Cardio Pathology Part 2 Flashcards
What are some causes of arrhythmias?
- Ischemic heart disease
- Cardiomyopathies
- Myocarditis
- Valvular disease
- Familial/congenital disordes
What is sick sinus syndrome?
- SA node is damaged leading to bradycardia
What is atrial fibrillation?
- Myocytes depolarize independently and sporadically with variable transmission to the AV node leading to an irregular HR
What can atrial fibrillation cause?
- Thrombus formation or thromboembolism (leading to stroke)
What is a heart block?
- Dysfunctional AV node
What is a first degree heart block?
- Prolonged PR interval
What is a second degree heart block?
- Intermittent transmission
What is a third degree heart block?
- Complete failure
What are hereditary channelopathies?
- Abnormal ion channels which cause arrhythmogenic disease
What is the most common hereditary channelopathy?
- Long QT syndrome
What is long QT syndrome?
- Cause of sudden death after exercise
- K+ and Na+ channel dysfunction leads to improper conduction
What are some risk factors for sudden cardiac death in younger patients?
- Drug abuse
- Hereditary conduction abnormalities
- Hypertrophic or dilated cardiomyopathy
- Myocardial hypertrophy
- Myocarditis
- Mitral valve prolapse
What does hypertensive heart disease lead to?
- Left ventricular hypertrophy
What can diastolic dysfunction lead to in hypertensive heart disease?
- Atrial enlargement ultimately leading to A fib
- Congestive heart failure
- Sudden cardiac death
What can acute cor pulmonale arise from?
- Large pulmonary embolus
What is the most common valve abnormality?
- Calcific aortic stenosis
What age group has the highest prevalence of calcific aortic stenosis?
- Older adults (60-80)
What causes the wear and tear on the valve in calcific aortic stenosis?
- Chronic HTN
- Hyperlipidemia
- Inflammation
What is the consequence of the calcifications on the aortic valve?
- Prevents complete opening
What is a caveat to bicuspid aortic valves in terms of calcification?
- Show an accelerated course due to asymmetrical closing
When do the symptoms of calcific bicuspid stenosis show in a patient?
- 1-2 decades earlier
What is more frequent on bicuspid valves?
- Bacterial endocarditis
What are some signs and symptoms of calcific aortic stenosis?
- Increased LV pressure causing concentric left ventricular hypertrophy
- Systolic murmur
- Angina
- Syncope
- CHF
What is the mortality rate of those with calcific aortic stenosis?
- Die with 5 years of developing angina
- Within 3 years of developing syncope
- Within 2 years of CHF
What is the treatment of calcific aortic stenosis?
- Surgical replacement of the valve
What is mitral annular calcification?
- Calcific deposits in the fibrous annulus
Who is more affected by mitral annular calcification?
- Females over 60 years old
What will the valve show in mitral annular calcification?
- Usually show as normal
What could mitral annular calcification lead to?
- Regurgitation
- Stenosis
- Arrhythmias (heart block, sudden death)
- Nidus for thrombus, infective endocarditis
What is mitral valve prolapse?
- When the valve leaflets prolapse back into the left atrium during systole
Who is mitral valve prolapse most likely seen in?
- Female (7:1)
What are the causes of mitral valve prolapse?
- No underlying cause identified
- Could be caused by a connective tissue disease (marfan)
- Complication of MI or rheumatic fever
What are the findings in someone with mitral valve prolapse?
- Leaflets are thickened and rubbery
- Interchondral ballooning of mitral leaflets
What are some clinical presentations of mitral valve prolapse?
- Most are asymptomatic
- Could lead to dyspnea due to mitral regurgitation
What are some serious complications of mitral valve prolapse?
- Infective endocarditis
- Mitral insufficiency
- Arrhythmias
- Thromboembolism
What is rheumatic fever?
- Multisystem inflammatory disease following a group A strep pharyngitis
What causes rheumatic fever?
- Antibodies and CD4+ T cell reactions against M streptococcal antigen which are reactive against antigens in the heart, joints, soft tissue, skin, and nervous system
When is the acute phase of rheumatic fever?
- 10 days to 6 weeks after the strep infection
What are the signs and symptoms of rheumatic fever?
- Fever
- Migratory polyarthritis
- Pancarditis
- Subcutaneous nodules
- Erythema marginatum
- Sydenham chorea (hopping, halting gait, asymmetric jerk, grimacing)
How is a rheumatic fever diagnosis confirmed?
- Looking at antibodies to streptolysin O and DNase B
Which valve is most likely to be affected in acute rheumatic heart disease?
