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Congenital Heart Disease

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Title: Congenital Heart Disease


1
Congenital Heart Disease 1 2005
Susan Etheridge, MD
2
Congenital Heart Disease
  • Objectives
  • Recognize that children can be born with heart
    problems
  • Recognize that heart disease in children has
    signs and symptoms that are different from adults
  • Identify congenital cardiac defects, presenting
    signs and symptoms
  • Application of general principles of cardiac
    physiology and pathophysiology to specific
    congenital heart lesions

3
Congenital Heart Disease (CHD)
  • Congenital - condition that is present at birth
  • 8-10/1000 live births
  • Increasing survival of children with CHD
    600,000 adults in US with CHD
  • Increasingly diagnosed in utero - may affect
    maternal management
  • Increasing complexity of the diagnoses of
    survivors

4
Risks in Adults with CHD
  • Stroke
  • Endocarditis
  • Heart failure
  • Pulmonary hypertension
  • Pregnancy complications
  • Arrhythmias (atrial fibrillation, atrial
    reentrant tachycardia, ventricular tachycardia,
    ventricular fibrillation)
  • Sinus node and AV node disease
  • Sudden death

5
Congenital Heart Disease Etiology
  • Multifactoral (most common)
  • Single gene mutations
  • Chromosome abnormalities
  • Environmental factors (alcohol, lithium)

DiGeorge 22q11
Trisomy 13
Trisomy 18
Trisomy 21
6
Classification
  • Chamber identity not be based on position
  • Chamber, vessel, valve can be anywhere
  • left ventricle can be on the right
  • Morphologic features of the chambers
  • and valves identify them
  • right ventricle heavily trabeculated
  • left ventricle smooth-walled
  • AV valves go with respective ventricles
  • the valve that empties into left ventricle is
    mitral valve

7
Congenital Heart Disease
  • May result in
  • no symptoms
  • heart failure
  • cyanosis

8
Heart Failure
  • Heart unable to pump sufficient blood to meet
    metabolic demands of body
  • Excessive work load on the heart
  • normal myocardium
  • volume load
  • pressure load
  • example congenital heart disease
  • Normal workload faced by damaged or abnormal
    myocardium
  • example myocarditis

9
Heart Failure Signs and Symptoms
  • due to
  • low cardiac output
  • systemic adaptation to low cardiac output
  • systemic and/or pulmonary venous congestion

10
Signs and Symptoms
  • Age-dependent
  • Fetus Hydrops
  • Infants
  • poor feeding, sweating with feeding, tachypnea,
    tachycardia, cool extremities, diminished pulses,
    mottling, hepatomegaly
  • Children (similar to adults)
  • exertional dyspnea, orthopnea, chronic cough,
    hepatomegaly, neck vein distention, peripheral
    edema

11
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12
Cyanosis and Hypoxemia
  • Hypoxemia - arterial saturation lt 90 in room air
  • respiratory causes (example pneumonia)
  • cardiac causes - shunting of blood from a right
    heart structure to a left heart structure
    (right-to-left shunt)
  • Cyanosis - bluish discoloration of skin
  • perceptible when gt 5 gm/dl of reduced hemoglobin
    in the capillaries

13
Infant with cyanosis
14
Cyanosis and Hypoxemia associated problems
  • Clubbing - widening and thickening of distal
    digits
  • occurs with hypoxemia
  • due to increased capillaries and increased blood
    flow through multiple arteriovenous aneurysms
  • increased connective tissue in terminal phalanges

15
Cyanosis and clubbing in a child with Tetralogy
of Fallot
16
Cyanosis and Hypoxemia associated problems
  • Polycythemia - increased total RBC in blood
  • low arterial O2 content acts via renal
    erythropoietin - stimulates bone marrow to
    increase RBC production increased O2 carrying
    capacity and O2 delivery
  • useful compensatory mechanism until hematocrit
    70-80 when blood becomes too viscous

17
Cyanosis and Hypoxemia associated problems
  • Squatting - posture assumed after exertion in
    children with some cyanotic congenital heart
    diseases
  • gt 1 - 2 year olds
  • increases O2 saturation by increasing venous
    return
  • increases peripheral resistance - decreases right
    to left shunt
  • not seen recently due to earlier repair of
    cyanotic infant
  • Other exercise intolerance, increased risk of
    brain abscesses and strokes

