Cardiovascular Medicine Book

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Hypertrophic Cardiomyopathy

Aka: Hypertrophic Cardiomyopathy
  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): 778-84

Hypertrophic Cardiomyopathy (C0007194)

Definition (CSP) myocardial disease characterized by hypertrophy, involving mainly the interventricular septum, interfering with left ventricular emptying.
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 (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.
Concepts Disease or Syndrome (T047)
MSH D002312
ICD9 425.11, 425.1
ICD10 I42.1
SnomedCT 15471000, 389998005, 389999002, 233873004, 45227007, 155351008, 266301006
English Obstructive hypertrophic cardiomyopathy, Cardiomyopathies, Hypertrophic, Cardiomyopathies, Hypertrophic Obstructive, Cardiomyopathy, Hypertrophic, Cardiomyopathy, Hypertrophic Obstructive, Hypertrophic Cardiomyopathies, Hypertrophic Obstructive Cardiomyopathies, Obstructive Cardiomyopathies, Hypertrophic, Obstructive Cardiomyopathy, Hypertrophic, hypertrophic myocardiopathy, CARDIOMYOPATHY, HYPERTROPHIC OBSTRUCTIVE, Hypertrophic Cardiomyopathy, Hypertrophic Obstructive Cardiomyopathy, HCM - Hypertrophic cardiomyop, HOCM - Obstruct cardiomypathy, Hypertrophic obstruct cardiomy, Cardiomyopathy, hypertrophic, SUBVALV STENOSIS, Primary hypertrophic cardiomyopathy (disorder), hypertrophic cardiomyopathy (diagnosis), obstructive hypertrophic cardiomyopathy, hypertrophic cardiomyopathy, obstructive hypertrophic cardiomyopathy (diagnosis), CARDIOMYOPATHY HYPERTROPHIC, Cardiomyopathy, Hypertrophic [Disease/Finding], hocm, Cardiomyopathy;hypertrophic, hypertrophic cardiomyopathy (HCM), cardiomyopathy hypertrophic, obstructive cardiomyopathy, Hyprtrophc obst cardiomy, Hypertrophic cardiomyopathy, Hypertrophic obstructive cardiomyopathy, Obstructive cardiomyopathy, Primary hypertrophic cardiomyopathy, HCM - Hypertrophic cardiomyopathy, HOCM - Hypertrophic obstructive cardiomyopathy, Hypertrophic cardiomyopathy (disorder), Hypertrophic obstructive cardiomyopathy (disorder), cardiomyopathy; hypertrophic, obstructive, cardiomyopathy; hypertrophic, hypertrophic; cardiomyopathy, Hyper. obst. cardiomyopathy, Primary hypertrophic cardiomyopathy (disorder) [Ambiguous], Cardiomyopathy, obstructive, HOCM Hypertrophic obstructive cardiomyopathy, HOCM
Italian Cardiomiopatia ipertrofica, Cardiomiopatia ipertrofica ostruttiva, Cardiomiopatia ostruttiva, Miocardiopatia ipertrofica ostruttiva, Miocardiopatia ipertrofica
Dutch HOCM, HOCM hypertrofische obstructieve cardiomyopathie, obstructieve cardiomyopathie, hypertrofische obstructieve cardiomyopathie, cardiomyopathie; hypertrofisch, obstructief, cardiomyopathie; hypertrofisch, hypertrofisch; cardiomyopathie, hypertrofe cardiomyopathie, Cardiomyopathie, hypertrofische, Cardiomyopathie, hypertrofische obstructieve, Hypertrofische cardiomyopathie, Obstructieve hypertrofische cardiomyopathie
