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1MAGDI AWAD SASI CARDIOMYOPATHY 2013Cardiomyopathies ` 2006 AHA defined cardiomyopathies as “a heterogeneous group ofdiseases of the myocardium associated with mechanical &/or electricaldysfunction that usually (but not invariably) exhibit inappropriateventricular hypertrophy or dilatation and are due to a variety of causesthat frequently are genetic.”FACTS: Cardiomyopathy is the 2ndmost common cause of sudden deathPrognosis for Cardiomyopathy is very poor** Undiagnosed until in advanced stages **DIAGNOSTIC EVALUATION:Echocardiography → confirms dilatedcardiomyopathyChest X-Ray → reveals cardiomegaly associatedwith any of the cardiomyopathiesCardiac Cath with possible Biopsy → can bedefinitive in diagnosing hypertrophiccardiomyopathyMost cardiac disease is secondary to some other condition (e.g., coronaryatherosclerosis, hypertension, or valvular heart disease). However, there aresome that are attributable to intrinsic myocardial dysfunction. Such myocardialdiseases are termed cardiomyopathies ( heart muscle diseases).
2MAGDI AWAD SASI CARDIOMYOPATHY 2013They are a diverse group that includes inflammatory disorders (myocarditis),immunologic diseases (e.g., sarcoidosis), systemic metabolic disorders (e.g.,hemochromatosis), muscular dystrophies, and genetic disorders of cardiacmuscle cells. In many cases, cardiomyopathies are of unknown etiology (termedidiopathic); however, several previously "idiopathic" cardiomyopathies havebeen shown to be caused by specific genetic abnormalities in cardiac energymetabolism or structural and contractile proteins.Classification
3MAGDI AWAD SASI CARDIOMYOPATHY 2013Dilated Cardiomyopathy(DCM) DCM is most common of all CMs(60%) Dilated cardiomyopathy (DCM) is characterized by progressive cardiacdilation and contractile (systolic) dysfunction. It is sometimes calledcongestive cardiomyopathy. Approximately 25% to 50% of DCM caseshave a familial (genetic) basis. Others result from a variety of acquiredmyocardial insults including toxic exposures (e.g., chronic alcoholism),myocarditis, and pregnancy . In some patients, the cause of DCM isunknown. Such cases are appropriately called idiopathic dilatedcardiomyopathy. Many in this category are likely to be of genetic origin.Regardless of the cause, all share a similar clinicopathologic picture. AetiologyIdiopathic (IDC)30%Myocarditis (9%) Viral / Bacterial InfectionIschemic (7%)Genetic disorders 50%(AD)HypertensionHyperthyroidismValvular Heart DiseaseChemotherapyPeripartum CMPCardiotoxic Effects of Drugs or alcohol Morphologically The heart in DCM is characteristically enlarged (two to three times itsnormal weight) and flabby, with dilation of all chambers. Because of the wall thinning that accompanies dilation, the ventricularthickness may be less than, equal to normal. Mural thrombi are commonand may be a source of thromboemboli.
4MAGDI AWAD SASI CARDIOMYOPATHY 2013 By definition there is no primary valve pathology; consequently, anyvalvular insufficiency is a secondary consequence of ventricular chamberdilation. The coronary arteries are usually free of atherosclerotic stenosis.Enlargement of RV & LV cavities without an increase in ventricular septalor free wall thickness → spherical shape & dilatation of heart →Displacement of papillary muscles → Regurgitant lesions despite valveleaflets being normalPathophysiology: Microscopically – fibrosis & scarring Systolic Dysfunction>>> Diastolic dysfunction STROKE VOLUME is initially maintained by ↑↑ EDV With disease progression→Marked LV dilatation with normal or thin wall→↑ Wall stress + Valvular Regurgitation →Overt Circulatory Failure .