18
Classification of Congenital Heart Disease
19
Left to Right Shunt Lesions
  • Abnormal shunting of blood from systemic
    circulation to pulmonary circulation
  • Oxygenated blood returns to lungs - increases
    pulmonary blood flow
  • Ventricular Septal Defects, Atrial Septal
    Defects, Patent Ductus Arteriosus,
    Atrioventricular septal defect, Aorticopulmonary
    window

20
Ventricular Septal Defect (VSD)
  • Defect (hole) in a portion of septum that
    separates right and left ventricle
  • Most common congenital cardiac lesion
  • Location
  • perimembranous septum
  • muscular septum
  • inlet septum (AV canal type)
  • subarterial septum

21
Perimembranous VSD
Muscular VSD
22
Ventricular Septal Defect (VSD)
  • Defect size more important than location
  • Small to medium sized defects smaller size
    restricts amount of left to right shunting
  • Large defect nonrestrictive, no resistance to
    flow across the defect
  • Shunt direction and volume determined by relative
    resistances of systemic and pulmonary circuits

23
VSD Pathophysiology
  • Oxygenated blood crosses LV to RV
  • Red blood from LV mixes with blue blood in RV
    (therefore no cyanosis)
  • Increased blood in RV (well-tolerated)
  • Increased blood in pulmonary arterial circuit
  • Increased blood returning to left atrium (LA) and
    LV volume overload and LV- enlargement

24
Pathophysiology Large VSD
  • Magnitude and direction of shunt determined by
    relative resistances of systemic and pulmonary
    circuits
  • Usually LgtR
  • SVR - higher resistance - systemic arterioles
    have thick muscular wall, narrow lumen
  • PVR- lower resistance - pulmonary arterioles have
    a thin wall, wide lumen
  • Reflected by higher aortic pressure compared to
    pulmonary artery pressure and hence higher LV
    pressure than RV pressure

25
Pathophysiology Large VSD
Red blood crosses from LV to RV through
VSD Large defect allows equalization of LV and
RV pressures Increased blood in pulmonary
circuit Increased pressure and O2 saturation in
pulmonary artery
92
92/6
LV
99
92/6
RV
26
Pathophysiology Large VSD
Increased LA volume - increased blood returning
to the LA from the pulmonary circuit Increased
blood in the LV - LVE Increased LV radius -
myocardial fibers lengthen Significant LV
dilation - myocardium cannot develop sufficient
tension to maintain pressure Heart Failure
27
Large VSD
  • Two hemodynamic loads
  • Equalization of RV and LV pressure -Pressure load
    on - RV
  • Increased blood passes across the VSD into - RV
    and subsequently into pulmonary circuit Increased
    blood then returns to LA and LV - Volume load on
    the LV
  • This results in heart failure

28
Large VSD
  • Try to imagine situations where a large VSD may
    shunt in the right to left direction keeping in
    mind the PVR and SVR relationship

29
Development of Heart Failure with Large VSD
  • LV volume overload (increased preload)
  • ? LV dilation
  • ? increased LV radius (R)
  • ? as LV radius increases LV tension (T) must
    increase to maintain pressure (P) (LaPlace
    relationship T P X R)
  • ? continued LV dilation
  • ? myocardium cannot develop sufficient tension to
    maintain pressure/volume relationship
  • ? Heart Failure

30
Large VSD and Heart Failure
  • Compensatory mechanisms to maintain myocardial
    performance and cardiac output
  • Stimulation of sympathetic nervous system
    -increased catecholamine release
  • ? increased force of contraction
  • ? increased heart rate
  • myocardial hypertrophy

31
Effects of Large VSD and CHF
  • decreased EF
  • increased residual volume in LV after contraction
  • increased LV EDP (end diastolic pressure)
  • increased LA pressure
  • increased pulmonary venous pressure
  • increased alveoli fluid
  • poor gas exchange

32
Clinical Picture
  • History
  • Symptoms of heart failure often 2 - 3 months of
    age
  • tachypnea (rapid breathing)
  • increased work of breathing
  • poor feeding
  • diaphoresis (sweating) with feedings
  • recurrent respiratory infections

33
Clinical Picture
  • Why are there so many pulmonary symptoms in
    children with a large VSD?
  • Why are the children tachycardic and diaphoretic
    (sweaty)?

