Cardiomyopathies Flashcards
What is cardiomyopathy
Myocardial disorder in which THE HEART MUSCLE IS STRUCTURALLY AND FUNCTIONALLY ABNORMAL in the absence of coronary artery disease, hypertension, valvular disease, and congenital heart disease
What are the three main types of CM
Hypertrophic
Dilated
Restrictive
Dilated cardiomyopathy pathophysiology DCM
Symmetric left ventricular LV dilation
Impaired LV systolic function
Can involve the right ventricle RV
Not caused by CAD, valvular disease or HTN
DCM presentation both symptoms and possible PE findings
Symptoms: EXERTIONAL INTOLERANCE WITH SOB, FATIQUE Chest pain Palpitations, presyncope/syncope Peripheral edema Abdominal pain
Posible PE findings: Mitral/tricuspid regurg murmur S3 gallop Increased jugular venous pressure Basal crackles
Etiologies of DCM
IDIOPATHIC Familial Infectious Peripartum Alcoholism Drugs [tachycardia-mediated Endocrine dysfunction Nutritional deficiencies
What is the most common etiology of DCM
Idiopathic DCM up to 50% (no identifiable cause
Primary indication for cardiac transplantation
Familial DCM criteria and important points
(+) FH of DCM in 2+ relatives
Seen >= 50% of patients with idiopathic DCM
Cardiac protein antibodies present in 30%
Infectious DCM important points
Myocarditis= inflammatory process 2 degree to infectious or non-infectious causes
Viral-most common in US
Parasitic (changes DZ) most common cause of DCM in Latin America
Present w/ viral sxs and/or heart failure sxs
Endomyocardial biopsy is GOLD STANDARD for diagnosis
Peripartum DCM risk factors and presentation
>30 African descent Multiparous H/o preeclampsia, eclampsia, postpartum HTN Maternal cocaine abuse >4wks use of oral tocolytic
Presentation
Generally 36 wks of gestation - 5 mos. post-partum
Dyspnea, orthopnea, PND, pedal edema, cough, hemoptisis
*diagnosis: Inc. BNP, Echo= EF <45
Alcoholic DCM risk factors, presentation, prognosis
Risk factors: 6 drink/day for 5-10 years
Presentation: HF symptoms, signs of long-term EtOH use
Prognosis: abstinence can lead to significant improvement in heart failure
If continue EtOH = poor prognosis, mortality 50% at 3-6 years
Cocaine-related DCM
Toxic effects on myocardium
Causes myocarditis
Chemotherapy-induced DCM
Anthracyclines
-including adriamycin (doxarubicin) -> breast CA, sarcoma and lymphoma
Early detection improves outcomes
Can present with HF symptoms
*obtain baseline echo to measure EF and continue to monitor
Tachycardia mediated DCM cause and treatment
Caused by sustained rapid ventricular rates (130-200)
May follow any type of chronic cardiac arrhythmia
Treatment of tachycardia/arrhythmia reverses myocardial dysfunction
Endocrine dysfunction DCM
Diabetes
-initially myocardial fibrosis and diastolic dysfunction(restrictive CM) later stages systolic dysfunction
Thyroid dysfunction
-thyroid hormone can alter preload, afterload, heart rate and contractility
Pheochromocytoma
-adrenal tumor secreting catecholamines
*cardiac dysfunction usually can be reversed by correction of endocrine disorder
Nutritional deficiencies DCM
Thiamine B1 deficiency
-consider in alcoholic or malnourished pt (diarrhea, dialysis, poor intake)
Carnitine deficiency
-impairs oxidation of fatty acids, resulting in lipid accumulation in myocete cytomplasm
*cardiac dysfunction usually can be reversed by correction of nutritional deficiency
DCM diagnostics
EKG:
-non-specific changes -> sinus tachycardia, left bundle branch block, atrial/ventricular arrhythmia
CXR:
-cardiomegaly (globular heart), pleural effusions
Labs:
-BNP, TSH, CBC, CMP, (+/-) endomyocardial biopsy
ECHO***
- dilated ventricle, reduced LV systolic function
- exclude unsuspected valve disease
DCM management
Treat or remove underlying cause
- d/c alcohol or cocaine use
- evaluate chemo dosing
Lifestyle modification
Close f/u
DCM med management
ACE inhibitors **1st line** -reduce afterload by vasodilation, BP reduction Diuretics -used for volume overload/reduce preload B-blockers -decrease oxygen demand Digoxin (+) inotrope Failure to respond to first line treatments Used in afib, fatigue, dyspnea
DCM management
Antiarrhythmics
-amiodarone if afib
Anticoagulation
-if afib, EF <30%, h/o thromboemboli, presence of mural thrombi (on wall of ventricle)
IMPLANTED CARDIOVERTER DEFIBRILLATOR
CARDIAC TRANSPLANTATION
Hypertrophic cardiomyopathy pathophysiology
LEFT VENTRICULAR HYPERTROPHY (LVH) IN THE ABSENCE OF A CAUSE
- diastolic filling abnormalities
- small LV cavity
Leading cause of sudden cardiac death (SCD) in young persons
HCM etiology
Familial
What is obstructive HCM
Factors WORSENING left ventricular outflow tract (LVOT) obstruction:
Tachycardia Hypovolemia Standing Valsalva \+ inotropes Diuretics Vasodilators
