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Clin Med II MHM - Cardio 2 > Congenital Heart Disease > Flashcards

Congenital Heart Disease Flashcards

1
Q

What are the stages of human heart embryology?

A
  1. Cardiac crescent
  2. Linear heart tube
  3. Looping heart
  4. Chamber formation
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2
Q

By ___, two cardiac cords form and become canalized

A

3 weeks

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3
Q

What is the cardiac tube slouch?

A
  • Fetal development
  • Cardiac tube elongates and loops as an “S”
  • Bulboventricular portion moves right
  • Atrium and sinus venosus move posterior to ventricle
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4
Q

What is the primitive atrium first divided by?

A

Septum primum membrane

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5
Q

In fetal development, left and right ventricles develop:

A

Side by side

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6
Q

How does the bulbocordis divide?

A
  • Subaortic portion (as muscular conus resorbs)

- Subpulmonary section (elongation of its muscular conus)

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7
Q

What does the spiral division of the common truncus arteriosus rotate and align?

A
  • Aortic valve posterior over LV outflow tract

- Pulm valve anterior over RV outflow tract

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8
Q

How does the AV canal form?

A
  • Endocardial cushions develop in dorsal and ventral walls of the heart in the AV canal region
  • Grow toward each other and fuse, dividing AV canal into R and L orifices
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9
Q

In fetal development, how are the venous systems initially?

A
  • Bilateral and symmetric

- Enter 2 horns of sinus venosus

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10
Q

Truncus arteriosus and aortic sac initially develop:

A

6 paired symmetric arches which curve posteriorly and become paired dorsal aortae

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11
Q

How and when do vascular rings and slings present?

A
  • Infancy
  • Stridor, wheeze, croupy cough
  • Usually worse in supine position
  • More severe with double aortic arch
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12
Q

What do vascular rings arise from?

A
  • Double aortic arch
  • Right aortic arch w/left ligamentum arteriosum
  • Patent ductus arteriosus
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13
Q

What is a vascular sling?

A

Left PA branches off right PA

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14
Q

How are vascular rings and slings diagnosed?

A

Barium swallow (showing esophageal compression)

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15
Q

How are vascular rings and slings treated?

A

Surgically corrected

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16
Q

What are the types of simple adult congenital heart disease?

A
  • Native disease

- Repaired conditions

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17
Q

What are the main examples of intermediate complexity adult congenital heart disease?

A
  • ASD
  • AV canal defects
  • Coarctation of aorta
  • Patent ductus arteriosus
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18
Q

What are the main examples of complex adult congenital heart disease?

A
  • Tetralogy of Fallot
  • Transposition of great arteries
  • Truncus arteriosus
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19
Q

What is the Still’s murmur and what congenital disease does it indicate?

A
  • Vibratory, left midsternal border

- VSD, subaortic stenosis, subpulmonic stenosis

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20
Q

What is a venous hum and what congenital disease does it indicate?

A
  • Continuous murmur at neck and under clavicles
  • Loudest when sitting
  • PDA, coronary AV malformation
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21
Q

What are the types of ASD?

A
  • Sinus venosus
  • Ostium primum
  • Ostium secundum
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22
Q

What is a sinus venosus ASD?

A
  • High in atrial septum near SVC

- A/w anomalous PV connection from R lung to SVC or RA

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23
Q

What is ostium primum ASD?

A
  • Adjacent to AV valves which may be deformed/regurgitant

- Common in Down syndrome

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24
Q

What is ostium secundum ASD?

A

-Involves fossa ovalis and is midseptal (as opposed to PFO)

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25
Q

How does ASD affect heart physiology in adults?

A
  • L to R shunt

- Creates diastolic overloading of RV, increased pulm blood flow

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26
Q

What determines the magnitude of L-R shunt in adult ASD?

A
  • ASD size
  • Ventricular diastolic properties
  • Impedance in pulm and systemic circulations
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27
Q

What are the S/S of ASD in adults?

A
  • Usually asymp in early life

- Past the 4th decade of life: atrial arrhythmias, PA HTN, bidirectional and then RL shunt of blood, R heart failure

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28
Q

What does ASD show on physical exam?

A
  • Prominent RV impulse and palpable PA pulsation
  • S1 normal or split
  • S2 widely split and relatively fixed
  • Mid diastolic rumbling murmur
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29
Q

What does an ostium secundum ASD show on EKG?

A
  • R axis deviation

- rSr’ pattern in right precordial leads

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30
Q

How does an ASD look on CXR?

