Cardiomyopathy Kono

Cardiomyopathy
How do we suffer broken hearts?

Alan Kono, MD
Assistant Professor of Medicine
Cardiology DHMC
Cardiomyopathy
Objectives
Review causes of Dilated Cardiomyopathy
Exclude Ischemic DCM (CAD) and VHD
Overview of some reversible causes
Review Non Dilated Cardiomyopathy

HCM
Infiltrative Diseases
Cardiomyopathy
Greek kardia heart + myo muscle
patheia suffering/disease
1980 WHO: heart muscle disease of unknown
cause
1995 WHO/Task force: diseases of the
myocardium associated with cardiac dysfunction
Cardiomyopathies
Ischemic
Hypertrophic CM
Idiopathic Dilated
Hypertensive
HF-Dilated
Konstam MA. J Card Failure. 2003.
HF-Non Dilated
Cardiomyopathy
Classification
Dilated Cardiomyopathy
Hypertrophic Cardiomyopathy
Restrictive Cardiomyopathy
Non Dilated
Arrhythmogenic RV cardiomyopathy
Unclassified Cardiomyopathy
Clinical Manifestations
SUBCLINICAL
CLINICAL
Asymptomatic
Minimally Symptomatic
II
Subtle symptoms overshadowed

Systemic illness
III
Congestive Heart Failure

Cardiovascular Collapse/SCD
IV
Etiologic Classification
1. Ischemic
2. Valvular
3. Hypertensive
4. Inflammatory
5. Metabolic
6. General Systemic Diseases
7. Muscular Dystrophies
8. Neuromuscular
9. Sensitivity and Toxic Reactions
10. Peripartum
Etiology of Dilated Cardiomyopathy

1230 patients-biopsy
Idiopathic
Myocarditis
Ischemic
Infiltrative
Peripartum
Other
50%
9%
7%
5%
4%
10%
HTN
HIV
CTD
Substance abuse
Adriamycin
Felker GM NEJM 2000;342:1077
4%
4%
3%
3%
1%
Viral Etiology by PCR

624 pts with +EMB Dallas criteria for
myocarditis
Serology, cultures, PCR
149 control pts with CHF & DCM, negative EMB
165 control pts with other cardiac disease
Myocarditis- ~30% viral pathogen detected

DCM- ~ 20% viral pathogen detected
Control- 1% + PCR
Bowles JACC 2003
Prognosis related to Etiology
Felker GM NEJM 2000;342:1077
Prognosis in DCM
Infiltrative Disease
Felker NEJM 2000;342:1077
Genetics
~20-35% DCM may be inherited
Risk of inheriting Gene
50% autosomal dominant
25 % autosomal recessive
Grunig JACC 1998

Mahon JACC 2002
Genetics
Familial Component-High Prevalence in Family Members

20-30%
445 patients w/ idiopathic DCM by echocardiography and
angiography
970 first and second degree relatives
definite familial DCM 11%
suspected DCM 24 %
189 consecutive patients with DCM
767 first and second degree relatives

Abnormalities found in 23%
DCM in 4.6%
LV dilatation w/o LV dysfunction 15%
J Am Coll Cardiol 1998 Jan;31(1):186-94
Ann Intern Med 2005 Jul 19;143(2):108-15.
Genetics
5 Phenotypes
DCM with muscular dystrophy
Juvenile DCM in male relatives no MD
DCM with segmental wall motion of LV
DCM with conduction defects
DCM with sensorineural hearing loss
Mestroni J Am Coll Cardiol 1999 Jul;34(1)
Phenotypes
Autosomal Dominant most common
56%
High incidence of cardiac autoantibodies
Autosomal recessive
16%
Younger age, worse prognosis
X linked
10%
Different mutations in dystrophin gene
Autosomal dominant + skeletal
7%
Restrictive pattern
Familial DCM with conduction defects 2%

