Cardiomyopathy Presentation
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A presentation I gave during an internship/Advanced Pharmacy Practice Experience (APPE) in cardiology at a hospital.
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Cardiomyopathy Presentation
- 1. CARDIOMYOPATHY Paul Pasco, PharmD Candidate UTHSC College of Pharmacy March 31, 2021 1
- 2. OBJECTIVES Define the most notable subtypes of cardiomyopathy, including dilated, hypertrophic, restrictive, and peripartum cardiomyopathies. Describe the epidemiology and pathophysiology of each subtype of cardiomyopathy. Explain the diagnostic criteria for each subtype, including relevant imaging strategies. Elucidate the pharmacological and non-pharmacological interventions associated with each subtype. 2
- 3. ABBREVIATIONS AICD = Automated implantable cardioverter-defibrillator ACEI = Angiotensin-converting enzyme inhibitor ARB = Angiotensin receptor blocker ARNI = Angiotensin receptor-neprilysin inhibitor BB = Beta blockers CAD = Coronary artery disease CRT = Cardiac resynchronization therapy DCM = Dilated cardiomyopathy EKG = Electrocardiogram EF = Ejection fraction HCM = Hypertrophic cardiomyopathy HF = Heart failure HFrEF = Heart failure with reduced ejection fraction HTN = Hypertension LV = Left ventricle LVAD = Left ventricular assist device LVOF = Left ventricular outflow tract MR = Mitral regurgitation MS = Multiple sclerosis MUGA = Multiple-gated acquisition RAAS = Renin-angiotensin-aldosterone system RCM = Restrictive cardiomyopathy RNA = Radionuclide angiography RNV/RVG = Radionuclide ventriculography SCD = Sudden cardiac death SNS = Sympathetic nervous system TTE =Transthoracic echocardiogram 3
- 4. WHAT IS CARDIOMYOPATHY? Structural and functional disease of the myocardium Two broad classifications: • Nonischemic cardiomyopathy • Not related to CAD • Includes dilated, hypertrophic, and restrictive cardiomyopathies • Ischemic cardiomyopathy • Related to CAD 4
- 7. DCM: DEFINITION, EPIDEMIOLOGY • Disease of the heart muscle in which the chambers of the heart dilate (enlarge) • Dilation → reduced contractility → often HFrEF Definition • Most common form of cardiomyopathy • Up to 50% of cases are genetic (likely underestimated) • Accounts for ~10,000 deaths and 46,000 hospitalizations annually in the USA • Lifetime incidence of DCM is approximately 30 cases per 100,000 people Epidemiology 7
- 8. DCM: PATHOPHYSIOLOGY May occur due to any physiological process that adversely affects the myocardium Excessive neurohormonal activation of SNS Excessive activation of RAAS Cardiac remodeling → impaired cardiac function, hypokinesis 8
- 10. DCM: DIAGNOSIS Arrythmias • Abnormal ECG, typically nonspecific changes • Up to 50% of patients may present with atrial and ventricular arrythmias • Arrythmias may result in SCD Symptomatic HF s/sx, including: • Dyspnea • Orthopnea • Peripheral and/or pitting edema • Paroxysmal nocturnal dyspnea • JVD 10 https://bit.ly/3rACElt
- 11. DCM: DIAGNOSTIC PROCEDURES & IMAGING 11 TTE • Abnormal • Dilated cardiac chambers • Hypertrophy • TR and MR • Helpful in differentiating among other types of cardiomyopathies RNA/RNV/RVG/MUGA • Abnormal • More accurate than echo • Involves gamma radiation • More expensive, invasive Endomyocardial biopsy • Sample of inner lining of heart (endocardium) • Not routinely recommended. Results usually unhelpful • Some utility in idiopathic dilated cardiomyopathy • Consider in new-onset HF (2- 12 weeks) with other factors • Serious risk for cardiac injury and cardiac death
- 12. DCM: PHARMACOTHERAPY The same as HFrEF • BBs • ACEIs/ARBs/ARNIs • Aldosterone antagonists • Loop diuretics • Vasodilators if AA and refractory • Influenza and pneumonia vaccinations Cornerstones include: 12 https://bit.ly/39scPO9
- 13. DCM: DEVICES & OTHER INTERVENTIONS Biventricular pacing/CRT AICD LVADs Cardiac transplantation 13 LVAD: https://bit.ly/3rFBwgw
