Cardiomyopathy - Exam 2 Flashcards
What is cardiomyopathy? What are the 5 different types?
Disorders characterized by morphologically and functionally abnormal myocardium in the absence of any other disease that is sufficient, by itself, to cause the observed phenotype.
Restrictive CM
Dilated CM
Hypertrophic CM
Arrhythmogenic RV CM and dysplasia
“Unclassified” Cardiomyopathies
What are the classic 3 types of cardiomyopathy?
Restrictive CM
Dilated CM
Hypertrophic CM
What is systolic dysfunction? What happens next? Eventually, what will develop?
decrease in myocardial contractility and reduction in LVEF
at the beginning the heart will compensate to maintain cardiac output but eventually the process fails and heart failure develops
What is the Frank-Starling relationship? When is it commonly seen?
Frank-Starling relationship (↑stretch = ↑contractility)
when the Left ventricle enlarges resulting in a higher stroke volume
What is diastolic dysfunction a result of?
cardiac dysfunction d/t abnormal LV relaxation and filling accompanied by elevated filling pressures
systolic dysfunction may also be present but it does NOT have to be present
diastolic dysfunction is often underestimated/missed
However, if systolic dysfunction comes first, _____ dysfunction follows
diastolic
What is myocarditis? What are 2 different causes? What does it result in?
An inflammatory, infiltrative process involving the myocardium
caused by infectious and noninfectious conditions
Results in necrosis and/or degeneration of myocytes
May lead to myocardial dysfunction and dilated cardiomyopathy
aka long term inflammation leads to scarring, which leads to necrosis
What is the MC pt population? **What is the MC cause in North America?
men aged 20-50
**viral: coxsackie B
What are the 3 common non-infectious causes of myocardititis? At what point do these patients need to be sent to cardiology?
medications, illicit drugs, and toxic substances
once LVEF drops below 40% or s/s of HF present
What are the 2 main mechanisms for myocarditis?
Host-mediated: direct cytotoxic effect of the causative agent
Autoimmune-mediated: secondary immune response
think mass inflammation
What are the 2 different phases of myocarditis?
acute and chronic
acute: first 2 weeks of disease
chronic: after 2 weeks
What is happening in the acute phase of myocarditis?
Myocyte death is a direct result of the causative agent, leading to cell-mediated cell toxicity
What is happening in the chronic phase of myocarditis?
A result of an inappropriate, overactive immune response
What are the top 3 infectious causes of myocarditis?
adenovirus -> COVID 19
coxsackie B virus
cytomegalovirus
What are the top 3 cardiotoxins that lead to myocarditis?
alcohol
anthracyclines (type of abx Daunorubicin, doxorubicin, and epirubicin)
cocaine
What is a typical myocarditis presentation?
Typically develops days to a few weeks after onset of acute febrile illness / respiratory infection if infectious cause with no known underlying cardiac pathology present
What are the classic symptoms of myocarditis?
SOB, pleural/pericardial chest pain that can be positional, +/- fever, chills
can happen gradual or abrupt with decreased CO, shock and severe LV systolic function
What will you hear on heart auscultation on a pt that has myocarditis?
pericardial friction rub, tachycardia, S3 or S4, murmur of mitral or tricuspid regur if ventricular dilation is severe
What 5 things would you want to order for a pt with suspected myocarditis?
EKG
cardiac biomarkers
labs
CXR
Echo: ALWAYS DONE!!!!
Bx: but not always done because it is VERY risky
When would you order a myocardial bx?
if the cause is unknown, other treatments have been tried and nothing is working AND it would change the outcome
should only be obtained if there is a high probability that results will change patient management
According to the AHA/ACC, when is a biopsy recommended?
What would a Cardiac MRI (CMR) tell you? Will it confirm the dx of myocarditis?
Cardiac MRI (CMR): helps assess extent of inflammation, myocyte necrosis and scarring, ventricular size / shape changes, wall motion abnormalities, and pericardial effusion
Can SUGGEST myocarditis, but sensitivity and specificity are limited and time-dependent
What is the tx for myocarditis? What is the ideal HR for these pts?