350 pts DCM, 281 relatives
Mestroni JACC 200234:181
Autosomal Dominant
Several Gene mutations identified
Cytosketal proteins
Actin, Lamin A and C, desmin
Sarcomere proteins
-myosin heavy chain, Troponin T
Also implicated in some forms of HCM
Familial DCM
Counseling of family members
The course and penetrance are not predictable, and
normal initial screening studies do not preclude the
future development of cardiomyopathy.
Initial symptoms of DCM are variable
(dyspnea, syncope, sudden death) and warrant prompt
evaluation.
Risk of inheriting the DCM gene

50 percent in families with autosomal dominant inheritance
25 percent with autosomal recessive inheritance.
EKG and Echo in first degree family members *

3-5 year intervals
*AHA CLASS IIa recommendation
No conduction abnormality
Intercalated discs proteins
Vinculin and isovinculin Chr 10q22.1-q23
Delta sarcoglycan, dystrophin protein

Mouse model, Verapamil improved phenotype
Phospholambin (inhibits SR Ca++ ATPaseSERCA2a)

LV dysfunction age 20-30, CHF ~10 yrs
Experimental data-> reduce phospholambin and increase
SERCA2a reduce CHF progression
Animal Models
Possible Contributions to DCM
Impaired SR Ca++ mechanics
Myosin autoantibodies
Nitric Oxide induced negative inotropic effects
DNAase may induce premature apoptosis
Calmodulin (CAM kinases)
Work up
History and PE
CXR and EKG
Echocardiogram
MRI or Nuc Scan
?Role of CT angio
Lab Tests
Work up
Laboratory tests
CBC
Electrolytes and BUN/Cr Ca 2+ Mg2+
LFTs & Hepatitis panel
UA
Thyroid Function Tests
FBS or HgbA1C
Iron Studies (ferritin and TIBC)
ESR
BNP vs ProBNP?
Other studies
ANA and serologies

Thiamine, carnitine, selenium
Antimyosin Ab
Metanephrine, VMA
Viral serologies, HIV
Work up
In age appropriate group
Cardiac Cath if ischemia
FTC assessment
TST nuclear Scan

Persantine/adenosine nuclear scan
Stress Echo
Dobutamine Stress Echo
Cardiac MRI
?64 Slice CT scan
Level IA
Level IIA
Goals of Therapy
Treat Congestive Symptoms
Initiate Evidence Based Therapies
Identify Diseases that can be Treated
CAD
VHD
Tachycardia induced
Thyroid Disease
Hemochromatosis
Sarcoidosis
Infections
ETOH
Drugs
Take notice!
Drug Induced
Cocaine
ETOH
Anthracycline (doxorubicin)
Trastuzumab
Tachycardia Induced
Peripartum Cardiomyopathy
Anemia
Hemochromatosis
Obstructive Sleep Apnea
Case Study 1: DCM

36 yo WM with a history of asthma
4 month h/o of worsening asthma
Albuterol inhaler 1x/day3-4x/day
Increased DOE and SOB
2 week history of LE edema, increased

abd girth and 3 day history of
scrotal/penile edema
40 lb wt gain in 3 months
Case Study 1: DCM

+PND, orthopnea No Chestpain,
palpitations or syncope
No HTN, DM, HPL or Family History
Works in Maintenance, ?exposure to
solvents
ETOH use since age 20, no drugs
cut back to of 1/5 of Bacardi
Case Study 1: DCM

BP= 142/93 P=123 Sinus tachycardia
Admission Wt = 142 kg
Lungs- bilat rales

Cardiac- RR, +S3/S4, JVP ~ 20 cm
Abd- obese, no HS megaly
Extrem- 3+ edema, scrotal edema, cool to ankles
Labs
Not anemic, Normal Lytes/Renal Function

Albumin= 3.8
Elevated TnT=.15 CPK=500 proBNP=1100
Tox screen- +MSO4
Blood Alcohol 1830 (>800 intoxication)
Case Study 1: DCM