5MAGDI AWAD SASI CARDIOMYOPATHY 2013Clinical Features Symptoms: GRADUAL , MIDDLE AGE, MIMIKING URTIThe fundamental defect in DCM is ineffective contraction. Hence in end-stage DCM, the cardiac ejection fraction is typically less than 25%.Typically pts c/o months of fatigue, weakness, reduced exercise toleranceSymptomatic HF (syncope, dyspnea, volume overload)LVF- Dyspnea, PND, orthopnea, cough, frothy sputum, palpitationRVF-Odema, abdominal swelling,constipation, RT hypochondrial painSome patients are asymptomaticMay also present as a Stroke, Arrythmia or Sudden Death.Fifty percent of patients die within 2 years, and only 25% survive longerthan 5 years Physical Signs( signs of heart failure)Tachycardia , pulsus alternansJugular venous distensionPulse pressure is narrowVariable degrees of cardiac enlargementDisplace apical impulse3rdor 4thheart sound are common (with the bell)MR=blowing scratchy systolicMitral/ tricuspid regurgitation may occurlung--Diminished breath sounds with effusionsRales if in failureSummary==== symptoms and signs of heart failureDiagnosis: CXR- Cardiomegaly , Pulmonary venous congestion ,pleural effusion ECG- Normal or low QRS voltage , abnormal axis, non specific ST segabnormalities, LV hypertrophy, conduction defects, Non sustainedVentricular tachycardia/Sinus tachycardia/atrial fibrillation, Left atrialabnormality, LBBB
6MAGDI AWAD SASI CARDIOMYOPATHY 2013 2D EchoChamber size - Wall thickness/shape( Eccentric hypertrophy/ Usually thin)Clot formationEjection Fraction -Normal 55-65%//Mild Dysfunction 41-55%Moderate Dysfunction -----------26-40%Severe Dysfunction----------------<26% Coronary Angiography- age >40-usually normal coronaries-coronary vasodilatation is impaired by ↑ LV filling pressures-distinguishes b/w Ischemic & Idiopathic DCM Endomyocardial Biopsyrarely valuable to identify the aetiology
7MAGDI AWAD SASI CARDIOMYOPATHY 2013Management Aim of treatment-Manage the symptoms-Reduce the progression of disease-Prevent Complications salt restriction of a 2-g Na+(5g NaCl) diet fluid restriction for significant low Na+ Mainstay of TherapyVasodilators + Digoxin + Diuretics + BB(CARDIOSELECTIVE)Reduce preload Reduce afterload_ Diuretics Arterial Vasodilators_ Venous Vasodilators ACE Inhibitors_ ACE Inhibitors_ Aldosterone antagonistsIncrease Contractility_ Decrease afterload_ DigoxinVasodilators (afterload reducing drugs) ACE Inhibitors-Indicated for all patients- Reduce symptoms & improve effort tolerance- Suppress ventricular remodelling & endothelial dysfunction-Reduce CV mortality SpironolactoneUsed along with ACE Inhibitors has shown to reduce mortality by 30% in alarge double blind randomized trial Digoxinclinically beneficial as reaffirmed by two large trials in adults
8MAGDI AWAD SASI CARDIOMYOPATHY 2013 β Blockers- carvedilolThey provide symptomatic improvement and substantial reduction insudden death in NYHA class II & III HF pts Amiodarone-High grade ventricular arrhythmias (Sustained VT or VF) are common inDCM→↑ risk of SCD-Preferred anti arrhythmic agent as it has least negative inotropic effect& proarrhythmogenic potential-Implantable Defibrillators are used for refractory arrhythmias. Anticoagulants-Indicated for pts with moderate ventricular dilatation+mod-severesystolic dysfunction-H/O stroke , AF or evidence of Intracardiac thrombus Hydralazine / nitrate combination anticoagulation for EF <30%, history of thromboemoli, presence of muralthrombi intravenous dopamine, dobutamine and/or phosphodiesterase inhibitorsPts refractory to Pharmacological therapy for CHF Dual Chamber Pacing Cardiomyoplasty LV Assist Devicesimproved pts sufficiently to avoid transplant or enable later transplant Cardiac Transplantationhas substantially prolonged survival in DCM pts with 5 yr survival rate of78%.
