Cardiac Pathology 2, Valvular Dz, Cardiomyopathies, Congenital HD, Shunts,Obstructive Lesions Flashcards
Mechanical difference between stenosis and insufficiency.
Stenosis - valve doesn’t open completely (Chronic) = PRESSURE overload hyptertrophy
Insufficiency - valve doesn’t close completely (AorC) = VOLUME overload hyptertrophy
Chronically, what do both stenosis and insufficiency result in?
Both result in overload hyperTrophy»_space; CHF
Stenosis = pressure, Insufficiency = volume
What is the most common valve abnormality, and why?
Calcific Aortic Stenosis. BICUSPID Valves accelerated course. Ossification and calcification prevent valve opening. “Wear and tear” with age, chronic HTN
Describe calcification in Mitral Annular Calcification and common results of this condition.
F>M, 60yo. Calcific deposits in the fibrinous annulus around MV. MV-PROLAPSE common.
Nodules may become sites for IE or thrombus formation.
What is MV-Prolapse? Describe physical characteristics.
MV leaflets prolapse back into LA during systole = "systolic click". Myxomatous degeneration (thickened/rubbery leaflets)
Describe the presentation of MV-Prolapse.
Females. Most asymptomatic, but may mimic angina-like pain or dyspnea.
May be the result of other regurgitation defects (dilated hypertrophy).
What are rare, but SERIOUS complications of MV-Prolapse?
IE, Mitral Insufficiency, Thromboembolism, Arrhythmias
Describe Rheumatic Fever (RF): What is it and what can it lead to?
Multisystem inflammatory disorder. Acute RF can evolve into chronic rheumatic heart disease.
RF Pathogenesis
Caused by grp A strep M proteins cross reacting with cardiac self-Ag.
Anti-streptolysin O, think what? And how long ago?
Grp A strep infection 10days-6wks ago, now Acute RF
What cardiac morphologic features are associated with Acute RF?
PANCARDITIS with ASCHOFF BODIES. Fibrinoid necrosis of endocardium and L-valves, with vegetations (VERRUCAE).
What are cardiac morphologic features are associated with Chronic RF?
Mitral leaflet thickening, fusion/shortening of commissures, fusion/thickening of tendinous cords. All resulting in MITRAL STENOSIS
If you see Mitral Stenosis, think what?
Chronic RHD!
What are clinical features of Chronic RHD?
LA enlargement leads to afib/thrombosis; pulm congestion/RHF.
What is Infective Endocarditis (IE)?
Infection of valves and endocardium with vegetations consisting of microbes and debris, associated with underlying tissue destruction.
Describe acute IE.
Rapidly progressing destruction of previously normal valve. Tx requires surgery and antibiotics.
Describe subacute IE.
Slowly progressing destruction of previously deformed valve (i.e. chronic RHD). Tx requires only antibiotics.
What predisposing conditions can lead to IE?
(1) Valvular abnormalities - RHD, prosthetic valve, MV-prolapse, calcific stenosis, bicuspid AV. (2) Bacteremia - dental work, needle, compromised epithelium, another site of infection.
What are the classic morphologic features of IE?
Friably, bulky, destructive valvular vegetations that lead to septic emboli.
Left-valves more often affected.
Clinical presentation of IE.
SICK, but nonspecific symptoms - fever, weight loss, fatigue.
Murmurs usually present with left-sided lesions.
Organisms that are often involved in IE.
Strep viridans, Staph aureus, Staph epidermidis, **HACEK (Hemophilus, Actinobacillus, Cardiobaterium, Eikenella, Kingella)
Associate mucinous-adenocarcinomas (MALIGNANCIES), sepsis, or catheter induced endocardial trauma with what type of valvular disease?
NBTE
What is NBTE?
Nonbacterial thrombotic endocarditis.
Small, sterile thrombi of cardiac valve leaflets, along line of closure.
What are the 3 major categories of cardiomyopathies?
(1) Dilated cardiomyopathy - most common! (2) Hypertrophic cardiomyopathy (3) Restrictive cardiomyopathy.
