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Cardiomyopathy
This document provides an overview of different types of cardiomyopathy including hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), restrictive cardiomyopathy (RCM), and arrhythmogenic right ventricular dysplasia (ARVD). It describes the pathology, diagnosis, and management of each type. HCM is characterized by unexplained left ventricular hypertrophy. DCM involves dilated chambers and systolic dysfunction. RCM causes increased myocardial stiffness. ARVD primarily affects the right ventricle with replacement by fibrofatty tissue. The document also briefly discusses left ventricular non-compaction and Takotsubo cardiomyopathy.
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Cardiomyopathy
- 1. CARDIOMYOPATHY What, How, Why,e.t.c ……………
- 2. CLASSIFICATION • Cardiomyopathy- • Idiopathic •Secondary • Cardiomyopathy- • Hypertrophic • Dilated • Restrictive • Arrhythmogenic RV dysplasia
- 3. HYPERTROPHIC (HCM) • ↑myocardial thickness • True myocyte hypertrophy • Absence of loading conditions accounting for degree of hypertrophy (HTN, AS, AR) • Genetics • Familial, Auto dominant, Incomplete penetrance • Variable phenotype and prognosis • Cardiac sarcomere mutation (60%)
- 4. HCM PATHOLOGY • Gross- •Hypertrophy – LV/Both • LV – Ant septum, Post septum, Free wall • RV – Symmetric • Histo- • Disorg muscle bundles – whorled pattern • Cell-cell orientation lost, Foci of disarray • Cells – Broad, short, bizzare shape, myofibrillar archit lost • Interstitial fibrosis • Result- • Systolic + Diastolic dysfunction • Electrical instability
- 6. HCM PATHOPHYSIOLOGY • Diast- •LV compliance ↓, Filling abnormal, LVEDP • Atrial press ↑ • Restrictive component + • Syst- • EF ↑ • LVOT obstruction – dynamic/static • Ischaemic- • ↓ Coronary flow reserve • ↑ demand, supply N/↓
- 7. HCM DIAGNOSIS • Unexplained/Disproportionatehypertrophy • Screening of relatives • History- • Autopsy – SCD • Any age • Paroxysmal symptoms • Exertional - dyspnoea, angina (typical/atypical), syncope • Exam- • JVP prominent a (RVH) • Forceful apex, palpable atrial beat • Pulse – rapid upstroke, low vol • Ausc – Loud S4, ESM AA, PSM apex
- 8. HCM DIAGNOSIS 2 •CXR- • LV enlargement • LA RA enlargement • PAH • ECG- • LVH, ST↓, T↓, deep S V1-V3 • P tall, M pattern • AF, IVCD • NSVT, VT, SVT • ECHO- • LVH, LV cavity↓, Diast dysfunc, LVOT gradient • LA cavity↑ • MR, SAM
- 9. HCM DIAGNOSIS 3 •MRI- Myocardial fibrosis • Cath- • Chamber pressures • Transvalvular & outlet gradients • Systolic narrowing of coronary artery
- 10. HCM MANAGEMENT • Asymptomatic- •Genetic testing • Screen relatives • Annual follow-up • Pharmacologic- • β blocker, CCB, Verapamil, Amiodarone • Anticoagulants • IE suspicion high • Surgical- • Myotomy, myomectomy • Papillary muscle remodelling • MV repair/replacement
- 11. HCM MANAGEMENT 2 •Alcohol septal ablation- • Septal artery • Elderly, failed medical • Pacing- • AICD, RV pacing, CRT (AV sequential) • Electrical cardioversion • Stratify risk of SCD- • Family history of sudden death (≥2 premature (<40 years) sudden deaths) • Unexplained syncope within previous year • Abnormal exercise blood pressure • Nonsustained ventricular tachycardia (≥3 beats at ≥120 beats/min) • Severe left ventricular hypertrophy (>3 cm) • Severe left ventricular outflow tract obstruction (>90 mmHg) • Cardiac arrest (or sustained ventricular tachycardia)
- 12. DILATED (DCM) • Chamberdilatation & Systolic dysfunction (LV, LV+RV) • Absence of CAD, valve disease, pericardial disease • Causes- • Genetic- • Autosomal dominant, incomplete penetrance • X-linked – Duchenne, Becker’s, Emery Driefuss • Acquired- • Infectious myocarditis • ChemoRx, RadioRx • Alcohol, cocaine • Nutritional deficiency – thiamine, calcium • Iron overload • Inflammatory, autoimmune disorders • Endocrinopathy – hypothyroidism, DM • Pregnancy, tachycardia
