Cardiac Path Flashcards
Define heart failure, and name key symptoms.
Cardiac pump dysfunction
- cardiac dilation
- dyspnea on exertion
- fatigue
- rales
- JVD
Name 3 classes of drugs used to treat heart failure.
- ACE inhibitors
- beta-blockers
- diuretics
Is right- or left-sided heart failure more common?
Left. (Right often follows left)
Contrast the causes of left-sided (systemic) vs. right-sided (pulmonic) heart disease?
Left (systemic problems): HTN, ischemia, MI, cardiomyopathy
Right (pulmonic problems): L side failure (or L–>R shunt, chronic lung disease/cor pulmonale)
The “nutmeg liver” is associated with what diagnosis?
Right-sided heart failure (pulmonary heart disease)
Contrast stable vs unstable angina.
Stable (angina pectoris):
- pain with exertion/stress,
- > 70% vessel occulsion
- <20 mins
- reversible injury
- ST depression
- predictable
Unstable:
- pain at rest
- 90% vessel occusion
- reversible injury (until MI)
- normal EKG
- unpredictable
Define prinzmetal (variant) angina.
Chest pain due to vasospasm of coronary artery
- normal EKG (except ST elevation only during a spasm)
Contrast NSTEMI and STEMI.
Both show elevated cardiac enzymes in labs ( troponin, CK, CK-MB)
- NSTEMI: normal EKG
- STEMI: abnormal EKG (ST elevation)
Which coronary arteries are most commonly occluded (in order)?
- LAD
- RCA
- L Circumflex
Where in the heart is affected by occluding the LAD?
- anterior LV near apex
- anterior 2/3 of septum
Where in the heart is affected by occluding the RCA?
- posterior LV
- posterior 1/3 of septum
- possibly some of the RV
Where in the heart is affected by occluding the L circumflex?
- lateral LV
What are possible causes of sudden cardiac death? (7)
- ischemia
- ventricular arrhythmia
- atherosclerosis
- mitral valve prolapse
- cocaine
- cardiomyopathy
- myocarditis
Splinter hemorrhages are indicative of what cardiac diagnosis?
Infectious endocarditis
Findings with hypertensive heart disease?
- High blood pressure
- Enlarged heart and irregular heartbeat
- Fluid in the lungs or lower extremities
- Unusual heart sounds
A systolic ejection click and crescendo-decrescendo systolic murmur indicate what?
Aortic stenosis
Generally, what is stenosis, and what population is it likely to affect?
Stenosis = fibrosis/calcification because of wear and tear
Normally affects >60 yrs old, unless the valve or vessel is previously compromised
High pitched, blowing, early diastolic decrescendo murmur indicates what?
Aortic regurgitation?
What can cause aortic regurgitation?
- Aortic root dilation
- Aneurysm, dissection
- Valve damage
What is Quincke pulse, and what diagnosis is it associated with?
Alternating filling and blanching of the capillary beds in the fingernails
Seen in aortic regurgitation
An opening snap with late diastolic rumble indicates what?
Mitral stenosis
Patients with aortic stenosis may present clinically with ___?
syncope
What developmental abnormality predisposes to aortic stenosis?
A biscuspid aortic valve
Explain mitral valve prolapse, and the causes.
= Systolic balooning of mitral valve
Causes
- marfan/ehlers-danlos
- rheumatic fever
- chordae rupture
Shortened fibrotic cordae tendinae are associated with what diagnosis?
Chronic rheumatic heart disease
Which diseases preferentially affects the mitral valve?
- Chronic rheumatic heart disease
What diagnosis results in the “fish mouth” mitral valve deformity?
Chronic rheumatic heart disease
What diseases preferentially affects the tricuspid valve?
- Libman-Sachs
- infective endocarditis
A holosystolic high pitched “blowing” murmur indicates what?
Mitral regurgitation
- (louder with squatting/expiration)
Name the JONES criteria and what diagnosis they are associated with?
Rheumatic heart disease:
- Joints (migratory polyarthritis)
- O for <3 (pancarditis)
- Nodules
- Erythema marginatum (rash on trunk/limbs)
- Sydenham chorea (Aschoff bodies of Anitschkow cells w wavy nuclei): vegetations of valves
List 5 infectious causes of endocarditis, and what predisposes for each.
