Title: CARDIOMYOPATHIES
1CARDIOMYOPATHIES
- Definition
- Cardiac dysfunction caused by myocardial disease.
- Five Categories
- 1- Dilated Cardiomyopathy ( genetic ).
- 2- Hypertrophic Cardiomyopathy ( genetic ).
- 3- Restrictive Cardiomyopathy.
- 4- Arrythmic Cardiomyopathy (genetic ).
- 5- Unclassified Cardiomyopathy .Disease has not
fature 1- 4 (Fibroelastosis and Mitochondrial
disease) - - Some of the Infiltrative and systemic diseases
cause Spesific heart muscle disease called
Secondary Cardiomyopathy or Specific
Cardiomyopathy.
2Secondary Myocardial Diseases
- Definition
- Myocardial disease of known origin. (Secondary
Cardiomyopaty) - 7 Subgroups
- 1- Ischemic Cardiomyopathy
- 2- Hypertensive Cardiomyopathy
- 3- Valvular Cardiomyopathy
- 4- Alcoholic Cardiomyopathy
- 5- Metabolic Cardiomyopathy
- 6- Muscular Distrophy Cardiomyopathy
- 7- Peripartum Cardiomyopathy
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4HYPERTROPHIC CARDIOMYOPATHY (HCM)
- Definition Massive ventricular hypertrophy with
no absolute cause. - Not all of these patients show IHSS (Idiopatic
Hypertrophic Subaortic Stenosis) or HOCMP
(Hypertrophic Obstructive Cardiomyopathy)
features. - There is no pressure gradient at left ventricle
outflow(LVOT) tract in 1/3 of patient. (Rest or
provocated gradient). - HCMP prevalance 1/500. HCMP is the most
prevalant genetic cardiovascular disease. - Nearly 60 inherited. Relation with mutation of
beta-myosin heavy chain and also other sarcomeric
proteins has been shown. (Troponin- T, -I, -C,
Myosin Related Protein- C etc.). - HCM is the most frequent cause of sudden cardiac
death among young people including athletes.
5- Structure of Human Sarcomere Contraction is
carried out by the interaction of actin and
myosin. - The process begins with the ligation of calcium
to troponin complex (I, C, T) and
alpha-tropomyosin. Then, actin binds to myosin
and activates ATPase of the spheric myosin, so
that contractile function is carried out. Cardiac
myosin binded protein C binds to myosin and
starts contraction.
6- HYPERTROPHIC CARDIOMYOPATHY Cellular changes of
myocytes. Cell disarrangement ise seen on
microscope. Left Organized and parallel cell
arrangement in normal myocardium. Right
Disarrangement affects impuls conduction and
promotes ventricular arrythmias.
7Common HCM types (A)- LV free wall, diffusely
hypertrophied IVS. (B)- Asymmetric septal
hypertrophy (C)- MassiveConcentric LV hpertrophy
( involve walls, papillary muscles).
(A)-
(B)-
-(C)
8HCMP Patophysiology
- Most patients have septum hypertrophy with
non-dilated LV and/or RV cavity. - Hypertrophy of the septum may be diffuse or
only the upper, mid or apical hypertrophy of the
septum may take place. - Hypertrophy is extednded to the LV free wall in
most of the patients. - Diastolic filling is impaired because of the
incomplete relaxation and compliance of the LV. - Hypertrophic LV empties most of its content in
the first half of systole. (Hyperdynamic systolic
function). - Mitral anterior leaflet is displaced towards
septum during mid systole and this causes
obstruction the the LV outflow tract during this
period. - At the obstructive phase of the disease mitral
regurgitation is always present. - There ise resting pressure gradient at LV outflow
tract in 35 of patients. - Gradient can be precipitted at the other 25 (by
augmenting myocardial contraction, and reducing
ventricular volume). - Fibrosis and occlusive disease in in small
coronary arteries. - The major coronary arteries, are wide and patent
unless occlusive atherosclerosis occurs.
9- HCM
- Symtomps Angina, Dyspne, Presyncope, Syncope.
- Physical Findings
- 1- Before LV beat, LA beat is palpated (S4) Is
present even in the absence of any gradient or
murmur. Impaired LV relaxation. - 2- LVOT Sistolik ejection Murmur
Crescendo-Decrescendo, starts with S1 and ends
with S2. - Best heard between apex and left sternal border.
Cervical radiation is weak. Augmented by
manouvers and drugs which decrease preload.
(Valsalva, standing, amyl nitrite). Attenuates
with increasing afterload (squating, handgrip
fenilefrin). - 3- MR murmur Heard at late systole, radiates
to axilla, and related with LV outflow
obstruction. Mitral diastolic rumble and
Paradoxic splitting of S2 may be heard. - 4- Hyperdynamic carotis pulse.
-
10HCM Clinical Presentation and Mechanisms
- Chest pain Ischemia, LVOT ob. Reduced coronary
perfusion pressure. - Exertional dyspnea Diastolic dysfunction.
