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RESTRİCTİVE CARDİOMYOPATHY (RCMP):
• Definitıon: The major abnormality is restriction of
ventricular filling, thus an increase in filling presures.
• The usual abnormality; impaired relaxation and
compliance.
• In contrast to Constrictive Pericarditis (CP); LV and RV
diastolic filling pressures are discordant in RCMP, but
concordant in CP.
• Discordant, means; the hemodynamic phenomenon of
dissociatıon between LV and RV diastolic filling
pressures during inspiration.
• “Concordant”; parallel changes in both LV and RV
diastolic pressures during respiratıon.
Classıfıcatıon (Ety):
1- Primary: (a) Löffler’s endocarditis, (b) Endomyocardial
fibrosis.
2- Secondary: (a) Infiltrative diseases, (b) Storage
disease. (c) Post- radiation disease.
RCMP:
Primary characteristic is inflammation ( due to parasitic infection,
autoimmun diseases, eosinophilic leukemia) and eosinophilia
associated this chronic inflammatory processes.
Systemic form; is classified by the spesific type of material
(amiyloid, sarcoid,hemochromatosis,neoplasm) deposition ( ie.
infiltration , storage or replacement).

Primary form; The most important cause of RCM is
Endomyocardial Fibrosis (EMF) which is seen in tropical regions.
Another form is (called acute form) Hypereosinophilic heart
disease (Löffler Disease).

Amiyloid deposition of myocardium may not be associated
with systemic amiyloidosis. (West of the world).
 Specific heart muscle disease: This form, usually produces
dilated CM with impaired both systolic and diastolic functıon .
Sarcoidosis, Hemosiderosis, Eosinophilic syndromes, Scleroderma,
Adriamycin toxicity, infectious diseases including tuberculosis.
Restrictive patophysiology; may be at pericardial (CP), endocardial
(EMF), or myocardial (HCM) level.
Restrictive/Infiltrative cardiomyopathy: The gross image on top shows a
heart with marked EMF which prevents diastolic compliance of the LV. Note the
markedly thick (white) endocardıum.
The lower part of the panel is a heart with patchy/ white areas involving the İVS
from the base to the apex and contiuning into the free wall of the LV owing to
infiltration by incaseating granulomata in a case of sarcoidosis.
RCMP: Patophysiology. Increased ventricular
diastolic pressure, decrased filling, and clinical endpoints.
Restrictive Cardiomyopathy:
Pathophysiology
73
RCMP: Clinical Presentation
• Intermittant fever, dyspnea, cough, palpitation, edema, tiredness.
• Eosinophlia with abnormal eoosinophil degranulatıon seen in
temperate climates (Löffler Syndrome).
• Eosinophilia is less severe in tropical EMF.
• S3 or S4 gallop may be visible an audible in the absence of HF.
• Symptoms and signs of HF and of moderate- to- severe MR and TR
owing to involvment of the papillary muscles serve differentiate
RCM from CP, as does the greater degreee of cardiac enlargement
on chest X-ray in the former condition.
Chest radiogram: Calsiffication may be seen on RV or LV apical
myocardium in EMF.
Echocardiogram: (a) Shows obliteratıon of apices of the ventricles by
echogenic masses, likened boxing glove.
(b) Numerous echogenic areas are usually observed througout the
ventricular myocardıum. (c) Also, myocardial calcificatıon may be
detected, (d) an in later stages; MR and TR.
ECG: Non-specific. ST-T wave changes and LVH may be present.
RCM: ECG
RCM: Treatment.
• There is no standart and effective therapy (No medical therapy
guideliness).
• Steroids: Beneficial in early inflammatory phase of
hypereosinophilia. Hydroxyurea and Vincristin are also
beneficial.
• Anticoagulation is needed as thromboembolism is frequent.
• Ventricular underfilling does not repond to digoxin, diüretics or
vasodiators.
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Digoxin may be used for rapid ventricular response in AF.
If dyspnea is prominent, ACEİ should be tried.
Tachyarrythmia may respond to low doses of beta blockers.
Amiodarone may be needed during lethal arrythmias.
• Resection of endocardial obliterating tissue and valve repair in
EMF may cause symptomatic relief.
