Cardiomyopathy Flashcards
What is the traditional definition of cardiomyopathy?
Dilated cardiomyopathy (ventricles enlarge)
Restrictive (cannot stretch)
Hypertrophic cardiomyopathy (HCM)
Who is hypertrophic cardiomyopathy common in?
Young athletes
What is the WHO/ISFC (international society and federation of cardiology) definition of cardiomyopathy?
Dilated cardiomyopathy (ventricles enlarge)
Restrictive (cannot stretch)
Hypertrophic cardiomyopathy (HCM)
PLUS
Arrhythmogenic right ventricular cardiomyopathy / dysplasia (ARVC/D) (seen in Europeans w/ RV dilation)
Unclassified cardiomyopathies (seen in babies born without enough cardiac tissue)
What are the different classes of cardiomyopathy?
Intrinsic (from heart) vs. Extrinsic (outside of heart, like meds)
Primary vs. Secondary
Ischemic vs. Nonischemic
Is it easy to diagnose the specific subtype of cardiomyopathy?
NO
most just present as heart failure
What is the MCC of cardiomyopathy?
MC involves the LV and adverse events during exertion
Can also be systole and dyastole
What are the symptoms of cardiomyopathy
Pulmonary edema
SOB (orthopnea - SOB when laying flat, paroxysmal nocturnal dyspnea)
Pitting edema
Ascities in liver
back up of blood d/t LV failure
What is the most helpful imaging for cardiomyopathy? What are some others?
Echo!
nuclear imaging, coronary angiography w/ left ventriculography, and cardiac MRI
Is cardiomyopathy permanent?
Can be transient if we treat quickly!
What does systolic dysfunction lead to?
Decrease contraction of LV, leading to decrease BF
As a result, the heart tries to beat harder d/t frank-starling
But this will eventually fail
Systolic dysfunction → decrease in myocardial contractility and reduction in LVEF
Compensatory mechanisms aimed to maintain cardiac output:
LV enlargement resulting in higher stroke volume
Frank-Starling relationship (↑stretch = ↑contractility)
Eventually this process fails and HF develops
What does diastolic dysfunction lead to?
Not enough filling of LV, leading to pulmonary edema, and back up of blood to the right side of the heart
Can you diastolic dysfunction without systolic dysfunction? Vice versa?
You can have diastolic dysfunction without systolic
BUT
Systolic will ALWAYS have dyastolic
so diastolic no matter what!
What does myocarditis lead to?
necrosis
may also lead to myocardial dysfunction and dilated cardiomyopathy (because the immune system attacks it, leading to ballooning)
What are the two pathogenesis of myocarditis?
UNKNOWN
but
Host-mediated: direct cytotoxic effect of the causative agent
Autoimmune-mediated: secondary immune response
What are the 2 main phases of myocarditis?
Acute: First 2 weeks
Myocyte death is a direct result of the causative agent, leading to cell-mediated cell toxicity
Chronic phase:
After 2 weeks
A result of an inappropriate, overactive immune response
What are the viral infectious causes of myocarditis?
Adenovirus
Coxsackie B Virus
Cytomegalovirus
COVID-19
CCCA
Are all common infections, but myocarditis is RARE
T Cs make an M (Myo) next two letters are CA (carditis)
What are some noninfectious causes of myocarditis?
Alcohol
Anthracyclines
Cocaine
If you include infectious causes, the mneomnic is
AACCCC
MC patient demographic of myocarditis?
YOUNG 20-50 yo men
worse outcomes in man as well
What are important questions to ask for infectious myocarditis?
Previously healthy
Acute febrile
SOB
Pleural/pericardial chest pain
palpitations
syncope
depressed LV systolic function
GRADUAL or ABRUPY onset
Why do patients with myocarditis often lead to arrhythmias?
Impaired conduction of one part of the heart leads to another
What heart sound can you hear from myocarditis?
A pleural friction rub that is heard best leaning forward (d/t inflamed heart)
S3 S4 (diastolic dysfunction d/t blood trying to go from the atria to a dtysfunctioning ventricle)
Mitral regurg
Tricuspid regurg
What leads to heart failure?
Volume overload
Apart from an echo (transthoracic), what diagnostics do you order?
EKG: (looking for ST elevations)
Cardiac biomarkers: (elevated troponin - order a cath if elevated and you will see clean coronaries)
CXR: nonspecific (sometimes see pleural perfusion)
Where do you see pulmonary edema oftentimes on xray
CVA angle
What do you sometimes see on EKG of mycarditis?
Isolated PVC (because the ventricle tries to squeeze a second time d/t blood build up and failure to eject)
Other than troponins, what labs do you order for cardiomyopathy?
CRP, ESR (elevated), CBC (eosinophilia), +/- rhematologic workup, antibodies, BNP
BNP over what is a red flag?
