Hypertrophic cardiomyopathy resident survival guide

Revision as of 18:22, 6 March 2015 by Steven Bellm (talk | contribs)
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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 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.[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

Life Threatening Causes

Common Causes

Complete Diagnostic Approach

 
 
 
 
 
 
 
A01
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
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.