Cardiovascular Medicine Book

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Hypertrophic Cardiomyopathy

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  1. See Also
    1. Cardiomyopathy
  2. Epidemiology
    1. Familial: Autosomal Dominant with variable penetrance
    2. Conferred by 11 mutant genes and over 500 transmutations
    3. Variable penetrance (not everyone is affected)
  3. Pathophysiology
    1. Primary ventricular hypertrophy
      1. Contrast with secondary Left Ventricular Hypertrophy
    2. Asymmetric thickening of ventricular septum
      1. Outflow obstruction (25%)
      2. Abnormal diastolic filling
    3. Sudden death secondary to Ventricular Tachycardia
  4. Symptoms (Often no history is elicited)
    1. Dyspnea
    2. Angina
    3. Dizziness
    4. Syncope with Exercise (60%)
    5. Family History of Sudden death (20%)
  5. Signs
    1. Harsh Systolic Murmur at Left Sternal Border
      1. Sounds like flow murmur
      2. Accentuated with standing
      3. Diminished with squatting
    2. Biphasic pulse
  6. Complications
    1. Heart Failure
    2. Sudden Death (may be how condition presents)
    3. Dynamic outflow obstruction by mitral valve (common)
  7. Diagnostics
    1. Electrocardiogram (changes seen in 90% of cases)
      1. Ventricular hypertrophy
      2. Deep Q waves without history of Coronary Artery Disease
      3. T-Wave Inversion
      4. Cardiac Ischemia
    2. Echocardiogram
      1. Definitive test for diagnosis
      2. Septum wider than 15mm confirms diagnosis
      3. Left venticular hypertrophy
      4. Reduced ventricular chamber volume
  8. Management: Pharmacologic
    1. See Systolic Dysfunction
    2. Exercise caution with Diuretics (hypertrophic Cardiomyopathy requires higher filling pressures)
  9. Management: Heart Failure
    1. Biventricular pacing
    2. Implantable Defibrillator
  10. Management: Obstructive hypertrophic Cardiomyopathy
    1. Septal myomectomy
    2. Septal Alcohol ablation
  11. Prevention
    1. Consider screening family members of those with hypertrophic Cardiomyopathy
      1. Up to annual echocardiogram, Electrocardiogram and physical is recommended in Europe
  12. References
    1. Wexler (2009) Am Fam Physician 79(9):778

Hypertrophic Cardiomyopathy (C0007194)

Definition (MSH)A form of CARDIAC MUSCLE disease, characterized by left and/or right ventricular hypertrophy (HYPERTROPHY, LEFT VENTRICULAR; HYPERTROPHY, RIGHT VENTRICULAR), frequent asymmetrical involvement of the HEART SEPTUM, and normal or reduced left ventricular volume. Risk factors include HYPERTENSION; AORTIC STENOSIS; and gene MUTATION; (FAMILIAL HYPERTROPHIC CARDIOMYOPATHY).
Definition (CSP)myocardial disease characterized by hypertrophy, involving mainly the interventricular septum, interfering with left ventricular emptying.
Definition (NCI)A condition in which the myocardium is hypertrophied without an obvious cause. The hypertrophy is generally asymmetric and may be associated with obstruction of the ventricular outflow tract.
ConceptsDisease or Syndrome (T047)
ICD9425.1, 425.4
MSHD002312
EnglishHCM - Hypertrophic cardiomyopathy, HOCM - Hypertrophic obstructive cardiomyopathy, Hyper. obst. cardiomyopathy, HYPERTR OBSTR CARDIOMYOP, Hypertrophic Cardiomyopathies, Hypertrophic cardiomyopathy, hypertrophic myocardiopathy, Hypertrophic Obstructive Cardiomyopathies, Hypertrophic obstructive cardiomyopathy, Obstructive cardiomyopathy, Primary hypertrophic cardiomyopathy
Spanishcardiomiopatia hipertrofica, cardiomiopatia hipertrofica primaria, miocardiopatia hipertrofica, miocardiopatia hipertrofica primaria, miocardiopatia obstructiva, miocardiopatia obstructiva hipertrofica
Parent ConceptsCardiomyopathies (C0878544), Cardiomegaly (C0018800), Aortic Stenosis, Subvalvular (C0003500), Hypertrophic Cardiomyopathy (C0007194), Left Ventricular Hypertrophy (C0149721), Ambiguous concept (C1274012), Duplicate concept (C1274013)
SourcesAOD, CSP, DXP, ICD9CM, MSH, MTH, MTHICD9, NCI, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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