- Mitral
- MAT (mitral>aortic>tricuspid)
What are the symptoms of acute rheumatic heart disease?
- Pancarditis
- Valvulitis with vegetation
- MacCallum Plaques
- Aschoff bodies with Anitschkow cells on histo
What is seen in chronic rheumatic fever disease?
- Valvular leaflet thickening
- Short chordae tendineae
- Fusion
- Regurgitation
Which valve is most likely to be affected in chronic rheumatic fever disease?
- Mitral
What is infective endocarditis?
- An infectious organism causes inflammation and fibrinous debris
Where does infective endocarditis usually affect?
- Valves or structural abnormalities
What is the most likely cause of infective endocarditis?
- Bacterial
What are some risk factors to infective endocarditis?
- Injection drug use
- Body piercings
- Male gender
- Poor dentition
- Invasive dental procedures
- Age >60
- Pre-existing heart conditions (rheumatic heart disease, MVP, calcific stenosis)
Which organism causes infective endocarditis that is native, structurally abnormal valves?
- Strep viridans
Which organism causes infective endocarditis from poor dentition or invasive dental procedures?
- HACEK organisms
Which organism causes infective endocarditis that affects prosthetic heart valves?
- Staph epidermidis
Which organism causes infective endocarditis in patients who abuse IV drugs?
- Staph aureus (causes right sided endocarditis due to traveling in veins)
What is the clinical presentation of someone with acute endocarditis?
- Rapid development of fever, chills, and weakness
What is the clinical presentation of someone with subacute endocarditis?
- Low grade fever and fatigue
How is infective endocarditis diagnosed?
- Duke criteria
- May have subungual/splinter hemorrhages, Janeway lesions, Osler nodes, or Roth spots
What is nonbacterial thrombotic endocarditis?
- A sterile, non-inflammatory valvular thrombi that is asymptomatic until embolization occurs
What causes a nonbacterial thrombotic endocarditis?
- Sepsis (proinflammatory, procoagulant cytokines)
- Cancer
- Antiphospholipid syndrome
- SLE (Libman-Sacks)
What is antiphospholipid syndrome?
- Autoantibodies against endothelial membranes
How does antiphospholipid syndrome manifest?
- Could manifest through fetal loss during pregnancy
- Could be primary or associated with lupus
What is Libman-Sacks endocarditis?
- Nonbacterial endocarditis associated with lupus
What is carcinoid heart disease?
- When bioactive compounds (serotonin) are secreted from carcinoid tumors, they induce plaque-like endocardial and valvular thickening
Why does carcinoid heart disease happen?
- The liver normally metabolizes serotonin, but if the tumor metastasis to the liver, serotonin travels to the lungs.
Where do the lesions occur in carcinoid heart disease?
- Right side of the heart due to MAO being present in the lungs
What are the signs and symptoms of carcinoid heart disease?
- Flushing
- Diarrhea
- Dermatitis
- Bronchoconstriction
What are the different types of mechanical prosthetic valves?
- Caged ball
- Tilting disk
- Hinged flap
What is a complication of having a mechanical prosthetic valve?
- Risk of thromboembolism
What are the different types of tissue prosthetic valves?
- Bovine or Porcine
What is a complication of having a tissue prosthetic valve?
- Mechanical failure like tearing or incompetence could occur
What is a complication that can affect both types of prosthetic valves?
- Infective endocarditis
What are some other risks involved in prosthetic valves?
- Anticoagulant related hemorrhage (mechanical require life long anticoag therapy)
- Dysfunction
- Hemolysis (hemolytic anemia)
What kind of a murmur does a calcific aortic stenosis cause?
- Harsh systolic murmur (crescendo-decrescendo)
What kind of a murmur does a mitral regurgitation cause?
- Holosystolic murmur
What kind of a murmur does a aortic regurgitation cause?
- Diastolic decrescendo murmur
What kind of a murmur does a PDA cause?
- Machine-like murmur
What are the three types of cardiomyopathy?
- Dilated
- Hypertrophic
- Restrictive
What is dilated cardiomyopathy?
- Progressive cardiac dilation and systolic dysfunction, usually with dilated hypertrophy
What are some causes of dilated hypertrophy?
- Familial 30-50% (mutation in Titin gene)
- Peripartum cardiomyopathy
- Alcohol
- Myocarditis
- Cardiotoxic drugs (doxorubicin)
- Iron overload (hereditary hemochromatosis or multiple transfusions)
What kind of cardiac morphology is seen in dilated cardiomyopathy?