34
Clinical Picture
  • Examination
  • murmur - noise made by turbulent blood crossing
    hole in the ventricular septum - high frequency
    holosystolic murmur
  • heart sounds increased second heart sound (S2)
    due to increased pulmonary artery pressure
  • cardiac enlargement (left chest enlargement)
  • signs of failure - tachycardia, tachypnea,
    dyspnea, retractions, growth failure,
    hepatomegaly

35
Clinical Picture
  • CXR
  • usually normal at birth
  • cardiac enlargement develops with time
  • LA and LV enlargement
  • increased pulmonary blood flow (increased size of
    the pulmonary vessels)

36
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37
RV
LV
Echocardiogram of a large VSD
38
Clinical Picture
  • Management
  • anticongestive medications
  • diuretics
  • afterload reduction
  • digoxin
  • surgical closure
  • Large defects do not close spontaneously

39
Large VSD Question
  • You have been following a 2-month-old with a
    large VSD.
  • She is lost to follow-up and returns to clinic at
    age 8 years.
  • On examination, she no longer has the signs and
    symptoms of a left -to-right shunt.
  • Should you be pleased because the large VSD has
    closed spontaneously?
  • What happens to the pulmonary vasculature
    exposed to increased blood flow and systemic
    pressures for a long time?

40
Small VSD
Small VSD pressure restrictive - does not allow
equalization of LV and RV pressures Shunt is
left to right because LV systolic pressure
greater than RV systolic pressure pressure
restrictive Normal pulmonary artery pressures L
gt R shunt is small
41
Small VSD Clinical Picture
  • History
  • usually asymptomatic, no heart failure
  • murmur typically at first newborn outpatient
    evaluation
  • murmur usually not present at birth because of
    high pulmonary resistance at birth and high RV
    pressures therefore little shunting of blood
    across VSD
  • even small VSDs often have very loud murmurs

42
Small VSD Clinical Picture
  • Examination
  • usually no heart failure symptoms or cardiomegaly
  • often a loud murmur
  • Natural history/management
  • many become smaller or close spontaneously
  • 50 of perimembranous get smaller or close,
    usually within 6 months - 1 year
  • muscular defects commonly close especially in
    infants

43
Patent Ductus Arteriosus (PDA)
  • Persistence of fetal communication between the
    aorta and pulmonary artery
  • should close by 1st day of life
  • embryonic left 6th arch
  • More common in premature infants
  • Direction and magnitude of shunt through the PDA
    depends on relative SVR and PVR

44
LPA
RPA
Constricting PDA
Aorta
Main PA
RVOT
45
Large PDA
  • Aortic pressure pulmonary artery pressure
    (large defect allows equalization of pressures)
  • But systemic resistance remains higher than
    pulmonary resistance (SVR gt PVR)
  • L gt R shunt

46
Hemodynamics of Large PDA
Shunting of red blood from the aorta to pulmonary
artery Increased pulmonary blood flow Increased
pulmonary artery pressure and increased O2
saturation in pulmonary artery
99/22
91
103/40
99
47
Hemodynamics Large PDA
  • Low diastolic blood pressure
  • run-off into pulmonary artery (PA)
  • Increased blood in pulmonary circuit
  • increased blood returning to LA
  • increased LA pressure
  • Poor gas exchange
  • low O2 sat in pulmonary veins and LA
  • Increased blood in LV results in
  • LV enlargement and increased
  • LVEDP
    Heart Failure

48
Clinical Picture Large PDA
  • History
  • More common in females (21)
  • Associated with maternal rubella, high altitude,
    Down syndrome, prematurity
  • Present with asymptomatic murmur within days to
    weeks of birth
  • Large PDA heart failure symptoms (similar to
    VSD)

