Cardio Final Exam Flashcards

1
Q

L –> R Shunts

A

(Late Cyanotic)

ASD
VSD
AVSD
PDA

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2
Q

R –> L Shunts

A

Early Cyanotic

Tetralogy
TGA
Truncus
TV Atresia
TAPVR
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3
Q

Atrial Septal Defect

- what are the 3 types?

A

3 types:

  1. Secundum = (90%) at fossa ovalis
  2. Primum = (5%) adjacent to AV valves
  3. Sinus Venosus = (5%) near SVC entrance
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4
Q

What are some characteristics of Patent Foramen Ovale (vs ASD)?

A
  • common = present in 1/3 of people
  • small remnant opening
  • no shunting
  • associated with paradoxical emboli, decompression sickness, migraines
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5
Q

Features of Ventricular Septal Defect

  • most occur where?
  • how common?
A

90% occur at membranous septum

  • most common congenital anomaly
  • 70% assoc with other anomalies
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6
Q

Muscular VSD

  • size?
  • does it close?
  • treatment?
A

– Defect is usually small
– Spontaneous closure by fibrous adhesions occurs in >60% of cases by 1 year of age
– Most do not need surgery
– Multiple muscular VSDs = “swiss cheese septum”

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7
Q

Perimembranous VSD

  • size?
  • does it close?
  • treatment?
A

– Defect is usually large
– Spontaneous closure by septal TV leaflet occurs in <10% of cases
– Requires surgical closure, usually around 1 year of age

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8
Q

Patent Ductus Arteriosus

  • features of normal closure
  • PE finding (upon auscultation)
  • other features
A

• Normal ductal closure
– Functional (~12 h)
– Structural (~3 mo)
– Delayed by prostaglandin E
– Closes later in preemies and at high altitude
• Classic physical exam finding
– Harsh, continuous, “machinery-like” murmur
• Other features
– Usually seen in isolation (90%)
– Necessary for survival in AV or PV atresia, others

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9
Q

Atrioventricular Septal Defect

  • definition
  • types
  • other features
A
• Definition
– Deficient AV septum, associated with MV and TV anomalies
• Two Types
– Partial:primumASD&cleftMV with MR
– Complete: AVSD & common AV valve (5 leaflets)
• OtherFeatures
– Down syndrome (40%) with complete AVS
– Needsearlysurgicalcorrection
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10
Q

Tetralogy of fallot

-definition and characteristics

A

• Most common form of cyanotic congenital heart disease
• Anteriosuperior displacement of the infundibular septum leads to
– Ventricular septal defect
– Subpulmonary stenosis
– Overriding aorta
– Right ventricular hypertrophy
• Clinical outcome depends on severity of subpulmonary stenosis.

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11
Q

Tetralogy of Fallot

-anatomy and physiology, PE findings

A
  • Heart may appear boot-shaped due to right ventricular hypertrophy
  • R-L shunt does not damage lungs because subpulmonary stenosis restricts pulmonary blood flow
  • Pulmonary outlet does not grow with child, so effects are worse with age
  • Surgery required
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12
Q

Transposition of the great arteries

  • Definition
  • Sequelae
  • Types
A

• Definition
– Aorta arises from RV
– Pulmonary artery arises from LV
• Sequelae
–Aorta lies anterior and to the right of the pulmonary artery
– Separate pulmonary and systemic circulations
–Right ventricular hypertrophy develops
– Pulmonary hypertension develops unless pulmonary stenosis is present
• Two types
– Intact ventricular septum (65%): unstable, needs prompt surgical intervention
– With VSD (35%): stable

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13
Q

Truncus arteriosus

  • definition
  • etiology
  • clinical sequelae
A

• Definition
– Origin of aorta & pulmonary artery from truncal artery
– Most have large VSD
• Etiology
– Developmental failure of separation of the embryologic truncus into the aorta and pulmonary artery.
• Clinical sequelae
– Mixing of blood
– Increased pulmonary blood flow & pulmonary HTN

