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John Steuter, MD

Bryan Heart

Cardiomyopathy

No Conflicts

Disclosures

Cardiomyopathy

WHO Classification

Anatomy & physiology of the LV1. Dilated

• Enlarged

• Systolic dysfunction

2. Hypertrophic• Thickened

• Diastolic dysfunction

3. Restrictive• Diastolic dysfunction

4. Arrhythmogenic RV dysplasia• Fibrofatty replacement

5. Unclassified• Fibroelastosis

• LV noncompactionCirc 93:841, 1996

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Dilated Cardiomyopathy

Dilation and impaired contraction of ventricles:

Reduced systolic function with or without heart failure

Characterized by myocyte damage Multiple etiologies with similar resultant pathophysiology

Many of the cases are idiopathic

incidence of idiopathic dilated CM 5-8/100,000

incidence likely higher due to mild, asymptomatic cases

3X more prevalent among males and African-Americans

DCM: EtiologyIschemic

Valvular (aortic and mitral valve disease)

Hypertensive (end stage)

Familial

Idiopathic

Inflammatory

Infectious

Viral –Cox B, CMV, HSV, HIV

Ricketsial - Lyme Disease

Parasitic - Chagas’ Disease, Toxoplasmosis

Non-infectious

Collagen Vascular Disease (SLE, RA)

Peripartum

Toxic

Alcohol, Anthracyclines (adriamycin), Cocaine

Metabolic

Endocrine –thyroid dz, pheochromocytoma, DM, acromegaly,

Nutritional

Thiamine, selenium, carnitine

Neuromuscular (Duchene’s Muscular Dystrophy--x-linked)

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Ischemic Cardiomyopathy

Patients with history of MI or revascularization (CABG or PCI)

Patients with >75% stenosis of the left main or proximal LAD

Patients with >75% stenosis of two or more epicardial vessels

Prognosis depends on Etiology

1230 pts. referred for unexplained CM. Felker GM. NEJM 2000;342:1077

40 year old female presents with “crushing chest pain”

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Echo: EF 45%

Inferior Wall hypokinesis

CATH:

Normal Coronaries

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Definition of mycocarditis

Inflammation of the heart muscle secondary to injury

– Ischemic damage

–Mechanical trauma

–Genetic cardiomyopathies

– Exposure to discrete external antigens

• Viruses, bacteria, parasites, toxins drugs

– Internal triggers

• Autoimmune activation against self antigens

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Incidence

Difficult to ascertain, depends on criteria used

Estimated 8 to 10 per 100,000

Unselected autopsy series as high as 1 to 4 per 100

Young adults with sudden cardiac death, estimated 8.6%

Idiopathic dilated cardiomyopathy patients only � 10-40% are secondary to myocarditis

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Population

Bimodal age distribution

Young children and teenagers: acute presentation

– Exuberant response to initial exposure of antigen

Older adults: Subtle and insidious symptoms of dilated cardiomyopathy and heart failure

–Mature immune system with greater tolerance

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Clinical presentation

Wide-ranging clinical presentation contributes to difficult diagnosis and

classification

– Asymptomatic ECG or echocardiographic abnormalities

– Cardiac dysfunction, arrhythmias, heart failure and hemodynamic collapse

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Acute Myocarditis Presentation

Fatigue 82%

Dyspnea on exertion 81%

Arrhythmias 55%

Palpitations 49%

Chest pain at rest 26%

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Acute Myocarditis Presentation

Acute ischemic syndrome type symptoms

– Elevated troponin

– ST-segment elevation on ECG

– Segmental wall motion abnormalities on echocardiography

Viral prodrome symptoms 20-80%

– Fever

– Chills

–Myalgias

– Constitutional symptoms

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Fulminant Myocarditis Presentation

Abrupt onset within 2 weeks of a viral illness

Hemodynamic compromise

Hypotension requiring pressors and mechanical support

Echocardiogram reveals diffuse global hypofunction

Thickening of the ventricular wall probably due to myocardial edema from myocardial inflammation and cytokine release

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Endomyocardial biopsy in fulminant myocarditis• Typical and diffuse myocarditis in each histologic section

Pathogenesis

Pathogenesis

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Diagnostics: Expanded Criteria for Diagnosis of Myocarditis

Category I: Clinical Symptoms

– Clinical heart failure

– Fever

– Viral prodrome

– Fatigue

–Dyspnea on exertion

– Chest pain

– Palpitations

– Pre-syncope or syncope

Category II: Evidence of Cardiac Structural or Functional Perturbation in the absence of Regional Coronary Ischemia

Echocardiography evidence– Regional wall motion abnormalities

– Cardiac dilation

– Regional cardiac hypertrophy

Troponin release–High sensitivity (>0.1 ng/mL)

Normal coronary angiography or

Absence of reversible ischemia by coronary distribution on perfusion scan

Category III: Cardiac Magnetic Resonance Imaging

Increased myocardial T2 signal on inversion recovery sequence

Delayed contrast enhancement after gadolinium-DTPA infusion

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Category IV: Myocardial biopsy – Pathologic or Molecular Analysis

Pathology findings compatible with Dallas criteria

Presence of viral genome of polymerase chain reaction or in situ hybridization

– 80-100% specificity when performed from myocardial biopsy

Treatments/Therapeutic Approaches

Supportive Therapy

Immunosuppression

Interferon

Intravenous Immune Globulin

Immune Adsorption Therapy

Hemodynamic Support

Vaccination

Supportive Therapy

First-line therapy

Only a small proportion of patient require hemodynamic support

Treat this group same as for clinical heart failure

– Diuretics

– IV Vasodilators: Nitroglycerin, Nesiritide

– ACEi, ARBs, B-blockers when stable

• Anti-inflammatory properties

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Hemodynamic Support

Patients with fulminant myocarditis and cardiogenic shock may require

– Intra-aortic balloon pump

– Ventricular assist devices

– Extracoporeal membrane oxygenation (ECMO)

Vaccination

Targeted vaccination in the future

Patients genetically susceptible to myocarditis

After the mumps vaccination

– Disappearance of endocardial fibroelastosis causing dilated cardiomyopathy

Prognosis

Most patients with acute myocarditis and mild cardiac involvement recover without long-term sequelae

Patient with advance cardiac dysfunction, varied outlook

Patients with severe hemodynamic collapse at presentation actually have a good prognosis

– 93% transplant-free survival in 11 years

30% of those with chronic myocarditis may recover

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Prognosis

Several studies have looked at clinical variables that predict adverse outcomes (death and transplantation)

– Syncope– Bundle branch block

– EF <40%

Other factors–NYHA Class III or IV

– PCWP <15mmHg

– Immunopathologic evidence of myocardial inflammation

– Failure to use B-blockers– BiV failure

–Giant cell or viral genome on biopsy

DCM: toxic

Alcoholic cardiomyopathy

Chronic use

Mechanism?:– Myocyte cell death and fibrosis

– Directly inhibits:

• mitochondrial oxidative phosphorylation

• Fatty acid oxidation

Risk vs intake

0

0.5

1

1.5

2

0 1-6d 7-13d 14-

27d

28-

41d

42-

69d

>69d

RR

PBRC 2010

Relative Risk of Mortality

Drinks/week

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Treatment

Guideline directed medical therapy

Reversible with abstinence

Questions