- strep viridans (most common)
- staph aureus (IV drugs; tricuspid)
- staph epidermidis (prosthetic valves)
4 strep bovis- underlying colorectal carcinoma
5 HACEK organisms- (-) BCx
Effects of smoking on the heart?
- Vasoconstriction
- Endothelial damage (vessel wall)
- Accelerates athersclerosis
- Exposure to CO (up to 5 or 6%)
Multifactorial contributor to ischemia of the heart
Who will not show the classic symptoms of an MI?
Women and diabetics
Splinter hemorrhages are associated with what diagnosis?
Infectious endocarditis
What are the causes of non-infectious of endocarditis? (2)
- hypercoagulable state
- adenocarcinoma (paraneoplastic)
Are valve vegetations due to rheumatic heart disease infectious or sterile?
Sterile. It is an remnant autoimmune reaction to the past illness.
Sterile vegetations on both sides of the mitral valve is typically due to what (diagnosis)?
Libman-sacks (Endocarditis from SLE)
Dilated cardiomyopathy can lead to….
(All 4 heart chambers dilated)
- systolic dysfunction
- biventricular CHF
- regurgitation
- arrhythmia
What is the most common type of congenital cardiac malformation?
Ventricular septal defects (VSD); accounts for about 40%
Explain how a VSD may change from an acyanotic to a cyanotic heart disease
Initially: VSD’s increased left ventricular pressure =left to right shunt 1) left ventricular overload 2) right ventricular and pulmonary artery hypertension 3) vascular changes that increase pulmonary vascular pressure and right ventricular pressure 4) reversal of the left to right shunt and cyanosis.
What are some causes of cardiomyopathy? (7)
- idiopathic
- genetic mutation
- myocarditis (coxsackie virus)
- alcohol abuse
- drugs
- pregnancy
- hemochromatosis
What is the histological hallmark of hypertrophic cardiomyopathy
myofiber hypertrophy and disarray (even before any symptoms or dysfunction)
What is the mian cause of hypertrophic cardiomyopathy?
- AD mutation in sarcomere protein, B-myosin heavy chain
Chelsea - is the B-mysoin the sarcomere protein or is that a separate cause?
What is the histological hallmark of hypertrophic cardiomyopathy
myofiber hypertrophy and disarray (even before any symptoms or dysfunction)
What are the common congenital heart malformations that result in late cyanosis?
left-to-right shunts:
VSD, ASD, Patent ductus arteriosus (PDA), AV septal defect
Some genetic or environmental causes of these congenital heart malformations include:
a. Down Syndrome
b. Trisomy 13 & 18
c. Turner syndrome
d. CHARGE association
e. VATER association
f. Congenital rubella -
g. Fetal alcohol syndrome
h. Fetal hydantoin syndrome
Down Syndrome causes congenital heart malformations:
VSD, AV septal defects
Trisomy 13 & 18 causes congenital heart malformations include:
VSD, PDA, ASD
Turner syndrome causes congenital heart malformations include:
bicuspid aortic valve, coarctation of aorta
Hemopericardium
Cardiac tamponade
blood in the pericardial sac of the heart
Compression of heart= dec CO
Sx: tachycardia, pulsus paradoxus (dec systolic BP on inspiration), kussmaul sign (inc JVP on inspiration)
Beck’s triad: hypotension, distended neck veins, distant heart sounds
EKG: low voltage QRS and electrical alternans (swinging heart)
Fetal alcohol syndrome causes congenital heart malformations include:
AS, PDA
Describe the 3 types of pericarditis
- Fibrinous: caused by Dressler syndrome (post-cardiac injury), uremia (renal failure), radiation
- Serous: viral and noninfectious inflammatory disease (RA, SLE)
- Suppurative: bacterial infections
Discuss the clinical features and complications of aortic stenosis.
Obstruction to LV outflow and combined with valvular incompetence and regurgitation. LVhypertrophy. Left sided congestive heart failure Angina pectoris Syncope Infection
What is the incidence and typical clinical presentation of mitral valve prolapse?
The overall incidence of MVP is 2-3% of the general population. The condition affects predominantly young
women. Clinical presentation is often asymptomatic with mid-systolic click/ late systolic murmur.
What is the current theory of the pathogenesis of MVP?
