitral regurgitation. HCM is usually recognized by maximal LV wall thickness ≥15 mm. Wall thickness of 13 to 14 mm is considered borderline if there are other compelling information (eg, family history of HCM), based on echocardiography. The diagnostic imaging mainly focused on echocardiography, however cardiovascular magnetic resonance (CMR) is used with increasing frequency.The risk of supraventricular and ventricular arrhythmias and for sudden cardiac death is increased.[1]
Classification
Left Ventricular Hypertrophy | |||||||||||||||||||||||||||||||||
Sarcomere Mutation | Without Extracardiac or Metabolic Findings + Genetic Substrate Unresolved | With Extracardiac or Metabolic Findings Associated With or Without Mutant Gene | |||||||||||||||||||||||||||||||
Hypertrophic Cardiomyopathy | Syndrome with Left Ventricular Hypertrophy | ||||||||||||||||||||||||||||||||
Causes
Common Causes
- Familial
- Gene mutation
- Hypertension
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 (at least 3 generations of family history, and others) | |||||||||||||||||||||||||||||||||
Physical examination: ❑ Vital signs:
❑ General appearance:
❑ Heart:
❑ Lungs:
❑ Abdomen:
❑ Extremities:
| |||||||||||||||||||||||||||||||||
Laboratory findings: ❑ Complete blood count
| |||||||||||||||||||||||||||||||||
Imaging and additional tests: ❑ Noninvasive imaging: | |||||||||||||||||||||||||||||||||
B01 | B02 | ||||||||||||||||||||||||||||||||
C01 | |||||||||||||||||||||||||||||||||
D01 | D02 | D03 | |||||||||||||||||||||||||||||||
E01 | E02 | E03 | |||||||||||||||||||||||||||||||
F01 | F02 | ||||||||||||||||||||||||||||||||
Treatment
Patients with HCM | |||||||||||||||||||||||||||||||||||||||||||||||
Treat comorbidities according to guidelines (hypertension, diabetes mellitus,etc) | |||||||||||||||||||||||||||||||||||||||||||||||
Obstructive physiology? | |||||||||||||||||||||||||||||||||||||||||||||||
Yes | No | ||||||||||||||||||||||||||||||||||||||||||||||
Avoid vasodilator therapy and high-dose diuretics | Heart failure symptoms or angina | ||||||||||||||||||||||||||||||||||||||||||||||
Heart failure symptoms or angina | Yes | No | |||||||||||||||||||||||||||||||||||||||||||||
No | Yes | Systolic function? | Annual clinical evaluation | ||||||||||||||||||||||||||||||||||||||||||||
Annual clinical evaluation | Beta-blockade/Verapamil/Disopyramide | LV-EF<50 percent | LV-EF≥50 percent | ||||||||||||||||||||||||||||||||||||||||||||
Persistent symptoms | Therapy as described in Heart failure | Beta-blockade | Verapamil | ||||||||||||||||||||||||||||||||||||||||||||
Invasive therapy | |||||||||||||||||||||||||||||||||||||||||||||||
Acceptable surgical candidate | Diuretics | ACE inhibitor or ARB | |||||||||||||||||||||||||||||||||||||||||||||
No | Yes | ||||||||||||||||||||||||||||||||||||||||||||||
Acceptable candidate for alcohol ablation? | Alcohol ablation | Surgical myectomy | |||||||||||||||||||||||||||||||||||||||||||||
Yes | No | ||||||||||||||||||||||||||||||||||||||||||||||
Consider Alcohol ablation | Consider DDD pacing | ||||||||||||||||||||||||||||||||||||||||||||||
Based on the ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy.[2]
Do's
Dont's
References
- ↑ American College of Cardiology Foundation/American Heart Association Task Force on Practice. American Association for Thoracic Surgery. American Society of Echocardiography. American Society of Nuclear Cardiology. Heart Failure Society of America. Heart Rhythm Society; et al. (2011). "2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines". J Thorac Cardiovasc Surg. 142 (6): e153–203. doi:10.1016/j.jtcvs.2011.10.020. PMID 22093723.
- ↑ Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS; et al. (2011). "2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines". Circulation. 124 (24): 2761–96. doi:10.1161/CIR.0b013e318223e230. PMID 22068435.