- 15. HCM: DEFINITIONS AND EPIDEMIOLOGY • Thickening of LV (hypertrophy) • Not completely due to abnormal LV loads Definition • Most common genetic heart defect • Affects 1 in 500 people, approximately 500,000 in the USA • Many are asymptomatic • Often undetected until cardiac event • More common in MS patients • Accounts for many SCD cases • 36% of SCD in young athletes have probable or definitive HCM Epidemiology 15
- 16. HCM: PATHOPHYSIOLOGY Pathophysiology LV hypertrophy without ventricular dilation Myocytes become hypertrophied and disorganized Interstitial fibrosis → cardiac remodeling, impaired ventricular function Hypertrophy most evident in interventricular septum May occur in other areas May be a combined product of genetic, HTN, and aging LV outflow tract obstruction Hypertrophic obstructive cardiomyopathy ➔ impaired systemic circulation May occur at rest Exacerbated by factors that ↑ myocardial O2 demand, ↓ preload, or ↓ afterload Normal myocardium HCM https://cle.clinic/2PCZfQX 16
- 17. HCM: CLINICAL PRESENTATION Clinical presentation Exertional dyspnea Orthopnea Fatigue Dizziness Presyncope Syncope Angina Ischemia (common) Impaired ventricular diastole, MR → pulmonary congestion Atrial and ventricular arrythmias SCD Increased risk of bacterial endocarditis https://bit.ly/3wajC8Z 17
- 19. HCM: GENETIC FACTORS Genetics Large genetic contribution Gene mutations affect sarcomere function Affect structural, contractile, calcium handling, or mitochondrial proteins ≥ 50% of people with s/sx have at least one mutation At least 30 genes that confer susceptibility identified The two most common mutations are: Myosin binding protein C (MYBPC3) Myosin heavy chain 7 (MYH7) Autosomal dominant Variable penetrance 19 https://bit.ly/2QRxpRF
- 20. HCM: DIAGNOSIS Absence of another disease that would produce similar hypertrophy in the patient LV wall thickness ≥ 15 mm Family history → 1st-degree relatives with SCD, HCM EKG ST- andT-wave abnormalities Physical examination Course systolic outflow murmur on auscultation Imaging 2D or Doppler echocardiogram to assess LVOF at baseline, every 1-2 years thereafter, and after each new event Cardiac MRI when echocardiogram not conclusive Holter monitor Cardiac stress test 20
- 21. HCM: GENERALTREATMENT APPROACHES Controversial in asymptomatic persons • Lack of evidence for medical therapy in this population Focus on symptom relief Prevent SCD with devices, BLS, ACLS Avoid strenuous physical activity → heavy weightlifting, competitive sports Moderate intensity aerobic exercise → safe and beneficial Practice good oral hygiene to reduce the risk of bacterial endocarditis 21
- 22. HCM: PHARMACOTHERAPY Reduce inotropy and chronotropy → reduce myocardial O2 demand Beta blockers NDHP CCBs Disopyramide in patients refractory to either group or in combination Avoid other concurrent antiarrhythmics Monitor QT interval Use vasodilators with caution (may worsen LVOT gradient) Digoxin is relatively CI (positive inotrope) Routine prophylaxis of bacterial endocarditis is not recommended If HCM presents with paroxysmal or chronicAF: Anticoagulate with DOACS or warfarin independent of CHA2DS2-VASc score Consider procainamide, disopyramide, or amiodarone for continuous AF suppression 22
- 23. HCM: DEVICES AICD Recommended with history of cardiac arrest,VF, orVT with hemodynamic instability Reasonable with recent, unexplained syncope Other considerations: LV wall thickness > 30 mm History of SCD in 1st-degree relatives History of non-sustainedVT on Holter Hypotension in response to exercise challenge Biventricular pacemaker May improve symptoms in a subset of patients No effect on survival May see up to 25% improvement of symptoms Only 10% of patients meet criteria 23 https://bit.ly/31vS7Zy