9MAGDI AWAD SASI CARDIOMYOPATHY 2013Hypertrophic CardiomyopathyHypertrophic cardiomyopathy (HCM) (also known as idiopathic hypertrophicsubaortic stenosis) is characterized by myocardial hypertrophy, abnormaldiastolic filling, and-in a third of cases-ventricular outflow obstruction. Theobstruction, in some cases, is dynamic, caused by the anterior leaflet of themitral valve. The heart is thick-walled, heavy, and hyper contracting. Systolicfunction is usually preserved in HCM, but the myocardium does not relax andtherefore shows primary diastolic dysfunction. Most common cause of sudden death in young athletes. Characterized by in appropriate and elaborate LV hypertrophy withmisalignment of the myocardial fibres. Hypertrophy may be generalized or confined largely to interventricularseptumHeart failure may develop because stiff non-compliant ventricles impedesystolic filling and decreased cardiac outputSeptal hypertrophy may cause dynamic LV outflow obstruction.Mitral regurgitation occur due to abnormal systolic anterior mitral valveleaflet.REMMEBER:Primary genetic cardiomyopathy and left ventricle disease.Effects men and women equallyHypertrophy of myocardial muscle massCause - transmitted genetically(autosomal dominant)Disarray of cardiac myofibrils with hypertrophy of myocytesCells take on a variety of shapesMyocardial scarring and fibrosis occurs
10MAGDI AWAD SASI CARDIOMYOPATHY 2013Pathophysiology Condition is genetic disorder with Autosomal Dominant transmission. Due to single point mutation in one of the genes that encode sarcomericcontractile proteins. Morphology : The essential gross feature of HCM is massive myocardial hypertrophywithout ventriculardilation .The classicpattern of HCM involvesdisproportionatethickening of theventricular septumrelative to the leftventricle free wall (so-called asymmetricalseptal hypertrophy);nevertheless, in about10% of cases there isconcentric hypertrophy.On longitudinalsectioning, theventricular cavity losesits usual round-to-ovoidshape and is compressedinto a "banana-like"configuration .Often present is an endocardial plaque in the leftventricular outflow tract, as well as a thickening of the anterior mitralleaflet. Both findings reflect contact of the anterior mitral leaflet with theseptum during ventricular systole and correlate with functional leftventricular outflow tract obstruction.
11MAGDI AWAD SASI CARDIOMYOPATHY 2013Pathophysiology( post graduate)1. Subaortic Obstruction2. Diastolic Dysfunction3. Myocardial Ischemia4. Mitral Regurgitation5. ArrythmiasA. Subaortic Obstruction1. Cause -Assymetrical Septal Myocardial Hypertrophy .2. Subaortic ObstructionEffect-Systolic anterior motion(SAM) of AML → accentuating obstructionMechanism of SAMThickened IVS→Restricted LVOT → ejection of blood at a higher velocitycloser to the AML → Drawing of AML closure towards the hypertrophiedseptum due to the venturi effect → Dynamic LVOT obstructionFactors aggregating SAM and cause dynamic obstruction(30-50%):↑ Contractility↓ Afterload (Aortic outflow resistance)↓ Preload (End diastolic volume) Therapeutically Myocardial depression, Vasoconstriction & Volumeoverloading should minimize obstruction & augment forward flowB. Diastolic DysfunctionSTIFF LEFT VENTRICLE/IMPAIRED RELAXATION/PASSIVE FILLINGATRIAL KICK (S4) IS ESSENTIAL TO MAINTAIN CARDIAC OUT PUTDECREASE LV CAVITY= DIASTOLIC DYSFUNCTION= LV FILLING