Echo PSLAX
Case Study 1: DCM

Echo PSLAX
Case Study 1: DCM

Echo PSLAX
Case Study 1: DCM

Cath
RA= 23 PA= 48/25 PCWP=24 mmHg
Case Study 1: DCM

Cath LV gram
Alcohol Induced Cardiomyopathy

3-4% of DCM
23-40% Prevalence
Incidence more common in Men
Alcoholic woman have a higher prevalence of DCM at
lower ETOH use
Etiology
Direct toxic effects of ETOH/Metabolites
Oxidative Stress
Accumulation of TG
Altered Fatty Acid Extraction
Decreased myofilament Ca++ sensitivity
Impaired protein Synthesis
Malnutrition
Contaminants (Cobalt)
?Genetic Predisposition
ACE receptor polymorphism

Murine Model
Over expression of
Alcohol Dehydrogenase
Acetaldehyde induced
myocardial damage
Liang Pharmacology 1999 291(2)
Alchohol DCM
Biological Proc 2003
American J Path 2005 165
Changes in left ventricular ejection fraction in patients with alcoholic

cardiomyopathy, according to daily ethanol intake during the first year of the study
N=55
Male
Nicolás, J. M. et. al. Ann Intern Med 2002;136:192-200
Abstinence Works
Case Study 1: DCM

ETOH withdrawal precautions
Aggressive IV po diuresis
Uptitration of carvedilol and Lisinopril
Outpatient Follow Up
Wt Loss 60 lbs/3 months
Stopped ETOH
Cooks homemade food without
processed food
Case Study 1: DCM

After Treatment
Case Study 1: DCM

After Treatment
Case Study 2: DCM

53 yo WF with history of palpitations
No Syncope, near syncope, chestpain,
PND/orthopnea, LE edema, or wt gain
Some exercise intolerance
PE normal BP, HR at rest. No rales/edema
Frequent PVCs on PE, no gallop/JVP
EKG
Echo
Holter
Case Study 2: DCM

Echo PSLAX
Case Study 2: DCM

Echo PSSAX
Case Study 2: DCM

Echo A4C
Case Study 2: DCM

Cardiac Cath LCA
Case Study 2: DCM

Cardiac Cath RCA
Case Study 2: DCM

Cardiac Cath LV
Case 2: DCM
EKG
Case 2: DCM
Tachycardia
Induced
EKG
Cardiomyopathy
Tachycardia Induced Cardiomyopathy

Potentially reversible DCM
First reported 1913 Gossage
Atrial Fibrillation DCM 1937 Brill
Animal model 1962 Whipple
LV dilation and Dysfunction occurs within

weeks and reverses with HR control
Tachycardia Induced DCM

Morphologic, structural and functional
changes secondary to chronic fast HR
Atrial and Ventricle variant
Systolic and Diastolic Dysfunction
Dilated chambers
MR secondary to MV annular dilatation
B-receptor down regulation
Neurohormonal activation
Abnormal Ca Channel function, QTprongation

Supraventricular
Atrial Fibrillation/Atrial Flutter

Atrial Tachycardia
AVRT AVNRT
PJRT
Ventricular
PVCs, multiple
Ventricular Tachycardia, ARVT
High atrial or ventricular pacing rates

Metabolic- Thyrotoxicosis glucoganoma

Treatment Control the HR!
B-blockers
Non Dihydropyridine CCB Verapamil,Diltiazem
Digoxin
Antiarrhythmia Rx (Sotalol, Amiodarone)
AV nodal ablation + permament pacer
RF ablation of VT focus or WPW
Case 2: Tachycardia induced DCM
Underwent EPSinducible VT
s/p Dual Chamber ICD
Started on Sotalol and Lisinopril
Had persistent VT on ICD interrogation
Underwent VT ablation
Changed Sotalol to Carvedilol

After RX and VT control

After RX and VT control
Case 3: DCM
68 yo WF with no prior cardiac disease, no DM,
HPL or Family history
PMH: HTN, mild COPD, Post menopausal
Remote Smoker, rare ETOH, no drugs
Recent epigastric pain, + Lipase/Amylase
Found to have cholelithiasis
Divorced, some financial and emotional stress

