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Block C - Circulation > Cardiology Lecture 5 -- Congenital Heart Disease > Flashcards

Cardiology Lecture 5 -- Congenital Heart Disease Flashcards

1
Q

7 acyanotic congenital heart defects

A
  • Atrial septal defects (ASD)
  • Ventricular septal defects (VSD)
  • Patent ductus arteriosus (PDA)
  • Congenital aortic stenosis (AS); Bicuspid aortic valve (BAV)
  • Pulmonary stenosis (PS)
  • Coarctation of the aorta
  • Congentially corected transposition of the Great Arteries (cc-TGA)
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2
Q

3 cyanotic congential heart defects

A
  • Tetralogy of Fallot
  • Complete transposition of the great arteries (D-TGA)
  • Eisenmenger Syndrome
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3
Q

O2 sat associated with cyanosis

A

80-85%

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4
Q

What causes cyanotic heart disease?

A

Defects that allow a R –> L shunt (poorly oxygenated blood goes from right side of heart to left, bypassing lungs)

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5
Q

Color of acyanosis

A

Pink

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6
Q

3 general condiitions included in acyanotic lesions

A

Intracardiac or vascular stenoses

Valvular regurgitation

L –> R shunts

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7
Q

When can acyanotic heart disease become cyanotic?

A

LARGE, uncorrected, longstanding L –> R shunts: Eisenmenger Syndrome

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8
Q

Cause of pulmonary arterial hypertension

A

Large L –> R shunts (by unknown mechanism)

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9
Q

Define the findings of pulmonary arterial hypertension

A

Hypertrophy of pulmonary arteriolar media

Intimal proliferation

Decreased cross-sectional area of the pulmonary vascular bed

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10
Q

Effect of decreased cross-sectional area of pulmonary vascular bed (i.e. in pulmonary arterial hypertension)

A
  • Increased resistance to blood flow
  • Vessel thrombosis Increased PVR
  • Decreased L –> R shunt
  • PVR > 2/3 SVR and shunt reverses (Eisenmenger)
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11
Q

Define Eisenmenger syndrome

A

Condition of severe irreversible pulmonary vascular obstruction that results from reversal of a large chronic left-to-right shunt to right-to-left with systemic cyanosis when PVR > 2/3 SVR

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12
Q

2 types of ASD

A

Secundum (2º ASD)

Primum (1º ASD)

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13
Q

What is 1º ASD associated with?

A

Endocardial cushion defects (AVCD)

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14
Q

2 conditions that are not true ASD’s

A

Sinus Venosus Defect (superior and inferior)

Patent Foramen Ovale (PFO)

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15
Q

Common locations of congenital shunts

A
  • Ductus arteriosis
  • Foramen ovale
  • Ductus venosus
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16
Q

Describe the atrial septum formation at 30 days

A

Septum primum extends downwards through the ostium primum towards the endocardial cushion

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17
Q

Describe the atrial septum formation at 33 days

A

Septum primum splits, the perforation through which is called the ostium secundum. The septum secundum extends downwards to the right of the septum primum’s upper portion

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18
Q

Describe the atrial septum formation at 37 days to birth

A

Bottom portion of the septum secundum from the endocardial cushion extends upwards to eventually form the foramen ovale, which is covered by the “flap valve” of the lower septum primum

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19
Q

Direction of atrial septal defect

A

LA –> RA

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20
Q

Effect of blood flow due to atrial septal defect on the heart chambers

A

Enlargement of the RA, RV and PA

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21
Q

Incidence of ASD

A

1 in 1500 live births

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22
Q

Where can ASD occur?

A

Anywhere along the interatrial septum (IAS) but most commonly in the area of the foramen ovale

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23
Q

Where does 2º (ostium secundum) ASD occur?