My words: mitral valve is close to septum and gets stuck. This causes regurg, and obstructed aortic outflow
Can be asymptomatic until exertion
HCM presentation
Asymptomatic
Found on echo
Death
Symptomatic Dyspnea on exertion Exertional angina Fatigue Pre-syncope, syncope Palpitations
HCM presentation continued
POTENTIAL PE FINDINGS W/ LVOT OBSTRUCTION
Brisk, bifid carotid pulse
Audible S4
Crescendo-decrescendo systolic ejection murmur
INCREASES with valsalva and standing
DECREASES with squat and isometric handgrip
HCM diagnostics
EKG
- LV hypertrophy w/ strain
- increased incidence of both supraventricular and ventricular arrhythmias
- ECHO*
- INCREASED LV WALL THICKNESS >= 15mm is diagnostic
- asymmetric septal hypertrophy
- systolic anterior motion of mitral valve
Stress/exercise echo:
-risk stratification assess LVOT
Sudden cardiac death risk factors
Prior sustained ventricular tachycardia (VT)
Non-sustained VT
Young age <30 years
Family history of sudden death due to HCM
Massive LVH (wall thickness >= 30mm)
Unexplainable syncope, especially in young patients
Brady arrhythmias
HCM and physical activity restriction
Avoid most competitive sports
Avoid high-intensity sports
HCM management
Asymptomatic: ->no prophylactic therapy
Symptomatic:
BETA BLOCKERS BB or NON-DIHYDROPYRIDINE CALCIUM CHANNEL BLOCKERS (Nd-CCB)
- (-) inotrope
-may improve chest pain and dyspnea
Diuretics w/ caution if significant LVOT obstructin and symptoms
Manage arrhythmias
ICD implantation if high risk for SCD, sustained ventricular tachyarrhythmia
Surgery for HCM
Indicated for SYMPTOMATIC LVOT obstruction w/ advanced heart failure refractory to medical therapy
Surgical septal myectomy
EtOH ablation
Mitral valve surgery (mitral valve replacement or plication)
Restrictive cardiomyopathy RCM pathophysiology
Non-dilated, rigid ventricles Diastolic dysfunction Restrictive filling Preserved systolic function HFpEF R and L atrial enlargement
RCM most common Etiology
Infiltrative
- Amyloidosis -> most common
- sarcoidosis
Storage diseases
-hemochromatosis
Glycogen storage disease
Other
Idiopathic
Scleroderma
Fibrosis 2nd chemotherapy, radiation
RCM presentation symptoms and PE
Symptoms:
Right > left heart failure
Edema, abdominal discomfort, ascites
Angina, syncope, dyspnea
PE
*S3 gallop not heard in constrictive pericarditis
Prominent JVP
+/- kussmaul’s sign (distensión of jugular vein during inspiration)
Possible MR/TR murmur (regurg)
Cardiac amyloidosis presentation and diagnostic studies
Presentation:
Elevated JVP
Hepatomegaly, ascites
Periorbital purpura with heart failure is pathognomonic (late finding)
Diagnostic studies:
Echo -> inc left ventricular wall thickness with diastolic dysfunction, later right ventricular diastolic dysfunction develops
Endomyocardial biopsy —> definitive
RCM diagnostic
EKG- non-specific findings
ECHO
Bi0atrial enlargement
Normal to small ventricular cavity (amyloid may result in wall thickness)
Nml Ef, abn filling
CARDIAC MRI
Chamber size, wall thickness, presence/absence of infiltration, inflammation, fibrosis
Endomyocardial biopsy
Diagnostic
RCM Managment
TREAT UNDERLYING CAUSE LOW DOSE LOOP DIURETICS - decrease systemic and pulmonary congestion Heart transplant consideration Poor prognosis
What is takotsubo cardiomyopathy (TCM)
“Ballooning”
TRANSIENT LV systolic and diastolic dysfunction in the absence of attributable coronary artery disease (CAD)
- preceded by emotional or physical trigger
- typically occurs in postmenopausal women
TCM presentation
-abrupt onset
Present like acute coronary syndrome
SUBSTERNAL CHEST PAIN, dyspnea, syncope
Symptoms of heart failure
TCM diagnostics
Labs: increased troponin, BNP
EKG: St-segment elevation, deep anterior T-wave inversions (which gradually resolve)
CXR: normal or pulmonary congestion
Echo: decrease EF, possible LVOT obstruction, eval for thrombus
Cardiac MRI: wall motion abnormalities
TCM managment
IMMEDIATE: similar to any acute MI, including cath lab
Resolve trigger
At discharge
-ASA, BB, and ACE-I until the LV fully recovers
-(+) thrombus = anticoagulation for minimum of 3 months
Long-term: depends on whether LV dysfunction persists
Prognosis: most recover systolic function w/in 4 wks. May have variable EKG changes for several months following event
Diagnostic criteria Takotsubo cardiomyopathy
- There is transient hypokinesis, dyskinesia, or alpines is of the left ventricle
- No obstructive coronary artery disease (CAD) or angiographic evidence of an acute plaque rupture
- New EKG abnormalities (ST-segment elevation and/or T-wave inversion) or modestly elevated levels of cardiac troponin
- Absence of pheochromocytoma or myocarditis