- Reduced functional capacity LVOTob, systolic
dysfunction, AFwith uncontrolled rapid
ventricular rate. - Palpitation SVT, AF, frequent VPB, non-sustained
VT. - Syncope/Presyncope Supraventricular arrythmia,
LVOTob, vasovagal, high VT rate . - Inadequate increase cardiac output during the
effort. - Cardiac arrest VT, SVT, AF, VF, bradyarrythmia.
11HCM Sudden death
- Supraventricular arrythmia is present in 20-50
of the patients, but sudden death is caused by
ventricular arrythmia. - Major Risk Factors of Sudden Death
- 1- Patient who survive after cardiac arrest
(sustained ventricular tachycardia). - 2- Arrythmia and abnormal blood pressure
response to excersize. - 3- Non-sustained ventricular tachycardia
(Holter). - 4- Family history of sudden cardiac death and
syncope in more than two first degree relatives
before 40 years. - 5- LV Wall thickness gt30 mm.
12HCM Other manifestations. Atrial Fibrillation
- Seen in 15 of patients with HCM.
- Absence of atrial systole. Rapid ventricular rate
causes pulmonary edema or hypotension. - Rapid ventricular rate causes detoriation of
functional capacity. - By conversion to sinus rythm or decreasing heart
rate functional capacity improves. - Endocarditis
- May occur on aortic or mitral valves. Unexpected
heart failure and IE symptoms or signs should
be suggest IE in HCM patiernts.
13HCM ECG and CHEST FILM
- Chest film May be normal-large left heart
chambers. . No aortic calcification. - ECG Is anormal in 97 of symptomatic HCMP, and
in 90 of asymptomatic HCMP patients. - AF is detected in 15.
- Non-sustained VT is frequent. .
- Q waves in DII, DIII, aVF and D1, aVL, V5, V6
(and less frequently in V1-3). This sign shows
hypertrophy, and causes pseudoinfarct patern. - Intraventricular conduction delay.
- High voltage findings of LVH.
- T waves of LVH.
- Huge negative T waves are frequently seen in
apical HCMP high precordial QRS voltage. - Short PR and pre-exitation may be seen, but is
infrequent.
14HCMP Echocardiographic Hallmarks
- Asymetric (dispropotionate) septal thickening
Septum to posterior wall ratio gt 1.5 - LV myocardial segment gt1.5 cm in thicknesss.
- Poor Septal contraction. Hypercontractile free
posterior wall. - Systolic anterior motion of the mitral valve
(SAM) when outflow tract gradient gt30 mmHg . - Mid-systolic closure of aortic valve.
- Small LV cavity.
- Mitral regurgitation is frequent.
- LVOT gradient at rest present in about 35 of
patients
15HCM Characteristic ECG paterns.
- Left axis deviation
- LBBB
- Pathologic Q wave on anterolateral leads.
- T wave inversion (commonly in Inferolateral
leads) - ST segment changes.
- Criteria for left atrial enlargement.
- V3-5 or V4-6 huge T wave inversion (Distal-
apikal HCM
16HCM Pseudoinfarction patern, Q wave.
17HCMP Management. Pharmacologic and surgical
intervention.
- OBSTRUCTIVE PHASE
- Beta bockers (Especially latent obstruction).
- Verapamil.
- Disopiramid. Amiodaron (SVT, VA ).
- Digoxin (contraindicated).
- DiĆ¼retic (contraindicated).
- ACEI (contraindicated).
- Surgery Septal myectomy.
- Septal ablation.
- ICD is indicated in severe VA.
- LAST PHASE
- Beta blockers (small doses)
- VerapamilContraindicate.
- DisopiramidContraindicate.
- Digoxin Beneficial.
- Diuretic Beneficial.
- ACEI Beneficial in patients with LV dilatation
and HF. - Surgery Transplantation.
18HOCM LVOT gradient.
19- Hypertrophic obstructive cardiomyopathy (HOCM)
As Mitral valve changesWhen the LVOTis narrowed,
blood rushes through the passageway toward the
aortic valve(like tight garden hose nozzle),
dragging the leaflets of the mitral valve with
it. Mitral valve normally functions keep blood
floiwing in direction from the left atrium
(upper heart chamber) to the LV. However
increased force of blood caused by HCM pulls the
valve open and may cause blood leak backward
(called regurgitation )into the LA.
NORMAL
LVOTobs.
20Anterior replacement of the papillary muscle in
HOCM MR, SAM
21HOCM Septal myectomy Before the operation There
is severe hypertrophy of the basal septum, whith
systolic anterior motion of the mitral valve
(-A-). This results in severe LVOT obs. as well
as MR. During the surgery (-B ), yhe portion of
the basal septum that project into the outflow
tract is removed by scalpel, resulting in
abolition of the LVOTobs. (-C-). There is no
longer SAM, and theMR abolished.
22HOCM Septal ablation (with absolute ethanol).
Indication LVOT gradient at rest gt 30-50 mmHg.
With provocation 75-100 mmHg.
23HOCM Decreased LVOT gradient after septal
ablation.