Cardiomyopathy

Definition:
A primary disorder of the heart muscle
associated with mechanical and/or electrical
dysfunction, which usually (but not invariably)
exhibit inappropriate ventricular hypertrophy
or dilatation, and are due to a variety of
etiologies that frequently are genetic.
Dilated Cardiomyopathy:latest
concepts and development
 WHO Classification
• Unknown cause • Specific heart
(primary) muscle disease
– Dilated (secondary)
– Hypertrophic – Infective
– Restrictive – Metabolic
– unclassified – Systemic disease
– Heredofamilial
– Sensitivity
– Toxic
 Functional Classification
• Dilated (congestive, DCM, IDC)
– ventricular enlargement and systolic
dysfunction
• Hypertrophic (IHSS, HCM, HOCM)
– inappropriate myocardial hypertrophy
in the absence of HTN or aortic stenosis
• Restrictive (infiltrative)
-abnormal filling and diastolic function
 Dilated Cardiomyopathy

 A disease of unknown etiology that

principally affects the myocardium
and there is
 LV dilatation and systolic dysfunction
 Classification of DCM :
• Idopathic Cardiomyopathy
• Ischemic cardiomyopathy
• Valvular cardiomyopathy
• Hypertensive cardiomyopathy
• Tachycardia induced cardiomyopathy
• Toxin : Alcohol, Chemotherapeutic agent, Cobalt,
Antiviral agent,lead, cocain,lithium etc.
• Infection induced
• Autoimmune/Collagen disorder
• Infiltrative disorder
• Peri/Postpartum dysfunction
• Neuromascular dystrophy
ETIOLOGIES OF DILATED CARDIOMYOPATHY

Felker et al NEJM 2000

 Idopathic Dilated
Cardiomyopathy(IDC)
Incidence and Prognosis
• 3-10 cases per 100,000
• 20,000 new cases per year in the U.S.A.
• death from progressive pump failure
1-year 25%
2-year 35-40%
5-year 40-80%
• stabilization observed in 20-50% of patient
• complete recovery is rare
IDIOPATHIC DILATED CARDIOMYOPATHY
PATHOLOGIC FINDINGS
 Pahophysiology :

– Cardiomegaly-LV/biventricular dilatation.
– Decreased systolic function
– Reduced LV contraction ability → decreased
cardiac output → increased residual volumes
in end-systole and end-diastole.
 Symptoms of heart
failure
– Pulmonary congestion (left HF)
dyspnea (rest, exertional, nocturnal),
orthopnea
– Systemic congestion (right HF)
edema, nausea, abdominal pain, nocturia
– Low cardiac output
fatigue and weakness
 Signs of Heart failure
• hypotension, tachycardia, tachypnea,
raised JVP,hepatomegaly,
• Gallop rhythm,
• murmur of MR or TR
 Complications :

 Heart failure
 Atrial or ventricular arrythmias
 Sudden death
 Systemic or pulmonary embolism
 Lab Studies
 CBC – anemia.
 Serum urea, creatinine,electrolytes
 LFT – may rise with hepatic congestion
 Fasting glucose – screen for DM
 TFT – thyrotoxicosis or hypothyroidism
 Fe/TIBC – if hemochromatosis is a risk
 BNP -- > indicator of failure
 ANA – possible lupus
 Viral studies
 Urine toxicology screen
 ECG :
 Sinus tachycardia
 Atrial or ventricular arrhythmia
 P wave shows either right or left atrial
enlargement.
 LBBB or RBBB or Fascicular block
 Conduction block.
 Holter monitoring: if lightheadedness,
palpitation or syncope
 X-ray chest of DCM
 Cardiomegaly
 Prominent upper lobe vessels
 Kerleys B line
 Pulmonary edema
 Pleural effusion
 Echo & Doppler :
 Dilation of all cardiac chamber
 Global or Regional wall motion
abnormality
 Wall thickness normal or slightly
decreased
 Functional MR or TR
 Coronary Angiogram

To exclude coronary artery disease

 if age >40,
 ischemic history,
 high risk profile,
 abnormal ECG
 Management of DCM