12MAGDI AWAD SASI CARDIOMYOPATHY 2013FILLING PRESSURES & PULMONARY CONGESTIONCompensation for decreased filling -> hyperdynamic systolicDysfunction ------------EF increases to 70-80%c.Mitral Regurgitation– Hypertrophied papillary muscles– Leaflets become calcified and thick– Atrial dilatation_ Enlargement of mitral valve annulusD. Myocardial Ischemia◦ Often occurs with normal coronary arteries.◦ Postulated mechanisms Abnormally small coronary arteries as a result of hypertrophy Inadequate number of capillaries for the degree of LV mass Early closure of aortic valve with decreased cardiac output Increased myocardial oxygen demand due to :1. Myocardial hypertrophy2. Diastolic dysfunction3. Myocyte disarray4. LVOT obstruction5. ArrhythmiaTypes Hypertrophic Cardiomyopathy• Asymmetric septal (ASH) - without obstruction• Asymmetric septal (ASH) - with obstruction• Symmetric hypertrophy - concentric• Apical hypertrophy• HCM or HOCM
13MAGDI AWAD SASI CARDIOMYOPATHY 2013Signs & SymptomsHCM is characterized by a massively hypertrophied left ventricle with diastolicfailure and systolic dysfunction. In addition, roughly 25% of patients havedynamic obstruction to the left ventricular outflow by the anterior leaflet of themitral valve. Many asymptomatic for years Incidence of sudden death often first presentation Identified during screening of relative of patient with HCM Symptoms related to severity of diastolic dysfunction ormitral regurgitation.1. Dyspnea on exertion (90%)/activity intolerance-LVF , HF ,ISCHEMIA2. Angina on effort (70-80%)3. Syncope on effort (20%)-LVOT4. Palpitations5. Sudden cardiac death1. Ventricular wall mass > 30 mm increased risk2. More common in patients under 403. Normally occurs during strenuous activitySIGNS(( MR+ AS)) Pulse- Irregular Pulse (with Atrial-fibrillation) Bisferiens Carotid Pulse (HOCM)– Brisk initial upstroke/ jerky pulse– Collapse of pulse then secondary rise– Must differentiate from AS – delayed upstroke Apex beat- forceful and brisk/LV heave /Double impulse at apex S4 MR murmur-Pansystolic murmurSystolic murmur with obstructive disease process
14MAGDI AWAD SASI CARDIOMYOPATHY 2013Sign of LV outflow obstruction- Mid systolic, best heard along left sternalboarder, usually does not radiate, crescendo-decrescendo, harsh or roughHOCM murmur louder during Valsalva’s maneuver Decreases venous return to the heart– Decreased preload _ _ Decreased left ventricular filling _ _obstruction Any factor that decreases venous return to the heart increases themurmur in HOCM– Squatting increases venous return– Standing decreases venous return Aortic stenosis murmur becomes quieter during Valsalva’s maneuverDiagnosis:.2/3 of patients have family H/O, the rest have sporadic mutations (de novo) HIGH INDEX OF SUSPECION IN YOUNG MALE WITH SYNCOPY.ECG-It is abnormal in 75 to 95% of patients, ↑ QRS voltage, ST-T changes, Axisdeviation, LV Hypertrophy +strain pattern, p wave abnormalities, Negative TWaves in V3-V5 (apical HCM),Abnormal Q waves may reflect septal hypertrophyCXR-Lt atrial enlargement or normalEcho Wall thickness -LV size, Hyperdynamic LV function, Atrial sizeMV leaflets, LV outflow obstruction
15MAGDI AWAD SASI CARDIOMYOPATHY 2013ManagementGoals– Relief of symptoms- Relax the ventricleSlow the Heart Rate– Use Negative Inotropes– Preventing complications– Preventing or reducing risk of sudden death– No evidence to support treatment of nonsymptomatic patientsDRUGS B-blocker and rate-limiting calcium antagonists (eg verapamil) Most patients are improved by therapy that promotes ventricularrelaxation. β Blockers- mainstay of therapyRelieves symptoms of exercise intolerance & dyspnoea associated with