- 13. DCM PATHOLOGY • Gross- •Dilated chambers – Vent mass↑, Vent thickness N/↓ • Mural thrombi, platelet aggregates • Histo- • Patchy fibrosis • T-lymphocyte & macrophage infiltration • Myocyte death • Myofibrillary loss • Result- • Slowly progressive dilatation • Progressive LV systolic dysfunction • Conduction defects - late
- 16. DCM DIAGNOSIS • History- •Progressive cardiac failure • Insidious fatigue, dyspnoea, ↓exercise tolerance • Arrhythmia – AF, VT, AV block • Systemic embolism • Incidental ECG, CXR, ECHO findings • Exam- • Normal • CCF – Hepatomeg, ascites, pedal edema , JVP↑ • Cardiomegaly, apex - lateral, diffuse & dyskinetic • Low pulse vol & press, Pulsus alternans, Low SBP • Ausc – S2 spilt/paradox, loud P2, S4, S3, MR, TR
- 17. DCM DIAGNOSIS 2 •ECG – Sinus tachy, nonspecific ST (fibrosis), arrhythmia, conduction defects • CXR – Cardiomeg, ↑pulm markings, pleural effusion • ECHO – • LV dilatation, syst & diast dysfunc, intracavit thrombus • Func MR TR • Pulm art press↑ • Cardiac biomarkers - ↑ CKMB, Trop I&T, ANP • MRI – Dimensions, fibrosis • Exercise testing – Func class, progress, pre-Tx • Cath – Pre-Tx
- 18. DCM MANAGEMENT • Aims- •Improve symptoms • Attenuate disease progress • Prevent complications (arrhythmia, stroke, sudden death) • Pharmac- • Diuretics • ACEi, ARB, β blockers • Digoxin, anticoagulants • Antiarrhythmics – Caution (neg intotropic, proarrhythmic) • Non-pharmac- • PPI – AICD, CRT • MVR • Dor & Batista Sx • LV assist device, artificial heart • Cardiac Tx
- 19. RESTRICTIVE (RCM) • ↑stiffness of myocardium • Vent press ↑↑ for small vol ↑ • Diast/syst volumes = N/↓ • N vent thickness • Causes- • Infiltrative – amyloidosis, sarcoidosis • Storage – haemochromatosis, Fabry’s, glycogen storage • Fibrotic – radiation, scleroderma, drugs (doxorubicin) • Metabolic – carnitine def, fatty acid metab disorders • Endomyocardial – endomyocardial fibrosis, hypereosinophilic syn • Misc – carcinoid syndrome
- 20. RCM PATHOLOGY • Heterogenousaetiology • Gross- • Biatrial dilatation • No LVH, N heart wt • Small vent cavity • Histo- • Idiopathic- • Nonspecific, patchy interstitial fibrosis, myocyte disarray • Endomyocardial fibrosis- • Necrotic – eosinophilic abscess, necrotic arteritis, mural thrombus • Thrombotic – endocardial + intracavitary thrombus • Fibrotic – dense fibrosis, fill cavity, impaired valve function • Pathophysiology- • Impaired relaxation, ↓ volume, sudden mid-diastolic pressure ↑
- 21. RCM DIAGNOSIS • Insidioussymptoms • Lt side - pulmonary congestion, MR • Rt side – JVP ↑, hepatomegaly, ascites, TR • CXR – cardiomegaly, pulmonary infiltrates • ECG – non-specific repolarisation defects, conduction defects • ECHO- • Enlarged atria • N ventricular size, intracavitary thrombus • Fibrosis ventricular myocardium • MR, TR • Cath – sudden mid-diastolic vent press ↑
- 22. RCM MANAGEMENT • Noeffective Rx for advanced cases • Symptomatic Rx • Diuretics for HF • Antiarrhythmics – sustained/symptomatic arrhythmias • Digoxin • Anticoagulants – warfarin • Antiplatelets • Poor prognosis • Surgery- • MVR/TVR • Decortication +/- • Selected patients
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- 24. Arrhythmogenic RV dysplasia •RV myocardium • Later LV and RV involved • 50% inherited, AD • RV wall thinning, aneurysm • Myocardium – fibrofatty, fibrosis, lymphocytic infiltration • Palpitation, syncope, chest pain, DoE • Ventricular arrhythmia, HF, SCD • ECG - T↓, wide QRS, IVCD, Vent arrhythmias • ECHO – RV (dilatation, hypo/dyskinesia, thinning, aneurysm, scarring), LV dilatation • Cardiac MRI, endomyocardial Bx
- 25. ARVD (contd) • Rxaccording to – • Symptoms • Arrhythmias • Risk of SCD • β-blockers, diuretics, ACEi • Anticoag, amiodarone • AICD • Cardiac Tx
- 26. LV NON-COMPACTION • Non-compactionof trabecular/spongiform layer • Assoc ASD, VSD, CoA • Echo – diagnostic • HF, systemic emboli, arrhythmia.
- 27. TAKOTSUBO • Stress cardiomyopathy •Apical LV dysfunction • Mimics MI • Chest pain, ST↑, biomarkers↑ • Emotional/physical stress • Conservative Rx • Spontaneous recovery