LVEF <40% → ACE-I, BB
Myopericardial Chest pain → NSAIDs (+/- colchicine)
HR less than 70
**______ is the MC type of cardiomyopathy. What is the MC type of pt? What is the mortality?
Dilated Cardiomyopathy
black pts
mortality is 50% at 5 years
What is dilated cardiomyopathy characterized by? What is their LVEF?
Characterized by dilation and impaired contraction of one or both ventricles, predominantly the LV
Defined by LVEF <40% without CAD or valvular disease
_____ is the #1 reason for heart transplant. What is the underlying cause?
dilated cardiomyopathy
cause is unknown
What is Chagas disease?
Caused by protozoan infection, Trypanosoma cruzi
Leading cause of DCM in Central and South America
How does Lyme Disease manifest in cardiomyopathy?
Usually manifests as conduction abnormality
May cause myocardial dysfunction due to myocarditis
dilated cardiomyopathy is genetic, how is it inherited?
Autosomal dominant predominantly
Inherited syndromes, such as muscular dystrophies, hemochromatosis, thalassemias
How will dilated cardiomyopathy present?
s/s of right and left HF
rales, elevated JVP, S3 gallop, murmur of mitral or tricuspid regurg, peripheral edema, ascites
arrhythmias: pulsus alternans
What diagnostics do you want to order if you suspect dilated cardiomyopathy? Which one is gold standard?
BNP or NT-proBNP
**Echocardiogram- gold standard
consider radionuclide ventriculography (MUGA) and cardiac MRI
Why do you need to order a BNP or NT-proBNP? What does it tell you?
are necessary to determine prognosis and disease severity - done if SOB/DOE is present
BNP gets released when the body has too much fluid, so it correlates with how much fluid is in the body
What is the tx for dilated cardiomyopathy?
treat underlying source
CHF managment
prevent sudden cardiac arrest
heart transplant
implant defibrillator if EF is less than 35%
When do you need to implant a defibrillator?
when EF is less than 35%
defibrillator will shock the heart to keep it in a normal rhythm
What is restrictive cardiomyopathy characterized by? What is it caused by? more likely to affect diastolic or systolic? atrial or ventricle?
nondilated ventricles with impaired filling
Caused fibrosis or infiltration of the ventricular wall
Causes diastolic dysfunction
lead to moderate to severe biatrial enlargement
aka crunchy and thick, atria are trying to push into a stiff ventricle
What are some main etiologies of restrictive cardiomyopathy?
Infiltrative disorders: Amyloidosis, sarcoidosis, fatty infiltration
storage diseases: Hemochromatosis, Fabry disease
radiation, chemo, carcinoid heart disease, hypereosinophilic syndrome
How will restrictive cardiomyopathy present?
RIGHT sided HF (diastolic, thinks backs up to the body)
JVD
Edema
Ascites
Hepatic enlargement
How do you dx restrictive cardiomyopathy?
Echo or cardiac MRI will assist in diagnosis
Endomyocardial biopsy may be considered
What is the tx for restrictive cardiomyopathy?
treat underlying cause if known
reduce pulm and systemic congestion with diuretics
What are the 4 underlying processes that need to be controlled for heart failure/dilated/restrictive cardiomyopathy management? How do you control each process?
What kind of pt is most likely to present with hypertrophic cardiomyopathy? Why is it a major problem?
young athlete that presents with cardiac arrest
thick wall blocks the aortic valve
What is the underlying root cause of hypertrophic cardiomyopathy? What is it characterized by? What does it lead to?
Caused by mutations of sarcomere genes
Characterized by LV hypertrophy NOT caused by pathologic loading conditions like HTN or AS
Leads to LV outflow tract obstruction and impaired filling leading to decreased systemic blood flow!!
How is Hypertrophic Cardiomyopathy inherited? What septum is more likely to be involved?
Autosomal-dominant trait with variable penetrance
Interventricular septum is commonly more prominently involved
What are the 3 key symptoms for hypertrophic cardiomyopathy? ______ due to mimicked aortic stenosis
Syncope, Angina, Dyspne
Carotid pulsus bisferiens : a double pulse in the carotid artery that’s characterized by two peaks separated by a dip in the middle
What murmur is associated with Hypertrophic Cardiomyopathy? Where do you need to listen to it?