 Limit activity based on functional

status
 salt restriction of a 2-g Na+ (5g NaCl)
diet
 fluid restriction for significant low Na+
 initiate medical therapy
 Management of DCM
– diuretics , ACE inhibitors, spironolactone
– beta blockers, ARNi(Angiotensin Receptor-
Neprilysin inhibitors):sacubitril/volsartan
– digoxin,SGLT2 inhibitors, Ivabradine,
Vericaguat
– anticoagulation for EF <30%, history of
thromboemoli, presence of mural thrombi
– amiodarone for symptomatic arrhythmia
– high normal potassium level
– intravenous dopamine, dobutamine and/or
 Management of DCM

 ICD or ICD plus biventricular pacing

for selective patients.
 Heart transplantation
 Ischemic
Cardiomyopathy
Definition :
Ischemic cardiomyopathy is commonly defined
as a dilated cardiomyopathy in a subject with a
history of MI or evidence of clinically significant
(i,e≥70 percent narrowing of a major epicardial
artery) coronary artery disease, in whom the
degree of myocardial dysfunction and
ventricular dilatation is not explained solely by
the extent of previous infarction or the degree
of ongoing ischemia
 Pathophysiology of ICM

 Dilatation of the left ventricle and a

decrease in ejection fraction occurs in 15
to 40 percent of subjects within 12 to 24
months following an anterior MI.
 Gross pathology includes transmural or
subendocardial scarring, representing old
MI that may comprise up to 50 % of the
LV chamber.
 Treatment of ICM
 Conventional treatment of heart failure plus
 ICD if EF less than or equal to 35% (at least
40 days post MI), NYHA functional class II
or III symtom while receving chronic optimal
medical therapy and who have expectation
of survival with good functional status> one
year.
 ICD plus biventricular pacing for patient with
intraventricular conduction defect.
 Heart transplantation
 Hypertensive
Cardiomyopathy
 Definition : A hypertensive dilated
cardiomyopathy is diagnosed when
myocardial systolic function is depressed
out of proportion to the increase in wall
stress.
 Treatment : as for ischemic
cardiomyopathy except that afterload
must be vigorously controlled.
Valvular Cardiomyopathy
 This most commonly occur in left sided
regurgitant lesions (mitral regurgitation and
aortic regurgitation), less commonly with
aortic stenosis and never with pure mitral
stenosis.
 Prognosis : The prognosis is variable and
depends on the number of associated
condition, nature and extent of the valvular
abnormality and most important, the severity
of cardiomyopathy at the time of surgical
correction.
 Treatment of Valvular
Cardiomyopathy
 Surgical valve replacement or repair as
soon as the cardiomyopathy is detected.
 Medical treatment may be the only
option in subject with aortic insufficiency
or mitral regurgitation whose LV function
is severely impaired.
 Peripartum
Cardiomyopathy
 Peripartum cardiomyopathy (PPCM) is
defined as the development of idiopathic left
ventricular systolic dysfunction (demonstrated
by echocardiography) in the interval between
the last month of pregnancy up to the first 6
postpartum months in women without
preexisting cardiac dysfunction.
 The incidence of PPCM in the United States
is estimated to be 1 in 3000 to 4000 live
births.
 The exact cause of PPCM is unknown
Risk factors associated with
the development of PPCM
 Pathophysiology of PPCM
 Unknown etiology
 Inflammatory cytokines may play a role in
PPCM
 (TNF, IL6, Fas receptor, ….)
 Myocarditis , evidence by endomyocardial
biopsies
 A maternal immunologic response to a fetal
antigen has been proposed as another
potential etiology of PPCM
 Familial or genetic etiology can not be
excluded
 Risk Factors for PPCM
 Age greater than 30 years
 Multiparity
 Women of African descent
 Pregnancy with multiple fetuses
 A history of preeclampsia, eclampsia, or
postpartum hypertension
 Association with maternal cocaine abuse or
selenium deficiency
 Long term (>4 weeks) oral tocolytic therapy
with beta adrenergic agonists such as :
Terbutaline.
 Treatment of PPCM
 Treatment of PPCM is similar to that for
other types of CHF. Anti-coagulations
also considered for severe LV dilation to
prevent thrombus formation.
 Intravenous immune globulin (IVIG) is of
benefit for some patients with myocarditis
and a dilated cardiomyopathy
 Heart transplantation
Infectious cardiomyopathy
Variety of infections can lead to myocarditis and
DCM :
 Viral (coxasackie virus,influenja virus,
cytomegalo virus,HIV, adenovirus,HSV)
 Rickettsial (Typhus, Rocky Mounted Spotted
fever)
 Bacterial (diphtheria. meningococcus,
streptococcus, Mycobacterial)
 Spirochetal
 Fungal (aspergillosis,candidiasis)
 Parasitic (Chagas disease,Toxoplasmosis
 Pathogenesis of myocarditis
• If immune response is not sufficient myocyte
injury occurs via :
• Direct cytotoxicity
• Persistent immune response against viral genomic
fragments.