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14
Q

Tricuspid atresia

A
  • Complete occlusion of the tricuspid valve orifice
  • Results from unequal division of the AV canal – mitral valve is enlarged
  • Needs coexisting ASD/PFO and VSD
  • Causes right ventricular hypoplasia
  • Symptomatic with high mortality
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15
Q

Total anomalous pulmonary venous return

A
  • Pulmonary veins do not directly drain into left atrium; left atrial hypoplasia
  • Instead, they connect via left innominate vein or coronary sinus
  • ASD/PFO allows oxygenated blood to enter systemic circulation
  • Occurs when common pulmonary vein fails to develop or regresses
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16
Q

Aortic coarctation

  • definition
  • types
  • clinical presentation
A
• Definition
– Constriction/narrowing of aorta
• Two types
– Preductal/infantile
– Tubular hypoplasia
with PDA
– Postductal/adult
– Ridgelike infolding at ligament without PDA
• Clinical presentation
– Bicuspid AV (50%) 
– Preductal
    – Lower body cyanosis
    – Requires surgery in neonatal period
– Postductal
    – Symptoms depend on degree of narrowing 
    – Surgically treatable HTN, upper > lower
    – Rib notching (CXR)
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17
Q

Pulmonary Stenosis

  • Definition
  • Isolated PV stenosis
  • other type of pathology…what is it? and describe?
A
• Definition
– Pulmonary valve obstruction due to hypoplasia, dysplasia, or abnormal number of cusps
• Isolated PV stenosis
– RV dilatation & hypertrophy
– Post-stenotic injury to PA
– May be asymptomatic until adulthood
• PV atresia with intact VS
– Hypoplastic RV and TV
– PDA needed to get blood to lungs
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18
Q

Aortic Stenosis

  • Definition
  • characteristics
A
• Definition
– Aortic valve obstruction due to hypoplasia, dysplasia, or abnormal number of cusps
• Isolated AV stenosis (80%)
– LV hypertrophy and LA dilatation
– AS may range from mild to critical
– Systolic murmur
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19
Q

AS- Hypoplastic Left Heart Syndrome

A

• Aortic valve atresia with intact VS
– Hypoplastic mitral valve and left ventricle
– Dependent on PDA for survival
– Requires staged surgical correction

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20
Q

Ebstein’s Anomaly

  • Definition
  • Secondary effects
  • Other Features
A
• Definition
– Inferiorly displaced and adherent septal and posterior leaflets
– Redundant anterior leaflet
– Dilated annulus with TR
• Secondary effects
– RV and RA dilatation
• Other features
– Arrhythmias, including WPW syndrome
– May be asymptomatic until adulthood
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21
Q

Morphology of HTN

  • Large/medium arteries
  • small arteries/arterioles
A
• Large/medium arteries
- Accelerated atherogenesis
- Degenerative changes in vascular walls 
- Increased risk of aortic dissection &
cerebrovascular hemorrhage
• Small arteries/arterioles
- Hyaline arteriolosclerosis
- Hyperplastic arteriolosclerosis
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22
Q

Hylaine Arteriolosclerosis

A

• Elderly patients
• Similar change in diabetics (microangiopathy)
• “Benign” nephrosclerosis
• Homogeneous pink, thickening of vessels with narrowing of lumen
▫ Leakage of plasma across endothelium due to HTN
▫ Excess matrix production by the smooth muscle cells occurs secondarily


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23
Q

Hyperplastic arteriolosclerosis

A

– Characteristic of malignant hypertension
– Onion-skinning, concentric laminated walls with luminal narrowing
• Due to reduplicated basement membrane and smooth muscle cells

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24
Q

Malignant HTN

A

– Necrotizing arteriolitis: term used when these changes are associated with fibrinoid necrosis

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25
Q

What is the morphology of Systemic HTN Heart Disease?

A

• Cardiomegaly: Concentric hypertrophy without dilatation, >1.5 cm wall thickness, 500 – 600 g.
• Thickness of left ventricular wall impairs diastolic filling and causes left atrial enlargement
• Myocyte hypertrophy
– Increased myocyte size & nuclear enlargement

26
Q

What are the clinical outcomes of systemic HTN Heart Disease?

