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RESTRİCTİVE CARDİOMYOPATHY (RCMP): PowerPoint PPT Presentation


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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.

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RESTRİCTİVE CARDİOMYOPATHY (RCMP):

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Restr ct ve card omyopathy rcmp l.jpg

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.


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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.


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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.


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RCMP: Patophysiology.Increased ventricular diastolic pressure, decrased filling, and clinical end-points.


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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.


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RCM: ECG


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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, 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.


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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.


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Right Ventricular Cardiomyopathy- Arrythmogenic 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 byfat 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.


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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.


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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.


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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.


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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.


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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.


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DCM (PostMI Remodelling): Infarct expansıon,+ nonİnfarct regions hypertrophy,+ global LV dilatation,+ HF.


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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) .


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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 enlargementand ventricular thrombus is frequently seen.

    Mild pericardial effusion is frequent.


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DCMP: ECG


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DCMP: Chest radiogram (Tele).


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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.


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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, RV- Failure, AF, 1 or 2 degree AV block, VT, LBBB.


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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) Anti-arrythmic 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.


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