cardiomyopathies Flashcards
according to dr. oaks, what is ischemic cardiomyopathy?
heart failure secondary to coronary artery disease-not a heart muscle problem at all
what is primary cardiomyopathies? (CMP)
soley or predominantly confined to the heart muscle
secondary cmp?
myocardial involvement is part of a generalized systemic disorder
what is a type of genetically acquired P CMP?
hypertrophic
what is are types of mixed PCMP?
dilated and restrictive
what are 2 types of acquired PCMP?
inflammatory (myocarditis) and peripartum
also stress-provoked “tako-tsubo”
types of secondary cmp?
infiltrative (amyloidosis)
toxicity, endocrine (DM, thyroid, phemochromocytoma, acopmegaly)
scleroderma, polyarteritis nodosa,
beriberi (tiamine def.)
examples of infections dz that can cause cardiomyopathy?
- diptheria
- Strep (rheumatic fever)
- gonococcal, pneumococcla tetanus, pertusses, typhoid fever
syphilis, lyme, parvovirus, HSV 6, influenza, all of them
etiologic classifications of cardiomyopathy?
genetic, metabolic, nutritional, hypokalemia, hematology, neoplastic dz, deposits (amyloid), neurologic/ neuromuscular) endomyocardial, inflammatory (scleroderma, sle, ankylosing spondylitis,
giant cell myocarditis
what can cause dilated cardiomyopathy?
- myocarditis
- kawasaki dz
- eosinophilic
- viral
- drugs
- pregnancy
- endocrine
- nutritional
- EtOH
- endocrinopathies
what population is DCMP more common?
men, especially black men
what are some causes of RCMP?
amyloidosis, scleroderma, carcinoid heart dz, metastic cancer. radiation, drugs
how is P HCMP characterized?
by a hypertrophied (particularly of the septum), non dilated and small LV, systolic anterior mitral motion, and diastolic dysfunction
what is systolic anterior mitral motion?
anterior movement of the mitral valve, either leaflets, during systole
maximal motion occurs before maximal posterior wall contraction, approximately 2/3rd of the way between systole
what causes HCMP?
variety of gene mutations encoding for contractile proteins of the cardiac sarcomere (at least 100 of them)
what population is more likely to get H CMP?
ppl of asian decent;
hypertrophic CMP in the elderly is a distinct form
what is a severe complication of HCMP?
sudden cardiac death!!!!
-occurs in pts younger than 30 yo at a rate of 2-3% yearly
what are the most common symptoms of HCMP?
dyspnea and angina
-syncope and arrhythmias are common
- can be asx
- may only manifest during exercise (increase in catecholamines)
- sudden death may be the initial presentation
what may physical exam reveal on a pt with HCMP?
- sustained PMI or triple apical impulse
- S4
- variable systolic murmur
- bisferiens carotid pulse
- JVD w/ a prominent “a” wave
what is bisferiens carotid pulse?
A double pulse is felt due to the backflow of blood in early diastole. The first carotid pulse felt is normal systole, while the second is actually early diastolic due to the regurgitating blood.
- Two pulsations are detected in systole. The first is caused by the pressure increase related to left ventricular ejection. The second systolic pulsation is caused by either arterial recoil reflected from the periphery, or early diastolic from the backflow of the regurgitated blood.
what is the most common CMP?
HCM
what is most common cause of sudden cardiac death in the young?
HCM
how is HCM transmitted?
its autosomal dominant
what is the PP of HCM?
-diasytolic dysfunction, LV outflow tract obstruction, mitral regurg, myocardial ischemia, and arrhythmia
why does diastolic dysfunction occur in HCMP?
-occurs secondary to the very “stiff” LV requireing high LA pressures to overcome the “stiffness”
why do patients with HCM become more symptomatic overtime?
mitral valves become incompetent, and pts become more symptomait and LV becomes ischemic
what cna HCMP lead to overtime?
heart failure
why do you see arrhythmieas with H CMP?
most likely due to myocardial ishemia
diagnostic studies for HCMP?
- EKG: nonspecific St/T wave changes, exaggerated septal Q waves, and LVH
- Echo: LVH, asymmetric septal hypertorphy, small LV, and diastolic dsyfuction
- can also get myocardial perfusion studies, cardiac MRI, and cardiac catherization
treatment of HCM?
-screen all first degree relatives, refrain from competitive athletics (moderate levels are ok)
medical tx of HCM?
BB or CCB
disopyramide is used for its nega antiarrhytmic affects
-may need dual-chamber pacing, implantable defibrillators, or Mitral valve replacement
what are some surgical interventions for HCM?
alcohol septal ablation (heart wont grow back)
what is most common form of CM according to PANCE?
- dilated (95%)
what is D CMP associated with?