Presents with recurrent epigastric discomfort/
chest discomfort and dyspnea not related to food
Case 3: DCM
+ Cardiac Troponin 2.5 CK-MB 3.5
In ER, Wellens pattern EKG
Case 3: DCM
Echo PSSAX
Case 3: DCM
Echo PSSAX
Case 3: DCM
Cath
Case 3: DCM
Cath
Takotsubo Cardiomyopathy
Stress Induced cardiomyopathy with
characteristic apical LV Ballooning
Usually reversible and overall good prognosis
Post-menopausal Females (~ 88-90%)

under emotional, financial or physical stress (~60-70%)
Clinical Presentation
Chestpain (67%), Dyspnea (17%)

HF, cardiogenic shock, or arrhythmias
EKG changes (ST elevation or T wave inversion)
Mildly Elevated Biomarkers
LV dysfunction
EF~ 20-49% on initial LV function assessment
Takotsubo DCM
Japanese Tako Octopus
tsubo Trap
tsubo Trap
tsubo Trap
tsubo Trap
LV angiogram in diastole (left) and systole (right) in right anterior oblique

projection demonstrating wall-motion abnormality characteristic of stress
cardiomyopathy
Sharkey, S. W. et al. Circulation 2005;111:472-479
Copyright 2005 American Heart Association
Coronary angiogram during chest pain in patient 2 with stress

cardiomyopathy
Diastolic and systolic cine CMR images (horizontal long-axis) from patient 4
And So I Died Of A Broken Heart

If only I'd died
Just once in my life
If only to try
To take a guess
To be the best.
A feeling of hope,
That runs this world
It keeps us alive
I want you to climb with me
Until I die of a broken heart
Of a broken heart
Until I die of a broken heart
Of a broken heart
Until I die
Lyrics by ZWAN
Hypertension
Viral
Ischemia
Myocardial Injury
Stress
Toxins
Valvular
Post
Partum
Myocardial Injury
Neurohormonal Activation
(NE, Ang II, Endothelin, Aldosterone, TNF-alpha)
Myocyte Loss
Myocyte
Hypertrophy/dysfunction
Chamber Enlargement
Progressive Systolic Dysfunction
Remodeling and Survival Effects by

Drug Class
Established
Rx
Remodeling Effects
Survival Effects
Benefit
Benefit
Benefit (+ACEI better)
Benefit (+ACEI better)
Aldo-B
Benefit
Benefit
Beta-B
Benefit
Benefit
Vasop-I
Benefit (ACEI-neutral)
Benefit (ACEI-neutral)
ET-1-B
No Benefit
No Benefit
TNF-B
Ibopamine
No Benefit
No Benefit
Adverse
Adverse
ACE-I
ARB
Other Rx
Heart Failure with

Normal Ejection Fraction
DHF
-Diastolic Dysfunction
- Hypertrophic Cardiomyopathy
- Infiltrative diseases
Abnormal
Load
Subclinically Depressed
Systolic Function
Afterload
(AV mismatch)
- Prior MI
- Ischemia
- DM
- Decompensated
Hypertrophy
- Valvular Disease
Preload
(volume overload)
- Pericardial Disease
*All subcategories may coexist
Cardiomyopathy
Classification
IHSS
ASH
HCM
HOCM
Thick as a Brick
Brock 1957 and Teare 1958
Brock RC - Functional obstruction of the left ventricle. Guys Hosp Rep 1957; 106: 221-38.
Teare RD - Asymetrical hypertrophy of the heart in young adults. Br Heart J 1958; 20: 1-8
Diagnosis
Suspected with heart murmur, family history,
symptoms or abnormal ECG
12 lead abnormal 75-95%
Modest relationship between voltage and LVH
Established with 2 D echo (MRI)

Hypertrophied and dilated chamber in absence of other
diseases (HTN/AS)
Mild: 13mm 15mm to severe >30 mm
Athletes (13mm-15mm)
Role for TDI?