Preop (top) and postop (bottom) RV Angiograms;
in patient with biventricular EMF: Characteristic, most pathognomic
finding in obliteration of the RV apex with a residual “bay- like”
formatıon. After decorticatıon, the RV becomes larger.
Right Ventricular CardiomyopathyArrythmogenic right ventricular
cardiomyopathy/displasy (RVC/D):
• Uncommon. Occurs in young and children.
• Male/Female=2-3/1. Autosomal dominant.
Pathology: İnvolve primarily RV. LV pathology is rare.
Characteristic: Segmentary, progressive, non-inflammatory myocyte
loss, replaced by fat and fibrosis.
Myocytes are replaced with “fibro-fatty” tissue in RVC/D.
Diagnosis: Cine Magnetic Resonance Imaging (cMRI). This can
differantiate between normal and fatty myocardium. Morphologic
changes can be detected. Regional and global ventricular
dysfunctional abnormalities can be detected. Correlation between
imaging and pathological tissue characteristics has been shown.
ARVD/C: Major and Minor criteria
• MAJOR CRITERIA
Familial disease documanted on autopsy or surgery.
ECG: Epsilon wave or QRS duration >110 ms in V1-3
Severe RV dilatation and systolic dysfunction. Mild LV pathology
may or may not be present.
Localized RV aneurisms (diastolic ballooning, localized dyskinetic
or akynetic regions). Severe RV segmentary dilatation.
Biopsy: Myocardium replaced by fibro-fatty infiltration.
• MINOR CRITERIA
Family history of sudden death (<35 y). Family history of clinical
diagnosis criteria.
Delayed potentials (signal averaged-ECG). T wave inversion in
V2,3 in the absence of RBBB.
>12 years: ECG holter, exercise testing: Tachycardia with LBBB
patern. >1000/24 hrs VES.
Mild RV dilatation/dysfunction: LV function normal/near normal,
mild RV segmentary dilatation. Regional hyperkinesis of RV.
Arrythmogenic Right Ventricular
Displasia/Cardiomyopathy: Fatty tissue infiltrates
RV free wall. This is seen in AV groove. Microscopic
view of RV free wall is seen at bottom.
ARVD/C: ECG: “Epsilon wave” is marked with arrow. This is
classic finding of ARVD. On right precordial leads, epsilon wave
and T wave inversion is present. There is conduction delay on
right precordial derivations.
Dilated Cardiomyopathy (DCM)
• Definition: Chronic heart muscle disease characterized by cavity
enlargement (getting spheric shape) and impaired globalsystolic
function of the LV or both ventricles.
3 Specific property: (1) decreased systolic function. (2) Global
dilatation and/or hypokinesis LV or RV. (3) If there is no CAD,
congenital, valvular, hypertensive, specific heart muscle disease,
and chronic alchohol consumption; in absence of these secondary
causes DCM must be considered in patients who have criterias
(1+2).
Alcholol does not cause dilated cardiomyopathy. But augments
dilated cardiomyopathy. Past viral infection is the estimated
diagnosis in %50 of patients.
It was thought that some of the disease were of familial origin. Now
the genetic origin is known.
Patients are between 20-50 years old. But DCM may also be seen in
children.
HF begins as NYHA clas III or IV in %75 of patients.
DCMP: Essential Process is: “Chronic volume overload”, due to
Primary- MR / idiopathic LV dysfunction, secondary- MR ( commonly
ischemic origin).
Last phase: LV cavity topography degenerated to spheric- shape with
manifest HF.
DCM (PostMI Remodelling): Infarct expansıon,+ nonİnfarct regions
hypertrophy,+ global LV dilatation,+ HF.
DCM: Clinical Presentation
• Dyspnea: Progressive exertional dyspnea.
• Signs of both LHF and RHF are common .
• Apical beat: Is replaced left, lateral and inferiorly because of LV
dilatation. Left sternal impuls are palpable because of RV dilatation.
• JVP: Is raised, and may show systolic wave of TR. (large V wave).
• MR/TR: Both MR and TR murmurs of I – III/IV degree may be heard
because by the dilatation of both LV, RV,and the AV anulus of the AV
valves both are dilate.
• S3 and S4: And sinus tachycardia are nearly always present.
Summation gallop is a frequent finding. S3 is heard in nearly all
patients and may also be present in the absence of heart failure.