A
• Normal longevity
• Progressive ischemic heart disease
– HTN potentiates ischemic heart disease 
• Progressive renal damage or stroke
• Progressive heart failure
• Sudden cardiac death
27
Q

What are 2 other organs that can be severely damaged in HTN heart disease?

A

Cerebral and Renal damage
1. Cerebral = • Cerebral vessels affected by arteriolosclerosis are weakened and more likely to rupture, causing intracerebral hemorrhage
• Lacunar infarcts
• Hypertensive
encephalopathy
- Headaches,confusion,
vomiting, convulsions
- Increased CSF pressure
2. Renal = • Benign hypertension
– Kidneys usually atrophic; granular, pitted surfaces
– Hyaline arteriolosclerosis of vessels results in ischemia and atrophy
– Glomeruli may become sclerosed
• Malignant hypertension
– Pinpoint petechial hemorrhages on surface
– Fibrinoid necrosis of arterioles
– Hyperplastic arteriolosclerosis and microthrombi lead to global ischemia

28
Q

What is Pulmonary HTN Heart Disease?

What is another term for it?

A

Another term = cor pulmnonale

Def:
• Right ventricular hypertrophy and/or dilatation and failure secondary to pulmonary hypertension
• Causes & morphology:
– Acute: massive pulmonary embolism
• Dilatation of right ventricle without hypertrophy
– Chronic: primary pulmonary hypertension or secondary
pulmonary hypertension due to chronic lung diseases
• Right ventricular hypertrophy, up to 1 cm in thickness, secondary to pressure overload
• Obstruction of pulmonary arteries/arterioles/septal capillaries

29
Q

What is Congestive Heart Failure?

A

• Inability of the heart to pump blood at a rate to meet the needs of active tissues
– Or can do so only from an elevated filling pressure.

30
Q

What is the prognosis of CHF?

A

• Common & recurrent condition with a poor prognosis
– Leading discharge diagnosis in hospitalized patients over 65 years
– 1 million hospital admissions, 50,000 deaths/year
– Increasing prevalence
– Symptomatic HF - one year mortality – 45%

31
Q

What is the pathogenesis of CHF?

- include specific mechanisms

A

Usually results from a slowly developing intrinsic deficit in contraction, (but occasionally occurs acutely)
- Mechanisms:
◦ Abnormal load presented to heart
- Acutely: fluid overload, MI, valve dysfunction
- Chronically: ischemic heart disease, dilated cardiomyopathy,
hypertension
◦ Impaired ventricular filling
- Acutely: pericarditis or tamponade
- Chronically: restrictive cardiomyopathy, severe left ventricular
hypertrophy
◦ Obstruction due to valve stenosis
 - Chronically: rheumatic valve disease (usually mitral valve)

32
Q

How do we characterize CHF by systolic and diastolic function?

A
– Systolic dysfunction: progressive deterioration of cardiac (contractile) function
• Ischemic heart disease
• Pressure or volume overload 
• Dilated cardiomyopathy
– Diastolic dysfunction: inability of heart to relax, expand, and fill sufficiently during diastole
• Massive left ventricular hypertrophy 
• Amyloidosis
• Myocardial fibrosis
• Constrictive pericarditis
33
Q

What does the primitive heart tube consist of?

A

the Primitive Left Ventricle and the Arterial and Venous poles

34
Q

What are features of the secondary heart field?

A
  1. Primitive Right Ventricle (bulbus cordis)
  2. Outflow region
  3. (common) Primordial Atrium
  4. AV Canal
  5. Sinus Venosus
35
Q

Connections at the Venous pole in heart devo

A
  1. Umbilical Veins
  2. Vitelline Veins
  3. Common Cardinal Veins
36
Q

Connections at the arterial pole in heart devo

A

pharyngeal (aortic) arch arteries originating from the aortic sac

37
Q

3 shunts in fetal circulation

A
  • Ductus venosus
  • ductus arteriosus
  • Oval Foramen
38
Q

Pressure in the ______ side of the heart is highest during development

A

RIGHT

39
Q

What are the compensatory mechanisms in CHF?