Dilated cardiomyopathy causes
Familial (TTN mutation), ALCOHOL (cirrhosis), Cardiotoxic drugs, myocarditis/Coxsackie.
What is dilated cardiomyopathy (3 things)?
(1) Progressive cardiac dilated hypertrophy of ALL chambers (GLOBULAR). (2) SYSTOLIC dysfunction. (3) Mural thrombi common. (4) Functional regurgitation of valves.
What is this describing? Ages 20-50, progressive CHF leads to dyspnea, exertional fatigue, and decreased EF. Arrythmias and embolism. S3/systolic dysfunction.
Presentation of dilated cardiomyopathy.
What is arrhythmogenic right ventricular cardiomyopathy - what part of heart and what morphological changes?
Right ventricular failure and arrhythmias. Myocardium of right ventricular wall replaced by adipose and fibrosis.
Muscle replaced by fat
What results from arrhythmogenic RVcardiomyopathy? What is its cause?
Causes ventricular tachycardia and fibrillation, sudden death.
Familial.
What is hypertrophic cardiomyopathy?
DIASTOLIC dysfunction. Often causes ventricular outflow obstruction.
Morphology of hypertrophic cardiomyopathy
Massive myocardial hypertrophy, often with SEPTAL hypertrophy.
Microscopically=myocyte disarray.
What is restrictive cardiomyopathy? What causes it?
DIASTOLIC dysfunction. Decreased ventricular compliance (stiff), leading to diastolic dysfunction - leads to ATRIAL enlargement.
Causes: amyloid or fibrosis (radiation).
Associate b-pleated sheets with what. This causes what?
Amyloid = restrictive cardiomyopathy.
Systemic=myeloma; restricted to heart=transthyretin
Testing for amyloidosis?
Apple green birefrigence.
Definition of myocarditis and most common causes of myocarditis.
Def - inflammation of myocardium, caused by:
Viral - **COXSACKIE A and B, echovirus
Also - Trypanosome (Chagas), RF, SLE
What do you see microscopically in viral myocarditis.
Lymphocytic infiltrate is seen in viral myocarditis.
Associate trisomy 21 with what?
Congenital heart disease
Three things that result in left-to-right shunts.
ASD, VSD, PDA.
When are ASDs dx?
Atrial septal defects may lead to what 3 things?
Tx?
Asymptomatic until adulthood. MUMUR and RV hypertrophy or dilated RA.
Vol. overload on right = Pulmonary HTN, right HF, paradoxical embolization (i.e. dt DVT)
Surgical closure.
Large VSDs may lead to what?
RV hypertrophy. Pulm HTN, which can reverse flow through the shunt and lead to to CYANOSIS.
Seen in infants - cyanosis and harsh, machinery-like murmur.
PDA
Postnatal cyanosis is associated with what type of shunt?
Right to left shunt!
Tetrology of Fallot is what type of shunt (classically) and what 4 cardinal features?
R-L shunt.
4 Features: VSD, obstruction of RV outflow tract, aorta overrides VSD, RV hypertrophy.
Associate a boot shape with what specific shunt and what anatomic feature?
Tetrology of Fallot, RV HYPERTROPHY
Transposition of the great arteries results in what?
Compatible with life?
Two sepearte circuits, incompatible with life after birth UNLESS SHUNT IS PRESENT! (Tx: surgery)
often VSD, PDA, or PFO present
What is an example of an obstructive lesion of the heart?
Coarctation of the aorta.
What L-R shunt is narrowing of the aorta associated with?
PDA
Difference between coarctation of aorta (distal to arterioles to UE/head) with PDA and without PDA. Each manifests at what stage of life?
With PDA (birth): cyanosis in lower half of body. Without PDA (adult): asymptomatic - UE HTN, LE hypoT and cold, concentric LV hypertrophy
All are clinical features of what?
Pancarditis, Syndham chorea, migratory polyarthritis, subcutanous nodules, rash.
RF
Worsening fatigue with dyspnea, palpitations (arrhythmias), fever over the past week. Elevated Troponin-I. Full recovery.
Viral myocarditis
Causes of NBTE
Hypercoagulable state, CANCER