A developmental anomaly of connective tissue is postulated in some cases because of the association of MVP in
Marfan syndrome
Discuss the possible complications of MVP
most asymptomatic palpitations, chest pain and fatigability, psychiatric manifestations/ anxious. serious complications: 1 mitral regurgitation (some with rupture of chordae tendineae), 2 infective endocarditis 3 systemic emboli( Cerebral emboli); or 4 Arrhythmia 5 Sudden Death
Angiosarcomas
- Proliferation of endothelial cells (cell marker CD31)
- breast, skin, liver
- Gross: Aggressive, poorly demarcated, variegated, fleshy masses
- Histo: dense clumps of Atypical cells lining vascular lumen
- Risk: older adults, PVC, arsenic
- (5 yr survival= 30%)
ANY change in microscopic cell arrangement, from disorganized growth or pathologic damage, may lead to?
Arrhythmias
A friction rub is associated with what diagnosis?
Pericarditis
Arrhythmogenic RV cardiomyopathy
- AD mutation of desmosomal adhesion proteins in myocytes
RV myocardial thinning and dilation - Histo: myocardial fatty infiltration and fibrosis (no inflammation)
- Leads to: ventricular arrhythmias, syncope, sudden death
Myocarditis
- Inflammation of myocardium leading to death of myocytes.
Lymphocytic infiltrate in myocardium
Common 1-10yo - Cause: Enteroviruses (coxsackie virus A or B), infections, Hypersensitivity, Giant cell myocarditis, Sarcoidosis
- Sx: CP, arrhythmia, sudden death, heart failure, flabby heart with biventricular dilation (dilated cardiomyopathy)
- Histo: lymphocytes in myocardium,
- T-cell infiltrate with myocyte necrosis.
Pericardial effusion
abnormal accumulation of fluid in the pericardial space
Orthopnea suggests what possible diagnoses?
- CHF (left-sided)
- pulmonary edema
- asthma
Ventricular septal defect (VSD)
L–>R shunt
- most common congenital defect
- Defect in septum dividing R/L ventricle
- Cause: fetal alcohol sx
- Sx: asymptomatic
- Sound: holosystolic, harsh murmur
- Leads to: Eisenmenger syndrome/cyanosis at birth, LV overload, heart failure
- Tx: surgical closure
Discuss the etiology of acute rheumatic fever/rheumatic heart disease
ARF is an immune-mediated hypersensitivity disorder (types 2 and 4) triggered by a preceding group A streptococcal
infection. The disease begins 2-4 weeks after the strep pharyngitis.
Explain the mechanism of cyanosis in Tetralogy of Fallot
A dominant right to left shuntfrom right ventricular outflow obstruction and VSD decreases pulmonary blood flow and the amount of erythrocytes that can be oxygenated.
how is nonbacterial thrombotic endocarditis different than acute rheumatic valvulitis
commonly in previously normal valves, associated with malignancies (50%), wasting, hypercoagulability,
or othewise normal individuals. More common on mitral and aortic valves, but underlying valves have no inflammation or
fibrosis.
classify the endocarditis (acute vs subacute)
subacute infective endocarditis: vague symptoms, underlying valvular abnormality
acute infective endocarditis:
rapid onset of fever, chills and lassitude. Petechiae or subungual hemorrhages
How would you diagnose infective endocarditis
Multiple blood cultures must be drawn before starting antibiotics
finding of a new or intensified cardiac murmur
Pulmonary stenosis and atresia
R–>L shunt
- obstruction to flow from the right ventricle (RV) to the pulmonary arterial vasculature
- Sx: asymptomatic, Cyanosis, exertional dyspnea, fatigue
- Leads to: angina, syncope, or sudden death, Peripheral edema, right heart failure
- a/w: patent foramen ovale, atrial septal defect, or ventricular septal defect
most likely causative organism for infective endocarditis: prosthetic valve
Staph. epidermis (coagulase negative staph)
most likely causative organism for infective endocarditis: rheumatic heart disease
Strep. viridans (alpha hemolytic strep - 50-60% of cases)
most likely causative organism for infective endocarditis: immune compromise
fungi
In what clinical setting are right-sided valves most commonly involved with infective endocarditis?