100
What imaging can cardio order for 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 100% confirmation of cardiomyopathy? When do you do this?
Endomyocardial biopsy (like a claw that picks up stuck food in your drain)
Cardio’s call for unexplained decompensation
GOLD STANDARD
What are some complications of endomyocardial biopsy?
Need a lot of tissue if you do not biopsy the right part of the heart, can lead to clots and pieces of tissue going to other parts
What is the treatment of myocarditis?
Consult cardiology!
Based on ejection fraction
ACE I (angiotensin increases d/t irritation, and ACE-I can combat this)
BB
NSAIDs (because this is an inflammatory response),
arrhythmias
What is the overview of the MCC of noninfectious myocarditis?
medications, illicit drugs, and toxic substances
What is the treatment of noninfectious myocarditis?
Monitor and remove as long as LVEF is >40%
once HF occurs (<40% EF) cardiology management
What is the MC cardiomyopathy and the MC population?
Dilated cardiomyopathy
3x more common in black patients
Characterized by dilation and impaired contraction
What defines the dilated cardiomyopathy?
Defined by LVEF <40% WITHOUT CAD or valvular disease
HUGE left ventricle
What is the only curative option for dilated cardiomyopathy?
Transplant :(
PRIMARY cause of heart transplant
mortality is 50% at 5 years
What is the MCC of dilated cardiomyopathy? What are some others?
Idiopathic MC
also
excessive alcohol consumption (very common)
Excessive alcohol consumption
Infectious
Genetic
Systemic Disorders
Peripartum
Endocrinopathies
Tachycardia induced (overworked muscle)
Arrhythmia associated
What is an interesting cause of dilated cardiomyopathy?
Lyme disease
What is the genetic component of dilated cardiomyopathy?
Autosomal dominant
20-25%
D = dilated = dominant
What is the peripartium dilated cardiomyopathy presentation and prognosis?
Unclear etiology
Occurs late in pregnancy or early postpartum
Presents as CHF or SCA
Typically normalizes after 2 to 3 months of therapy
What is the etiology mneomnic of dilated cardiomyopathy?
A Bunch of stuff Can Cause Cardiac Dilation
Alcohol abuse
Beriberi (wet)
Cox B
Chronic cocaine use
Chagas disease
Doxorubicin
Presentation of dilated cardiomyopathy
Gradual HF development
PE: rales, elevated JVP, S3 gallop, murmur of mitral or tricuspid regurg, peripheral edema, ascites
Arrhythmias
Tachycardia, pulsus alternans, LBBB
Sudden death possible
How is dilated cardiomyopathy diagnosed?
If dyspnea is present, BNP or NT-proBNP are necessary to determine prognosis and disease severity
Echo!
Excludes valvular disease and confirms ventricular dilation, reduced LV systolic function (and RV systolic dysfunction if applicable), or pulmonary HTN
Possibly radionuclide ventriculography (MUGA), cardiac MRI (cardios call for these)
What is the treatment of dilated cardiomyopathy?
Treat underlying source if known, which commonly resolves LV dysfunction
CHF management
Prevention of SCA (sudden cardiac arrest)
Heart transplant
What is restrictive cardiomyopathy?
nondilated ventricle with impaired filling
don’t lose tissue, but you have hardened tissue
What can cause restrictive cardiomyopathy?
fibrosis or infiltration of the ventricular wall
What kind of dysfunction is MC in restrictive cardiomyopathy and why?
Fibrotic tissue cannot stretch (diastolic dysfunction)
It can contract oftentimes (systolic contraction)
What can cause infiltrative disorders for restrictive cardiomyopathy?
Amyloidosis, sarcoidosis, fatty infiltration
What are the storage diseases of restrictive cardiomyopathy?
Hemochromatosis, Fabry disease¹
What is the MCC of restrictive cardiomyopathy in the US?
Radiation = Restrictive = R
also chemo, carcinoid heart disease, hypereosinophilic syndrome
What is the diagnosis and treatment of restrictive cardiomyopathy?
Diagnosis
Echo or cardiac MRI will assist in diagnosis
Endomyocardial biopsy may be considered
Treatment
Treat underlying cause if known
Reduce pulmonary and systemic congestion
with diuretics (remember - diastolic dysfunction)
What is hypertrophic cardiomyopathy and what causes it?
Seen in kids
Genetic predisposition d/t enlarged tissue
NOT caused by
pathologic loading conditions, such as HTN and valvular disease (AS)
Where is the buildup of myocytes in obstructive hypertrophic cardiomyopathy ?
Intraventricular septum
Hypertrophy can lead to mechanical block of aortic valve which is the issue
if it is towards the apex, it is without obstruction and less worrisome becaause it does not block the aortic valve
How can excerise lead to problems with hypertrophic cardiomyopathy?
