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CARDIOMYOPATHIES

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CARD OMYOPATH ES Definition: Cardiac dysfunction caused by myocardial disease. Five Categories: 1- Dilated Cardiomyopathy ( genetic +). 2- Hypertrophic ... – PowerPoint PPT presentation

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Title: CARDIOMYOPATHIES


1
CARDIOMYOPATHIES
  • 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.

2
Secondary 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

3
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4
HYPERTROPHIC 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.

7
Common HCM types (A)- LV free wall, diffusely
hypertrophied IVS. (B)- Asymmetric septal
hypertrophy (C)- MassiveConcentric LV hpertrophy
( involve walls, papillary muscles).
(A)-
(B)-
-(C)
8
HCMP 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.

10
HCM 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.

11
HCM 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.

12
HCM 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.

13
HCM 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.

14
HCMP 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

15
HCM 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

16
HCM Pseudoinfarction patern, Q wave.
17
HCMP Management. Pharmacologic and surgical
intervention.
  • OBSTRUCTIVE PHASE
  • Beta bockers (Especially latent obstruction).
  • Verapamil.
  • Disopiramid. Amiodaron (SVT, VA ).
  • Digoxin (contraindicated).
  • Diüretic (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.

18
HOCM 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.
20
Anterior replacement of the papillary muscle in
HOCM MR, SAM
21
HOCM 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.
22
HOCM Septal ablation (with absolute ethanol).
Indication LVOT gradient at rest gt 30-50 mmHg.
With provocation 75-100 mmHg.
23
HOCM Decreased LVOT gradient after septal
ablation.
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