Cardiomyopathy Flashcards
Case presentation
19 y/o Male, presents to your office with complaints of palpitations and occasional chest pain especially when he has been practicing for his football team
Fam Hx: Uncle passed away at age 25, Cousin has heart problem
Definition of cardiomyopathy
a heterogeneous group of diseases of the myocardium associated with mechanical &/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic. Cardiomyopathies either are confined to the heart or are a part of generalized systemic disorders, often leading to cardiovascular death or progressive heart failure-related disability
Dilated cardiomyopathy
Dilatation and impaired contraction of the left or both ventricles (chamber enlargement)
- Ventricular dilation, hypokinetic left ventricle, and systolic dysfunction
- can be inherited
- congestive presentation
Hypertrophic cardiomyopathy
Left and/or right ventricular hypertrophy, often asymmetrical, which usually involves the interventricular septum.
-Inappropriate myocardial hypertrophy, with or without left ventricular obstruction
Restrictive cardiomyopathy
Restricted filling and reduced diastolic size of either or both ventricles with normal or near-normal systolic function.
-Abnormal ventricular filling with diastolic dysfunction
Arrhythmogenic right ventricular cardiomyopathy
Progressive fibro-fatty replacement of the right ventricle
-Fibroadipose replacement of right ventricle
Common causes of Dilated Cardiomyopathy
- Idiopathic
- Toxin induced (alcohol, anthracycline, cobalt, catecholamines)
- Radiation
- Infection (viral, HIV, parasite)
- Metabolic (starvation, thiamine deficiency like beriberi, thyrotoxicosis)
- Sarcoidosis
- Hemochromatosis
- Peripartum/postpartum
- Genetic
Dilated cardiomyopathy incidence
- most common of all
- myocyte injury and fibrosis
- Third most common cause of heart failure
- Middle age, men > women
- Annual mortality: 12% !
- Most frequent cause of heart transplantation
- complete recovery is rare
S/S of Dilated cardiomyopathy
- Pulmonary congestion-left heart failure: dyspnea (rest, excertional, nocturnal), orthopnea
- Systemic congestion- right heart failure: edema, nausea, abdominal pain, nocturia
- Hypotension (low CO), tachycardia, tachypnea, fatigue and weakness
- Arrhythmia
Atrial fibrillation, conduction delays, complex PVC’s, sudden death
During dilated cardiomyopathy cardiac exam shows
S3, S4 and murmur of TR and/or MR
CXR
shows enlarged heart, CHF
Treatment for dilated cardiomyopathy
- Limit activity based on functional status
- Salt restriction
- Fluid restriction
- Diuretics
- Beta Blockers, ACE/ARB (improve mortality)
- Hydralazine/nitrate combination if cannot tolerate ACE/ARB
- Spironolactone if EF
Dilated cardiomyopathy is the most common indication for ________
cardiac transplantation
Survival after transplant is
80% one year
70% 5 years
-or can do left vent reshaping
People with Hypertrophic Cardiomyopathy (HCM) also have:
- Small LV cavity
- septal hypertrophy (huge)
- systolic anterior motion of the mitral valve leaflet +/- obstruction of left ventricular outflow
- low stroke volume
- elevated EF
Usually patients with Hypertrophic Cardiomyopathy (HCM) are
very young
may lead to MI, mitral regurgitation or arrhythmias
What type of dysfunction is Hypertrophic Cardiomyopathy and what does it lead to?
