cardiomyopathy Flashcards
what is restrictive cardiomyopathy?
impaired DIASTOLIC function with relatively preserved contractility
ventricular rigidity impedes ventricular filling ( decreased compliance)
the stiff ventricle only fills with great effort
etiologies of restrictive cardiomyopathy
infiltrative disease: amyloidosis-MCC
sarcoidosis, idiopathic myocardial fibrosis, chemo and radiation, scleroderma, hemochromatosis, etc.
clinical manifestations of restrictive cardiomyopathy
R sided HF symptoms more common (SOB, fatigue, swelling of leg/abdomen), poorly tolerated tacchyarrythmias
physical exam of restrictive cardiomyopathy
Kussmaul’s sign (JVP increasing with inspiration)
stiff inelastic RV–> impaired filling of RV–> causes increased blood flow to back up in venous system
signs of HF
may be an S3 sound present
Diagnostic Studies in restrictive cardiomyopathy
- Chest x-ray: normal heart size, enlarged atria, may have pulmonary congestion/pleural effusion
- EKG- low voltage, may be arrhythmia-low amplitude signal results in small QRS complex
- ECHO: 1)ventricles non dilated with normal thickness,2) marked dilation in both atria, 3)diastolic dysfunction. may also see systolic dysfunction in advanced restrictive cardiomyopathy
dilated atria is common in restrictive cardiomyopathy and can lead to ___
pulmonary hypertension and a-fib and AVNRT
management of restrictive cardiomyopathy
no specific treatment
TREAT UNDERLYING DISORDER
treatment is symptomatic
What is dilated cardiomyopathy?
systolic dysfunction that results in ventricular dilation which can lead to dilated, weak heart
“dysfunction in heart’s ability to contract”
MC age for dilated cardiomyopathy
20-60 yrs
is dilated cardiomyopathy more common in men or women
men
etiologies of dilated cardiomyopathy
- idiopathic-postviral, probably MCC
- viral myocarditis- enterovirus MC, HIV, Lime dx, PB19, chugs dx, etc.
- toxic: alcohol abuse, cocaine, anthrocycluines, radiation treatment
- other: pregnancy, infiltrative autoimmune, metabolic syndrome (thyroid)
clinical manifestations of dilated cardiomyopathy
Heart failure- systolic HF symptoms: S3, fatigue, signs of L or R sided CHF, lateral displaced PMI, may be mitral or tricuspid regurgitation
Embolic events
Arrhythmias
Chest pain on exertion
diagnostic studies for dilated cardiomyopathy
echo: 1)LV dilation (thin ventricular walls), large ventricular chambers, 2) decreased EF, 3) regional or global left ventricular hypokinesis
chest x-ray: cardiomegaly, pulmonary edema, pleural effusion
EKG: sinus tach or arrythmias
management of dilated cardiomyopathy
standard HF treatment
hypertrophic cardiomyopathy is an inherited genetic disorder of ___ hypertrophy.
LV and/or Rv
especially septal
in hypertrophic cardiomyopathy, sub aortic outflow obstruction narrows LV tract secondary to hypertrophied septum and systolic anterior motion of the mitral valve and papillary displacement.
increased systolic anterior motion is seen with what two things?
- increased contractility-> exercise, digoxin, beta agonists
- decreased left ventricular volume–> decreased venous return, dehydration, valsalva maneuver
diastolic dysfunction: stiff ventricular chamber results in impaired _____
ventricular relaxation/filling
clinical manifestations of hypertrophic cardiomyopathy
Dyspnea, fatigue
angine pectoris
syncope: pre-syncope and dizziness- due to inadequate output on exertion
arrhythmias: palpitations, a-fib, v-tavh, v-fib
sudden cardiac death: especially in young people during times of extreme exertion
what type of murmur will you hear on a patient with hypertrophic cardiomyopathy
harsh systolic crescendo-decrescendo murmur
the murmur with hypertrophic cardiomyopathy is best hear where?
left lower sternal border (sounds similar to Aortic Stenosis)
in hypertrophic cardiomyopathy, when performing handgrip, there is increased venous return which causes the murmur to be ___
decreased
in hypertrophic cardiomyopathy, when performing the valsalva maneuver, there is decreased venous return which results in the murmur to be ______
increased
on physical exam of hypertrophic cardiomyopathy, there will usually be no carotid radiation, but there may be ____.