- Dilation of all four chambers
- Often hypertrophic
- Functional regurgitation of valves
What are some clinical features of dilated cardiomyopathy?
- Manifests between ages 20-50
- Progressive CHF leading to dyspnea, exertional fatigue, and decreased EF
- Systolic dysfunction
- Arrhythmias (sudden death)
- Thrombi formation with embolism
What is Takotsubo cardiomyopathy?
- AKA broken heart syndrome
- Associated with emotional distress which causes sudden surge of catecholamines
What are the signs and symptoms of takotsubo cardiomyopathy?
- Apical ballooning of the left ventricle
- Similar presentation to MI
Who is most likely to suffer from takotsubo cardiomyopathy?
- Women 60-75 YO
What is arrhythmogenic right ventricular cardiomyopathy (ARVC)?
- Defective cell adhesion proteins in desmosomes that link adjacent cardiac myocytes
What is seen clinically in ARVC?
- Right ventricular failure and arrhythmias
What is Naxos syndrome?
- ARVC with plantar and palmar hyperkeratosis and wooly hair
What gene is affected in Naxos syndrome?
- Plakoglobin
What happens to the right ventricular wall in naxos syndrome?
- Adipose and fibrosis leading to v tach/fib causing sudden death
Who is affected by hypertrophic cardiomyopathy?
- Genetic disorder that affects predominantly males
What is hypertrophic cardiomyopathy characteristically marked by?
- Myocyte hypertrophy and myocyte disarry
What mutations are associated with hypertrophic cardiomyopathy?
- Numerous mutations are known, involving sarcomeric proteins
- Most common is B-myosin heavy chain
What are the signs and symptoms of hypertrophic cardiomyopathy?
- Ventricular arrhythmias
- Systolic ejection murmur
- Exertional dyspnea
- Exertional chest pain
- Palpitations
What can the ventricular arrhythmias cause in hypertrophic cardiomyopathy?
- Unexplained death in athletes (most common presentation)
What causes the systolic ejection murmur in hypertrophic cardiomyopathy?
- Mitral valve pushed toward septum
What causes the exertional chest pain in hypertrophic cardiomyopathy?
- Microvascular thrombi
What causes the exertional dyspnea in hypertrophic cardiomyopathy?
- Impaired diastolic filling– diminished cardiac output and pulmonary congestion
What is restrictive cardiomyopathy?
- Decreased ventricular compliance leading to diastolic dysfunction
What happens to the left ventricle in restrive cardiomyopathy?
- Systolic function of LV remains normal
What could cause restrictive cardiomyopathy?
- Could be secondary to deposition of material within the wall (amyloid) or increased fibrosis (radiation)
What do the atria look like in restrictive cardiomyopathy? How do they compare to the ventricles?
- Atria are enlarged
- Ventricles are normal
What is amyloidosis?
- Extracellular deposition of proteins which form an insoluble B-pleated sheet
What stain is used to show amyloidosis?
- Congo red stain
How does amyloidosis show up on stain?
- Apple green birefirngence
What are some causes of amyloidosis?
- Myeloma
- Chronic inflammatory states
- Mutated version of transthyretin
- Senile amyloidosis
What what endomyocardial fibrosis? Where is it found?
- Fibrosis of endocardium and subendocardium
- Found in children and young adults in tropical or subtropical areas
What is Loeffler endocarditis?
- Part of spectrum of endomyocardial fibrosis
- Shows eosinophilic infiltration
- Associated with myeloproliferative disorders
What is endocardial fibroelastosis? When does it show up?
- Fibroelastic thickening of left ventricle endocardium
- Shows up in first 2 years of life
- Associated with congenital heart defects
What is myocarditis?
- Inflammatory disease of cardiac muscle
What is the most common cause of myocarditis?
- Viral infection (coxsackievirus B)
What causes chagas disease?
- Parasite: Triatomine bug
What causes trichinosis?
- Eating undercooked pork
What causes lyme disease
- Ixodes tick in NE US
When is myocarditis seen in lyme disease?
- In the early disseminated phase
What other symptoms are seen in lyme disease?
- Migratory polyarthritis
- Facial palsy
- Meningitis
What is lymphocytic myocarditis?
- Most common form of myocarditis
- Due to viral/post viral infection, autoimmune process, or idiopathic
What is eosinophilic myocarditis? What must you exclude from differential?
- Marked increased eosinophils
- There is a hypersensitivity reaction due to an underlying allergy
- Could be idiopathic and must exclude parasitic infection
What is idiopathic giant cell myocarditis?
