Medical Archive

Definition and classification

HTN?? Congenital/Acquired valvular, coronary, pericardial abnormality? ?? ? ?? ?? ?? ?? ??? primary ??? ?? ??

General presentation

Dilated cardiomyopathy

Causes

Acute viral myocarditis, Obesity

• Inflammatory myocarditis
  • Infective
  • Noninfective
• Toxic
  • Alcoholic abuse: ?? ???? 80%? 3? ? ??
  • Anthracycline, doxorubicin, cyclophosphamide, herceptin, cocaine
• Metabolic
  • Nutritional deficiencies: thiamine, selenium, carnitine
  • Electrolyte deficiencies: calcium, phosphate, magnesium
  • Endocrinopathy
    • Thyroid disease
    • Pheochromocytoma
    • Diabetes
  • Obesity
  • Hemochromatosis
• Inherited metabolic pathway defects
• Familial
• Overlap with nondilated cardiomyopathy
• Idiopathic
• Miscellaneous (shared elements of above etiologies)
  • Peripartum: ?? ?? 6??, ?? ???? ??? ?? ????.

Stress-related
Likely caused by a catecholamine surge in the setting of physical or emotional stress
Catecholamines may cause microvascular spasm leading to ischemia and myocardial stunning.
Takotsubo. ???? ??? ??~?? ? ???? ?? ?? ??. 

??? ??

AD inheritance with incomplete penetrance.

Truncating mutations( usually nonsense mutations) affecting the TTN gene, which encodes for the sarcomere protein titin, are the m/c cause of familial DCM.

Echo

Dilated, normal~thin wall
Systolic dysfunction: ?? EF <30%

Complications

Mitral and tricuspid valve regurgitation and arrhythmia.

Treatment

Digitalis, diuretics / Heart transplant

Overlapping types of cardiomyopathy

Hypertrophic cardiomyopathy

Cause

50%? AD inheritance, 3? ???? ? ??.
Mutations in genes encoding sarcomeric proteins, such as myosin binding protein C and ?-myosin heavy chain.
?? ??? ?? ?? ??.

Biopsy shows myofiber hypertrophy with disarray.
(High rate of myosin mRNA synthesis)

Clinical manifestations

• Symptoms
  • Syncope
    • In large part it’s d/t outflow obstruction from the hypertrophied myocardium
    • Secondary to arrhythmia, ischemia, and a ventricular baroreceptor response that inappropriately causes vasodilation
  • Dyspnea
  • Chest pain
• Auscultation
  • 4th heart sound
  • Systolic murmur
    • Crescendo-decrescendo
    • Along the left sternal border
    • Without carotid radiation
  • Afib ?? ??

Echo

Asymmetric LVH. EF>60% (septal wal? 1.3? ?? ? ???. ???? RCMP)
Systolic ant. Motion (SAM) of mitral valve -> ??? ??
SAM?? ?? IHSS(idiopathic hypertrophic subaortic stenosis) ??

Apex? LVOT ?? ??? ?

To avoid

Treatment

Medical treatment

• BB
  • Contractility ??? ?? ??. SCD ???? ??.
  • Propranolol 160-320mg/d, ?? ?? ?? ? 480mg/d ?? ?? ?? (cf. HF?? ?? ??)
• CCB (non-DHP)
  • ??? ????? ???? ?? ??. SCD ???? ??.
  • Verapamil
  • Dilitazem
• Disopyramide (NaB; Class Ia anti-arrhythmics)
  • Contractility? ????? afterload? ????. SCD ???? ??.
  • BB? ?? ?? ? ?? ??. ?? ???? ??.
• To avoid
  • ??? ?: Digitalis, ? agonist
  • Preload ?: Diuretics, nitrate
  • Afterload ?: DHP CCB, vasodilator
  • Alcohol

Septal myotomy-myectomy

?? ??? ?? ?? ? ??

ICD for prevent SCD

• Primary prevention
  • HCM Risk-SCD score: 5-year risk? ???? ICD ?? ?? ??.
  • 5? ??? ?6%(????)? ? ICD ?? ??.
• Secondary prevention
  • ??? ? ???
  • ???/??? ????
• SCD? ?? ????
  • ??? ???
  • ??? ??
  • Vtac
  • ?? ??>30mm
  • ?? ? ???? fail
  • FHx, ?? Gene mutation

• Clinical course? ??? ?? ???
  • Afib
  • Infective endocarditis
  • Burnt out HCMP (??? ?? ????)

Restrictive cardiomyopathy

Causes

• Infiltrative (between myocytes)
  • Amyloidosis
    • Primary (light chain amyloid)
    • Familial (abnormal transthyretin)
    • Senile (normal transthyretin or atrial peptides)
    • P4 S2 C108 Amyloidosis
  • Inherited metabolic defects
• Storage (within myocytes)
  • Hemochromatosis (iron)
  • Inherited metabolic defects
    • Fabry’s disease <Nelson> P11 C86 Defects in Metabolism of Lipids
    • Glycogen storage disease (II,  III)
• Fibrotic
  • Radiation
  • Scleroderma
• Endomyocardial
  • Endocardial fibroelastosis (children)
  • Löffler syndrome (endomyocardial fibrosis with an eosinophilic in filtrate and eosinophilia)
• Overlap with other myocardiopathies
  • Sarcoidosis
• Idiopathic

Echo & physical exam

?? ???? ??.  Symmetric LV wall thickness

Low-voltage EKG with diminished QRS amplitude.
Prominent x & y, square root sign, S3, S4(??? ??? ???), Kussmaul’s sign

• RV failure sign
  • ?? ??, ??, ???, ???? ??

Treatment

??? ?? x
?? ??: ???
?? ??? ??: CCB
?????? ??

Myocarditis

Cause

• Viral – adeno, coxsackie B, parvo B19, HIV, HHV-6
• Parasitic – Trypanosoma cruzi, Toxoplasma gondii
• Bacterial – Borrelia burgdorferi, Mycoplasma, pneumoniae
• Toxins – CO, black widow venom
• Rheumatic fever
• Drugs – doxorubicin, cocaine
• Autoimmune – Kawasaki disease, sarcoidosis, SLE, polymyositis/dermatomyositis

??? viral (Coxsackie B ?)
1-2? ? URI hstory ?????.

Clinical presentation

??: Pericarditis? ?? (ST?, pleuritic pain, low tidal volume)
???? ??? ??. S3, MR? ???. Cardiac marker ?
Fulminant? ?? ????? ?????? ?? ?
Lymphocytic myocarditis ? ?? ???? ???? ??.

Treatment

???? HF? ??? ??? ???. Digoxin? ???? ? ???? ?? ???? ??.
????: EKG, ?? ??? ? ??