Download Hıpertrophıc cardıomyopaty

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.
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 ejectıon 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).
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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.
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4- Hyperdynamic carotis pulse.
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 .
İnadequate increase cardiac output during the effort.
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Cardiac arrest: VT, SVT, AF, VF, bradyarrythmia.
HCM: Other manifestations.
Atrial Fibrillation
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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 İE in HCM
patiernts.
HCM: ECG and CHEST FİLM
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.
HCM: Characteristic ECG paterns.
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Left axis deviation
LBBB
Pathologic Q wave on anterolateral leads.
T wave inversion (commonly in İnferolateral leads)
ST segment changes.
Criteria for left atrial enlargement.
V3-5 or V4-6 huge T wave inversion (“Distal- apikal HCM”
HCM: Pseudoinfarction patern, Q wave.
HCMP: Echocardiographic Hallmarks
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Asymetric (dispropotionate) septal thickening: Septum to
posterior wall ratio > 1.5
LV myocardial segment >1.5 cm in thicknesss.
Poor Septal contractıon. Hypercontractile free posterior wall.
Systolic anterior motıon of the mitral valve (SAM) when outflow
tract gradient >30 mmHg .
Mid-systolic closure of aortic valve.
Small LV cavity.
Mitral regurgitation is frequent.
LVOT gradient at rest present in about %35 of patients
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Hypertrophic obstructive cardiomyopathy (HOCM): As Mitral
valve changes:When the LVOTis narrowed, blood rushes through the
passageway toward the aortic valve dragging the leaflets of the mitral valve
with it. Mitral valve normally functıons keep blood floıwing 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 regurgitatıon )into the LA.
NORMAL
LVOTobs.
Anterıor replacement of the papillary muscle in HOCM :
MR, Systolic anterior motion (SAM)
HCM vs Athlete’s Heart
HCM
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Unusual pattern of LVH
LV cavity <45 mm
LV cavity >55 mm
LA enlargement
Bizarre ECG paterns
Abnormal LV filling
Female gender
 thickness with deconditioning
Family history of HCM
Circulation 1995; 91:1596
Athlete
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HOCM: Septal myectomy: Before the operatıon There is severe hypertrophy of the basal
septum, whith systolic anterior motıon of the mitral valve (-A-). This results in severe LVOT
obs. as well as MR. During the surgery (-B ), yhe portıon of the basal septum that project into
the outflow tract is removed by scalpel, resulting in abolitıon of the LVOTobs. (-C-). There is
no longer SAM, and theMR abolished.
HOCM: Septal ablation (with absolute ethanol). İndication: LVOT
gradient at rest > 30-50 mmHg. With provocation 75-100 mmHg.
HOCM: Decreased LVOT gradient after septal ablation.
Restictive Cardiomyopathy
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Tanım:
Ventriküler sertliğin daha farklı
mekanizma/patolojilerle artış gösterdiği bir grup
bozukluğu temsil etmektedir. İnfiltrtif ,
inflamatuar veya metabolik hastalıkların
sonucunda restriktif kardiyomyopati gelişebilir.
Idiopathic Dilated
Cardiomyopathy
IDC - Definition
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a disease of unknown etiology that principally
affects the myocardium
LV dilatation and systolic dysfunction
pathology
increased heart size and weight
 ventricular dilatation, normal wall thickness
 heart dysfunction out of portion to fibrosis
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Incidence and Prognosis
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3-10 cases per 100,000
20,000 new cases per year in the U.S.A.
death from progressive pump failure
1-year
2-year
5-year
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25%
35-40%
40-80%
stabilization observed in 20-50% of patient
complete recovery is rare
Observed Survival of 104 Patients
120
104
100
80
72
60
56
51
45
40
37
35
31
24
20
19
16
0
0
1
2
3
4
5
6
7
Years
Am J Cardiol 1981; 47:525
8
9
10
Predicting Prognosis in IDC
Predictive
Possible
Not Predictive
Clinical factors
symptoms
age
duration
viral illness
Hemodynamics
LVEF
Cardiac index
LV cond delay
complex VPC
alcoholism
peripartum
family history
LV size
atrial pressure
AV block
atrial fibrillation
myofibril volume
Dysarrhythmia
Histology
Neuroendocrine
hyponatremia
plasma norepinephrine
atrial natriuretic factor
simple VPC
other findings
Clinical Manifestations
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Highest incidence in middle age
blacks 2x more frequent than whites
 men 3x more frequent than women
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symptoms may be gradual in onset
acute presentation
misdiagnosed as viral URI in young adults
 uncommon to find specific myocardial disease on
endomyocardial biopsy
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History and Physical
Examination
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Symptoms of heart failure
pulmonary congestion (left HF)
dyspnea (rest, exertional, nocturnal),
orthpnea
 systemic congestion (right HF)
edema, nausea, abdominal pain, nocturia
 low cardiac output
fatigue and weakness
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hypotension, tachycardia, tachypnea, JVD
Cardiac Imaging
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Chest radiogram
Electrocardiogram
24-hour ambulatory ECG (Holter)
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lightheadedness, palpitation, syncope
Two-dimensional echocardiogram
Radionuclide ventriculography
Cardiac catheterization
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age >40, ischemic history, high risk profile, abnormal
ECG
Clinical Indications for
Endomyocardial Biopsy
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Definite
monitoring of cardiac allograft rejection
 monitoring of anthracycline cardiotoxicity
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Possible
detection and monitoring of myocarditis
 diagnosis of secondary cardiomyopathies
 differentiation between restrictive and constrictive
heart disease
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Management
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Limit activity based on functional status
salt restriction of a 2-g Na+ (5g NaCl) diet
fluid restriction for significant low Na+
initiate medical therapy
ACE inhibitors, diuretics
 digoxin, carvedilol
 hydralazine / nitrate combination
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Management
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consider adding ß-blocking agents if
symptoms persists
anticoagulation for EF <30%, history of
thromboemoli, presence of mural thrombi
intravenous dopamine, dobutamine and/or
phosphodiesterase inhibitors
cardiac transplantation
myocardial noncompaction