- Giant cells are admixed with variable inflammation (typically increased eosinophils)
What is the prognosis of idiopathic giant cell myocarditis?
- Poor with survival rate of less than 3 months
What is myocardial sarcoidosis?
- An idiopathic process, rare cause with variable presentation
- Giant cells with non-necrotizing granulomas
What are the two cardiotoxic drugs that cause dilated cardiomyopathy?
- Doxorubicin
- Daunorubicin
What is the normal volume of fluid surrounding the heart?
- > 50 mL
How much fluid can build up in a slow accumulation? Is the patient symptomatic or asymptomatic?
- <500mL can build up
- Patients are typically asymptomatic
What happens in an acute pericardial effusion?
- Occurs in less than a week
- 200-300 mL rapidly accumulate and causes symptomatic cardiac tamponade
What are some examples of pericardial effusions?
- Hemopericardium (blood in pericardial space)
- Serous effusion (CHF)
- Purulent pericarditis (pus)
What is pericarditis?
- Inflammation of the pericardial sac
What are some signs and symptoms of pericarditis?
- Chest pain
- Pericardial friction rub
- Pericardial effusion
- EKG changes
- Low grade fever
What are the different types of pericarditis?
- Fibrinous and serofibrinous
- Serous pericarditis
- Purulent or suppurative pericarditis
- Caseous pericarditis
- Hemorrhagic pericarditis
- Constrictive pericarditis
What is the most common type of pericarditis?
- Fibrinous and serofibrinous
What causes the fibrinous and serofibrinous pericarditis?
- A fibrinous inflammatory exudate causing a variable amount of fluid build up
What can cause a fibrinous or serofibrinous pericarditis?
- Acute MI
- Dressler’s syndrome
- Uremia
- Chest irradiation
- RF
- SLE
- Trauma
What causes a serous pericarditis?
- Viral or noninfectious inflammatory diseases
What causes a purulent or suppurative pericarditis?
- Active infection caused by microbial invasion
What causes a caseous pericarditis?
- Tuberculosis
- Occasionally fungal
What causes a hemorrhagic pericarditis?
- Most commonly due to malignant tumor spread
- Could be due to trauma
What causes a constrictive pericarditis?
- Occurs when the heart is encased in a dense, fibrous or fibroelastic scar that limits diastolic expansion and cardiac output
What is a cardiac myxoma?
- Most common primary tumor
- Stromal tumor of mesenchymal origin
Where is a cardiac myxoma usually located?
- Left atrium and begins in septal region near fossa ovalis
What are some signs and symptoms of a cardiac myxoma?
- “ball-valve” obstruction
- mechanical valve damage
- Embolization
- Fever
- Malaise
- IL-6 elaboration by tumor (acute phase response)
What will you hear on auscultation with a cardiac myxoma?
- Tumor “plop”
What familial syndromes are cardiac myxomas associated with?
- McCune-Albright syndrome
- Carney complex
What is McCune-Albright syndrome?
- A GNAS1 mutation
- Symptoms include: polyostotic fibrous dysplasia, cafe au lait spots, endocrine abnormalities
What is Carney complex?
- PRKAR1A mutation
- Symptoms include: skin changes, endocrine dysfunction
What is a lipoma?
- Localized mass composed of mature lobulated fat
- Occurs throughout the heart
What is a papillary fibroelastoma?
- Typically incidental
- Often located on valves
What does a papillary fibroelastoma look like?
- Sea anemone
What is a rhabdomyoma?
- Most frequent primary tumor of the pediatric heart
What causes a rhabdomyoma?
- 50% are sporadic
- 50% are associated with tuberous sclerosis
What is an angiosarcoma?
- Malignant endothelial neoplasm that primarily affects older adults
How can non-cardiac tumors affect the heart?
- Mass effect (limiting cardiac filling)
- Decreased myocardial contractility
- Symptomatic pericardial effusion
- SVC syndrome
What is an allograft rejection?
- Rejection that can be cellular mediated or antibody mediated
What is the cellular mediated rejection?
- T cell mediated, lymphocytic response
What is the antibody mediated rejection?
- Antibody mediated with vascular neutrophilic infiltration
What is allograft vasculopathy?
- A late, progressive, and diffusely stenosing intimal proliferation
- In 50% of patients in 5 years, in all patients after 10 years
What does allograft vasculopathy cause?
- Silent MI due to denervated transplanted heart (no angina)
What are some complications to chronic immunosuppression?
- Infection
- Malignancy