RESTRICTIVE CARDIOMYOPATHY (RCMP):

1
RESTRICTIVE CARDIOMYOPATHY (RCMP)

• Definition 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
  dissociation between LV and RV diastolic filling
  pressures during inspiration.
• Concordant parallel changes in both LV and RV
  diastolic pressures during respiration.
• Classification (Ety)
• 1- Primary (a) Löfflers endocarditis, (b)
  Endomyocardial fibrosis.
• 2- Secondary (a) Infiltrative diseases, (b)
  Storage disease. (c) Post- radiation disease.

2

• 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,neo
  plasm) 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 function .
• Sarcoidosis, Hemosiderosis, Eosinophilic
  syndromes, Scleroderma, Adriamycin toxicity,
  infectious diseases including tuberculosis.
• Restrictive patophysiology may be at pericardial
  (CP), endocardial (EMF), or myocardial (HCM)
  level.

3
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) endocardium.The
lower part of the panel is a heart with patchy/
white areas involving the IVS 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.
4
RCMP Patophysiology. Increased ventricular
diastolic pressure, decrased filling, and
clinical end-points.
5
RCMP Clinical Presentation

• Intermittant fever, dyspnea, cough, palpitation,
  edema, tiredness.
• Eosinophlia with abnormal eoosinophil
  degranulation 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 obliteration of apices
  of the ventricles by echogenic masses, likened
  boxing glove.
• (b) Numerous echogenic areas are usually observed
  througout the ventricular myocardium. (c) Also,
  myocardial calcification may be detected, (d) an
  in later stages MR and TR.
• ECG Non-specific. ST-T wave changes and LVH may
  be present.

6
RCM ECG
7
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.
• Digoxin may be used for rapid ventricular
  response in AF.
• If dyspnea is prominent, ACEI 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.

8
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 formation. After decortication, the
RV becomes larger.
9
(No Transcript)
10
Right Ventricular Cardiomyopathy- Arrythmogenic
right ventricular cardiomyopathy/displasy (RVC/D)

• Uncommon. Occurs in young and children.
• Male/Female2-3/1. Autosomal dominant.
• Pathology Involve 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.

11
ARVD/C Major and Minor criteria

• MAJOR CRITERIA
• Familial disease documanted on autopsy or
  surgery.
• ECG Epsilon wave or QRS duration gt110 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 (lt35 y). Family
  history of clinical diagnosis criteria.
• Delayed potentials (signal averaged-ECG). T wave
  inversion in V2,3 in the absence of RBBB.
• gt12 years ECG holter, exercise testing
  Tachycardia with LBBB patern. gt1000/24 hrs VES.
• Mild RV dilatation/dysfunction LV function
  normal/near normal, mild RV segmentary
  dilatation. Regional hyperkinesis of RV.

12
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.
13
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.
14
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 (12).
• 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.

15
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.
16
DCM (PostMI Remodelling) Infarct expansion,
nonInfarct regions hypertrophy, global LV
dilatation, HF.
17
(No Transcript)
18
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) .
  

19
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.

20
DCMP ECG
21
DCMP Chest radiogram (Tele).
22
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 degeneration and necrosis.
• HOLTER Monitorisation Helps diagnose lethal
  arrythmias.

23
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 lt3 of these were known heart
  disease.
• PROGNOSIS 1 year mortality was 25.
• ACEI 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 Is the
  worse prognostic factor.
• 2- Echocardiography Global hypokinesis, EF
  lt20, spheric LV geometry, reduced LV mass
  volume, and RV dilatation.
• 3- Clinical parameters Aging, history of
  syncope, S3 gallop, RV- Failure, AF, 1 or 2
  degree AV block, VT, LBBB.

24
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
  Prevention) (1) RAAS blockers (ACEI/ARB/AA,
  BBl). , (2) Statins, ASA (3) Anticoagulation, (4)
  Anti-arrythmic drugs, device (Amiodarone, ICD).
• 4. Cardiac Transplantation Young, refractory HF,
  NYHA IV. Maximal oxygen consumption at
  cardio-pulmoner exercise testing lt 12 mL/kg/min,
  LVEFlt12, low quality of life. Contraindications
  Presence of non cardiac other comorbid diseases
  (Pulmonary, Hepatic, Renal, Psyciatric, and
  Alcoholism).
• 5. CRT. Indication NYHA class 3- 4,NSR, QRS gt120
  ms.