Cardiomyopathies

Definition :
Cardiomyopathies
Dilated or congestive c.m Hypertrophic or asymmetric hypertrophic c.m Restrictive
Or HOCM O for obstructive
Large baloonised of ventricles both Pathological hypertrophy of myocardium, Pathological infiltrations in myocardium
Global hypokinesia = global impairement of especially in the upper part of septum => firm myocardium , thicker
myocardium contractility Hyperkinetic heart => Failure to relaxation
Systolic failure During systole, the septum becomes shorter, thee Diastolic failure
septal obstruction buldges further into the cavity => back flow
=> obstruction to the outflow which is different
from aortic stenosis or HTA symmetric
hypertrophy (equal in all parts)
Echo Echo Echocardio
Poorly contracting heart Vigorously contracting heart
Very bug heart with big cavity and thin myocardial Hypertrophy which is asymmetrical
wall Upper part of septum is more hypertrophied then
the free wall
Banana shaped cavity

Dynamic obstruction (aortic stenosis fixed

obstruction)
100 % gee defect
50% familial AD, 50% sporadic
Most important cause of death in young during
vigorous activity => more dynamic heart =>
obstruction more pronounced => less outflow =>
underfieled aorta + more blood flow to muscles =>
severe myocardial ischemia => sudden cardiac
arrhythmia => death

force generatig mechanisms impared Defect in sarcomeres proteines => impaired force
- structural defect or generation
- functional defect
Energy generation
Dilated CM
Progressive cardiac dilatation with impaired contractility
Biventricular systolic failure
LV failure => poor contractility =>  pressure backward => PVP PCP => pulmonary edema
- cough, dyspnea, PND, orthopnia
- bilateral crepitations
RV failure =>  pressure in vena cava =>
-  Jugular veinous pressure
- congestive hepatomegaly
- veins can’t empty well to the right heart => capillaries can’t empty to veins => ascites and generalized edema
Congestion in :
- pulmonary circulation due to LV failure
- systemic circulation due to RV failure
Both are congested => congestive heart failure
The only c.m which causes from the beginning congestive heart falure

Weak pulse
OAP
 JVP hepatomegaly ascites and
S3 : overfilled ventricle is rapidly filled during diastole
Dilatation of ventricles => annulous dilatation => mitral/ tricuspid cusps don’t close
Functional MR, TR+
Dvpt of thrombi stagnant blood flow
Dilated C.m is the most frequent C M
Causes : ABCCCDHP
A Alcohol
B Beri Beri thiamine deficiency
C Coxsachi virus
C Cocaine
C Chagas disease
D Drugs Doxorobacin
H Hemochromatosis
P Pregnancy
Idiopathic +++
Genetic factors
Genetic factors :
A special protein stabilizing sarcomeres by attaching them to the basement membrane through the cell membrane
Dystrophin <= mutation
=> systolic failure sarcomeres are there but can’t work well => can t eject => get dilated

 𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡
P = 𝛼𝛼   P =>  cardiac output
 𝑅𝑅

- mutant genes => defect in enzyme oxidative phosphoralation => not enough energy
So force generatig mechanisms impared
- structural defect or
- functional defect
Myocardial cells can use fatty acid as energy fuel
- the enzyme concered to break down fatty acid are mutated
Hypertrophic CM
Underlying mutation
Asymmetric hypertrophy especially in the upper part of septum => LV cavity banana shaped
=> dynamic obstruction to LV outflow
During systole => septal shortening => the hypertrophied part bulges into the cavity =>
HOCM
100% genetic defect
50% familial AD
50%
Defect :
Heavy chain myosin especially beta myosin
Troponine , tropomyosine
Defect in sarcomere proteines => impaired force generation => the cell releases growth factors cause it can’t generate
enough force => growth of myocardial cells => hypertrophy => hypercontractile heart
Histologically Microscope :
Bundle of myocytes disarray disorganized hazardely arranged large myocyte, proeminent nuclei
Individual myocyte sarcomeres disorganized
At the peak of systole => maximum of buldge => blood flows with high velocity => mitral valve touches the
obstructive septum venture effect => mitral regurgitation =>
With every systole anterior mitral valve beats against the dynamic obstruction => thickened
Angina pain
Exercise => fatal tachy arrhythmia
Heart generates more force => need more oxygen
Because of the obstruction => coronary arteries are hypo perfused
Exercional dyspnea
Harsh systolic murmur
blood passing in narrowed space => high velocity => murmur => harsh systolic murmur => similar murmur in aortic
stenosis
in both outflow obstruction and there is hypertrophy but here it s asymmetric hypertrophy
EDV AS HOCM Why
   Points of obstruction are far from each other because of more blood in the cavity
   Points of obstruction are near

Evolution Risc of
Fatal arrhythmia
Infective endocarditis (abnormal endothelium)
Atrial fibrillation : LA hypertrophied
Thrombosis
LV failure
LA hypertrophied => vigourous contraction => S4

Slow donw the heart beta blockers

Surgical excision
Catether => alcohol inection => localized myocardial infarction => fibrosis => reduce obstruction

Restrictive cardiomyopathy
Ventricular filling is obstructed
Heart 2 functions
Diastolic : ventricle should relax enough to fill adequately
Systolic : stroke volume and cardiac output
Normal size but thick
Causes
1- Idiopathic
2- Post radiation
 3- Amyloidosis : extra cellular deposition of amyloid protein
Transthyretin a protein that transforms thyroid hormones and retinoid acid
Beta plated => no enzyme to digest it (contrarily to alpha)
Any protein which made it to beta plateing
Deposition in extra cellular and around capillaries = ischemic changes
=> firm hardened heart
4- Saroidosis multisystemic granulomatous disease
A collection of epitheloid cells (modified macrophages) surrounded by +/- geant cells
5- Glycogen storage diseases defect in enzymes that degrade glycogen => accumulation
6- Metastatis
7- Endomyocardial fibrosis : fibrotic process of endocardium starts in th apex involves LV +++ : Africa
Mural thrombi formation
8- Loeffler endomyocarditits a lot of circulating eosinophils : eosinophilic leukemia
Leukemia malignancy of hematopoietic cells
While they circulate, they degranulate and release their products MBP major basophilic protein in the
endocardium => damage of endo and myo cardium => endo myocarditis
=> thrombi formation
9- Endocardial fibroelastosis

Constrictive pericarditis : scar of pericardium