A
  • Rapid = increase preload dilation (attempt to sustain cardiac performance), activation of neurohumoral systems (release of NE, activated Renin-angiotensin-aldosterone, release of atrial natriuretic peptide)
  • Cardiac Hypertrophy (inc sarcomeres, not myocytes; weeks to months; extent varies with underlying cause)
40
Q

Patterns of hypertrophy:

pressure overload vs volume overload

A

Pressure overload = concentric hypertrophy
- HTN & aortic stenosis

Volume overload = hypertrophy with dilation
- mitral or aortic regurg

41
Q

Signs of cardiac hypertrophy leading to cardiac failure

A
  1. Increased myocyte size results in decreased capillary density, increased intercapillary distance and increased fibrous tissue
  2. Higher cardiac oxygen consumption
  3. Altered gene expression and proteins
  4. Loss of myocytes due to apoptosis
42
Q

Causes of Left Sided HF

A
– Ischemic heart disease
– Hypertension
– Aortic and mitral valve diseases (insufficiencies)
– Non-ischemic myocardial diseases
• Cardiomyopathies 
• Myocarditis
43
Q

Cascade of events the lead to Left-sided HF

A

Secondary enlargement of left atrium –> atrial fibrillation –> stagnant blood in atrium –> thrombus, embolic stroke

44
Q

Left-sided heart failure: effect on kidneys and brain

A

Kidneys:

  • decreased perfusion causes R-A-A system activation –> inc BV
  • if perfusion deficit is severe –> prerenal azotemia (= impaired kidney function due to low perfusion)
45
Q

Causes of Right Sided HF

A
  1. Secondary to left-sided failure, usually
  2. Pulmonary hypertension
  3. Primary myocardial disease
  4. Tricuspid or pulmonary valvular disease
46
Q

Right sided HF Effects:

  • heart
  • liver
  • kidneys
  • brain
A

 Heart:
◦ Right ventricle responds to the increased workload with
hypertrophy and often dilatation
 Liver and portal system:
◦ Elevated pressure in the portal vein leads to congestive
hepatosplenomegaly, cardiac cirrhosis, ascites
 Kidneys:
◦ congestion, fluid retention, peripheral edema, azotemia (more
marked with right heart failure than left)
 Brain:
◦ venous congestion and hypoxic encephalopathy

47
Q

Clinical signs of Right Sided HF

A
  1. Pleural and pericardial effusion, atelectasis
  2. Peripheral edema
    ◦ At ankle (pedal)
    ◦ Presacral
  3. Eventual anasarca (generalized massive edema)
48
Q

Types of vasculitis by vessel size:

  • large
  • medium
  • small
A

Large = giant cell, Takayasu
Medium = polyarteritis nodosa (complexes), kawasaki (antibodies)
Small = no asthma/granulomas-microscopic polyangiitis;
Yes grans / no asthma - wegner
Yes to both - churg/strauss

49
Q

What are the inherited CV diseases? (5)

A
  1. Arrhythmias (LQTS, Brugada synrome, familialr a-fib, CPVT)
  2. Cardiomyopathies
  3. Aneurysm sydromes
  4. Familial Hypercholesterolemia
  5. Pulmonary Arterial HTN
50
Q

Romano-Ward syndrome (RWS)

  • what is it?
  • how is it inherited?
A

• Primary electrophysiologic disorder due to ion channel abnormalities
• Autosomal dominant disorder
– Reduced penetrance: 50% of individuals with a disease-causing
mutation will not show symptoms
– Pleiotropy: 10 genes known to be associated with RWS

51
Q

Treatment and management for LQTS

A

• Beta blockers
– Consider non-compliance • Pacemakers
• Access to defibrillators
• Implantable cardioverter-defibrilators (ICDs) for individuals resistant to medications