Right sided involvement is most common in intravenous drug abuse settings, and the causative organisms
are predominantly skin flora (S. aureus)
Discuss potential complications of infective endocarditi
1) valve insufficiency or stenosis with heart failure,
2) myocardial ring abscess, possible perforations,
3) suppurative
pericarditis,
4) dehiscence of artificial valve,
5) septic emboli, infarcts to brain, heart, spleen, kidney, lungs (right sided),
6)renal complications - glomerulonephritis
(4) features of Tetralogy of Fallot
1 Overriding Aorta
2 Right ventricular hypertrophy
3 Subpulmonary stenosis
4 Ventricular septal defect
Pericarditis (signs and symptoms)
- swelling and irritation of the pericardium, the thin sac-like membrane surrounding your heart
- Sx: sharp pain, worse on inspiration, tripoding
Sound: friction rub - EKG: widespread ST elevation, PR depression
Describe the 3 types of pericarditis
- Fibrinous: caused by Dressler syndrome (post-cardiac injury), uremia (renal failure), radiation
- Serous: viral and noninfectious inflammatory disease (RA, SLE)
- Suppurative: bacterial infections
“most prevalent cardiac malformation” that results in cyanosis and diminished pulmonary blood flow
Tetralogy of Fallot
Explain the mechanism of cyanosis in Tetralogy of Fallot
A dominant right to left shuntfrom right ventricular outflow obstruction and VSD decreases pulmonary blood flow and the
amount of erythrocytes that can be oxygenated.
List the congenital cardiac malformations associated with cyanotic heart disease
(5 T’s). A. Tetralogy of Fallot B. Transposition of the great vessels C. Tricuspid atresia D. Total anomalous pulmonary venous return E. Truncus arteriosus
tumors affect the heart: most common primary tumor of adults
Myxoma (90% in atria)
What are some of the effects of noncardiac tumors on the heart?
Direct consequence of tumor:
Pericardial and myocardial metastases
Large vessel obstruction
Pulmonary tumor emboli
What is the most common primary tumor of the heart?
Myxoma
Describe a myxoma
- most common in adults
- Benign mesenchymal tumor
- Common: in LA
- Gross: gelatinous appearance
- Pedunculated mass in LA
- Leads to: syncope due to mitral valve obstruction
Lipomas
- A benign tumor of fat
the most common soft tissue tumor of adulthood
soft, mobile, and painless (except angiolipoma) - Cured by simple excision, rarely recur
well-encapsulated mass of mature adipocytes - Histo: Normal fat, Clear cytoplasm, Low mitoses- small nuclei
Rhabdomyomas
- most common in kids
- Benign tumor of striated muscle
- A/w: tuberous sclerosis (multi-system benign tumors)
- Circumscribed
- Tx: excision
- Common: in ventricle
- Histo: Eosinophilic spider-cells (“legs”), Low mitotic activity, Well formed
Angiosarcomas
- Proliferation of endothelial cells (cell marker CD31)
- breast, skin, liver
- Gross: Aggressive, poorly demarcated, variegated, fleshy masses
- Histo: dense clumps of Atypical cells lining vascular lumen
- Risk: older adults, PVC, arsenic
- (5 yr survival= 30%)
Rhabdomyosarcomas
- A malignant tumor of striated muscle
- Characteristic cell: rhabdomyoblast
- (+) Desmin and Myo D1 immunostain
- Most common malignant soft tissue tumor in children
- Mutations are genetically heterogenous: Fusions of FOXO1 or FKHR to either PAX3 gene (alveolar) or t(2;13) translocation= chimeric proteins
- Tx: surgery, chemotherapy, radiation therapy
What are the most common metastatic heart tumors?
Breast, lung, melanoma, lymphoma
Atrial septal defect (ASD)
L–>R shunt
1) ostium secundum defect- area of the foramen ovale
2) ostium primum- endocardial cushion defect (a/w Down Sx)
- Sound: split S2 (delayed pulmonary valve)
- Leads to: paradoxial emboli, heart failure, asymptomatic
What is seen histologically with dialated cardiomyopathy?