Myocardial Velocity <7m/s during early diastole
Sensitivity 100% specificity 93% (13pts with only gene
abnormality)
Pathophysiology
Prevalence 1:500
VT 613,000 = 1,225
NH 1,259,000 = 2, 500
Heterogeneous with respect to:
disease causing mutation

presentation
prognosis
Treatment
Predominately a non obstructive disease (%75)

Most common cause of SCD < 25 yrs
Diagnosis
HCM
HCM GENOTYPE
Autosomal Dominant
Sarcomeric >Non sarcomeric protein abnormality
10 genes identified, >200 mutations
3 /10 highest prevalence Myosin heavy chain
myosin-binding Protein C
Troponin T
HCM PHENONTYPE
Early Onset (age 4-12)
Adolescent Onset (13-18)
Late Onset (?middle age)
Maron & Seidman J Am Coll Cardiol, 2004; 44:2125-2132
Genetics
Why?
Hydrolysis of ATP to ADP
in the presence of Ca
Head binds to Actin
Conformational change
that tugs on actin
Head releases ADP and
disengages
Why?
Hypothesis
Hypertrophy develops as compensatory response to sarcomeric
dysfunction
Normal myocytes react to excessive mechanical loads with altered patterns
of gene expression and the release of autocrine growth factors (AT II) e.g
HTN model
In HCM, normal systolic pressures sensed by abnormal myocyte as
excessive load, initiating further hypertrophy
In the short term increased LV thickness, and small cavity size decreases
wall tension for systolic contraction
Hemodynamic dysfunction precedes histopathology

Upregulated genes producing 2o hypertrophy
HCM Phenotype
Considerable variance
characteristically asymmetric with anterior septum
predominant
1/3 have wall thickening localized to a single segment
many with diffuse LVH
Distribution of wall thickness does not predict

outcome
Apical variant may have a benign outcome
Eriksson: Long Term Outcome in Patients with Apical HCM. JACC 2002;4:638
Phenotype
Phenotype - Septal
Phenotype - Apical
Phenotype - Diffuse
Cellular
Architecture
disorganized, hypertrophied myocytes
bizarre shapes
multiple intracellular connections
chaotic alignment at oblique and perpendicular
angles
Abnormal collagen matrix
Cellular - normal
Cellular - HCM
HCM Prognosis
Genetic Differences
Individuals with the Phe110Ile mutation in the troponin T gene have a good prognosis
that is comparable to that of patients with the benign Phe513Cys mutation of the
beta-cardiac myosin heavy chain gene. The survival of patients with the Arg719Trp
mutation of the beta-cardiac myosin heavy chain gene is significantly lower (p =
0.0002).
Data from Anan, R, Shono, H, Kisanuki, A, et al, Circulation 1998; 98:391.
HCM
Role of Genetic Testing?
Rapid DNA sequencing now available
Detects the five most common HCM genes
myosin heavy chanig

Myosin binding protein C
Cardiac Troponin T
Cardiac Troponin I
Tropomyosin
Costly ~ $4,400
HCM Screening for Relatives

<12 yrs old
Optional unless:
Malignant family history of premature HCM death or other adverse

complications
Competitive athlete in an intense training program
Onset of symptoms
Other clinical suspicion of early LV hypertrophy
12 to 1821 yrs old
Every 1218 months
>1821 yrs old
Probably every 5 yrs
or more frequent intervals with a family history of late-onset HCM

and/or malignant clinical course*
Maron & Seidman J Am Coll Cardiol, 2004; 44:2125-2132
Primary Treatment Strategies for Subgroups Within the

Hypertrophic Cardiomyopathy Clinical Spectrum
SCD
Family Hx
LVH >3cm
LVOT >30mmHG
Syncope
Hypotension
Copyright restrictions may apply.
Maron, B. J. JAMA 2002;287:1308-1320.
B-blockers
CCB
Norpace
Amiodarone
ETOH Ablation
ETOH Ablation
64 pts followed 3 years

Resting gradient > 30 mm and stress gradient > 60 mm
Multiple septal branches attempted
Results
No death
Marked reduction in NYHC (72% to none in III or IV)
HOCM- Pre Septal Ablation

REST
VALSALVA
HOCM- Pre Septal Ablation MR

VALSALVA
HOCM- Septal Ablation
HOCM- Post Septal Ablation

REST
VALSALVA
Cardiomyopathy
Classification
Non dilated Ventricle w/ normal wall
thickness
Need to Exclude Constrictive Pericarditis
Preserved LV systolic function (normal EF)

