Cardaic Pathology Pt 2 Flashcards
What is the MC structural birth defect?
Congenital heart disease
What is congenital heart disease?
Abnormality or defect that affects the heart or great vessel which is present before birth
What are some causes of congenital heart disease?
Most commonly due to sporadic genetic mutations
Also environmental factors (ex fetal alcohol syndrome)
What are the most common congenital heart disease?
Septal defects (VSD #1)
How do ASD and VSD affect RV and pulmonary outflow pressure and volume?
They both increase RV and pulmonary outflow volumes and pressure
Whereas PDA increases pulmonary outflow volume and pressure only
What is the result of increased pulmonary pressure?
Increases pulmonary vascular resistance which will result in Eisenmenger syndrome
Does staph aureus cause acute or subacute endocarditis?
Acute
Is early (<2 months) prosthetic valve infection typical with staph aureus infection?
Yes
What are risk factors for infective endocarditis caused by staph aureus?
IV drug abuse, very common with prosthetic valves
What is the #1 cause of endocarditis?
Staph aureus
What are unique features of staph aureus?
Right sided (tricuspid valve) endocarditis associated with IV drug abuse
What are causes of subacute infective endocarditis?
Strep viridans, enterococci, staph epidermidis, HACEK group
Is early (<2 months) prosthetic valve infection typical with strep viridans, enterococci, staph epidermidis, or the HACEK group?
Only for staph epidermidis
What are risk factors for strep viridans?
Dental procedures/poor dentition
What are unique features of strep viridans?
1 for native, structurally abnormal
What are risk factors and unique features for staph epidermidis?
Very common with prosthetic valves
Unique feature is that its present in normal skin flora
What are risk factors for the HACEK group?
Dental procedures/poor dentition
What are unique features of the HACEK group?
Rare
<2% of endocarditis cases
Which organisms are apart of the HACEK group?
Haemophilus Aggregatibacter (previously actinobacillus) Cardiobacterium Eikenella Kingella
What are the two types of prosthetic valves?
Mechanical and tissue valves
What are mechanical valves?
Caged ball, tilting disk, hinged flap (bi-leaflet)
Risk of thromboembolism
What are tissue valves (bioprostheses)?
Bovine (cow) or porcine (pig)
Risk of mechanical failure (tearing, incompetence)
Both types of prosthetic valves have a risk of what?
Infective endocarditis
What are other risks associated with prosthetic valves?
Anti-coagulant related hemorrhage (lifelong anticoagulation with mechanical valves)
Dysfunction (regurgitation or exuberant healing)
Hemolytic anemia
Which prosthetic valve lasts longer?
Mechanical valves last a long time (greater than 25 years) whereas tissue valves last 10-15 years
What type of dysfunction is seen with dilated cardiomyopathy?
Systolic dysfunction
What is the principal cardiac abnormality in dilated cardiomyopathy?
Enlargement of all chambers
What are some additional features of dilated cardiomyopathy?
Most common type
Multiple causes
What type of dysfunction is seen with hypertrophic cardiomyopathy?
Diastolic dysfunction
What is the principal cardiac abnormality seen with hypertrophic cardiomyopathy?
Thickened left ventricular wall (septal)
What are additional features associated with hypertrophic cardiomyopathy?
Risk of sudden death in young athletes
Beta myosin heavy chain mutation (common mutation)
What type of dysfunction is seen with restrictive cardiomyopathy?
Diastolic dysfunction
What is the principal cardiac abnormality seen with restrictive cardiomyopathy?
Rigid ventricular walls
What are additional features associated with restrictive cardiomyopathy?
Least common type Multiple causes (especially amyloidosis)
What is iron overload cardiomyopathy?
Hereditary hemochromatosis caused by mutation in HFE gene
How does hereditary hemochromatosis affect different systems of the body?
Skin (pigmentation), pancreas (diabetes) = bronze diabetes
Heart (dilated cardiomyopathy and arrhythmia)
Liver (hepatosplenomegaly), bone (arthritis), testicles (hypogonadism)
What are other types of restrictive cardiomyopathy?
Endomyocardial fibrosis
Loeffler endocarditis
Endocardial fibroelastosis
What is endomyocardial fibrosis?
Children and young adults in tropical and subtropical regions (Africa)
Fibrosis of endocardium and subendocardium
Sx: Dyspnea with exertion, fatigue, paroxysmal nocturnal dyspnea, orthpnea
What is Loeffler endocarditis?
Part of spectrum of endomyocardial fibrosis
Shows eosinophilic infiltration
Associated with myeloproliferative disorders (leukemia/lymphoma)
Seen in subtropical areas
Sx: Fever, cough, rash
What is endocardial fibroelastosis?
Fibroelastic thickening of left ventricular endocardium
Presents in the first 2 years of life
Associated with congenital heart defects (aortic stenosis, atresia, etc)
S/S: Infant with feeding difficulty, breathlessness, failure to thrive and wheezing
What is the most common functional pattern?
Dilated
What is the least common functional pattern?
Restrictive
What is the mechanism of heart feature in dilated functional pattern?
Impairment of contractility (systolic dysfunction)
What are the causes of the phenotype associated with dilated functional pattern?
Genetic Alcohol Peripartum Hemochromatosis Doxorubicin toxicity
What is the mechanism of heart failure associated with hypertrophic functional pattern?
Impairment of compliance (diastolic dysfunction)
What are the causes of the phenotype associated with hypertrophic functional pattern?
Genetic (hypertrophic cardiomyopathy)
What is the mechanism of heart failure for restrictive functional pattern?
Impairment of compliance (diastolic dysfunction)
What are the causes of the phenotype associated with restrictive functional pattern?
Amyloidosis
Radiation induced fibrosis
What is the MC form of myocarditis?
Lymphocytic myocarditis
Seen both in viral/post viral infection, autoimmune or idiopathic
What is eosinophilic myocarditis?
Characterized by marked increased eosinophils
Hypersensitivity myocarditis due to underlying allergy (hypersensitivity) reaction (typically drug reaction)
Can be idiopathic
Must exclude parasitic infection
What are the types of myocarditis with giant cells?
Idiopathic giant cell myocarditis
Myocardial sarcoidosis
What is idiopathic giant cell myocarditis?
Aggressive, poor prognosis, survival less than 3 months from onset
Giant cells admired with variable mixed inflammation (typically increased eosinophils)
What is myocardial sarcoidosis?
Idiopathic process, rare cause of myocarditis, variable presentation
Giant cells with non-necrotizing granulomas