16MAGDI AWAD SASI CARDIOMYOPATHY 2013CHF by- negative inotropic effect-HR reduction , lower myocardial O2 demand- longer diastolic filling times , improve filling of LV CCB-Verapamil is indicated if β Blockers not tolerated or ineffective-It improves diastolic function & ventricular relaxation causing improvedfilling, decreased obstructive features in 50% pts-CCBs with strong vasodilatory effect are C/I in pts with obstructivesymptoms Cardioversion (ATRIAL-Fib to Sinus) Anticoagulants Amidarone ACE-I AND NGT to be avoided. Diuretic - with cautionSURGERY:Indications1. Subaortic gradients≥ 50mmHg frequently associated with CHF & arerefractory to medication2. Marked outflow obstruction3. On maximum medical therapy4. NYHA Class III or IV Septal Myotomy +Partial Mymectomy through a transaortic approachrelieves the obstruction, reduces the LVOTO gradient, SAM & MR MV Replacement or repair at same time (increases operative mortality) Improvement noted immediately and last 20-30 years May need pacer due to development of LBBB. Complications –CHB or septal perforation (0-2%) Mortality rate-1to 3% Percutaneous Alcohol SeptalAblationNot appropriate if MVR needed, Better for patients > 55Catheter in septal perforatorEthyl alcohol injected -MI occurs-Enlarged septum eventually shrinks
17MAGDI AWAD SASI CARDIOMYOPATHY 2013Ablation of AV Node (HOCM),Dual Chamber Pacemaker (HOCM)Heart TransplantCOMPLICATION:1. Atrial fibrillation, VT , V.fibrillation.2. Mural thrombus formation3. IE of the mitral valve4. CHF5. Sudden death. PrognosisAdults - 2-3% SCD per yAdolescents - 4-6% SCD per yearMARKERS OF INCREASED RISK1. Prior cardiac arrest or spontaneous sustained VT.2. Family history of premature HCM death3. Syncope or near syncope (exertional or recurrent)4. Multiple and repetitive burst of NSVT5. Hypotensive response of BP to exercise6. LVH greater than 30 mm(septum)7. Myocardial ischemia (with angina)
18MAGDI AWAD SASI CARDIOMYOPATHY 2013Restrictive CMWHO in 1995 defined RCM as restrictive filling & reduced diastolic volume ofeither or both ventricles with normal or near normal systolic function & wallthickness.Restrictive cardiomyopathy is characterized by a primary decrease in ventricularcompliance, resulting in impaired ventricular filling during diastole ( the wall isstiffer). The contractile (systolic) function of the left ventricle is usuallyunaffected. Thus, thefunctional state can beconfused with that ofconstrictivepericarditis orhypertrophiccardiomyopathy.Restrictivecardiomyopathy canbe idiopathic orassociated withsystemic diseases thatalso happen to affectthe myocardium .Least common form ofCardiomyopathy5% of 1ry heart muscle Disease. Ventricular filling is impaired because ventricles are stiff. Lead to high atrial pressure with atrial hypertrophy, dilatation and lateratrial fibrillation. Amyloidosis is the most common cause
19MAGDI AWAD SASI CARDIOMYOPATHY 2013Pathophysiology:Ventricular chamber has limited ability to expand during filling (diastolicdysfunction)- Filling Impairment- Rate of LV filling is slowDecreased volume available to eject- Elevated LV filling pressurePressure Overload -------- Often elevated LV EF.stroke volume & cardiac output1. volume and pressure in atria(INCREASE)2.Dilated atrium due to increase volume & pressure3.Increase volume & pressure in pulmonary system4.Heart failure symptoms3 problems:1. Stiffness of the ventricle2. Ventricular Filling Reduced3. The rigidity of the myocardium causes failure to completely contractduring systole-------------((End-result is decreased CO))----------CAUSES Primary---idiopathic• MYOCARDIAL1. NoninfiltrativeIdiopathic, Scleroderma2. InfiltrativeAmyloid 90% north AMERICA ,Sarcoidosis ,Gaucher disease,Radiation carditis , Hurler disease3. Storage DiseaseHemochromatosis, Fabry disease, Glycogen storage• ENDOMYOCARDIALEndomyocardial fibrosis, Hyperesinophilic syndrome, Carcinoidmetastatic malignancies, radiation, anthracyclineSigns & Symptoms:HEART FAILURE IS THE END RESULT OF CARDIOMYOPATHY.