49
Clinical Picture - Large PDA
  • Examination
  • murmur noise made from continuous shunting of
    turbulent blood from aorta to PA
  • classic murmur - continuous machinery murmur
  • wide pulse pressure
  • low diastolic blood pressure
  • due to run-off of blood from Aorta to Pulmonary
    artery
  • bounding pulses-due to large difference btw
    systolic and diastolic pressure

50
Clinical Picture - Large PDA
  • CXR
  • increased pulmonary blood flow
  • cardiac enlargement
  • prominent aortic and pulmonary trunks
  • small PDA - normal CXR

51
Clinical Picture - Large PDA
  • Catheterization - only if planning coil occlusion
  • Management
  • ligation CHF
  • some small PDAs close spontaneously but usually
    not after age 1 year
  • ligation of large or moderate PDA to prevent
    pulmonary vascular disease or CHF
  • PDAs (even tiny PDA) are usually closed (after
    age 1 year or so) to prevent endocarditis
    (infection of the heart) - risk 0.45 per year

52
Ligation of PDA
53
Large PDA Coil Occlusion
54
Atrial Septal Defect (ASD)
  • Defect (hole) in septum btw the right and left
    atrium
  • usually in region of fossa ovalis
  • may be single or multiple
  • Location
  • Secundum (fossa ovalis) - most common
  • Sinus venosus (near SVC/RA junction) may be
    associated with anomalous venous drainage
  • Primum defect AV canal type defect
  • Location important because of associated defects

55
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ASD Locations
  • a. secundum
  • b. sinus venosus
  • c. primum

b
a
c
58
Hemodynamics ASD
  • Amount of shunt depends on relative RV and LV
    compliance not size or location of defect
  • RV more compliant than LV - direction of shunt is
    from least compliant toward the most compliant
    chamber
  • L gt R shunt - red blood from LA crosses ASD into
    right atrium (RA)
  • no cyanosis (acyanotic)

59
ASD in the Neonate
  • Neonates have decreased RV compliance immediately
    after birth
  • less L gt R shunt at birth
  • may even be R gt L shunt - cyanosis
  • RV compliance increases with age and L gt R shunt
    increases

60
Hemodynamics ASD
Red blood crosses from LA to RA Increased O2
saturation in RA and increased RA
volume Increased O2 saturation in RV and
increased RV volume Increased blood in pulmonary
circuit - normal PA pressures and resistance
100
93
61
Clinical Picture ASD
  • History
  • more common in females
  • cyanosis occurs (rarely) in neonates due to R gt L
    shunt due to decreased RV compliance
  • usually diagnosed in preschool and school aged
    children with a murmur
  • usually asymptomatic
  • heart failure and growth failure rare in children

62
Clinical Picture ASD
  • Adult unrepaired ASD patients
  • pulmonary vascular disease (gt 20 years) more
    common in females
  • atrial arrhythmias (atrial fibrillation, atrial
    flutter)

63
Clinical Picture ASD
  • Examination
  • RV precordial bulge (due to enlarged RV)
  • murmur- systolic ejection murmur due to increased
    blood flow across the pulmonary valve
  • murmur is not due shunting across the ASD, this
    is a low velocity shunt and does not make noise

64
Clinical Picture ASD
  • S2 (second heart sound) widely split - due to
    delayed emptying of volume-overloaded RV
  • fixed split - due to unlimited communication
    between the 2 atria
  • ASD allows for equalization of the influence of
    respiratory variation on RV and LV output

65
Clinical Picture ASD
  • CXR
  • increased pulmonary blood flow
  • prominent right heart border due to enlargement
    of RA and RV

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Natural History and Management
  • surgical / device closure at 3 - 5 years if L gt
    R shunting is proven (rarely close spontaneously
    after age 3 years)
  • prevents development of pulmonary vascular
    disease, late rhythm problems (atrial
    tachycardia)
  • unrepaired ASDs result in pulmonary vascular
    disease in 5 - 10 of patients
  • unrepaired there is a risk of paradoxical emboli
    stroke

69
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Septal occluder in position
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ASD Question
  • A 45 year- old woman with known pulmonary
    hypertension due to sarcoidosis.
  • She presents with a stroke and is noted to have
    an ASD on an echocardiogram.
  • Why did the neurologists order an echocardiogram?
  • Why did she not have a murmur or widely split
    second heart sound? What might you notice on
    physical exam?

