Myocarditis, Cardiomyopathies and Hypertensive Heart Disease Flashcards Preview

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Flashcards in Myocarditis, Cardiomyopathies and Hypertensive Heart Disease Deck (38)
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1

cardiomyopathy

•abnormality or disease of the cardiac muscle cells, occurring in the absence of other known mechanisms for myocardial injury (excluding, for example, ischemic heart disease, valvular and congenital abnormalities, hypertensive heart disease, etc...)

2

primary cardiomyopathy

•primary involvement is myocardial and of unknown etiology

3

secondary cardiomyopathy

•associated with, but not completely explained by, another form of heart disease (e.g. myocarditis) or secondary to a systemic condition (e.g. pregnancy, amyloidosis or hematochromatosis) 1. not all patients with myocarditis develop a cardiomyopathy 2. only rare pregnant women develop postpartum cardiomyopathy

4

dilated cardiomyopathy

•60% of cases present with idiopathic, primary form of dilated cardiomyopathy, many of which are also familial and heritable - gene mutations •the other 40% present as secondary cardiomyopathy including: 1. alcoholism 2. previous myocarditis 3. pregnancy (postpartum cardiomyopathy) 4. exposure to certain drugs or toxins, especially doxorubicin (a chemotherapy agent) •physiologic consequences: systolic disorder (decreased contractility of LV with decreased ejection fraction), with LV hypertrophy and dilatation, and arrhythmias; may result in heart failure or sudden death

5

hypertrophic cardiomyopathy

•hypertrophy of ventricular septum (often asymmetric, being most prominent near the mitral valve; septum markedly hypertrophied but other areas of the ventricle are not) •myocyte disarray seen in septum •100% of cases are associated with gene mutations that encode various sarcomere proteins •physiologic consequences: diastolic disorder (decreased LV compliance with decreased LV filling), with asymmetric septal hypertrophy (but no dilatation) and dynamic LV outflow tract obstruction; often results in sudden death at a young age, especially in young athletes

– Increased blood flow pulls the anterior leaflet of the mitral valve into the outflow tract, where the septum is already protruding

– Obstruction increases with decreased ventricular volume and decreases with increased ventricular volume

– Exacerbated by exercise

6

restrictive cardiomyopathy

•increased cardiac wall stiffness )decreased compliance) which limits ventricular stretch and results in decreased cardiac filling •50% of cases are associated with amyloidosis - myocardium becomes stiff and waxy •blood eosinophilia (35% of cases) --> endocardial fibrosis and stiffening of the ventricles •other rare associations also occur •physiologic consequences: diastolic disorder (decreased ventricular compliance with decreased ventricular filling), bilateral dilation and usually normal systolic function, may result in heart failure or sudden death

•least common

7

arrhythmogenic cardiomyopathy

•rare •fibrosis and fatty replacement of the ventricles, esp right ventricle, with right ventricular dilatation •many cases are familial, associated with gene mutations •physiologic consequences: systolic disorder (decreased contractility of involved ventricle (s), with decreased ejection fraction, especially of the RV), with arrhythmias, often results in sudden death at a young age

• AKA: Arrhythmogenic Right Ventricular Dysplasia

• Etiology: genetic

• Develops gradually

• Ultimately, right ventricle is severely thinned

– Myocyte loss

– Fatty infiltration

– Interstitial fibrosis

• May progress to involve left side as well

8

myocarditis

inflammatory disease of the heart, characterized by inflammatory infiltrates in the myocardium, associated with myocyte damage

9

clinical features of myocarditis

•arrhythmias, EKG changes, heart failure, fever, fatigue, dyspnea

10

etiologies of myocarditis

1. infectious (particularly viral (e.g. Coxsackie virus, influenza) but may be bacterial, TB, fungal or parasitic (Chaga's disease) 2. hypersensitivity or autoimmune reactions (e.g. drug reactions, systemic lupus erythematosus) 3. rejection of cardiac implant 4. idiopathic (sarcoidosis, Fiedler's myocarditis, idiopathic giant cell myocarditis)

11

pathologic features of myocarditis

•gross: may appear normal or with dilated ventricles, often the heart appears "flabby" •microscopic: disintegration or necrosis of myocytes, associated with inflammatory cell infiltrates (typically mononuclear in viral myocarditis and transplant rejection, neutrophilic in bacterial infections, eosinophilic in hypersensitivity myocarditis and granulomatous in fungal, TB or giant cell myocarditis)

12

outcome of myocarditis

•most patients recover •treatment is supportive •sudden death may result from arrhythmia or hear failure •long term prognosis is variable •important outcome in some patients is evolution into dilated cardiomyopathy

