Cardiomyopathies/Myocarditis, Vasculitis

Question 1

Lymphocytic Myocarditis

Answer 1

Most common type of myocarditisTypically YOUNG patientUsually caused by virusesEnterovirus (Coxsackie B3) AdenovirusMOA: autoimmune injury (molecular mimicry) to cardiac muscleDx? Serology and/or PCRRare to have positive culturesClinical: arrhthymia, CHF and possible sudden deathMost (~2/3) patients recover. Some eventually develop scarring and dilated cardiomyopathyGross -> flabby, dilated heartHistology -> myocyte necrosis and lymphocytic infiltration

Question 2

Hypersensitivity Myocarditis

Answer 2

No specific age groupAllergic reaction, usually to medicationEspecially antibiotics Pathology: eosinophilic infiltrate, especially around vasculature, with little necrosisNO scarringNO dilated cardiomyopathy

Question 3

Giant Cell Myocarditis

Answer 3

AdultsUnclear cause -> thought of as a more severe form of Lymphocytic Myocarditis but NO underlying viral infectionAssociated with other autoimmune diseasesPathology: macrophage giant cells and myocyte necrosis (a lot of necrosis – pretty bad so it makes sense that you will see a lot of necrosis) Prognosis: very bad -> rapidly fatal

Question 4

Toxic Myocarditis

Answer 4

Damage to myocytes caused by toxic insults Can be caused by:Excess catecholamines (i.e. cocaine abuser or patient with pheochromocytoma)Adriamycin (aka doxorubicin) -> Dilated cardiomyopathyLithiumArsenic Pathology: meutrphils w/ contraction bands & large necrosis

Question 5

Sarcoid Myocarditis

Answer 5

NON-INFECTIOUSChronic disease: ~5% of all patients with sarcoidosis will have heart involvementCan cause arrhythmia and sudden deathSarcoidosis can affect pretty much any of your organsPathology:Gross -> epicardial scarHistology: non-caseating granuloma with scar

Question 6

Infectious Myocarditis

Answer 6

Generally occurs in immunocompromised patients (i.e. AIDS, transplant, chemo, congenital immunodeficiency)ImmunocompromisedUsually viral, especially CMVToxoplasmosis (protozoa)ImmunocompetentVaricella Chagas disease (T. cruzi, endemic in South America)Rickettsia (RMSF)Borrelia burgdorfi (Lyme)

Question 7

Dilated Cardiomyopathy

Answer 7

failing, dilated heart w/o apparent causedyspnea, heart failure, arrhythmiaspathology: non specific, 4 chamber dilation, myocyte atrophy/hypertrophy, fibrosisassoc w/: myocarditis (viral), autoimmune, alcohol, genetic, pregnancy, Chagas (apical aneurysm), toxicites (adraimycin)complications: systemic, pulmonary emboli bc of mural thrombi

Question 8

Hypertrophic Cardiomyopathy

Answer 8

thick, hypertrophied L ventricle w/ small cavityventricle asymmetricdisorganized myocytessubaortic stenosis bc of obstruction of outflow**FAMILIAL - beta-myosin heavy chainthickened intramural coronary arteriesSx: chest pain, syncope, dyspnea, sudden death (exercise related)rare, male 20-50yoabnormalities in sarcomeric proteins

Question 9

Restrictive Cardiomyopathy

Answer 9

stiff L ventricle, doesn’t expand as it should during diastolelacks compliancedeposition of fibrillary proteinsheart appears normal, but is fibrosedblood backs up into atria (atrial dilation)Causes: amyloidosis (positive red congo stain)radiation, sarcoidosis, eosinophilic (Loeffler’s endocarditis), idiopathic endocardial fibrosis of the tropics (Davies disease)

Question 10

R Ventricular Cardiomyopathy

Answer 10

R ventricular aneurysms thinning of R ventricular wallsudden death, often w/ exercise

Question 11

Giant Cell Arteritis

Answer 11

Granulomatous inflammation involving branches of the carotid arteryMost commonly Temporal ArteryUsually in elderly womenPresents with HA, visual changes, and jaw claudication. May have polymyalgia rheumatica (joint and muscle pains)Labs: look for elevated ESR and/or CRPPathology: inflamed vessel wall including giant cellsSegmental lesions -> so might skip parts of vesselTreatment: high dose corticosteroidsRisk of blindness (from involvement of Ophthalmic A) if not treated promptly

Question 12

Takayasu

Answer 12

“Pulseless Disease”Commonly occurs in younger women (

Question 13

Polyarteritis Nodosa

Answer 13

Rare vasculitis affecting medium sized arteriesUsually attacks multiple organs (especially kidney) but lungs are sparedClassic presentation: abdominal pain and malignant HTN in a younger patient with HBV infectionSkin lesions are called “livedo reticularis” -> nodules or patchy erythemaPathology: fibrinoid necrosis with “string of pearls” appearance due to alternating areas of fibrosis and aneurysm

Question 14

Kawasaki Disease

Answer 14

Important disease to know because it usually presents with non-specific symptoms Key finding: rash on palms and solesUsually occurs in young kidsCan have disastrous consequences if untreated -> coronary artery aneurysmLifelong follow-up Treat with aspirinOne of the only/few times you give a child aspirin (because of risk for Reye’s syndrome)

Question 15

Wegener’s Granulomatosis

Answer 15

Rare autoimmune disease that attacks small vessels, so may involve many different organsClassic Triad: involvement of upper respiratory tract, lung and kidney(glomerulonephritis)Auto-antibodies to neutrophils = cANCA (less commonly known as “anti-proteinase3”)Pathology: necrotizing granulomasTreatment: cyclophosphamide and steroids

Question 16

Microscopic Polyangiitis

Answer 16

similar to Wegener’spositive for pANCA (anti-myeloperoxidase)MPA -> similar to Wegener lung and kidney but NO nasopharyngeal involvement No granulomas

Question 17

Henoch-Schonlein Purpura

Answer 17

Common vasculitis among childrenDue to IgA immune complex depositionCauses damage to blood vessels potentially leading to GI bleeding and hematuria, among other vague symptomsSpecific buzzword: palpable purpura