Myocardial Disease 1x PDF

APPROACH TO PATIENTS WİTH MYOCARDIAL DISEASE Bülent MUTLU, Prof. MD. Marmara University School of Medicine Department of Cardiology  Dilated Cardiomyopathy NonDilated Cardiomyopathy Hypertrophic Cardiomyopathy Restrictive Cardiomyopathy Arrhythmogenic Right...

APPROACH TO PATIENTS WİTH MYOCARDIAL DISEASE Bülent MUTLU, Prof. MD. Marmara University School of Medicine Department of Cardiology  Dilated Cardiomyopathy NonDilated Cardiomyopathy Hypertrophic Cardiomyopathy Restrictive Cardiomyopathy Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)  DILATED CARDIOMYOPATHY Idiopathic D-CMP is a disease of unknown etiology that principally affects the myocardium leading to LV dilatation and systolic dysfunction. Most common of the cardiomyopathies Prevalence: 36 per 100.000 population Third common cause of heart failure and most common cause of transplantation Complete recovery is rare 50% die within 2 years and 25% survive longer than 5 years  Causes of D-CMP GENETIC: 20-50% are familial Autosomal dominant-predominant pattern Mutations in genes encoding dystrophin, sarcoglycan and troponin.. Myocarditis Alcohol and other toxins Childbirth (peripartum CMP)  Dilated Cardiomyopathy Myocyte injury Arrhythmias (Afib, VT) Contractility Sudden death Thromboembolism  Stroke volume Chest pain Forward LV Ventricular CO dilatation filling pressure Exertional dyspnea Fatigue, P. Congestion weakness MR S. Congestion  Morphology of D-CMP Heart enlarged, heavy, flabby Mural thrombi common Dilatation of all chambers (especially in advanced cases), both ventricular hypertrophy Microscopically-atrophic and hypertrophic myocardial fibers, cardiac myocytes show degenerative changes Interstitial and endocardial fibrosis  Clinical Features Highest incidence in middle age Symptoms may be gradual in onset Acute presentation - Misdiagnosed as URI in young adults Symptoms signs of heart failure: Pulmonary congestion, dyspnea, orthopnea systemic congestion, edema, hypotension, tachycardia, tachypnea, fatigue and weakness Arrhythmia; AF, conduction delays, Sudden Cardiac death Physical signs A poateint visits Er with edema in legs and jungular vein distension, upon futher investigation you hear rales on the lungs and the ECG shows Pulsus alternans, Jugular venous Murmurs Rales on Peripheral plusus alternus , what is Tachycardia distension pulmonary edema your diagnosis auscultation  Management Diuretics ACE inhibitors / Angiotensin receptor antagonists / ARNI (Sacubitril/Valsartan) Beta blockers SGLT inhibitors Spironolactone Digoxin Amiodarone Anticoagulants  Interventional Therapy Biventricular pacing (Cardiac resynchronization therapy) Left Ventricular assist device (LVAD) Cardiac transplantation  Patients is an adulensent you see normal examination finding non dilated LV sinus ruthme with minor inteventricualr delay , which one the cardiac morphology mostly will NonDilated Cardiomyopathy be seen ? Subepicardial scar  HYPERTROPHIC CMP Characterized by myocardial hypertrophy, abnormal diastolic filling, intermittent ventricular outflow obstruction. Ventricular outflow obstruction related to defects in force generation owing to altered sarcomeric function Leading cause of LVH, unexplained by other clinical/pathologic cause Caused by mutation of genes encoding sarcomeric proteins  Pathogenesis Autosomal dominant with variable penetrance Remaining are sporadic Mutations are mostly missense Mutations causing HCM found in genes encoding β MHC (myosin heavy chain), cardiac TnT, α tropomyosin, myosin binding protein C.  The major abnormality of the heart in HCM is excessive thickening of the muscle. Thickening usually begins during early adolescence and stops when growth has finished. Left ventricle almost always affected Hypertrophy is usually greatest in the septum, associated with obstruction to the flow of blood into the aorta.  Asymmetric septal hypertrophy with obstruction to the outflow of blood from the heart may occur. The mitral valve touches the septum, blocking the outflow tract. Some blood is leaking back through the mitral valve causing mitral regurgitation.  Histologic features Myofiber disarray Extensive myocyte hypertrophy Interstitial and replacement fibrosis  Pathophysiology Impaired diastolic filling-----reduced stroke volume Which one of the myocardial diseases of Reduced CO and increase in pulmonary venous pressure---exertional dyspnea the below ia accompanied with Diastolic dysfunction mitral regurgitation? –Impaired diastolic filling, filling pressure Myocardial ischemia Mitral regurgitation Arrhythmias  Clinical features Asymptomatic –Echocardiographic finding only Symptomatic –Dyspnea in 90% –Harsh systolic ejection murmur –Angina pectoris in 75% –Fatigue, pre-syncope, syncope, risk of SCD –Palpitation, PND, CHF, dizziness –Atrial fibrillation, thromboembolism  Management of H-CMP Pharmacological (B-blockers, CCB) Surgical Non Surgical Alternatives Implantable Cardioverter Defibrillator (ICD) Cardiac Transplantation  What are the Surgical Correction treatmentts done for HCM ? Indications Subaortic gradients≥ 50mmHg frequently assoc with CHF & are refractory to medication (high dose b blockers or CCB) Septal Myotomy + Partial Myectomy through a transaortic approach relieves the obstruction, reduces the LVOTO gradient, SAM & MR Complications: septal perforation (0-2%) Mortality rate: 1% to 3% Surgical Myectomy  Non surgical treatments Septal Ablation with Ethanol  Non surgical septal reduction therapy  2-5 ml of Ethanol is injected with an angioplasty balloon catheter lumen to the first major septal perforator of the LAD  Reduce LVOT grad in 85-90% pts immediately  Further ↓in grad & sympt improvement seen over next 3-6 mths  Permanent