A
  • Enlarged RA, RV, PA

- Increased pulm vascular markings of LR shunt

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31
Q

What is the surgical treatment of ASD?

A
  • Patch of pericardium or prosthetic material

- Percutaneous transcatheter device closure

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32
Q

What is the medical management of ASD?

A
  • Prompt tx of resp. tract infections
  • Antiarrhythmics for AF or SVT
  • Tx of HTN, CAD, CHF
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33
Q

What is the MC of all cardiac birth defects?

A

Ventricular septal defect (VSD) either as an isolated defect or component of a combo of anomalies

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34
Q

How does VSD present in adults?

A
  • Depends on size and status of pulm vascular bed
  • Only small to moderate size are first seen in adults
  • Most w/isolated large VSD come to medical attention early in life
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35
Q

When is spontaneous closure of a VSD more common?

A

In patients born with a small VSD

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36
Q

What determines the clinical manifestations and feasibility of repair in VSD?

A

Status of pulmonary vascular bed

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37
Q

What is Eisenmenger’s syndrome?

A
  • Large communication b/w 2 circulations at aortopulm, ventricular, or atrial levels
  • Bidirectional or RL shunts bc of high resistance and obstructive pulm HTN
38
Q

When should large VSDs be corrected?

A

Early in life when pulm vascular disease is still reversible or not yet developed

39
Q

What are the symptoms in adults with Eisenmenger’s syndrome?

A
  • DOE
  • Chest pain
  • Syncope
  • Hemoptysis
40
Q

What predicts a good surgical outcome in Eisenmenger syndrome?

A

Pulm vasc resistance less than 1/3 of systemic

41
Q

How does a resolving VSD present on physical exam?

A

Asymp murmur may not develop until 1st few months of life as VSD constricts and then disappears

42
Q

How does a restrictive VSD present on physical exam?

A

Holosystolic murmur with normal S2

43
Q

How does a muscular VSD present on physical exam?

A

Short systolic not extending to S2 (small defect closes as muscle contracts)

44
Q

Surgery is NOT recommended for which patients with VSD?

A

Normal PA pressures with small shunts

*Pulm to systemic flow ratios of less than 1.5:1 to 2:1

45
Q

Surgery is indicated in VSD with pulm to systemic flow ratios of:

A

greater than 1.5:1 or 2:1

46
Q

Which congenital heart defect is pregnancy not recommended?

A

VSD - puts both mother and fetus at risk

47
Q

Why are oral contraceptives often contraindicated in cyanotic women with VSD?

A

Enhanced risk of vascular thrombosis

48
Q

Describe patent ductus arteriosus

A
  • Ductus arteriosus is a vessel leading from bifur of PA to aorta
  • Normally it is open in fetus but closes immediately after birth
  • PDA is when it remains open
49
Q

How does PDA present in most adults?

A
  • Pulm pressures normal
  • A gradient and shunt from aorta to PA persists through cardiac cycle
  • Characteristic thrill and continuous “machinery” murmur
50
Q

What are the leading causes of death in adults with PDA?

A
  • Cardiac failure
  • Infective endocarditis
  • Pulm vascular obstruction
51
Q

When is closure recommended for adults with PDA?

A

Without severe pulm vascular disease and RL shunt

52
Q

Describe congenital aneurysm of aortic sinus of Valsalva with fistula

A
  • Separation or lack of fusion b/w media of aorta and annulus of aortic valve
  • Rupture usually occurs 3-4th decades of life
53
Q

What is the MC fistula rupture in congenital aneurysm of aortic sinus of Valsalva?

A

Fistula between R coronary cusp and R ventricle

54
Q

What is a coronary ateriovenous fistula?

A
  • Communication between a coronary artery and another cardiac chamber
  • Shunt is usually small and myocardial BF NOT compromised
55
Q

What commonly occurs within the first year of anomalous origin of LCA from PA?

A

MI and fibrosis leading to death

56
Q

How is an anomalous origin of LCA from PA corrected surgically?

A

CABG

57
Q

How do bicuspid aortic valves present?

A
  • Not always detectable
  • Harsh systolic murmur
  • Males
  • Stenosis or regurge with time
  • Dilation of ascending aorta over time
58
Q

What is supravalvular aortic stenosis?

A
  • Narrowing of ascending aorta just above level of coronaries
  • Coronary arteries subjected to elevated systolic pressures
59
Q

How is a supravalvular aortic stenosis caused?

A

-Most have genetic defect (chromosome 7)

60
Q

What is Williams-Beuren syndrome?