A

In the area of the foramen ovale

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24
Q

Most common type of ASD

A

Ostium secundum ASD

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25
Q

Causes of ostium secundum ASD (5)

A
  • Inadequate formation of septum secundum
  • Too much resorption of septum primum
  • A combination of the previous two
  • Sporadically (most common) OR Inherited:
    • Familial septal defect
    • Holt-Oram syndrome
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26
Q

Define partial ostium primum ASD

A

1º ASD usually with a cleft mitral valve

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27
Q

Define intermediate ostium primum ASD

A

1º ASD, VSD and 2 separate AV valves

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28
Q

Define complete ostium primum ASD

A

1º ASD, VSD and common AV valve

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29
Q

Location of ostium primum ASD

A

Inferior portion of the interatrial septum

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30
Q

Cause of ostium primum ASD

A

Failure of septum primum to fuse with the endocardial cushions

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31
Q

In what chromosomal disorder is ostium primum ASD often found?

A

Trisomy 21

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32
Q

Why isn’t a sinus venosus defect a true ASD?

A

Interatrial septum is intact (however, it is functionally identical to an ASD)

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33
Q

Cause of sinus venosus defect

A

Abormal development of the sinus venosus located postero-superior (superior defect) or, rarely, postero-inferior (inferior defect) to the oval fossa

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34
Q

What is sinus venosus defect often associated with?

A

Partial anomalous pulmonary venous return (PAPVR)

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35
Q

Define partial anomalous pulmonary venous return (PAPVR)

A

Anomalous connection of the right upper pulmonary vein (most commonly) often associated with a superior sinus venosus defect

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36
Q

ASD pathophysiological effects after birth

A
  • Increased RV compliance, decreased RV wall thickness
  • L –> R shunt across IAS
  • Volume overload = dilatation of RA and RV
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37
Q

ASD symptoms in infants

A

Asymptomatic

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38
Q

ASD symptoms in adults

A

Palpitations (atrial arrhythmias precipitated by RA enlargement)

Decreased exercise tolerance

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39
Q

Important ASD auscultation finding

A
  • Fixed split S2
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40
Q

Fixed split S2 manifestations on inspiration

A
  • Increased venous return to RA
  • Increased RAP
  • Decreased L –> R shunt
  • Non-shunted LA blood keeps LV volume constant
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41
Q

Fixed split S2 manifestations on expiration

A
  • Decreased venous return to RA
  • Decreased RAP
  • Increased L –> R shunt
  • Shunted blood keeps RV volume constant
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42
Q

EKG rhythm of ostium secundum ASD

A

Sinus

Atrial fib/flutter

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43
Q

EKG axis of both ASD’s

A
  • 2º ASD = right axis deviation
  • 1º ASD = left axis deviation
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44
Q

EKG conduction pattern for 2º ASD

A
  • Partial/complete right bundle branch block
  • Increased PR interval
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45
Q

2º ASD R wave behavior

A

Crochetage of R waves in II, III, AVF with rSr’ (bunny ears)

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46
Q

CXR manifestations of large ASD with pulmonary hypertension

A
  • RA and RV enlargement
  • Prominent pulmonary arteries
  • May or may not have increased vascular markings
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47
Q

Use of Cardiac Catheterization in ASD

A
  • Rarely required
  • Useful to evaluate pulmonary pressures/ vascular resistance
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48
Q

When is closure of an ASD necessary? (2)

A

If shunt is significant or unrestrictive:

  • Symptomatic patient: palpitations/ decreased exercise tolerance
  • Right sided chamber enlargement
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49
Q

Goals of closing the ASD

A

Prevent right-heart failure and irreversible pulmonary hypertension and improve/stop arrhythmias

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50
Q

How to close an ASD

A
  • Percutaneously with a device (preferred is feasible)
  • Surgically by direct suture closure
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51
Q

Define a patent foramen ovale

A

An unclosed foramen ovale where the septum primum “flap” is often firmly stuck to the septum secundum since LAP >> RAP

52
Q

Consequences of PFO

A
  • Usually of no clinical significance
  • Increased risk of paradoxical embolus (i.e. stroke)
  • Increased RAP –> straining, coughing, PHTM, RHF
  • Can precipitate a R –> L shunt
53
Q

Consequence of R –> L shunt due to PFO

A

Deoxygenated blood

Thrombus

Air (especially if patient is coughing)

54
Q

Recommended treatment to manage PFO

A

Air filters on IVs

55
Q

What activities are not recommended for PFO patients?