loud S4, mitral regurgitation, S3 or pulses bisfereins
diagnostic studies for hypertrophic cardiomyopathy
echo: asymmetrical wall thickness, especially septal; systolic anterior motion of the mitral valve, small LV chamber size, dynamic outflow obstruction. there may or may not be mitral regurgitation
ekg: LVH, atrial enlargement, anterolateral and inferior pseudo q waves
TTE
chest x ray: cardiomegaly
management of hypertrophic cardiomyopathy
counseling to avoid dehydration and extreme exertion!
- medical: beta blockers 1st line
- surgical: myoectomy
- alcohol septal ablation
- last resort= heart transplant
what is cardiomyopathy?
disease of myocardium resulting in cardiac dysfunction
5 types of cardiomyopathies
- dilated
- hypertrophic
- restrictive
- arrhythmogenic right ventricular cardiomyopathy
- unclassified
3 key histologic features in cardiomyopathy
fibrosis
cellular necrosis
myocyte hypertrophy
physical exam: 5 useful clinical signs to establish presence of CM and severity
- general appearance- cachexia and dyspnea at rest (severe impairment)
- hypotension
- tachycardia
- elevated jugular venous pressure (4cm elevated)
- displaced LV point of maximal impulse (PMI)
useful marker for diagnosis, severity, and prognosis of patients in HF
BNP
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a clinical entity characterized by_______ and a specific ventricular pathology.
ventricular arrhythmias
in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), there is a scarred appearance with fibrous or fibro-fatty replacement of myocardium in the inflow tract, outflow tract, and/or apex of the _____
right ventricle
in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), The RV myocardial scarring initially produces typical regional wall motion abnormalities but later may involve the free wall and become global, producing ______. The tissue replacement can also involve areas of the left ventricle (LV) with relative sparing of the septum
RV dilation
The 5 most common abnormalities on an EKG for cardiomyopathies in general
(1) Q waves (from a previous myocardial infarction)
(2) Diffuse ST-segment abnormalities
(3) LBBB - (or any intraventricular conduction delay)
(4) Atrial fib
(5) Abnormal P waves
biphasic in leads V1 and V2—“left atrial overload”
hypertrophy of the myocardium more than 1.5 cm, without an identifiable cause
defines what type of cardiomyopathy?
hypertrophic cardiomyopathy
hypertrophic cardiomyopathy is what kind of genetic disorder?
autosomal dominant
hallmark of hypertrophic cardiomyopathy
myocardial hypertrophy that is inappropriate and often asymmetric and that occurs in the absence of an obvious inciting hypertrophic stimulus
hypertrophic cardiomyopathy is diastolic or systolic dysfunction?
diastolic
____ is the most common genetic CV disease
Cardiomyopathy
Genetic mutations resulting in HCM 1⁰ involve the cardiac _____ but may also involve the connective tissue matrix
sarcomere
In young adults, ____is the most common cause of sudden cardiac death with exertion*
hypertrophic cardiomyopathy
The classic auscultatory finding for HCM
a crescendo-decrescendo systolic murmur along the left sternal border that increases with the Valsalva maneuver
Almost all cardiac murmurs_____ in intensity during Valsalva -except HCM, so this maneuver is a crucial part of the cardiac examination if HCM is suspected
decrease
The Valsalva maneuver decreases preload, which results in decreased filling of the ____
left ventricle
hand grip increases_____ and decreases_____
hand grip increases ventricular volume and decreases murmur intensity and LVOT gradient
valsalva increases_____ and decreases_____
valsalva increases murmur intensity and LVOT gradient and decreases ventricular volume
differentiate HCM from valvular aortic stenosis (AS) and a subvalvular aortic membrane
In AS – calcified aortic valve w/restricted mobility
In HCM - obstruction occurs below the aortic valve, valve structure and function preserved
what is the standard for Dx of HCM
TTE (Transthoracic Echocardiography)
hypertrophic CM Complications
CHF Arrhythmias Infective mitral endocarditis Atrial fibrillation with mural thrombosis formation Sudden death