A
  • A/w supravalvular aortic stenosis
  • “Elfin” facies, low nasal bridge
  • Mental retardation w/retained language skills
61
Q

What is coarctation of aorta?

A
  • Narrowing of aortic lumen, MC distal to origin of L subclavian artery
  • MC males
  • Turner’s syndrome pts
62
Q

What is the main determinant of clinical course and symptoms in pulmonary stenosis?

A

Severity of obstructing lesion (NOT site)

63
Q

Describe Tetralogy of Fallot

A
  1. Malaligned VSD
  2. RV outflow obstruction
  3. Overriding aortic (over VSD)
  4. RVH
64
Q

What determines the clinical presentation of Tetralogy of Fallot?

A

Severity of RV outflow obstruction

65
Q

What is a Tet Spell?

A

Children with Tetralogy of Fallot exhibit bluish skin during episodes of crying or feeding

66
Q

How do you treat a Tet Spell?

A
  • Increase SVR
  • Increase R filling pressure
  • Reduce catecholamine release
  • Supplemental O2
67
Q

What shows on CXR with Tetralogy of Fallot?

A

“Boot shaped” heart due to RV hypertrophy

68
Q

What is the MC cause of reoperation in adults with Tetralogy of Fallot?

A

Severe pulm regurge

69
Q

Describe complete transposition of great arteries

A
  • Dextro-transposition
  • Aorta rises R anteriorly from RV
  • PA emerges L posteriorly from LV
  • MC in males
70
Q

What does complete transposition of arteries result in?

A

2 separate parallel circulations

71
Q

How is the clinical course determined in complete transposition of great arteries?

A
  • Degree of tissue hypoxemia
  • Ability of each ventricle to sustain an increased workload w/reduced coronary arterial oxygenation
  • Associated CV anomalies
  • Status of pulm vasc bed
72
Q

How is intra-atrial switch repaired?

A

Mustard operation (rearranging venous returns)

73
Q

What is the Mustard operation?

A
  • Intra-atrial switch

- Rearranging venous returns

74
Q

What is the Jantene procedure?

A
  • Arterial switch
  • Transpose both coronaries to PA
  • Transect, contrapose, anastomose aorta and PA
75
Q

What is the Rastelli procedure?

A
  • VSD w/severely obstructed LV outflow tract

- PA is replaced

76
Q

What is total anomalous venous return?

A

Oxygenated blood returning from lungs is routed back into SVC rather than LA
*ASD must be present for this to happen

77
Q

What must be present for a total anomalous venous return to occur?

A

ASD

78
Q

What is tricuspid atresia and its associated lesions?

A
  • Absence of tricuspid valve
  • Interatrial communication
  • Hypoplasia of RV and PA
79
Q

What is the Fontan procedure?

A
  • To treat tricuspid atresia

- Direct RA to PA anastomosis

80
Q

What is Ebstein’s anomaly?

A
  • Downward displacement of tricuspid valve into RV
  • Anomalous attachment of tricuspid leaflets
  • Tricuspid regurge
  • RV often hypoplastic
81
Q

What is situs inversus totalis?

A

Reversal of organs (CXR looks backwards)

82
Q

What is Kartagener’s syndrome?

A
  • Autosomal recessive
  • Abnormal ciliary motion
  • Impaired clearance
  • Situs inversus
83
Q

How does Kartagener’s syndrome present?

A
  • Complete situs inversus
  • Chronic sinusitis
  • Bronchiectasis
  • Telecanthus (widened interpupillary distance)
84
Q

What is dextrocardia?

A

Cardiac apex positioned to R side of chest

85
Q

What is mesocardia?

A

Cardiac apex positioned in midline of chest

86
Q

What is isolated levocardia?

A

Cardiac apex positioned on L side of chest but abnormal position of viscera

87
Q

What is ectopia cordis?

A
  • Rare congenital defect
  • Herniation of the heart through sternal, pericardial and/or abdoinal defect
  • Most don’t survive
88
Q

What is Pentalogy of Cantrell?

A
  1. Defects in diaphragm
  2. Midline supra-umbilical abdominal wall defect
  3. Pericardium hear
  4. Lower sternum
  5. Congenital intracardiac abnormalities
    * A/w ectopia cordis
89
Q

What is the only surgical repair of a congenital defect that doesn’t usually leave behind some abnormality?

A

Ligation of an uncomplicated PDA

90
Q

What do cardiac operations involving the atria typically cause later in life?

A 
Sinus or AV node dysfunction
Atrial arrhythmias (esp. A flutter)

Clin Med II MHM - Cardio 2 (12 decks)

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