A

Scuba diving and space travel

56
Q

Incidence of ventricular septal defect (VSD)

A

1.5 - 3.5 per 1000 live births

57
Q

4 examples of types of VSD

A

Based on location in the interventricular septum (IVS)

  • Membranous (70%)
  • Muscular (20%)
  • Subarterial (below aortic/pulmonary valves)
  • AV septal VSD (adjacent to AV valves)
58
Q

Which VSD is common in Asians?

A

Subarterial VSD (below aortic/pulmonary valves)

59
Q

Degree if shunting and hemodynamic effects of VSD are related to:

A
  • Size of VSD
  • Pulmonary and systemic vascular resistances
60
Q

Heart sound produced by a small insignificant/restrictive VSD

A

Small hole = loud systolic murmur

61
Q

Consequence of large significant/unrestrictive VSD

A
  • Volume overload of RV, pulmonary circulation, LA and LV
  • Initially, SV is increased (Frank-Starling mech)
  • Left-sided structures will dilate with time –> systolic dysfunction –> heart failure
  • Pulmonary overcirculation –> pulmonary hypertension by age 2
62
Q

In what type of VSD is RV volume overload seen? Which types do you not typically see it?

A

Typically only occurs with muscular VSD

Rarely with perimembranous and subarterial VSD

63
Q

Name the clinical manifestation associated with small, restrictive VSD

A

Asymptomatic

64
Q

Name the clinical manifestatin associated with large, moderately restrictive VSD

A

Dyspnea

65
Q

Name the clinical manifestation associated with large, non-restrictive VSD

A

Eisenmenger

66
Q

Heart failure symptoms in infants with large unrestrictive VSDs

A
  • Tachypnea
  • Poor feeding
  • Failure to thrive (FTT)
  • Frequent LRTI (lower resp. tract infection)
67
Q

VSD symptoms if Eisenmenger

A

Dyspnea

Cyanosis

68
Q

VSD palpation findings

A

Thrill

69
Q

VSD auscultation findings

A

If restrictive:

  • Harsh, high frequency holosystolic murmur
  • Grade 3 - 4/6 at LLSB

If non-restrictive:

  • No murmur
70
Q

Small VSD CXR findings

A
  • Normal
  • With or without dilated proximal PA’s
71
Q

Moderate VSD CXR findings

A

Central PA enlargement

LV dilation

Vascular markings

72
Q

Large VSD CXR findings

A

Central PA enlargement

Peripheral pruning

Olligemic lung fields

RV enlargement

73
Q

Effect of VSD on the heart chambers

A
  • Left atrial enlargement (LAE)
  • Left ventricular hypertrophy (LVH)
  • Right ventricular hypertrophy (RVH) if pulmonary vascular disease is present
74
Q

O2 saturation in R heart chambers in VSD

A

Increased O2 saturation in the RV compared to the RA

  • RA O2 sat = 70%
  • RV O2 sat = 92%
75
Q

VSD treatment options (4)

A
  • No treatment: 50% of small and moderate-sized VSD will partially or completely close by age 2
  • Surgical closure in infancy: Large VSD with associated heart failure or pulmonary vascular disease
  • Correction in later childhood/early adulthood: moderate VSD w/o pulmonary vascular disease but significant L –> R shunt
  • Catheter-based device closure: usually muscular VSD
76
Q

Define coarctation of the aorta

A

Discretely narrowed aortic lumen

77
Q

Incidence of aortic coarctation

A

1 in 6000 live births

78
Q

What is aortic coarctation associated with?

