Hypertrophic cardiomyopathy resident survival guide: Difference between revisions

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:❑  Discontinuation of medications (reasons?)
:❑  Discontinuation of medications (reasons?)
:❑&nbsp;&nbsp;Medications that may exacerbate [[HF]]<br>
:❑&nbsp;&nbsp;Medications that may exacerbate [[HF]]<br>
❑&nbsp;&nbsp;Diet (restriction of sodium and fluid intake?)</div>}}  
❑&nbsp;&nbsp;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>
❑&nbsp;&nbsp;Vital signs:
❑&nbsp;&nbsp;Vital signs:
:❑&nbsp;&nbsp;Pulse (strength and regularity/may be brisk in upstroke and bifid through  midsystolic obstruction)
:❑&nbsp;&nbsp;Pulse (strength and regularity/may be brisk in upstroke and bifid through  midsystolic obstruction)
:❑&nbsp;&nbsp;Blood pressure  
:❑&nbsp;&nbsp;Blood pressure  
:❑&nbsp;&nbsp;Respiratory rate
:❑&nbsp;&nbsp;Respiratory rate
❑&nbsp;&nbsp;General appearance:
❑&nbsp;&nbsp;General appearance:
:❑&nbsp;&nbsp;BMI(weight loss/weight gain)
:❑&nbsp;&nbsp;BMI(weight loss/weight gain)
:❑&nbsp;&nbsp;Peripheral edema
:❑&nbsp;&nbsp;Peripheral edema
:❑&nbsp;&nbsp;[[JVD]] may show a prominent "a" wave
:❑&nbsp;&nbsp;[[JVD]] may show a prominent "a" wave
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::❑&nbsp;&nbsp;mitral regurgitation:mid-late systolic murmur at the apex
::❑&nbsp;&nbsp;mitral regurgitation:mid-late systolic murmur at the apex
::❑&nbsp;&nbsp;LVOT obstruction murmur is similar to valvular aortic stenosis and subaortic stenosis, Valsalva increases systolic murmur in LVOT
::❑&nbsp;&nbsp;LVOT obstruction murmur is similar to valvular aortic stenosis and subaortic stenosis, Valsalva increases systolic murmur in LVOT
::❑&nbsp;&nbsp;eventually have paradoxic split of S²
::❑&nbsp;&nbsp;eventually have paradoxic split of S²
::❑&nbsp;&nbsp;eventually forceful LV apical impulse, presystolic apical impulse, systolic thrill at apex
::❑&nbsp;&nbsp;eventually forceful LV apical impulse, presystolic apical impulse, systolic thrill at apex
❑&nbsp;&nbsp;Lungs:
❑&nbsp;&nbsp;Lungs:
:❑&nbsp;&nbsp;Rales
:❑&nbsp;&nbsp;Rales
:❑&nbsp;&nbsp;Pleural effusion
:❑&nbsp;&nbsp;Pleural effusion
❑&nbsp;&nbsp;Abdomen:
❑&nbsp;&nbsp;Abdomen:
:❑&nbsp;&nbsp;[[Hepatomegaly]] and/or [[ascites]] (volume overload)
:❑&nbsp;&nbsp;[[Hepatomegaly]] and/or [[ascites]] (volume overload)
❑&nbsp;&nbsp;Extremities:
❑&nbsp;&nbsp;Extremities:
:❑&nbsp;&nbsp;Temperature of lower extremities</div>}}
:❑&nbsp;&nbsp;Temperature of lower extremities</div>}}
{{familytree | | | | | | | | H01 |H01=<div style="text-align: left;"><b><u>Laboratory findings:</u></b><br>
{{familytree | | | | | | | | H01 |H01=<div style="text-align: left;"><b><u>Laboratory findings:</u></b><br>
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:❑&nbsp;&nbsp;Urinalysis</div>}}  
:❑&nbsp;&nbsp;Urinalysis</div>}}  
{{familytree | | | | | | | | I01 |I01=<div style="text-align: left;"><b><u>Imaging and additional tests:</u></b><br>
{{familytree | | | | | | | | I01 |I01=<div style="text-align: left;"><b><u>Imaging and additional tests:</u></b><br>
❑&nbsp;&nbsp;<b>Noninvasive imaging:</b>}}
❑&nbsp;&nbsp;<b>Noninvasive imaging and tests:</b>
:❑&nbsp;&nbsp;ECG (i.e. repolarization changes/Prominent abnormal Q waves/P wave abnormalities/Deeply inverted T waves/signs ventricular hypertrophy/Left axis deviation)
 
 
 
 
:❑&nbsp;&nbsp;2D Echocardiography with Doppler:
::❑&nbsp;&nbsp;LV hypertrophy (HCM confirmed when unexplained increased LV wall thickness ≥15 mm/ ≥13 mm may be considered if family member with HCM)
::❑&nbsp;&nbsp;Systolic anterior motion of the mitral valve
 
:❑&nbsp;&nbsp;LVOT obstruction


:❑&nbsp;&nbsp;</div>}}
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{{familytree | | | | |,|-|-|-|^|-|-|-|-|.| | | }}
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{{familytree | | | B01 | | | | | | | | B02 | | |B01=B01|B02=B02}}

Revision as of 21:25, 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)
❑  Duration and onset of illness
❑  Severity and triggers of dyspnea and fatigue, presence of chest pain, exercise capacity, physical activity, sexual activity (NYHA?)
❑  Weight loss/weight gain (cachexia/volume overload?)
❑  Palpitations/(pre)syncope/ICD shocks(adverse prognosis)
❑  Symptoms of transient ischemic attack or thromboembolism (anticoagulation necessary?)
❑  Presence of peripheral edema or ascites (volume overload?)
❑  Problems with breathing at night/ sleep
❑  Medical history:

❑  Prior hospitalizations for HF (adverse prognosis?)
❑  Discontinuation of medications (reasons?)
❑  Medications that may exacerbate HF
❑  Diet (restriction of sodium and fluid intake?)
 
 
 
 
 
 
 
Physical examination:

❑  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:

❑  Systolic murmur
❑   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
 
 
 
 
 
 
 
Laboratory findings:

❑  Complete blood count
❑  Chemistry:

❑  Troponin, BNP or NT-proBNP
❑  Serum electrolytes (including calcium and magnesium)
❑  Blood urea nitrogen
❑  Serum creatinine
❑  Glucose
❑  Fasting lipid profile
❑  Liver function tests
❑  Thyroid-stimulating hormone
❑  Consider Screening for hemochromatosis, HIV, rheumatologic diseases, amyloidosis, or pheochromocytoma
❑  Urinalysis
 
 
 
 
 
 
 
Imaging and additional tests:

❑  Noninvasive imaging and tests:

❑  ECG (i.e. repolarization changes/Prominent abnormal Q waves/P wave abnormalities/Deeply inverted T waves/signs ventricular hypertrophy/Left axis deviation)



❑  2D Echocardiography with Doppler:
❑  LV hypertrophy (HCM confirmed when unexplained increased LV wall thickness ≥15 mm/ ≥13 mm may be considered if family member with HCM)
❑  Systolic anterior motion of the mitral valve
❑  LVOT obstruction
❑  
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
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

  1. 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.
  2. 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.