Miscellaneous Heart Disease Flashcards
diverse group of heart diseases involving the _____
myocardium
What are the 3 functional classes of cardiomyopathy
- dialed (imparted contractility ad systolic function
- hypertrophic ???
- restrictive ??
a group of genetic diseases sharing hypertrophy as a compensatory mechanisms for mutations in cardiac contractile proteins
hypertrophic cardiomyopathy
epidemiology of hypertrophic cardiomyopathy
males in mid 20s
Pathophys of hypertrophic cardiomyopathy
-dec compliance and inc LV, LA, and pulmonary pressures
30% also have obstruction due to rapid flow and venturi forces ???
Gross pathology of hypertrophic cardiomyopathy
thickened wall
asymmetric hypertrophy of upper septum and anterior LV
thickened atrial leaflet of mital valve???
Microscopic pathology of hypertrophic cardiomyopathy
myocyte disarray (oriented in diff directions**cannot contract as a unit anymore) and hypertrophy
interstitial and replacement fibrosis in a haphazard plexiform pattern
Symptom of hypertrophic cardiomyopathy
***very similar to aortic stenosis: but these pts are much younger!!!!*** dyspnea angina syncope lightheadedness sudden death
Signs of hypertrophic cardiomyopathy
S4
rough crescendo-decrescendo systolic murmur at left sternal boarder that inc with standing or valvsa maneuver
Tx of hypertrophic cardiomyopathy
- BB
- CCB
- amiodarone
- surgical resection of hypertrophied septum
- injection of poison into first septal perforator to infarct hypertrophied septum
- implanted defibrillator
STOP PLAYING COMPETITIVE SPORTS (can jog)
How is the hypertrophy as compensation for HTN or valvualr disease different than the hypertrophy due to genetic disease?
concentric hypertrophy vs asymmetrical hypertrophy
due to deposition of abnormal protein in the interstitium of the heart
cardiac amyloid
epidemiology of cardiac amyloidosis
older pts
gross pathology of cardiac amyloidosis
enlarged, waxy heart
microscopic pathology of cardiac amyloidosis
intertilial depostion of hyalin smooth eosinaphillic material
** initially in blood vessels
congo red + apple green bifringence
diff than collagen bc it is smooth (not wavy)
What type of amyloid is deposited in cardiac amyloidosis
P, A
**can be target for immuno stain
symptoms of of cardiac amyloidosis
similar to CHF (left and right)
signs of of cardiac amyloidosis
FAMILIAL
JVD kussmauls sign (JVD worse with ?? peripheral edema ascities enlarged tender liver pulmonary rales
~left and right heart failure
Diagnosis of of cardiac amyloidosis
CT
MRI
commonly in multiple other organs (which you can biopsy if they are in failure as well)
ITS FAMILIAL
multiorgan inflammatory disease with may be autoimmune
sarcoidosis
epidemiology of cardiac sarcoidosis
young adults (20s and 30s) AA > caucasians
symptoms pf of cardiac sarcoidosis
non-specific: fatigue, malaise, weight loss etc
+ syncope
AFFECTS LUNGS (dyspnea)
pathology of cardiac sarcoidosis
granulomas in LUNGS, lymph nodes, liver spleen, BM, skin, eyes and HEART
- giant cell and eventual fibrosis
- astroid body
favors the base of the heart and commonly involves conduction system!!
complications of cardiac sarcoidosis
arrhythmias (VT) and sudden death
precipitation of cytoskeletal proteins
astroid bodies (common in sarcoidosis biopsy)
In giant cell or by themselves???
sign of cardiac sarcoidosis
usually have arrhythmias
treatment of cardiac sarcoidosis
steroids and immunosuppresive therapy
cardiac??
non-specific end stage heart disease with dilation and no cause evident
idiopathic cardiomyopathy
Epidemiology of idiopathic cardiomyopathy
middle age with slowly progressive heart failure but with some acute compensation
gross pathology of idiopathic cardiomyopathy
enlarged, soft, flabby heart
all 4 chambers symmetrically dilated
commonly have mural thrombi
microscopic pathology of idiopathic cardiomyopathy
- myocyte atrophy, irregular hypertrophy
- interstitial and perivascualr fibrosis
- or healthy looking
complications of idiopathic cardiomyopathy
mural thrombi and emboli
mitral and tricuspid regurg
arrythmias
sudden death
diagnosis of idiopathic cardiomyopathy
determine that the heart is dilated and then must exclude all other causes of dilation
= diagnosis of exclusion!
BENIGN gelatinous mesenchymal neoplasms of the endocardium
cardiac myxomas
epidemiology of cardiac myxomas
women, rare
Where in the heart do cardiac myxomas form?
left atrium
gross path of cardiac myxomas
pedunculated polypoid soft gelatinous tumor, friable, focally hemorrhagic
microscopic path of cardiac myxomas
? stellate cells
symptoms of cardiac myxomas
non-specific... SOB orthopnea cough, hemoptysis fatigue fever
signs of cardiac myxomas
simialr to mitral stenosis!!
loud first heart sound, diastolic murmur, diastolic tumor plop
mitral regurg w/ holosystolic murmur if the tumor hangs into the LV
complications of cardiac myxomas
mitral obstruction embolization
Tx of cardiac myxomas
surgical excision (curative)