A
  • Bicuspid aortic valve
  • Turner’s syndrome (45, XO)
79
Q

Where does aortic coarctation usually occur?

A

Region of the ligamentum arteriosum

80
Q

3 types of aortic coarctation and which one is most common?

A

Post-ductal

Preductal

Juxta-ductal

81
Q

3 thoeries behind the cause of aortic coarctation

A
  • “No flow, no grow” = decreased antegrade flow through left heart and ascending aorta during fetal life –> aortic hypoplasia
  • Ectopic muscular tissue from ductus arteriosus extends into aorta during fetal life and constricts at the same time as the ductus closes
  • A manifestation of more diffuse aortic disease
82
Q

Pathophysiological changes due to aortic coarctation (3)

A
  • Increased LV afterload
  • Decreased blood flow in descending aorta and lower extremities
83
Q

Why is the blood flow to the head and upper extremities preserved in aortic coarctation?

A

Vessels branch off BEFORE the coarctation site

84
Q

Consequence of leaving aortic coarctation uncorrected (2)

A
  • LVH
  • Dilated collateral vessels formed from intercostal arteries to bypass coarctation and supply blood to descending aorta –> can erode surface of ribs (rib notching on CXR)
85
Q

Aortic coarctation symptoms in infants

A

Heart failure symptoms

Differential cyanosis if coexistent PDA

86
Q

Coarctation symptoms in older children and adults

A

Can be asymptomatic

Symptomatic:

  • Unexplained HTN or difficult to control HTN
  • Epistaxis
  • Headache
  • Leg weakness on exertion
  • Claudication
87
Q

Physical observational findings of coarctation in physical exam

A
  • Less developed lower body (narrow hips, short legs)
  • More developed upper body (broad shoulders, long arms)
  • Upper extremity hypertension
88
Q

Where to measure BP in coarctation patients

A

In both arms and one leg

89
Q

BP findings in coarctation

A
  • BP difference >30 mmHg between R and L arms if coarctation proximal to L subclavian
  • Systolic BP difference <10 mmHg between brachial and popliteal arteries
  • Diastolic BP equal in both UE and LE
90
Q

Palpation findings in coarctation physical exam

A
  • Diminished pulse in lower extremities
  • Radial/brachial-femoral delay
  • Precordium: LVP overload –> sustained apex
91
Q

Auscultation findings of coarctation

A
  • Bicuspid valve
  • Interscapular systolic murmur from coarctation site (more severe = longer murmur)
  • Anterior systolic murmurs from intercostal collateral arteries (interclavicular areas, sternal edge, axillae)
92
Q

Coarctation ECG

A

LVH with or without LAE is most common abnormality

93
Q

Coarctation CXR findings

A
  • “3 sign” prestenotic and poststenotic dilatation
  • Rib notching
    • On inferior ribs 3 - 8
    • From dilated intercostal arteries
    • Usually bilateral unless subclavian arises below coarctation
94
Q

Coarctation treatment for neonates

A

Prostaglandin infusion to keep ductus arteriosus patent

95
Q

Coarctation treatment for younger children

A
  • Elective repair often done to prevent hypertension
  • Types of surgery:
    • End-toend anastomosis
    • Subclavian flap aortoplasty
    • Arch augmentation
    • Interposed graft
96
Q

Coarctation treatment for older children, adults and those with recoarctation post repair

A
  • Transcatheter balloon dilatation +/- stent preferred
  • Surgery:
    • End-to-end anastomosis
    • Arch augmentation
    • Interposed graft
    • Jump graft bypassing coarct segment
97
Q

Most common form of cyanotic heart disease

A

Tetralogy of Fallot

98
Q

Incidence of Tetralogy of Fallot

A

5 in 10 000 live births

99
Q

Associated cardiac defects with TOF

A
  • Right aortic arch (25%)
  • ASD (10%) = pentalogy of Fallot
  • Anomalous left coronary anomaly (LAD comes off RCA and crosses over RVOT)
100
Q

15% of TOF cases are associated with what genetic disorder?

