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Cardiac Myopathies_Tumors.ppt

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  • 1.
    • Cardiomyopathies
    • Heart disease resulting from a primary
    • abnormality in myocardium
    • most causes are idiopathic
    • a) most likely genetic defects
    • Types:
    • a) Dilated
    • i) “congestive”
    • ii) most common (~90%)
    • b) Hypertrophic
    • c) Restrictive (lease common)
  • 2.  
  • 3.  
  • 4.
    • 1.- Dilated Cardiomyopathy (DCM)
    • Progressive cardiac dilation
    • a) systolic dysfunction
    • ~35% of patients have genetic form
      • a) “primary”
    • large variety of acquired DCM (2 o )
    • a) toxicities (e.g., alcohol)
    • b) myocarditis (inflammations)
    • c) pregnancy
    • d) unknown (“idiopathic DCM”)
    • i) likely to be genetic
  • 5.
    • Myocarditis (inflammatory)
    • a) viral in nature (most often)
    • i) coxsackievirus A and B
    • ii) other enteroviruses
    • - cleave dystrophin (later)
    • b) progression from myocarditis to DCM in some patients
    • Alcohol (alcoholic cardiomyopathy)
    • a) alcohol toxicity or 2 o nutritional disturbances may be cause
    • b) ethanol metabolite –acetaldehyde-
    • have direct toxic effect on heart
  • 6. i) however, no distinguishing features from DCM Toxicity a) chemotherapeutic agents i) doxorubicin (Adriamycin) ii) Cobalt Pregnancy-associated a) “peripartum cardiomyopathy” i) late in pregnancy or weeks to months postpartum
  • 7.
    • ii) poorly understood
    • - hypertension
    • - volume overload
    • - nutritional deficiency
    • - etc. ?
    • Genetic Influences
    • a) ~ 35% of patients have familial occurrence
    • b) autosomal dominant predominates
    • c) affects on cytoskeleton, oxidative
    • Phos., fatty acid β – oxidation, etc
  • 8. d) Mitochondrial defects most frequently cause DCM in children e) X-linked induces DCM in teenagers i) rapidly progressive ii) linked to gene for “dystrophin” - cytoskeleton protein iii) diseases with dystrophin mutations (myopathies such as Duchenne and Becker muscular dystrophy) present with DCM
  • 9. f) Clinical: i) common at 20-50 yrs ii) progress slowly - shortness of breath - easy fatigue - poor exertional capacity iii) may rapidly slip in decompensation - EF < 25% - 50% die within 2 yrs - 25% survive > 5 yrs. iv) hypo contracting
  • 10.
    • Arrhythmogenic Right Ventricular
    • Cardiomyopathy (right ventricular
    • dysplasia)
    • a) associated with right sided heart
    • failure
    • b) various rhythm disturbances
    • i) Ventricular tachycardia
    • c) right ventricular thinning
    • i) due to loss of myocytes
  • 11.
    • 2.- Hypertrophic Cardiomyopathy(HCM)
    • Is a genetic disease
    • other names:
    • a) idiopathichypertrophic subaortic stenosis
    • b) hypertrophic obstructive cardiomyopathy
    • Characteristics:
    • a) hypertrophy (mainly septal vs. free wall) 1:3 ratio)
    • b) abnormal diastolic filling
    • c) hyper contracting
  • 12.
    • Primary diastolic dysfunction
    • Systolic function is preserved
    • 2 diseases which must be
    • differentiated from HCM are:
    • a) amyloidosis
    • b) hypertensive heart disease
    • Most cases familial
    • a) autosomal dominant
    • b) several gene mutations found
    • i) see fig. 12-35
    • - β -myosin heavy chain is most common
  • 13.
    • - MYBP-C and troponinT are next in frequency
    • - these 3 genes account for ~80% of all cases of HCM
    • c) these gene mutations mainly effect proteins of sarcomere
    • Clinical:
    • a)  compliance
    • b)  chamber size
    • c)  V s
    • d)  diastolic filling
  • 14. e) ~ 25% have obstruction to ventricular outflow i) systolic ejection murmur f) focal cardiac ischemia and angina g) major problems: i) atrial fibrillation ii) mural thrombus formation iii) embolization iv) IE h) one of most common causes of death in young athletes !!
  • 15. An illustration of the obstructive form of HCM during relaxation (diastole, left) and during active contraction (systole, right). The thickened septum and the mitral valve leaflets come in close proximity, effectively narrowing the pathway that blood must travel to get out of the heart. Additionally, the forces on the mitral valve can lead to variable degrees of backward leakage (regurgitation) that can also lead to symptoms. SYSTOLE DIASTOLE
  • 16.
    • 3.- Restrictive Cardiomyopathy
    • Impairs diastolic function
    • a) primary  in compliance
    • - impaired diastolic filling
    • least common of the CM
    • Ventricles approximately normal size
    • Systolic function unaffected
    • Biatrial dilation commonly seen
    • Myocardial fibrosis
    • a) radiation
    • b) amyloidosis
    • c) sarcoidosis
  • 17. d) metastatic carcinoma e) sarcoid granulomas f) any factor which increases endomyocardial fibrosis
  • 18. Primary Cardiac Tumors BENIGN MALIGNANT Myxoma Rhabdomyosarcoma Lipoma Mesothelioma Hemangioma Fibrosarcoma Mesothelioma Lymphoma Fibroma Osteosarcoma Teratoma Angiosarcoma Rhabdomyoma Liposarcoma Neurofibroma Leiomyosarcoma
  • 19.
    • Myxoma
    • a) most common 1 o tumor of heart
    • i) most within atria (~90%)
    • ii) left-to-right prevalence (4:1)
    • b) benign
    • c) almost always single neoplasm
    • d) 10% familial (i.e., “Carney syndrome”)
    • e) Clinical:
    • i) “ball-Valve” obstruction
    • ii) constitutional effects
    • iii) embolization
  • 20.
    • Rhabdomyomas
    • a) most common cardiac tumor in children
    • i) often diagnosed within 1 st year of life
    • - obstructive disease
    • Non Cardiac Neoplasms
    • a) most common are metastases from
    • i) lung
    • ii) breast
    • iii) melanomas
    • iv) leukemias and lymphomas
  • 21. b) clinical: i) pericardial spread ii) obstruction (i.e., filling) iii) cardiomyopathy via radiation

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