- 24. HCM: SURGICAL INTERVENTION Septal reduction • Myectomy • Most common • Use intraoperativeTTE to assess MV function • Effective in up to 95% of patients with < 1% mortality • Alcohol septal ablation • Catheter • Ethanol • Both techniques provide symptom relief but no survival benefit in those who failed medications Cardiac transplantation • Refractory patients • End-stage HCM with symptomatic HF 24
- 25. HCM SEPTAL REDUCTION: MYECTOMY 25 https://cle.clinic/2PCZfQX
- 26. HCM SEPTAL REDUCTION: ALCOHOL ABLATION 26 https://bit.ly/3dcdOTK
- 28. RCM: PATHOPHYSIOLOGY 28 Ventricular stiffening (1° or 2° process) Systole is normal Diastole is impaired LV is nondilated LVEF is normal Distinct from constrictive pericarditis Diverse etiology Cause often unknown
- 31. RCM: DIAGNOSIS Clinical presentation HF s/sx Exertional dyspnea Dyspnea at rest Fatigue Peripheral edema Weight gain Abnormal EKG Low voltage Poor R-wave progression 31 • Imaging • 2D or Doppler echocardiogram • Cardiac MRI • PET • CT • Cardiac catheterization • Endomyocardial biopsy for unknown etiology/confirmation of protein- based disease
- 32. RCM:TREATMENT Correct underlying cause Chemotherapy for light-chain amyloidosis. Avoid digoxin and BBs Iron depletion via venipuncture or chelation therapy with deferoxamine in hemochromatosis Glucocorticosteroids, other immunomodulators for sarcoidosis Enzyme replacement in Fabry disease AICD in those with syncope and/or ventricular arrythmias 32 https://bit.ly/3rzU81p
- 33. PERIPARTUM CARDIOMYOPATHY (PP CMP) 33 https://bit.ly/3sBqJVR
- 34. PP CMP: DEFINITION, EPIDEMIOLOGY, PROGNOSIS Definition: An induced type of DCM LV systolic dysfunction Last month of pregnancy or up to 5 months postpartum Epidemiology 1 in 1000-4000 pregnancies in the USA More common and severe in later pregnancies Prognosis Better probability of recovery to baseline compared to other subtypes Improvement at 6 months → indicator of overall recovery Recovery may continue up to 3 years after pregnancy Subsequent pregnancies may result in maternal mortality 34
- 35. PP CMP: PATHOPHYSIOLOGY Unclear LV dysfunction may be 2° to unrestricted viral replication Relative immunosuppression of pregnancy Adenovirus, herpesvirus, and others may have a role Other potential causes: Fetal microchimerism: infiltration of maternal circulation → myocarditis Prolactin metabolism → cardiotoxic metabolites Risk factors Advanced maternal age Multiple pregnancies Gestational HTN AA race/ethnicity 35
- 36. PP CMP: DIAGNOSIS S/sx of HF Typically, NYHA class III and IV, but may be milder Dyspnea on exertion Peripheral edema Orthopnea PND PP CMP vs. late-stage pregnancy? New MR Abnormal EKG LV hypertrophy ST- andT-wave abnormalities Abnormal echocardiogram with reduced LVEF +/- dilation 36
- 37. PP CMP: PHARMACOTHERAPY ACEIs/ARBs if postpartum (Category D, harm) Hydralazine if still pregnant BBs Cardioselective to reduce uterine relaxation if still pregnant Digoxin for (+) inotropy and (-) chronotropy, if needed Loop diuretics Heparin in those withTE while pregnant (Category C, benefits > risks) Warfarin after delivery (Category X, significant harm, absolute CI) 37
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- 42. REFERENCES 1. Ommen SR, Mital S, Burke MA, et al. 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: Executive Summary. Circulation. 2020;142(25). doi:10.1161/cir.0000000000000938 2. Bozkurt B, Colvin M, Cook J, et al. Current Diagnostic and Treatment Strategies for Specific Dilated Cardiomyopathies: A Scientific Statement From the American Heart Association. Circulation. 2016;134(23). doi:10.1161/cir.0000000000000455 3. Wu LA, Lapeyre AC, Cooper LT. Current role of endomyocardial biopsy in the management of dilated cardiomyopathy and myocarditis. Mayo Clinic Proceedings. 2001;76(10):1030-1038. doi:10.4065/76.10.1030 4. Crees Z, Fritz C, Heudebert A, et al. The Washington Manual of Medical Therapeutics. 36th ed. Wolters Kluwer; 2020. 42