Diastolic Dysfunction and restrictive filling
Abnormal Echo Doppler or Cardiac MRI
R/L cardiac cath to assess hemodynamics
Etiology
Idiopathic
Sarcoidosis
Amyloidosis
Hemochromatosis
Chemotherapy or Radiation Therapy
Hyper-eosinophilic Syndrome
Endomyocardial fibrosis
Endomyocardial-Biopsy Specimens from Patients with Idiopathic Restrictive Cardiomyopathy
Kushwaha S et al. N Engl J Med 1997;336:267-276
Endomyocardial-Biopsy Specimens from Patients with Cardiac Amyloidosis
Hemodynamic Tracings
PA
LV
RV
Echocardiogram
Echocardiogram
The Differential Diagnosis of

Restrictive Cardiomyopathy and Constrictive Pericarditis
Cardiomyopathy
Classification
ARVC- arrhythmias and specific RV morphology
1/5000, marked geographic variation
High Incidence in Northern Italy
Autosomal Dominant, spontaneous

11% of SCD, 22% SCD in athletes (Italy)
Fat tissue infiltration of RV free wall
Fibrofatty
Fatty
Arrhythmogenic RV
cardiomyopathy
Global and/or regional dysfunction and
structural alterations
Repolarization abnormalities on the ECG
Depolarization/conduction abnormalities
on the ECG
Arrhythmias
- Monomorphic VT with LBBB
ARVC
Morphologic Changes
Increased Fatty or fibrofatty RV infiltrate

EM demostrates Desmosomal Abnormalities
plakoglobin, desmoplakin, and plakophilin-2
Genetics
Sporadic
Autosomal Dominant
100% penetrance
Autosomal Dominant Transmission

Part of one of 11 autosomal-dominant families with arrhythmogenic right ventricular
cardiomyopathy linked to 3p25 showing family members at high risk (n = 197), low risk (n =
92), and unknown (n = 78) status
Hodgkinson, K. A. et al. J Am Coll Cardiol 2005;45:400-408
Copyright 2005 American College of Cardiology Foundation. Restrictions may apply.
Time to death or last follow-up (censored) in high-risk males and females with
arrhythmogenic right ventricular cardiomyopathy linked to 3p25
Hodgkinson, K. A. et al. J Am Coll Cardiol 2005;45:400-408

Copyright 2005 American College of Cardiology Foundation. Restrictions may apply.
Cardiomyopathy
Classification
Noncompaction LV
Noncompaction LV
Summary
r/o reversible causes
guarded prognosis, depends on etiology
initiate evidence based therapies
Non Dilated Cardiomyopathy

exclude Hypertensive Cardiovascular Disease
New Approach to the

Classification of Heart Failure
A
B
C
D
Stage
Patient Description
High risk for

developing heart
failure (HF)
Asymptomatic HF
Symptomatic HF
Refractory
end-stage HF
Hunt SA et al. J Am Coll Cardiol. 2001;38:21012113.
Hypertension
CAD
Diabetes mellitus
Family history of cardiomyopathy
Previous MI
LV systolic dysfunction
Asymptomatic valvular disease
Known structural heart disease
Shortness of breath and fatigue
Reduced exercise tolerance
Marked symptoms at rest despite
maximal medical therapy (eg, those
who are recurrently hospitalized or
cannot be safely discharged from
the hospital without specialized
interventions)
Classification of HF: Comparison

Between ACC/AHA HF Stage and
NYHA Functional Class
ACC/AHA HF Stage1
A At high risk for heart failure but without
structural heart disease or symptoms
of heart failure (eg, patients with
hypertension or coronary artery disease)
B Structural heart disease but without
symptoms of heart failure
C Structural heart disease with prior or

current symptoms of heart failure
D Refractory heart failure requiring

specialized interventions
NYHA Functional Class2

None
I Asymptomatic
II Symptomatic with moderate exertion

III Symptomatic with minimal exertion
IV Symptomatic at rest
Hunt SA et al. J Am Coll Cardiol. 2001;38:21012113.
New York Heart Association/Little Brown and Company, 1964. Adapted from: Farrell MH et al. JAMA. 2002;287:890897.
Heart Failure in One Minute