20MAGDI AWAD SASI CARDIOMYOPATHY 2013Fatigue, weakness ,SyncopePalpitations with arrhythmiasDyspnea, OrthopneaEdemaChest PainSIGNS –Pale/ cool-------Peripheral pulse decreased, Pulsus paradoxusJVP-prominent x and y descents Kussmaul’s sign-↑ JVP during inspirationMurmur of Mitral Regurgitation-----Systolic Murmur, 5th ICS MCLM.R.- Dilation of atrium , Papillary muscle dysfunction, Fibrosis of leafletsS4----------Left Lateral Position, Bell of StethoscopeDiagnosis: Diagnosis is difficult Rule Out Other Causes of Diastolic Dysfunctiona. Aortic Stenosisb. Hypertrophic Cardiomyopathyc. Hypertensive Cardiovascular Disease Differentiate from Constrictive Pericarditis.Clinical FeaturesConstrictivePericarditisRestrictiveCardiomyopathyHistoryPrior history ofpericarditis orcondition that causespericardial diseaseHistory of systemicdisease (e.g..Amyloidosis,Hemochromatosis)Heart SoundsPericardial knock, highfrequency soundPresence of louddiastolic fillingsound S3, low frequencysoundMurmurs No murmursMurmurs of mitral andtricuspidinsufficiencyArrhythmias
21MAGDI AWAD SASI CARDIOMYOPATHY 2013Heart PressuresL & R filling pressures upandequal(Elevated JVP)L sided filling pressures >R sidedfilling pressures Require complex Doppler echocardiographyCXR- pulmonary congestion, small heart size, Dilated atrium ,Congestion if in HFCalcified pericardium can be seen inconstrictive pericarditis. ECG- Low QRS voltage, No-specific ST-T wave Changes, P waveabnormalities,Arrhythmias,Conduction abnormalities, BBBs, low voltage,QR or QS complexes, An abnormal heart rhythm is customary• Echo-Doppler abnormal mitral inflow pattern prominent E wave (rapid diastolic filling) reduced deceleration time ( LA pressure)Impaired ventricular relaxation & abnormal Compliance causes rapidfilling in early diastole & impeded filling during rest of diastoleCharacteristicVentricular diastolic waveform of Dip & Plateau (Square root sign)-RA pressure waveform-M or W shaped due to rapid y descent
22MAGDI AWAD SASI CARDIOMYOPATHY 2013 Pressure in the ventricle rises precipitously in response to small volume Both ventricles appear thick(Increased in infiltrative disorders )with smallcavities in contrast to corresponding dilated atria Lt sided Pulmonary venous pressure >Rt sided venous pressure by 5mmHg PASP↑↑ upto 50mmHg Speckled appearance on myocardium with amyloidosisCardiac Catheratization Full cath not necessary -Hemodynamic measurements valuable_ Elevated LVEDP_ Elevated PAOP_ Elevated RA Pressures_ Elevated pulmonary pressures CT and MRI and endomyocardial biopsy may be done.Endomyocardial BiopsySeptal wall of RV, Multiple sitesEssential for diagnosis of RCMExclusion “Guidelines• LV end-diastolic dimensions 7 cm• Myocardial wall thickness 1.7 cm• LV end-diastolic volume 150 mL/m2• LV ejection fraction < 20%
23MAGDI AWAD SASI CARDIOMYOPATHY 2013TREATMENTThere is no cure for the disease. Similar to most types of cardiomyopathiesthere are ways to reduce symptoms and prevention.The purpose OF medical treatment is to alleviate the problems of heart rateand prevent blood clots.Transplantation is the best treatment. IdiopathicDiuretics-To relieve congestionB-blockers, Amiodarne, CCBs- Control of HRvasodilators may decrease filling pressureLong term anticoagulation-MR,TR,LA LARGE,AF, FIBROSISCCBs, ACEI- To enhance myocardial relaxationDual Chamber Pacing- AV blockCardiac Transplantation- Refractory Heart Failure Amyloidosis- Melphelan, prednisone, H+L transplant