Diastolic dysfunction (impaired filling, increased filling pressure)
Leads to pulmonary congestion (have LV outflow obstruction and hyper dynamic systolic function bc of so much muscle, especially septum)
“Classic” feature of hypertrophic cardiomyopathy is
Dynamic left ventricular outflow tract obstruction
absent in about half of people and obstruction varies greatly
The severity of LV outflow obstruction increases with
hypertrophic obstructive= HOCM
**exercise
-positive inotropic agents
volume depletion
Anything that decreases filling:
- *sudden assumption of upright posture
- tachycardia
- *Valsalva maneuver
S/S of hypertrophic cardiomyopathy
- Asymptomatic (Echo finding only)
- Dyspnea in 90%
- Angina pectoris in 75% (ISCHEMIC CHEST PAIN)
- Fatigue, pre-syncope, SYNCOPE (incr. risk of SCD)
- Palpitation, PND, CHF, dizziness
- Atrial fibrillation, thromboembolism
- SUDDEN CARDIAC DEATH
In HCM, ejection murmur is heard at
left sternal border
-does NOT radiate to the carotid arteries
HCM, the EKG will show
*LVH (Strain pattern) and *abnormal Q waves
may see giant T wave inversions or LA enlargement, bbb or arrhythmias
In HCM, the valsava will
decrease LV volume and INCREASE THE MURMUR
-standing will do this too
In HCM, squatting will
increase LV volume and DECREASE THE MURMUR
Echo findings in HCM
- Echocardiogram is best test
- Left ventricular hypertrophy > 1.3 cm
- Septal to posterior wall ratio > 1.3:1
- Mitral regurgitation
- Systolic anterior motion of the mitral valve (SAM)
- Premature midsystolic closure of the aortic valve
- Asymmetric septal hypertrophy (ASH)
- Diastolic dysfunction
- Left ventricular outflow tract obstruction (LVOT)
Recommendation when there is a family history of HCM
Serial ECHOs are recommended up to the age of 20 yr where there is a family history of HCM
-LVH usually develops between 5-15 y/o
Accounts for 36% of deaths in
athletes
Management of HCM
- careful family history focused on sudden cardiac death
- counseling regarding avoidance of strenuous exercise, avoidance of dehydration
- all first-degree family members should be periodically screened with an echo
HCM treatment
- FIRST- Beta blockers
- CCB
- Anti-arrhythmics – Amiodarone, disopyramide
- Pacemakers (ICD) / Defibrillators (AICD)
* **Myomectomy (resection of septum)
* **Alcohol septal ablation (controlled MI through septal perforator perfusing basal septum), leads to wall thinning which decreases LV outflow obstruction
Beta blockers help HMC by
*NEGATIVE inotropic effect decreases outflow gradient
-decreased myocardial demand results in reduced
ischemia
-prolonged diastolic filling time results in improved LV
filling as well as improved coronary perfusion
-may have an antiarrhythmic effect
IN HCM, usually, signs and symptoms _____ with
age
Leading cause of death: _____________
increase
sudden death in asymptomatic patients
Restrictive Cardiomyopathy (RCM) background
- rare in western world
- can affect both ventricles
- systolic fx usually normal (especially early on)
- SMALL ventricular cavity size
RCM is a ________ dysfunction and involves __________
- DIASTOLIC DYSFUNCTION
- elevated vent filling pressures (small ventricular cavity size)
RCM has a ________ with impaired ventricular filling
rigid ventricular wall
RCM bears some functional resemblance to _________
constrictive pericarditis
-Importance lies in its differentiation from operable constrictive pericarditis
RCM is much less common then DCM or HCM outside the tropics, but frequent cause of death in ________ primarily because of the high incidence of endomyocardial fibrosis in those regions
Africa, India, South and Central America and Asia
Non-infiltrative causes of RCM
*Idiopathic
*Scleroderma
Familial
Hypertrophic
Infiltrative causes of RCM
Amyloid (most common)
*Sarcoid
Gaucher’s
Storage disease causes of RCM
Hemochromatosis
Glycogen storage disease
Fabry’s
Endomyocardial causes of RCM
*Endomyocardial fibrosis
*Hypereosinophilic syndrom
*Radiation
*Chemotherapy toxicity
Carcinoid
Metastatic malignancy
Rigid myocardium –> ______ diastolic vent pressure —> venous congestion –> ________
increased
-Jugular venous distention, Hepatomegaly, Ascites
Rigid myocardium–> _____ ventricular filling –>
_____ CO –> ________
decreased
decreased
fatigue and weakness
in RCM, Echo shows
- *thick walls, reduced systolic fxn**
- Abnormal mitral inflow pattern
- Prominent E wave (rapid diastolic filling)
- Reduced deceleration time (increased LA pressure)
procedure that is commonly necessary in RCM
Right heart cath
-can help with endomyocardial biopsy
Consider RCM in any patient with
R sided heart failure without evidence of either cardiomegaly or systolic dysfunction
RCM diagnosis
possibly biopsy
RCM treatment
CHF THERAPY
- No Clear medical therapy
- Treat reversible causes
- Transplant maybe only option
GOOD TABLE COMPARING 3 ON SLIDE 56
slide 56
Arrhythmogenic RV Cardiomyopathy is characterized by
fibroadipose replacement of segments of the free wall of the right ventricle
Arrhythmogenic RV Cardiomyopathy is predominately found in ____
young adults; cause of young adult sudden death
Arrhythmogenic RV Cardiomyopathy- all patients who are symptomatic with arrhythmias get
ICD implantation