A

22q11 deletion (AD) (DiGeorge Syndrome, CATCH-22)

101
Q

Etiology of TOF

A

Abnormal anterior and cephalad displacement of the infundibular (outflow tract) portion of the IVS

102
Q

4 anomalies of the TOF

A
  • Malalignment VSD
  • Subvalvular +/- valvular pulmonary stenosis
  • RVH
  • Overriding aorta
103
Q

ToF pathophysiology (2)

A
  • Increased R by pulmonary stenosis –> increased deoxygenated blood shunting R –> L through VSD –> systemic hypoxemia and cyanosis
  • Magnitude of shunt flow across VSD = function of severity of pulmonary stenosis. Acute changes in systemic and pulmonary vascular R can also have effect
104
Q

Why doesn’t pulmonary hypertension occur in ToF?

A

Pulmonary stenosis protects against pulmonary overcirculation

105
Q

Define tet spell and associated symptoms (5)

A

Children have dyspnea on exertion

  • Irritability
  • Cyanosis
  • Hyperventilation
  • Syncope
  • Convulsions
106
Q

Cause of tet spells

A

After an activity that results in systemic vasodilation such as physical exertion, crying or feeding = increased R –> L shunt

107
Q

How children alleviate tet spell. Explain why this works

A

Squatting

  • Increased systemic vascular R by kinking the femoral arteries
  • Decrease R –> L shunt through VSD so blood will preferentially be directed towards lungs
108
Q

Inspection findings in ToF physical exam

A

Mild cyanosis (lips, mucus membranes, digits)

Digital clubbing

109
Q

ToF palpation findings

A

RV heave at LLSB

110
Q

Auscultation findings in ToF

A
  • Single S2 (P2 soft and inaudible)
  • SEM at LUSB
  • No VSD murmur
111
Q

What causes SEM at LUSB in ToF

A

Turbulent flow thruogh stenotic RVOT

112
Q

Why is there not VSD murmur in ToF?

A

VSD is large and non-restrictive

113
Q

ToF CXR findings

A
  • Boot-shaped heart (RV enlargement, decreased MPA segment)
  • Decreased pulmonary vascular markings
114
Q

ToF ECG findings

A

RVH and right axis deviation

115
Q

ToF Catheterization details the… (5)

A
  • RV outflow tract anatomy
  • Malaligned VSD
  • Aortic relationship with ventricles
  • RVH
  • Associated defects
116
Q

When is definitive repair of ToF usually done?

A

By 6 - 12 months

117
Q

3 general procedures that ToF surgical repair involves

A
  • VSD closure
  • Repair associated defects
  • Relieve RVOT obstruction
118
Q

7 ways to relieve RVOT obstruction

A
  • Resection of infundibular muscle
  • Transannular patch
  • RV subannular outflow patch
  • RV to PA conduit
  • Pulmonary valve replacement
  • Pulmonary valvotomy
  • Pulmonary arterioplasty
119
Q

ToF surgical repair procedures in the past (2)

A
  • Right ventriculotomy appraoch (increase incision)
  • Generous transannular patch augmentation
120
Q

ToF Surgical repair complications in adults due to procedures performed before

A

Severe free PR –> decreased RV function, arrhythmias, sudden cardiac death

121
Q

ToF surgical repair procedures today

A
  • Transatrial/transpulmonary approach
  • Limited RV incision for patch augmentation of the RVOT +/- PV annulus
122
Q

Goal of today’s approach to ToF surgical repair

A

Avoid PR at the expense of some residual PS

123
Q

Incidence of congenital heart disease in population

A

0.8%

124
Q

Frequent result of acynotic lesions on the dynamics of the ventricle(s)

A

Volume or pressure overload of the ventricles

125
Q

Consequence of large L –> R shunts

A

Pulmonary overcirculation –> PHTN

Shunt reversal and cyanosis = Eisenmenger Syndrome

Block C - Circulation (26 decks)

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