- reduced strength of ventricular contraction that results in the dilation of the left ventriicle
what is the most frequent indication for heart transplant?
dilated CMP
what are some causes of D CMP?
genetic abnormalilites, excessive alcohol consumption, postpartum state, chemotherapy toxicity, endocrinopathies, and myocarditis
-may be idiopathic
how cna DCMP b-e transmitted
- autosomal dominant or recessive
- x’linked or matrilinear
what are clinical features of dcmp?
signs and symptoms of left or biventricle heart failure
- arrhythmias
- thromboembolic complications
- sudden death
what ist he most common presentation of DCMP?
dyspnea
what may you see on PE in a pt with D CMP?
- S3
- pulmonary crackles (rales)
- increased JVD
what is a work up for DCMP?
- exclude CAD, valvular heart dz,
- echo, stress tes, cardiac cath, endomyocaridal bx
- r/o nutritional deficencies, endocrine disorders,
what will a EKG show in dilated cardiomyopathies?
- nonspecific ST/ T wave changes
- conduction abnormalities
- ventricular ectopy
what would a CXR show in DCMP?
-long standing: cardiomegaly and pulmonary congestions
what would an echo show in DCM?
- LV dilation and dysfunction w/ high diastolic pressures and low CO
LV EF less than 40%
-impaired contraction of the ventricles
what are some examples of primary acquired cardiomyopathies?
- myocarditis (viral)
- peripartum CMP
- stress provoked
- tachycardia induced
- infants born to insulin dependent diabetic mothers
secondary acquired cardiomyopathies
Ischemic Valvular Hypertensive Infectious Anthracycline Postpartum Alcohol Chagas
myocarditis?
inflammation of the cardiac myocytes and is apparent on endomyocardial biopsy
**common cause of dilated CM
what is the most common cause of myocarditis?
-viral:
coxsackievirus, influenza, adenovirus, CMV, HIV
what ist he PP of myocarditis?
infx may directly damage the myocyte or elicit immune responses that damage the myocyte
what are other causes of myocarditis?
- toxoplasma godii, leptospirosis,
- lyme
- group A strept
- Chagas: parasite dz
what is the most common cause of CHF in Central and south america?
Chagas dz
what is eosinophilic myocarditis?
allergic rxn to various drugs, mos common abx
what is giant cell myocarditis?
is a rare but aggressive disease that is typically progressive. It occurs in young adults and only 20 % survive one year unless they are transplanted. Giant cell myocarditis may recur in the transplanted heart
what is takotsubo CMP?
stress-induced; after major catecholamine discharge and results in hypocontractility of the left ventricular apex; presentation may be indistuguishable from an MI
clinical features of takotsubo?
restrosternal chest pain
diagnostic studies for takotsubo?
-may see EKG changes and mild cardiac enzymes suggestive of an MI
what will cardiac catherization reveal in takostubo?
- hypocontractility of the left V
- patent coronary arteries
tx of takostubo?
supportive
- avoid inotropes
- most pts return to baseline in 2 mnths
what is restrictive CMP?
non-dilated, non hypertrophied ventriles with impaired ventricular filling. systolic function remains normal at least early in the dz
(heart just cant move: fibrosis)
what can cause R CMP?
fibrosis or infiltration of the ventricular wall bc of collagen-defect dz,
-most commonly amyloidosis, radiation, postoperative changes, DM, and endomyocardial fibrosis
what must R CMP must be differentiated from ?
constrictive pericarditis
what are sx R CMP
right sided heart failure in the absence of cardiomegaly (advanced dz)
- decreased exercise intolerance
- pulmonary htn, low pulse volume due to reduced stroke volume and tachycardia
RCMP secondary to amyloidosis
amyloid: interstitial deposition of abnormal protein fibrils in the heart, kidney, liver, and nerves
what is the most common condition that produces amyloidosis?
disorder of plasma cells and the production of immunoglobulin light chains
how is the diagnosis of amyloidosis made?
-serum and uring electrophoresis or endomyocardial biopsy
what will a CXR show in R CMP?
mild to moderately enlarged cardiac silhouette
what is the key to dx R cmp?
- echo
- cardiac emir may sho normal or midly reduced lvfunction
tx of restrictive CMP?
diuretics may help severe dz
what is peripartum cardiomyopathy?
- virchow in 1870
- most common in obese multiparous black women older than 30
- pts rpresent with heart failure in last trimester or first 6 mnths after deliver
- possibly viral or autoimmune causes
ischemic cardiomayopathy?
refers to heart failure secondary to multiple myocardial infarctions and coronary artery disease
*not a true cardiomyopathy)
valvular cardiomyopathy?
seen with left sided regurgitant lesion (AI and MR)
- must be distinguished from the mitral regurg that is commonly associated with any dilated cardiomyopathy
what chemo meds can cause CMP?
anthracyclines: include doxorubicin, daunorubicin and epirubicin
cyclophosphamide (acute myocarditis)
-trastuzumab