heart blocks can develop after this procedure (5-10%) Alcohol septal ablation Alcohol septal ablation  Non surgical treatments Dual Chamber or AV Sequential Pacing (DDD) Exact mechanism unknown Possible mech: Excitation of the septum of LV contracts it away from apposing wall which may reduce the LVOT gradient Now rarely recommended since symptoms actually worsen despite gradient reductions  RESTRICTIVE CMP RCM as restrictive filling & reduced diastolic volume of either or both ventricles with normal or near normal systolic function & wall thickness Contractile functions are generally normal and abnormal diastolic function is main problem. Rigid ventricular wall with impaired ventricular filling Much less common then DCM or HCM Characterized by primary disease in ventricular compliance resulting in impaired ventricular filing during diastole. Classification of R-CMP  Morphology Ventricles are of normal size Cavities are not dilated Myocardium is firm and non compliant Biatrial dilatation is common Patchy/diffuse interstitial fibrosis  Management Idiopathic Amyloidosis: Melphelan, prednisolone, Tx Diuretics Hemochromatosis: Phlebotomy, B-Blockers, Amiodarone, CCB Desferrioxsamine Long term anticoagulation Carcinoid: Somatostatin analogs, Valvular ACE-I, SGLT inh. surgery Internal pace maker Sarcoidosis: Pacing, steroids, Tx Cardiac transplantation  Arrythmogenic RV Cardiomyopathy (ARVC) Progressive replacement of RV myocardium with fat & fibrous tissue creating an excellent environment of fatal arrythmias Typical involves regional RV→ Global RV→ Partial LV involvement with sparing of septum Autosomal dominant inheritance  Arrythmogenic RV Cardiomyopathy (ARVC) In which type of myocardial diseases can we see ectopic beat intitation ? Presentation  Onset of arrythmias from RV range from VES-VF  SCD 75% due to VT/VF in sports related exercise  CHF 25%  Progressive RV & LV Dysfunction  Arrythmogenic RV Cardiomyopathy (ARVC) Diagnosis  Genetics, ECG, Serial Echo, EM Biopsy  ECG-Inverted T waves (Right precordial leads)  Epsilon wave, QRS>110ms  Ventricular extrasystoles, tachycardia with LBBB morphology MYOCARDITIS  It is characterized by: 1. Inflammatory cells infiltrates in myocardium 2. Myocyte degeneration or necrosis  Pathogenesis Myocardial inflammation, necrosis and fibrosis. Cardiomegaly and reduced systolic function occur due to myocardial damage. Typical signs of HF develop which may progress to cardiogenic shock, arrhythmias and sudden cardiac death.  Viruses act on myocardium in three phases. 1. Acute phases: replication of virus 2. Autoimmun Injury phase 3. Dilated CMP phase or chronic phase.  Endomyocardial biopsy in acute myocarditis: Arrow shows a collection of lymphocytes infiltrating the cardiac muscle in response to a viral infection.The arrowhead shows an area of cardiac muscle damage induced by the virus directly or to the cytotoxic immune response to the viral infection.  Fulminan myocarditis Acute myocarditis Fulminant myocarditis is characterized by moreextensive and diffuse lympocytic infiltration and myocyte necrosis than acute myocarditis Myocarditis represents a clinically and pathogenetically highly variable disease entity.  Diagnosis Myocarditis is a challenging diagnosis due to the heterogeneity of clinical presentations Clinical presentation: Myocarditis presents in many different ways, ranging from mild symptoms of chest pain and palpitations associated with transient ECG changes to life-threatening cardiogenic shock and ventricular arrhythmia  Signs and symptoms Chest pain (often described as "stabbing" in character). CHF (leading to edema,breathlessness and hepatic congestion). Palpitations (due to arrhythmias). Sudden death (in young adults, myocarditis causes up to 20% of all cases of sudden death). Fever (especially when infectious)  Signs and symptoms Since myocarditis is often due to a viral illness, many patients give a history of symptoms consistent with a recent viral infection, including fever, diarrhea, joint pains, and easy fatiguability. Myocarditis is often associated with pericarditis, and many patients present with signs and symptoms that suggest concurrent myocarditis and pericarditis.  Diagnostic Tests ECG- Sinus tachycardia, Non-specific ST segment and T-wave abnormalities Biomarkers: CK-MB and Troponin may be elevated Inflammatory markers: ESR and CRP levels are often raised, but they do not confirm diagnosis Chest X-Ray- Variable (Normal to Cardiomegaly) Echocardiogram: Normal/ wall motion abnormality or reduced systolic function, mural thrombus Cardiovascular Magnetic Resonance: Extent of scarring, LV function, inflammatory injury Endomyocardial biopsy- There are risks and not used for every case but is definitive for myocarditis  Treatment Acute myocarditis resolves in about 50% of cases in the first 2–4 weeks, but about 25% will develop persistent cardiac dysfunction and 12–25% may acutely deteriorate and either die or progress to end-stage DCM with a need for heart transplantation. The main principles of treatment in myocarditis are optimal care of arrhythmia and of heart failure  Treatment and Life Style Changing Patients with LV dysfunction or symptomatic HF should follow current HF therapy guidelines, including diuretics and ACE inhibitors or ARBs. Beta-blockers can be used cautiously in the acute setting. Digoxin should be avoided in patients suffering from acute HF induced by viral myocarditis Restrict salt intake to 2-3g of sodium per day Exercise especially during the acute phase of virus myocarditis enhances viral replication rate, enhances immune mechanisms and increases inflammatory lesions and necrosis. Resumption of physical activity can take place within 2 months of the acute disease.