Mid-systolic, harsh, louder with valsalva, quieter with squatting
3rd and 4th intercostals
Why is the mid-systolic harsh murmur louder with valsalva and quieter with squatting?
louder with valsalva due to the decreased blood flow
quieter with squatting because the heart is stretching out and getting the larger septum out of the way
What is noted on the EKG for hypertrophic cardiomyopathy? What is the diagnostic modality of choice? How thick does the LV wall need to be?
EKG may demonstrate LVH pattern
Echocardiogram is the diagnostic modality of choice
LV wall >1.5 cm thick
**What is the management for hypertrophic cardiomyopathy?
Avoid volume depletion: LOTS of water
Avoid diuretics and vasodilators
**Activity restriction
**Beta-blockers or Verapamil (helps relax contractility)
Septal myectomy or alcohol septal ablation
Screening of 1st degree relatives
How often due to first degree relatives need to be screened for hypertrophic cardiomyopathy?
Annual echo until age 20 and then Q5 yrs
______ is the MC cause of HF in the US. What are 4 common causes?
Ischemic Cardiomyopathy
CAD
cocaine
vasospams
thrombus
Ischemic Cardiomyopathy is characterized by _______. Mainly affects the ____ but can involve ____. How does it present?
systolic dysfunction
LEFT ventricle but can involve the right or both ventricles
presents like CHF
What are the diagnostic things you would need to order in ischemic cardiomyopathy? What will each show?
EKG: possible Q waves
CXR: may have pulm edema
Echo: decreased LVEF and/or regional wall motion abnormalities
coronary angiography: recommended!! especially if LV dysfunction cause is unknown
What is ischemic cardiomyopathy management?
Revascularization (PCI or CABG)! For acute ischemia/infarction
Consider nuclear viability study to determine if myocardial dysfunction is due to scarring or hibernating myocardium
manage the CHF
How do you prevent sudden cardiac arrest?
External wearable defibrillator
or implanted defibrillator
What is Arrhythmogenic Right Ventricular Cardiomyopathy characterized by? What is the underlying cause?
Characterized by ventricular arrhythmias and a specific myocardial pathology
inherited, autosomal dominant
What is happening in Arrhythmogenic Right Ventricular Cardiomyopathy? What does it lead to?
RV free wall myocardium is replaced by fibrous/fatty tissue, producing RV dilation and myocardial thinning
Leads to abnormal RV function and possible LV dysfunction later on
Arrhythmogenic Right Ventricular Cardiomyopathy can lead to ____ in young adults. What is the mean age of presentation?
can cause sudden death in young adults
Mean age of presentation is 30yo
What is the presentation of Arrhythmogenic Right Ventricular Cardiomyopathy? What is the highlighted symptom? What is the management?
palpitations syncope, SCA, chest pain, dyspnea
management:
manage CHF symptoms
antiarrhythmics, ablation, or ICD for vent arrhythmias
heart transplant
What is the MC presenting arrhythmia in Arrhythmogenic Right Ventricular Cardiomyopathy? What diagnostic tests should you order?
MC presenting arrhythmia is VT with LBBB pattern
echo and cardiac MRI
What is Left Ventricular Noncompaction? How does it present?
Rare congenital cardiomyopathy that results from altered myocardial wall due to intrauterine arrest of compaction of the loose interwoven meshwork
CHF, thromboembolism, and ventricular arrhythmias occur
How do you diagnose Left Ventricular Noncompaction? What is the tx?
Echo to start; Cardiac MRI to confirm
Consider cardiac transplant
What is Stressed Induced Cardiomyopathy? What is the MC pt?
Causes an ACS (even STEMI) in the absence of critical CAD, due to a high catecholamine surge a result of intense psychological or physical stress
Primarily occurs in postmenopausal women
How do you diagnosis stress induced cardiomyopathy? What is the tx?
Characterized by LV apical ballooning on echo or LV angiography (systolic dysfunction of the apex and/or mid segments)
Almost all patients recover in a few weeks
Treat with beta blockers for at LEAST 1 YEAR to try and prevent CV events
What are 2 other names for Stressed Induced Cardiomyopathy?
broken heart syndrome and Takotsubo cardiomyopathy
At what point is stenosis in heart vessels considered significant?
70% and greater is considered significant stenosis
What is this? What cardiomyopathy is it associated with?
LV apical ballooning on echo or LV angiography
Stressed Induced Cardiomyopathy
