Cardiomyopathies

Definition

*Primary disease of the myocardium, excluding myocardial dysfunction due to

ischemia, chronic valvular disease, hypertensive heart disease and pulmonary
heart disease.

*Diagnosis by exclusion.
 Physiologic Classification

1] Dilated
2] Hypertrophic
3] Restrictive
4] Arrhythmogenic RV cardiomyopathy (ARVC), or
arrhythmogenic RV dysplasia (ARVD)
5] Noncompaction of the ventricular myocardium
Arrhythmogenic RV cardiomyopathy (ARVC), or
arrhythmogenic RV dysplasia (ARVD):
is caused by progressive fibrofatty replacement of the RV
myocardium; occasionally, the LV is involved, with
relative sparing of the septum

? Noncompaction of the ventricular myocardium

is characterized by extensive trabeculation of the

ventricular wall and deep intratrabecular recesses
 (1)
Dilated
Cardiomyopathy
 Definition and prevalence:
Dilated cardiomyopathy (DCM) is a myocardial disorder characterised by
dilatation and contractile dysfunction of the left right ventricles.

DCM may be caused by a diverse range of conditions that promote

cardiomyocyte injury or loss, e.g.. coronary artery disease, viral myocarditis,
alcohol excess.

In approximately 50% cases, an underlying cause is unable to be identified.

This group has traditionally been termed idiopathic DCM.

It is now recognised that approximately 25% of cases of idiopathic DCM

have a positive family history, suggesting that an inherited gene defect might
be the cause of the disorder (familial DCM).
 Causes of DCM

Most cases of DCM are idiopathic (an exact cause is

not known)
Sometimes, Infection (Chagas disease, Post viral)
may be responsible
Occasionally it may be inherited (familial
cardiomyopathy)
Heart valve disease (valvular cardiomyopathy)
Alcoholism (heavy drinking, alcoholic
cardiomyopathy)
Drug abuse or taking d rugs that are toxic to the heart
Thyroid disease
Diabetes
Women after childbirth (peripartum cardiomyopathy).
Tachyarrhythmia
Stress (Tako-tsubo or Broken Heart Syndrome)
Toxic (alcohol, Medications, Cobalt, Snake bite)
The hallmarks of dilated cardiomyopathy (DCM):

the most common cardiomyopathy, [DCM accounts for approximately 25% of the
cases of congestive heart failure in the United States]

enlargement of one or both of the ventricles and

systolic dysfunction
Factors Associated with an Adverse Outcome in Dilated Cardiomyopathy

CLINICAL NONINVASIVE INVASIVE

NYHA Class III/IV Low LV ejection fraction High LV filling pressures

Increasing age Marked LV dilation

Low exercise peak oxygen

Low LV mass
consumption

Marked intraventricular
Moderate mitral regurgitation
conduction delay

Complex ventricular arrhythmias Abnormal diastolic function

Abnormal contractile reserve

Evidence of excessive
sympathetic stimulation Right ventricular dilation or
dysfunction

Protodiastolic gallop (S3)

Elevated serum BNP
Elevated uric acid

Decreased serum sodium

Predictors of poor prognosis in idiopathic dilated cardiomyopathy

Biochemical features : Elevated levels of:

Angiotensin II
Atrial natriuretic factor
Epinephrine (adrenaline)
Norepinephrine (noradrenaline)
Clinical features
History of syncope
Male sex
New York Heart Association class IV
Older age
Persistent third heart sound, gallop rhythm
Signs of right heart failure
Electrocardiographic features
Atrial fibrillation
First-degree or second-degree AV block
Left bundle branch block
Ventricular tachycardia
Exercise test feature
Peak oxygen consumption < 12 mL/kg/minute
Hemodynamic features
Cardiac index
High right atrial pressure
Low mean arterial pressure
Pulmonary capillary wedge pressure > 20 mm Hg
Ventriculographic features
Decreased ventricular mass-volume ratio
Global diffuse wall motion abnormality
Low left ventricular ejection fraction
Large left ventricular end-diastolic dimension
Right ventricular dilatation
Spherical left ventricular geometry
 Stress (Tako-tsubo or Broken Heart Syndrome)
An acute cardiomyopathy provoked by a stressful or emotional situation or exposure
to high doses of catecholamines (sympathomimetic drugs).
most common among middle-aged women,
appears to be related to catecholamine release,

Prognosis: most cases is fully reversible with supportive care.

Diagnosis: ECG: findings of myocardial infarction in the presence of LV

dysfunction and absence of epicardial coronary stenoses.
Endomyocardial biopsy is of value to exclude myocarditis, which can also mimic
acute myocardial infarction, and demonstrates contraction band necrosis.
 Takotsubo cardiomyopathy
The diagnosis: pathognomonic wall motion abnormalities, in which the base of LV is contracting
normally or is hyperkinetic while the remainder of LV is akinetic or dyskinetic [apical ballooning].
 Peripartum Cardiomyopathy
Manifesting between the last month of pregnancy and 6 months post partum.

Etiology is unclear, but ?? inflammatory factors + a high incidence of lymphocytic

inflammation.

Common in Africa but also is manifested in the developed world;

Prognosis is worse in the developing world.

