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Cardiomyopathy
Cardiomyopathy, which literally means
"heart muscle disease", is the deterioration
of the function of the myocardium (i.e., the
actual heart muscle) for any reason
Cardiomyopathies can generally be categorized
into two groups, based on WHO guidelines:
extrinsic cardiomyopathies and intrinsic
cardiomyopathies
primary pathology is outside the myocardium itself.
Most cardiomyopathies are extrinsic, because by far
the most common cause of a cardiomyopathy is
ischemia
Causes of Extrinsic CMP:
•Coronary artery disease
•Congenital heart disease
•Nutritional diseases as deficiency of thiamin (vitamin B-1),
selenium, calcium and magnesium
•Hypertensive cardiomyopathy
•Valvular cardiomyopathy
•Inflammatory cardiomyopathy
• CMP secondary to a systemic metabolic disease like
diabetes, hyperthyroidism, lysosomal storage diseases and
the muscular dystrophies ,hemochromatosis ,amyloidosis
•Alcohol cardiomyopathy
•Chronic rapid heart rate (TICMP)
Intrinsic cardiomyopathies
An intrinsic cardiomyopathy is weakness in the muscle
of the heart that is not due to an identifiable external
cause
intrinsic cardiomyopathies are generally classified
into four types:
•Dilated cardiomyopathy DCM
•Hypertrophic cardiomyopathy (HCM or HOCM)
•Arrhythmogenic right ventricular cardiomyopathy
(ARVC)
•Restrictive cardiomyopathy (RCM)
•Dilated cardiomyopathy
(DCM), the most common form, and one of the
leading indications for Heart transplantation. In DCM
the heart (especially the LV) is enlarged and the
pumping function is diminished. It can affect people of
all ages, it occurs most often in middle-aged people, and
is more likely to affect men. Some people with dilated
cardiomyopathy may have a family history of the
condition. Approximately 40% of cases are familial,
but the genetics are poorly understood compared
with HCM. In some cases it manifests as peripartum
CMP(nigros,multiparous,obeise), and in other cases
it may be associated with alcoholism.
Symptoms of DCM
S&S of heart failure
Fatigue, shortness of breath, edema ,nocturia ,orthopnea
,pedal edema .Arrhythmia are common(AF,PVC s and VT).
Stagnated blood my complicate by DVT,PTE and stroke
raised JVP, displaced apex beat,S3 g ,S4 ,systolic Murmur
of functional MR and TR
Treatment: antifailure
ACEi,Ald,B2B,L.diuretics,anticoagulant, transplantation , LV
assist device (LVADs),stem cell therapy
•Hypertrophic cardiomyopathy
in this condition, the muscle mass of the left ventricle enlarges or
"hypertrophies."
In one form of the disease, the IV septum becomes enlarged and
obstructs the blood flow from the left ventricle. the thickened wall
sometimes distorts one leaflet of the mitral valve, causing it to leak.
HCM is known as hypertrophic obstructive cardiomyopathy (H.O.C.M.)
or asymmetric septal hypertrophy (A.S.H.). It's also called idiopathic
hypertrophic sub aortic stenosis (I.H.S.S.). Rare type known as
Yamagoshi syndrome (HCM in Japanis)
Hypertrophic cardiomyopathy is the most common inherited heart
defect, occurring in one of 500 individuals caused by various
mutations in genes encoding sarcomeric proteins β-myosin heavy
chain and Troponin T.
Close blood relatives (parents, children or siblings) of such persons
often have enlarged septums, although they may have no symptoms.
This disease is most common in young adults.
The symptoms of hypertrophic cardiomyopathy include shortness of
breath on exertion, dizziness, fainting and angina pectoris
The obstruction to blood flow from the left ventricle increases the
ventricle's work, and a heart murmur may be heard(systolic murmur at
the left sternal border, the pulses are jerky, the apex is not displaced
but feel of double apical paradoxical impulses
Some people have cardiac arrhythmias. in some cases can lead to
sudden death. Often an implanted cardioverter defibrillator (ICD) is
needed to shock the heart to restart a normal heart rhythm and
prevent sudden death ,beta blocker (such as propranolol) or a
calcium channel blocker. Surgical treatment of the obstructive
form is possible in some cases if the drug treatment fails.
Alcohol septal ablation is a type of nonsurgical treatment for
hypertrophic obstructive cardiomyopathy. It involves injecting
alcohol down a small branch of one of the coronaries to deaden
the extra heart muscle. This allows the extra heart muscle to
thin out without having to cut it out surgically(myomectomy).
Restrictive cardiomyopathy (RCM)
is an uncommon cardiomyopathy. The walls of the ventricles
are stiff, but may not be thickened, and resist the normal filling
of the heart with blood .the person with restrictive
cardiomyopathy often complains of being tired, may have
edema of hands and feet, and may have difficulty breathing on
exertion.
This type of cardiomyopathy can occur at any age, it most often
seen in the elderly
It's the least common type of cardiomyopathy and can occur
for no known reason (idiopathic). The condition may also be
caused by diseases elsewhere in the body that affect the heart,
such as amyloidosis
This disease mimic constrictive pericarditis and often difficult to
differentiate
The only effective therapy is transplantation!