Increase demand of blood
heart pushes harder against aortic valve
less perfusion of blood to brain
ALOC
passing out
death
What can hypertrophic cardiomyopathy lead to?
May lead to LV outflow obstruction, myocardial ischemia and/or mitral regurgitation
Presentation
Fatigue, chest pain, CHF, syncope, SCA
Carotid pulsus bisferiens d/t mimicked aortic stenosis
Increased risk for arrhythmias (afib, SVT, and VT/VF) and SCA
Murmur to listen for on sports physicals
Mid-systolic, harsh, 3rd and 4th intercostals, louder with valsalva, quieter with squatting
What is the heart sound that changes with position of hypertrophic cardiomyopathy?
TEST QUESTION
Murmur to listen for on sports physicals
Mid-systolic, harsh, 3rd and 4th intercostals, louder with valsalva, quieter with squatting
What is the diagnosis of hypertrophic cardiomyopathy and the diagnostic modality of choice?
EKG may demonstrate LVH pattern
Echocardiogram is the diagnostic modality of choice
LV wall >1.5 cm thick (shows hypertrophy)
What is the managemnt of hypertrophic cardiomyopathy?
Avoid volume depletion
Activity restriction
Beta-blockers or Verapamil (helps relax contractility)
Avoid diuretics and vasodilators
Septal myectomy or alcohol septal ablation (kills off tissue so that you can
Why do you not want to use diuretics in hypertrophic cardiomyopathy
decreases preload! Problem is not getting enough blood
what is the screening protocol for hypertrophic cardiomyopathy?
Screening of 1st degree relatives (if father had it for example)
Annual echo until age 20 and then Q5 yrs
What is ischemic cardiomyopathy?
TISSUE DIES
What dysfunction does ischemic cardiomyopathy
Characterized by systolic dysfunction
Can be transient or permanent
Predominantly affects LV, but can involve the RV or both ventricles
Presentation
CHF (edema, dyspnea, JVD)
What is the MCC of heart failure in the US and what can cause it?
Ischemic cardiomyopathy
Results from death/damage/hibernation of myocardium d/t reduced O₂
Typically results from CAD, but can be from any source of ischemia
Cocaine, vasospasm, thrombus
What do you see on an EKG for iscehmic cardiomyopathy?
Possible Q waves on EKG
What is the modality of choice for ischemic cardiomyopathy and what will it show?
Start with Echo → decreased LVEF (because some of the heart does not work), regional wall motion abnormality
Coronary angiography recommended, especially if LV dysfunction cause unknown
What is the treatment of ischemic cardiomyopathy?
Revascularization (PCI or CABG)! For acute ischemia/infarction
Appropriate CHF management (stay tuned!)
If you are unsure if a heart is ischemic or hibernating, what can you order?
Consider nuclear viability study to determine if myocardial dysfunction is due to scarring or hibernating myocardium
What prevents SCA (sudden cardiac arrest) if ischemic cardiomyopathy?
External wearable defibrillator
Implanted cardioverter defibrillator
what is the management of ischemic cardiomyopathy
cardio rehab +External wearable defibrillator
Implanted cardioverter defibrillator
What is arrythmognic right ventricular cardiomyopathy?
Characterized by ventricular arrhythmias and a specific myocardial pathology
RV free wall myocardium is replaced by fibrous/fatty tissue, producing RV dilation
Leads to RV function abnormal
Important cause of sudden death in young adults
More prevalent in Europe; rare in US
What does arrythmognic right ventricular cardiomyopathy present as?
Presents with chest pain, palpitations, syncope, SCA
Diagnose with echo and cardiac MRI
Management
Manage systemic congestion with diuretics
Manage ventricular arrhythmias with antiarrhythmics, ablation or ICD
What is Left Ventricular Noncompaction?
Congental
not enough cardiac tissue
present with CHF symptoms
sudden cardiac death
consider a transplat right away
loose muscular tissue on imaging
What is an unclassified cardiomyopathy aka?
Stress-Induced Cardiomyopathy
AKA broken heart syndrome and Takotsubo cardiomyopathy
What causes unclassified cardiomyopathy and what does it present as?
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
What is the MC demographic of unclassified cardiomypathy?
Primarily occurs in postmenopausal women
What do you see on echo or LV angiography of stress-induced cardiomyopathy (aka broken heart syndrome)
Characterized by LV apical ballooning on echo or LV angiography (systolic dysfunction of the apex and/or mid segments)
What do you treat unclassified cardiomyopathy?
Almost all patients recover in a few weeks
Treat with beta blockers for at least 1 yr to try and prevent CV events
When putting together all of the information of cardiomypathy, what is important to keep in mind?
They all present with HF
Look for the differences!