Women who recover are at increased risk of recurrences with subsequent pregnancies.
 Tachycardia-Induced Cardiomyopathy

The most common association is with atrial fibrillation or supraventricular tachycardia.

There is a high rate of full recovery with control of the arrhythmia.

This cardiomyopathy is notable for the degree to which it resembles idiopathic DCM
phenotypically, yet it is characterized by a remarkable degree of recovery in left
ventricular function once the arrhythmia is controlled.

Patients presenting with an atrial or supraventricular arrhythmia should undergo

definitive therapy to control heart rate and to restore normal sinus rhythm.

In addition, patients should be treated with standard neurohormonal blocking agents

and carefully monitored with echocardiography in the weeks to months after
presentation for signs of recovery.
Macroscopic Examination:
four-chamber enlargement + ventricular walls are increased in thickness
consistent with the myocyte hypertrophy that accompanies this disorder.

Histologic evaluation of the myocardium:

myocyte hypertrophy and interstitial fibrosis.

DCM Physiology

1. Impaired contractility of the LV

2. Reduced CO

3. Elevated LV-EDP
DCM: Symptoms
1. Low cardiac output

2. Pulmonary venous congestion

3. Systemic venous congestion

Complications

1. ventricular and supraventricular arrhythmias.

2. conduction system abnormalities.
3. thromboembolism.
4. sudden or heart failurerelated death.
 Electrocardiography
No specific ECG findings signifying DCM.
Sinus tachycardia is often present in proportion to the degree of heart failure.
Typical changes in the QRS complex include poor R wave progression,
intraventricular conduction delays, and left bundle branch block.

A wide QRS complexes a worse prognosis and has now emerged as a clinical
indicator of responsiveness to cardiac resynchronization therapy.

Patients with substantial left ventricular fibrosis may exhibit anterior Q waves even
in the absence of a discrete scar or epicardial coronary artery obstructions.

Nonsustained ventricular tachycardia is extremely common on 24-hour ambulatory

monitoring and represents a predictor of all-cause mortality.

Persistent supraventricular or ventricular tachyarrhythmias represent an important

etiologic factor for ventricular dysfunction, and restoration of sinus rhythm or heart
rate control may lead to recovery of ventricular function. Control of atrial
fibrillation is also important because of atrial transport issues contributing to cardiac
output. In addition, atrial fibrillation should prompt consideration of tachycardia-
induced cardiomyopathy.
 DCM: Echocardiography
Echocardiography is a cornerstone in the
evaluation and management of patients with
DCM
M-mode findings:
1] Increased LVESD and LVED
2] Dilated LA
3] Reduced EF & FS
4] Increased EPSS ( normal 5mm )
5] Reduced AV cusp separation in
systole.
6] Reduced anteroposterior aortic root
motion.
DCM:
Echocardiography
2 D findings
1. Characterized by four chamber
enlargement.
2. Impaired systolic function of
both ventricles
3. TR, MR.
4. Global decreased in wall
motion.
5. Thrombus.
Dilated Cardiomyopathies
Doppler

Mitral and tricuspid

regurgitation due to
stretching of AV
rings due to
ventricular
dilatation
CXR-DCM
 Treatment of DCM
Indeed, treatment with neurohormonal antagonists to prevent disease
progression and the use of diuretics to maintain the volume balance are the
therapeutic cornerstones for the management of patients with DCM.

I] Lifestyle Changes:
Diet: restriction of salt (sodium) intake to 2,000 to 3,000 mg per day. Most salt ingested
comes from processed food.
Exercise: Most people with cardiomyopathy are encouraged to do non-competitive
aerobic exercise. Heavy weight lifting may not be recommended.

II] Medications:
Medications are used for two reasons:
1. To improve cardiac function.
2. To treat symptoms and prevent complications.
by taking a beta-blocker and ACE inhibitor even when not having symptoms. If
symptoms occur and/or worsen, digoxin, diuretics, and aldosterone inhibitors may be
added.
III] Implantable Devices:

1] Cardiac Resynchronization Therapy (CRT, biventricular pacing): In some patients

with advanced heart failure, biventricular pacing improves survival, reduces symptoms
and increases exercise capacity. or tolerance.

2] Implantable Cardioverter Defibrillators (ICD): ICDs are suggested for people

at risk for life-threatening ventricular arrhythmias or sudden cardiac death. The ICD
constantly monitors the heart rhythm. When it detects a very fast, abnormal heart rhythm,
it delivers energy (shock) to the heart muscle to cause the heart to beat in a normal rhythm
again.
IV] Surgery:

1. left ventricular assist device insertion: This procedure requires the patient to meet strict
criteria and have advanced, end-stage heart failure. The VAD can be used as a bridge-to-
transplant, which means it can help a patient survive until a donor heart becomes available for
transplant.
A left ventricular assist device (LVAD) receives blood from the left ventricle and delivers it to
the aorta. A right ventricular assist device (RVAD) receives blood from either the right atrium
or right ventricle and delivers it to the pulmonary artery. Some VADs can perform the functions
of either an LVAD or a RVAD, or both (BiVAD).
2. Heart transplant.

V] Emerging Specific Therapies :

Only recently are specific etiology-based therapies being evaluated. These include
agents to eradicate persistent viral infections and immunomodulatory agents. Stem
cells for cardiac regeneration and gene therapy approaches are in clinical trials