• Triphasic disease process:

Phase I : Viral Infection and Replication
Phase 2 : Autoimmunity and injury
Phase 3 : Dilated Cardiomyopathy
 Clinical presentations
 Cardiac involvement typically occurs 7 to 10
days following a systemic illness.
 Antecedent viral syndrome with fever, myalgias
and malaise
 Chest pain occur in 35% of patients
 Acute dilated cardiomyopathy from lymphocytic
myocarditis can produce mild, moderate or
fulminant heart failure.
 Occasionally patients present with clinical
syndrome identical to an acute myocardial
infarction with ischemic chest pain and ST
segment elevation on ECG.
 Treatment and follow up
 The cornerstone of therapy with acute
myocarditis is supportive care
 Mechanical circulatory support devices
can be used to bridge patients either to
cardiac transplantation or to recovery
 Follow up: Initially at 1 to 3 months
interval for drug and physical activity
titration,
 Serial Echocardiography
 Hypertrophic
Cardiomyopathy
Definition:
Heart muscle disease of unknown
oetiology characterized by
inappropriate ventricular
hypertrophy which is not due to
systemic hypertension or valvular
stenosis.
 Pattern of hypertrophy
 Asymmetric hypertrophy (septum &
anterior wall)—65%
 Concentric--35 %
 Apical or lateral wall—10% (25% in
Japan/Asia)
65% 35%

10%

www.kanter.com/hcm
 Pathophysiology
● Systole
-- dynamic outflow tract gradient which increases with
decreased preload,
decreased afterload and
increased contractility
-- ventury effect: Anterior Mitral Valve Leaflet and
chordae sucked into LV outflow tract.
-- increased obstruction causes eccentric jet of MR in
mid-late systole
● Diastole
-- impaired diastolic filling, increased filling pressure
● Myocardial ischaemia
-- increased muscle mass, filling pressure, increased
O2 demand
-- vasodilator reserve, capillary density
-- abnormal intramural coronary arteries
● Arrhythmias
 Clinical manifestation
• Asymptomatic, (detected after
Echocardiographic & ECG findings)
• Symptomatic
-- dyspnoea in 90%
-- angina pectoris in 75%
-- fatigue, presyncope, syncope-
-- increased risk of SCD in children and
adolescents
-- palpitation, PND, CHF, dizziness less
frequent
 Examination

 jerky pulse
 forceful apical impulse
 S4
 ejection systolic murmur augmented by
Valsalva manoeuvre
 ECG
 may be normal
 LVH criteria
 repolarization (ST/T) abnormalities
 large septal voltages (V2-V4)
 inferior Q waves
Holter monitoring
 nonsustained VT common (25%)
 Echocardiography
 pattern of hypertrophy
 systolic anterior mitral (SAM) valve
motion
 LVOT gradient
 Natural history

• annual motality- 3% (6-8% with NSVTach)

• risk of SCD higher in children
may be as high as 6% per year
majority have progressive hypertrophy
• clinical deterioration usually is slow
• progression to DCM occurs in 10-15%
 Risk factors of SCD
• Young age ( <30 years)
• Malignant family history of sudden death
• Aborted sudden cardiac death
• Sustained VT or SVT
• Recurrent syncope in the young
• Nonsustained VT (Holter Monitoring)
• Brady arrhythmias (occult conduction disease)
• IVS>30mm
• Two or more risk factors
 Management

Prophylactic
 All first degree relatives screening with
echocardiography/genetic counseling
 Avoid competitive athletics
 Prophylactic antibiotics before medical &
dental procedures
 Medical & Surgical
 beta blockers—Atenolol 25-200mg/d, or
Metoprolol 25-100mg bd
 calcium antagonist—Verapamil 240-480
mg/d, Diltiazem 200-500mg/d
 disopyramide
 amiodarone, sotalol
 Dual chamber pacing
 septal ablation by alcohol
 myotomy-myectomy
 plication of anterior mitral leaflet
 Hypertensive HCM of the
elderly
 modest concentric LV hypertrophy
(22mm)
 small LV cavity size
 reduced outflow tract
 sigmoid septum and “grandma SAM”
 associated HTN
Questions?

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