52
Q

Jervell and Lange-Nielson syndrome

  • what is it?
  • how is it inherited?
A

• Congenital, profound, bilateral sensorineural
deafness and QT prolongation often >500 ms
– increased risk for SIDS
– >50% of untreated children with JLNS die prior to age 15
• Autosomal recessive inheritance
– 2 genes known to be associated with JLNS
• The most common genes include: – KCNQ1 (LQTS Type 1) 90%
– KCNE1 (LQTS Type 5) 10%

53
Q

Brugada Syndrome

  • prevalance
  • what is it?
  • how does it present?
A
  • Prevalence: 1:2,000
  • Inherited Arrhythmia: Cardiac conduction abnormalities ST-segment elevation in the right precordial leads (V1-V3), high risk for ventricular arrhythmias
  • Presents as syncope, SIDS or sudden unexpected nocturnal death syndrome (SUNDS)
54
Q

Catecholaminergic polymorphic ventricular tachycardia (CPVT)

  • prevalance
  • what is it?
  • how does it present?
  • genes associated?
A
  • Prevalence: 1 in 10,000
  • Distinct bidirectional or polymorphic ventricular tachycardia that can degenerate into ventricular fibrillation and cause sudden death
  • Syncope caused by exercise or acute emotion in an individual without structural heart disease
  • Genes: RYR2, CASQ2
55
Q

Management of Cardiomyopathies

A
• Pharmacologic therapy – ACE inhibitors
– Diuretics
• Lifestyle modifications
– Avoiding competitive sports – Healthy lifestyle
• Mechanical devices: pacemakers, ICD
• Surgical intervention
– Septal myectomy
– Alcohol ablation
• Cardiac transplantation
56
Q

Genetics of Familial Hypercholesterolemia

- 3 genes assoc with it and %

A
LDL-R = 60-80%
APOB = 1-5%
PCSK9 = <3%
57
Q

what is Atrial Natriuretic Peptide?

A
  • released from atria in response to increased Blood Volume and atrial pressure
  • causes generalized vascular relaxation
  • constricts renal efferent arterioles, dilates afferent arterioles (via cGMP mediated mech), thus promotes diuresis
58
Q

Baroreceptors:

  • what nerve projects from aortic arch? what does it sense?
  • what nerve projects from carotid sinus? what does it sense?
A

Aortic arch = vagus nerve; less sensitive, only senses increases in BP

Carotid sinus = glossopharyngeal nerve; more sensitive/influential, senses increase and decreases in BP

59
Q
Evolution of an MI: what occurs at each time point?
0-4 hrs
4-12 hrs
12-24 hrs
2-4 days
5-10 days
7 wks
A

0-4 hrs = nothing
4-12 hrs = wavy fibers, early coag necrosis, edema, hemorrhage
12-24 hrs = neutrophil emigration, release of necrotic cell content into blood, contraction bands
2-4 days = neutrophils, extensive coag necrosis, tissue around infarct shows acute inflammation
5-10 days = granulation tissue; at risk for free wall rupture, tamponade, pap-muscle rupture, VSD rupture
7 wks = contracted scar complete

60
Q

Genetic Associations of Coarctation types:

  • infantile:
  • adult:
A
  • infantile = Turner’s

- Adult = Bicuspid Aortic Valve

61
Q

Kaposi Sarcoma:

  • what is it assoc with?
  • How to treat 3 specific populations:
    1. Eastern European Men
    2. AIDS pts
    3. Transplant recipients/immunosuppressed
A

Assoc with HHV-8

Treatments:

  1. East European Men = tumor localized to skin, surgical removal
  2. AIDS pts = tumor spreads early, tx: antiretroviral agents-which allow immune system so recover and naturally kill the virus (HHV-8) thus curing disease
  3. Transplant pts = tumor spreads early; tx: decrease immune suppression (allows same mech as for AIDS pts)
62
Q

Angiosarcomas (specifically of the liver) are associated with what?

A

Thorotrast, arsenic, PVC