13

systemic hypertension

•persistent systolic pressure greather than 140mmHg or diastolic pressure greater than 90 mmHg •prevalence in US is 20-35% of the adult population; African Americans, women and elderly adults are at greater risk •about 95% of cases are idiopathic ("essential hypertension") •rare cases are due to renal arterial narrowing or tumors (pheochromocytomas) that produce vasoactive hormones that constrict blood vessels

14

criteria for diagnosing hypertensive heart disease

1. cardiac enlargement, characterized by LV hypertrophy without dilatation 2. absence of other etiologic factors which would produce LV hypertrophy 3. history of hypertension

15

pathogenesis of hypertensive heart disease

•vascular changes: systemic arterioles become narrowed (arteriosclerosis), increased peripheral vascular resistance (increased afterload) ---> LV hypertrophy •mild myocardial hypoxia: increasing size of myocytes, diffusion distance increases for O2 transport from capillaries to individual myocytes, leading to mild hypoxia in the myocardium

16

additional factors for hypertensive heart disease

1. hypertrophied myocytes do not contract as efficiently 2. interstitial collagen increases, reducing ventricular compliance 3. superimposed atherosclerosis of coronary arteries (accelerated in hypertension) further decreases blood supply to myocardium and exacerbates myocardial hypoxia

17

pathologic features of hypertensive heart disease

•gross: increased heart size and weight - due to LV hypertrophy, thickening of LV wall •microscopic: increased myocyte diameter with increase in nuclear size, where nuclei often appear "squared off" or "box car" shaped

18

complications and causes of death in hypertension

1. most common COD is CHF (40% of cases) 2. other complications: coronary atherosclerosis, intracerebral hemorrhage (stroke), arteriolar nephrosclerosis (which may lead to chronic renal failure)

19

what does end stage heart disease look like?

• There is no one diagnostic appearance • In all causes the cardiac muscle has been subjected to the stresses of either increased intracardiac pressures, volumes, or both – If the primary stress pressure overload, the predominant alteration is hypertrophy of the myocardium – If the primary stress is volume overload, chamber dilation is the predominant alteration • Most cases will have elements of both hypertrophy and dilation

20

•left ventricular hypertrophy without dilatation and a normal heart

21

•normal heart and left ventricular hypertrophy with dilation

22

pathologic cardiac hypertrophy

• Increased myocyte mass is not accompanied by increase in capillary capacity – Hypertrophied myocytes are relatively hypoxic – Contractility is decreased

• Ultimate increase in muscle mass (normal heart weight=250-350 g):

– 350-600 g in pulmonary hypertension and ischemic heart disease

– 400 to 800 g in hypertension, aortic stenosis, mitral regurgitation, dilated cardiomyopathy

– 600 to 1000 g in aortic regurgitation or hypertrophic cardiomyopathy

23

•myocarditis

• In acute phase, may show mottling of the myocardium, dilated chambers, and flabby/floppy consistency

• In chronic/healed disease, after inflammation resolves, looks like every other dilated cardiomyopathy

24

•lymphocytic myocarditis

• Most common type of myocarditis

• Infiltrate is predominantly lymphocytes

• Typical of viral etiology, transplant rejection

25

•parasitic myocarditis - Chaga's disease

•Myofiber filled with trypanosomes at arrow

• Affects up to ½ of population in endemic areas of S. America.

• Most cases involve heart

• Inflammatory infiltrate of neutrophils, lymphs, macrophages, occasional eosinophils

• Parasites seen in scattered myofibers

• Individual fiber necrosis

26

•hypersensitivity myocarditis

– Prominent eosinophils

– Associated with drug allergies

27

•giant cell myocarditis

– Prominent giant cells

– Often extensive necrosis

– Poor prognosis

-granulamatous

28

•healed myocarditis

• Healed myocardium may show no residual changes or only nonspecific patchy fibrosis, as seen here

• Once healed, causes of myocarditis are indistinguishable

29

toxic cardiomyopathy

•dilated cardiomyopathy

• Alcohol

– Pathogenesis unclear

• Direct toxicity of alcohol and its metabolites?

• Associated nutritional deficiency?

• Chemotherapeutic agents

– Anthracyclines (adriamycin)

– Others

• Other therapeutic and illicit drugs • Misc. others

30

peripartum cardiomyopathy

•dilated cardiomyopathy

• Development of congestive heart failure in the third trimester of pregnancy or the first 6 months postpartum

• Very rare and poorly understood

– Pregnancy associated hypertension?

– Volume overload?

– Metabolic or nutritional imbalance?

– Immune response?

• Spontaneous recovery occurs in many patients in about 6 to 12 months