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.
1- Primary:(a) Löffler’s endocarditis, (b) Endomyocardial fibrosis.
2- Secondary:(a) Infiltrative diseases, (b) Storage disease. (c) Post- radiation disease.
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).
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.
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.
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.
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.
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.
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.
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.
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.
Cardiomegaly, frequently all chambers are enlarged.
Pulmonary vascular evidence of left atrial enlargement. Pulmonary venous congestion, interstitial edema, and pleural effusion.
Severe dilatation in both ventricules.EF is generally between %10-30.
Atrial enlargementand ventricular thrombus is frequently seen.
Mild pericardial effusion is frequent.
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.
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.
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.