Haemochromatosis- Phlebotomy, Desferrioxamine Treat Rhythm AF Control - Loss of atrial kick, Digoxin cautiously in amyloidosis Conduction abnormalities ---------May require pacemakerOutcomes_ Poorest mortality of all cardiomyopathies_ 90% mortality rate at 10 years (Kavinsky & Parrillo, 2000)._ Amyloid Heart 80% mortality at 2 yearsArrhythmogenic Right Ventricular Dysplasia Patches of the right ventricular myocardium are replaced with fibrousand fatty tissue. Inherited as autosomal dominant trait Dominant clinical problems are ventricular arrhythmia Sudden death Right sided cardiac failure ECG shows inverted T waves in the right precordial leads. MRI useful diagnostic tool and used to screen 1stdegree relatives fromhaving the same pathology.
24MAGDI AWAD SASI CARDIOMYOPATHY 2013Obliterative Cardiomyopathy Involves the endocardium of one or both ventricles and is characterizedby thrombosis and elaborate fibrosis with gradual obliteration of theventricular cavities. Mitral and tricuspid valves are regurgitant. Heart failure and pulmonary and systemic embolism are prominent. Associated with eosinophilia Eg: eosinophilic leukemia, churg strauss syndrome Mortality is high (50% at 2 years of developing the symptoms) Anticoagulation and antiplatelet therapy is advisable and diuretics mayhelp symptoms of HF. Surgery valve replacement with decortication of endocardium may behelpful in certain cases.MyocarditisIn myocarditis there is inflammation of the myocardium with resulting injury.It is important, however, to emphasize that the presence of inflammationalone is not diagnostic of myocarditis; for example, inflammatory infiltratescan also occur as a secondary response to ischemic injury. In myocarditis, theinflammatory process is the cause of-rather than a response to-myocardialinjury.Lymphocytic myocarditis is most common .If the patient survives the acutephase of myocarditis, the inflammatory lesions either resolve, leaving noresidual changes, or heal by progressive fibrosisPathogenesisINFECTIONS Viruses-coxsackie virus, HIV, influenza,ECHO,cytomealovirusChlamydiae PsittaciRickettsiae typhi , typhus feverBacteria-Diphtheriae, Neisseria meningeocccus,Lyme diseaseFungus- CandidaProtozoa-Trypanosome (chagas disease), ToxoplasmosisHelminths- Trichinosis
25MAGDI AWAD SASI CARDIOMYOPATHY 2013IMMUNE MEDIATED REACTIONSPost viralPost streptococcal-Rheumatic feverSystemic lupus erythematosusTransplant rejectionDrug hypersensitivity- methyl dopa, sulphonamidesUNKNOWNGiant cell myocarditisSarcoidosisClinical FeaturesThe clinical spectrum of myocarditis is broad.At one end, the disease is asymptomatic and patients recover withoutsequelae, and at the other end is the precipitous onset of heart failure orarrhythmias, occasionally with sudden death.Between these extremes are the many forms of presentation, associated with avariety of symptoms (e.g., fatigue, dyspnea, palpitations, pain, and fever).The clinical features of myocarditis can even mimic those of acute MI.Occasionally, over many years, patients can progress from myocarditis to DCMClinical PresentationAcute Fulminant Chronic Nonspecific cardiacsymptoms Heart failure, AcuteMI, or SCD More common inchildren/teenagers +/- viral prodrome Cardiogenic shock+/- acute heart failure Biopsy doesn’t matchthe clinical severity. High levels ofcytokines reversible cardiacdepression betterprognosis Subtle, insidiousonset Already have DCMHF symptoms Biopsy with fibrosisusually