French Cardiomyopathie hypertrophique asymétrique, Cardiomyopathie obstructive hypertrophique (COH), CMOH, CMO (Cardiomyopathie Obstructive), Cardiomyopathie hypertrophique, Cardiomyopathie hypertrophique idiopathique, Cardiomyopathie hypertrophique primitive, Cardiomyopathie obstructive, Myocardiopathie hypertrophique
German obstruktive Kardiomyopathie, Kardiomyopathie hypertrophisch obstruktiv, HOCM, HOCM hypertrophische obstruktive Kardiomyopathie, Hypertrophische obstruktive Kardiomyopathie, hypertrophische Kardiomyopathie, Kardiomyopathie, hypertrophische, Kardiomyopathie, hypertrophische obstruktive
Portuguese Miocardiopatia obstrutiva hipertrófica, Cardiomiopatia obstrutiva, Cardiomiopatia Hipertrófica, Cardiomiopatia Hipertrófica Obstrutiva, Miocardiopatia Hipertrófica Obstrutiva, Cardiomiopatia hipertrópica
Spanish CMOH, Cardiomiopatía obstructiva hipertrófica, Miocardiopatía obstructiva hipertrófica, Cardiomiopatía obstructiva, Cardiomiopatía Hipertrófica, Cardiomiopatía Hipertrófica Obstructiva, Cardiomiopatia Hipertrofica, Cardiomiopatia Hipertrofica Obstructiva, Miocardiopatía Hipertrófica Obstructiva, Miocardiopatia Hipertrofica Obstructiva, Hypertrophic cardiomyopathy, Hypertrophic obstructive cardiomyopathy, Hyper. obst. cardiomyopathy, cardiomiopatía hipertrófica primaria, cardiomiopatía hipertrófica, miocardiopatía hipertrófica (trastorno), miocardiopatía hipertrófica primaria (concepto no activo), miocardiopatía hipertrófica primaria (trastorno), miocardiopatía hipertrófica primaria, miocardiopatía hipertrófica, miocardiopatía obstructiva hipertrófica (trastorno), miocardiopatía obstructiva hipertrófica, miocardiopatía obstructiva, Cardiomiopatía hipertrófica
Japanese 閉塞性心筋症, ﾍｲｿｸｾｲｼﾝｷﾝｼｮｳ, ﾋﾀﾞｲｶﾞﾀｼﾝｷﾝｼｮｳ, ﾋﾀﾞｲｶﾞﾀﾍｲｿｸｾｲｼﾝｷﾝｼｮｳ, 特発性肥厚性大動脈弁下狭窄症, 非対称性中隔肥大, 肥大心筋症, 閉塞性肥大型心筋症, 特発性肥大型大動脈弁下狭窄症, 肥大性心筋, 心筋症-肥大性閉塞性, 大動脈弁下狭窄症-特発性肥厚性, 特発性肥厚性大動脈弁下部狭窄, 肥厚性心筋症, 肥大性心筋症, 肥大型心筋症, 肥大型閉塞性心筋症, 大動脈弁下狭窄症-特発性肥大型, 心筋症-肥大型閉塞性, 心筋症-肥大性
Swedish Kardiomyopati, hypertrofisk
Czech kardiomyopatie hypertrofická, Hypertrofická kardiomyopatie, Hypertrofická obstrukční kardiomyopatie, HKMP Hypertrofická obstrukční kardiomyopatie, Obstruktivní kardiomyopatie, HoKMP, HKMP, obstruktivní hypertrofická kardiomyopatie
Finnish Hypertrofinen kardiomyopatia
Russian ASSIMETRICHNAIA GIPERTROFIIA MEZHZHELUDOCHKOVOI PEREGORODKI, PODKLAPANNYI STENOZ IDIOPATICHESKII GIPERTROFICHESKII, KARDIOMIOPATIIA GIPERTROFICHESKAIA, IDIOPATICHESKII GIPERTROFICHESKII PODKLAPANNYI STENOZ, IDIOPATICHESKII GIPERTROFICHESKII SUBAORTAL'NYI STENOZ, АССИМЕТРИЧНАЯ ГИПЕРТРОФИЯ МЕЖЖЕЛУДОЧКОВОЙ ПЕРЕГОРОДКИ, ИДИОПАТИЧЕСКИЙ ГИПЕРТРОФИЧЕСКИЙ ПОДКЛАПАННЫЙ СТЕНОЗ, ИДИОПАТИЧЕСКИЙ ГИПЕРТРОФИЧЕСКИЙ СУБАОРТАЛЬНЫЙ СТЕНОЗ, КАРДИОМИОПАТИЯ ГИПЕРТРОФИЧЕСКАЯ, ПОДКЛАПАННЫЙ СТЕНОЗ ИДИОПАТИЧЕСКИЙ ГИПЕРТРОФИЧЕСКИЙ
Korean 폐쇄성 비대성 심장근육병증
Polish Kardiomiopatia przerostowa
Hungarian HOCM, Hypertrophiás cardiomyopathia, Hypertrophiás obstructiv cardiomyopathia, HOCM hypertrophiás obstructiv cardiomyopathia, Obstructiv cardiomyopathia
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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