fibrosis and myocyte variability
Patent foramen ovale
L–>R shunt
- septum primum and septum secundum fail to fuse
- Allows interatrial shunting after birth
- Tx: surgical closure with occluder device
Premature closure of the foramen ovale
L–>R shunt
- Loss of intrauterine blood flow from the right to the left atrium
- Leads to: LV hypoplasia, right heart failure, aneurysm of the atrial septum, arrhythmias, pericardial effusion, pleural effusion, death
Ventricular septal defect (VSD)
L–>R shunt
- most common congenital defect
- Defect in septum dividing R/L ventricle
- Cause: fetal alcohol sx
- Sx: asymptomatic
- Sound: holosystolic, harsh murmur
- Leads to: Eisenmenger syndrome/cyanosis at birth, LV overload, heart failure
- Tx: surgical closure
Patent ductus arteriosus (PDA)
L–>R shunt
- Ductus arteriosus fails (pulmonary artery–>aorta) to close
- Cause: congenital rubella, premature
- Sound: continuous “machine like” murmur
- Leads to: Eisenmenger, lower extremity cyanosis
- Tx: indomethacin (decreases PGE)
Tetralogy of fallot
R–>L shunt
- pulmonary stenosis/RV outflow
- RV hypertrophy
- VSD
- aorta overrides VSD- aorta is displaced to the right so that it appears to arise from both ventricles
- Xray: boot shaped heart
- Sx: cyanotic spells (pt’s squat to decrease shunt)
- Cause: mutation in NOTCH pathway
Transposition of great arteries
R–>L shunt
- LV–>pulmonary artery
- RV–>aorta
- Cause: maternal diabetes, aorticopulmonary septum fails to spiral
- Sx: cyanosis, RV hypertrophy, LV atrophy
- Tx: surgical shunt and PGE to maintain PDA
Truncus arteriosus
R–>L shunt
- Truncus fails to divide= Single large vessel from both ventricles
- a/w VSD
- Sx: cyanosis, blood mixes
Tricuspid atresia
R–>L shunt
- Absence of tricuspid valve
1. ASD
2. VSD
3. RV atrophy
4. LV hypertrophy
Pulmonary stenosis and atresia
R–>L shunt
- obstruction to flow from the right ventricle (RV) to the pulmonary arterial vasculature
- Sx: asymptomatic, Cyanosis, exertional dyspnea, fatigue
- Leads to: angina, syncope, or sudden death, Peripheral edema, right heart failure
- a/w: patent foramen ovale, atrial septal defect, or ventricular septal defect
Coarctation of the aorta
- Narrowing of aorta
- Excessive ductal closure
Contrast the infantile vs adult form of coarctation of the aorta
- Infantile form
- Narrowing distal to aortic arch, proximal to PDA
- Sx: lower extremity cyanosis
- Cause: Turner’s syndrome - Adult form
- Narrowing distal to ligamentum arteriosum
- Sx: HTN in upper extremities, hypotension/weak pulses lower extremities (radiofemoral delay), diminished renal blood flow (renin=HTN)
- Cause: bicuspid aortic valve
- Xray: notching of ribs, engorged intercostal arteries from developing collateral circulation
Dextrocardia
apex of the heart is situated on the right side of the body
Situs inversus
major visceral organs are reversed or mirrored from their normal position
DiGeorge sequence
- congenital heart disease
- Sx: defects in the palate, neuromuscular problems with closure (velopharyngeal insufficiency), learning disabilities, mild differences in facial features, and recurrent infections.
- 22q11.2 deletion
CHARGE syndrome
- Sx: Posterior coloboma, heart defect, choanal atresia, retardation, GU, ear disorders
- AD Mutation: chromodomain helicase DNA-binding protein-7
Eisenmenger’s syndrome
- Pulmonary vascular disease caused by 1) cardiac defect with L–>R shunt, and 2) pulmonary build up reverses R–>L
- Leads to: pulmonary HTN, late central cyanosis, clubbing of nails, polycythemia
Noonan’s syndrome
- Cause: mutation in RAS-MAPK pathway signaling
- Sx: congenital heart defect (typically pulmonary valve stenosis; also atrial septal defect and hypertrophic cardiomyopathy), short stature, learning problems, pectus excavatum, impaired blood clotting, webbed neck and a flat nose bridge.
Interrupted aortic arch
- Abnormal loss of 4th aortic arch on both sides
- Sx: poorly oxygenated blood, patent ductus arteriosus, high O2 sat to UE but low O2 to LE
Double or Right aortic arch
- Can form a vascular ring around the trachea and esophagus
- Tx: ligate and divide
- Cause: coarctation, DiGeorge, CHARGE
What are possible causes of obstruction in the inferior vena cava? (4)
- Double inferior vena cava
- Absence of the inferior vena cava
- Left superior vena cava
- Double superior vena cava