26MAGDI AWAD SASI CARDIOMYOPATHY 2013Diagnosis:Symptoms: non-specificLaboratory Testing: also non-specific Positive cardiac biomarkers ECG: T wave inversion, ST segment elevation, bundle branch blocksECHO Differentiate fulminant from acute myocarditis Detect thrombi, valvular abnormalities, and pericardial involvement Rule out other cardiomyopathies (HOCM, Takotsubo)Cardiac MRINon-invasiveVisualize entiremyocardiumUse to guide biopsyFollow disease courseand response to therapyCoronary Angiography Rule out other congenital, rheumatic, or ischemic heart disease Determine need for inotropic or mechanical support based onhemodynamic parameters Elevated pulmonary artery pressures are independent predictors ofmortalityEndomyocardial Biopsy Although controversial, still the current gold-standard test for diagnosis 1-6% complication rate Consider when suspicious for:
27MAGDI AWAD SASI CARDIOMYOPATHY 20131. Giant cell myocarditis2. Hypersensitivity/eosinophilic myocarditis3. Cardiac involvement in a systemic disease All other patients, consider only if pt is deterioratingTreatment Circulation: Intra-aortic balloon pump counterpulsation Ventricular assist device Cardiopulmonary assist device Medical therapy1. ACE-inhibitors2. Beta-blockersMost therapy used in HF patients appears to benefit those with HF due tomyocarditis – with the exception of digoxin Immunosuppressive therapy- N0 RULE
28MAGDI AWAD SASI CARDIOMYOPATHY 2013 Intra-aortic balloon pump Electrocardiographic synchronized phased pulsationo Inflation with aortic valve closureo Deflation just before systole Reduce systolic arterial pressure (afterload)o Reduces myocardial oxygen consumption Augment diastolic arterial pressureo Enhances coronary blood flow Mean pressure unchangedIntra-aortic balloon pump Benefits: Diminish myocardialischemia 10-20% increase in CO Diminish heart rate Increase urine output Risks: Damage/perforation ofaorta Distal ischemia Thrombocytopenia Hemolysis Renal emboli Mechanical failure –balloon ruptureVentricular-assist device Centrifugal pump or Archimedes’ screw type Inflow from LV and outflow into aorta Has been used as a bridge in myocarditis until recovery or transplant
29MAGDI AWAD SASI CARDIOMYOPATHY 2013 Disadvantages:1. Surgical implantation2. infection3. thrombosis4. hemolysis**Centrifugal pump vs. corkscrew“Loose” rule of third’s… 1/3: recover 1/3: residual ventricular dysfunction 1/3: transplantation or death
30MAGDI AWAD SASI CARDIOMYOPATHY 2013Diagnosis Expanded Criteria Suspicious formyocarditis = 2 positivecategories Compatible withmyocarditis = 3 positivecategories High probability ofbeing myocarditis = all 4categories positive Category I: Clinical symptoms Category II: Evidence of Cardiacdysfunction in the Absence ofregional coronary ischemia Category III: Cardiac MRI Category IV: Myocardial biopsy- Pathological or MolecularAnalysis Most common cause is viruses (adeno and coxsackie) Highly variable clinical manifestations Cardiac MRI looks promising for diagnosis Biopsy is the gold standard but should be pursued in only select patients Aggressive, supportive care is the first line therapy because of highincidence of recovery Immunosuppressive therapy does not affect mortality Supportive therapy is mainstay therapy Most medical therapies for HF seem to benefit myocarditis patients withthe exception of digoxin
31MAGDI AWAD SASI CARDIOMYOPATHY 2013 Immunosuppressive therapy does not seem to play a role in survivalBIOPSY