This finding differantiates DCM from ischemic CM. Mild S3 is heard
in the prescence of HF. In the absence of HF, and if LV aneurysm
occured, no S3 can be heard.
• BP; Is frequently low and is a sign of poor prognosis.
• Diastolic murmur: Is frequently abscent. This finding rules out HF of
specific origin (as AR) .
DCM: Lab. Tests (I):
• ECG:
Sinus tachycardia, AF in %25.
T wave inversion or flattening. Mild LVH may be masked because of
low voltage.
Pseudoinfarct pattern: Q wave, or poor R wave progression in V2-4.
Conduction disturbances in %75 of patients.
Intraventricular conduction delay: LAH, and in a small group, LBBB or
RBBB, infrequently.
• Chest Radiogram (Tele):
Cardiomegaly, frequently all chambers are enlarged.
Pulmonary vascular evidence of left atrial enlargement. Pulmonary
venous congestion, interstitial edema, and pleural effusion.
• Echocardiography:
Severe dilatation in both ventricules. EF is generally between %10-30.
Atrial enlargement and ventricular thrombus is frequently seen.
Mild pericardial effusion is frequent.
DCMP: ECG
DCMP: Chest radiogram (Tele).
DCMP: Lab. Tests (II):
• ENDOMYOCARDIAL BIOPSY: Indications:
To rule out myocardial disease.
Especially in myocarditis in which immunsuppressive therapy is
planned.
To diagnose viral particles.
Pathologic properties: Myocyte degeneration and necrosis.
Interstitial fibrosis. Myocyte hypertrophy.
In some patients, histologic findings are non-specific.
Interstitial fibrosis points out past viral myocarditis.
Pathologic myocarditis diagnosis is made according to “Dallas
Criteria”:
Active myocarditis: Myocytolysis and necrosis near the regions of
infiltration.
Borderline myocarditis: Increased infiltrates, without myocyte
degeneratıon and necrosıs.
HOLTER Monitorisatıon: Helps diagnose lethal arrythmias.
DCM: Prognosis.
• Previous viral infections is suspected up to %50 of patients.
• Cardiomegali and reduced EF is present in %20 of patients with
DCM.
• In %26 of DCM patients autoimmune antibodies were detected. But
<%3 of these were known heart disease.
PROGNOSIS: 1 year mortality was %25.
ACEİ decrease %50 mortality, when was given early phase of HF.
Poor Prognostic Parameters:
LV systolic function is the most important prognostic factor.
1- “Low EF, or high NYHA class ≥III: ” İs the worse prognostic factor.
2- Echocardıography: Global hypokinesis, EF <%20, spheric LV
geometry, reduced LV mass volume, and RV dilatation.
3- Clinical parameters: Aging, history of syncope, S3 gallop,
Failure, AF, 1 or 2 degree AV block, VT, LBBB.
RV-
DCM: Treatment.
•
BASIC PRINCIPLE: Preventing recurrence of HF,and systemic
embolization, arrythmia, sudden death, and other life threating
complications.
1. Treatment of chronic DHF:
(a) Bed rest, salt restriction, diet, restriction of alcholol consumption.
b) NYHA -III, -IV and Killip ≥2 patients must be hospitalized, and take standart
therapy: IV diuretic, vasodilators, inotropic agents, oxygen therapy.
c) Precipitating factors DHF must be evaluated, and treated. (Infection,
anemia, arrythmia, tolerance to diet and drug therapy).
2. Treatment of acute DHF: Increase diuresis, decrease volume overload,
relief symptoms.
3. Complementary treatment( Secondary Preventıon): (1) RAAS blockers
(ACEI/ARB/AA, BBl). , (2) Statins, ASA (3) Anticoagulation, (4) Antiarrythmic drugs, device (Amiodarone, ICD).
4. Cardiac Transplantation: Young, refractory HF, NYHA IV. Maximal oxygen
consumption at cardio-pulmoner exercise testing < 12 mL/kg/min,
LVEF<%12, low quality of life. Contraindications: Presence of non cardiac
other comorbid diseases (Pulmonary, Hepatic, Renal, Psyciatric, and
Alcoholism).
5. CRT. İndication: NYHA class 3- 4,NSR, QRS >120 ms.