Confirm the clinical diagnosis with an
echocardiogram
?new role for BNP
Determine the etiology and reverse it, if

possible (Rx for HTN, stop alcohol,
reverse or treat ischemia, control HR in
AF)

Add Diuretic if neck veins remain elevated (Block secondary
effects of RAA System)
Attempt euvolemic status
Add Aldactone in FTC III-IV pts (RALES)
?Eplerenone in post MI (EPHESUS)
Add ACEI Therapy (Block RAA System)

May use ARB if ACE intolerant (ELITE II, ValHFT, CHARM, VALIANT)
Add Beta Blockers to lower HR to about 60 beats per minute

Metoprolol, Carvedilol, Bisoprolol

Control BP ( 80 +EF)
May add Hydralazine/Isosorbide (VeHFT Trial)
Avoid CCB, except for Amlodipine, Felodipine
May add Digoxin in symptomatic pts, or Afib

AVOID
ASA (in non-ischemia DCM) & NSAIA
Coumadin (unless Afib)
Multidiscipline Approach emphasizing CARE MANAGEMENT

Move away from Episodic Care
Diagnosis
How is the diagnosis confirmed
Endomyocardial Biopsy- RV > LV

Definitive diagnosis
Sample bias
1/250 risk for perforation, 1/1000 risk death
Sensitivity= 35- 60% Specificity= 80%
Endomyocardial Biopsy
Technique
Bioptome
And When do we biopsy?
Does Biospy Help?

Cardiomyopathy Kono

Uploaded by

Document Information

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Cardiomyopathy Kono

Uploaded by

Copyright:

Available Formats

Cardiomyopathy

How do we suffer broken hearts?

Review Non Dilated Cardiomyopathy

Subtle symptoms overshadowed

Congestive Heart Failure

Etiology of Dilated Cardiomyopathy

Felker GM NEJM 2000;342:1077

Viral Etiology by PCR

Myocarditis- ~30% viral pathogen detected

Felker GM NEJM 2000;342:1077

Felker NEJM 2000;342:1077

Grunig JACC 1998

Familial Component-High Prevalence in Family Members

189 consecutive patients with DCM

767 first and second degree relatives

Mestroni J Am Coll Cardiol 1999 Jul;34(1)

High incidence of cardiac autoantibodies

Younger age, worse prognosis

Different mutations in dystrophin gene

Autosomal dominant + skeletal

Familial DCM with conduction defects 2%

Risk of inheriting the DCM gene

EKG and Echo in first degree family members *

Delta sarcoglycan, dystrophin protein

Phospholambin (inhibits SR Ca++ ATPaseSERCA2a)

ANA and serologies

TST nuclear Scan

Case Study 1: DCM

2 week history of LE edema, increased

Case Study 1: DCM

Case Study 1: DCM

Lungs- bilat rales

Not anemic, Normal Lytes/Renal Function

Case Study 1: DCM

Case Study 1: DCM

Case Study 1: DCM

Case Study 1: DCM

RA= 23 PA= 48/25 PCWP=24 mmHg

Case Study 1: DCM

Alcohol Induced Cardiomyopathy

Alcohol Induced Cardiomyopathy

Liang Pharmacology 1999 291(2)

Biological Proc 2003

Alcohol Induced Cardiomyopathy

American J Path 2005 165

Changes in left ventricular ejection fraction in patients with alcoholic

Nicol&aacute;s, J. M. et. al. Ann Intern Med 2002;136:192-200

Case Study 1: DCM

Case Study 1: DCM

Case Study 1: DCM

Case Study 2: DCM

Case Study 2: DCM

Case Study 2: DCM

Case Study 2: DCM

Case Study 2: DCM

Case Study 2: DCM

Case Study 2: DCM

Tachycardia Induced Cardiomyopathy

LV dilation and Dysfunction occurs within

Tachycardia Induced DCM

Tachycardia Induced Cardiomyopathy

Atrial Fibrillation/Atrial Flutter

High atrial or ventricular pacing rates

Tachycardia Induced Cardiomyopathy

Nicolás, J. M. et. al. Ann Intern Med 2002;136:192-200