73
ASD in a woman with pulmonary hypertension
74
Obstructive Lesions - Basic Hemodynamic
Principles
  • outflow obstruction more common than inflow
  • elevated pressure proximal to obstruction leads
    to hypertrophy of chamber proximal to obstruction
  • smaller orifice greater pressure needed to
    deliver cardiac output beyond obstruction
  • cardiac output is maintained
  • signs and symptoms are due to pressure elevation
    proximal to obstruction and to chamber
    hypertrophy

75
Obstructive Lesions
  • Obstruction leads to
  • hypertrophy
  • hypertrophy results in increased O2 demand
  • fibrosis results if there is inadequate O2 to
    meet demands
  • fibrosis leads to chamber dilation
  • Heart failure

76
Coarctation of the Aorta
  • narrowing of descending Aorta
  • usually discrete area of stenosis
  • juxtaductal - opposite the site where the ductus
    arteriosus entered)
  • intimal thickening and medial ridges that
    protrude posteriorly and laterally into the
    aortic lumen

77
Monosomy X - Turner syndrome
  • Occurs ins 1/2,500 females
  • High miscarriage rate
  • Clinical features
  • Lymphedema
  • Webbed neck
  • Short stature

78
Coarctation Pathology
79
Coarctation
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81
Coarctation of the Aorta
  • Upper extremity hypertension
  • Upper extremity blood pressure gtgt lower extremity
    blood pressure
  • Associated bicuspid aortic valve in approx. 50

82
Hemodynamics of Coarctation
mechanical obstruction pressure elevation
proximal to obstruction decreased pressure
distal to obstruction
83
Hemodynamics of Coarctation
Collaterals (internal mammaries, intercostals)
to bypass the obstruction LV hypertrophy
helps maintain wall stress systolic function
normal LV EF LV diastolic function may
not be normal due to decreased compliance of
hypertrophied LV
84
Clinical Picture Coarctation
  • History
  • M gt F (in females consider Turner syndrome)
  • 10 present in infancy with CHF
  • may be life-threatening in infancy usually
    requiring immediate and aggressive treatment
  • older children present with hypertension,
    decreased lower extremity pulses, murmur,
    claudication (leg pain with walking), and
    headaches

85
Clinical Picture - Examination
  • normal growth and development in children out of
    newborn period
  • neonates may present with severe CHF, low
    cardiac output, shock
  • children upper extremity blood pressure at least
    20 mmHg greater than lower extremity blood
    pressure
  • decreased femoral pulses with pulse delay (lower
    extremity pulse later than upper extremity pulse)

86
Clinical Picture - Examination
  • murmur- systolic ejection murmur best heard
    between scapulae, left sternal border, apex,
  • extends into diastole
  • turbulent blood crossing the coarctation site

87
CXR - Coarctation
neonate/infant cardiac enlargement, pulmonary
edema, pulmonary venous congestion
88
CXR - Coarctation
children normal heart size rib notching
dilation of descending aorta 3 sign
89
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91
Angiogram - Coarcation
92
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96
Coarctation - Natural History and Management
  • Neonates with decreased cardiac output -
    emergency
  • Prostaglandin E to maintain PDA patency until
    surgery- allows blood to bypass coarctation to
    promote perfusion of the organs supplied by the
    descending aorta
  • Surgical repair once medically stabilized

97
Coarct
PDA
Aorta
Pulmonary Artery
98
Coarctation - Natural History and Management
  • Children
  • if hypertensive
  • significant UE - LE gradient, LV hypertrophy
    surgical repair
  • Recoarctation may occur after repair, increased
    in smaller children (lt 1 years)
  • Hypertension may persist after repair

99
Coarctation Repair
Repair end-to-end anastomosis, Other types of
repair subclavian flap interposition
graft balloon angioplasty
100
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102
Valvar Pulmonary Stenosis
  • Systolic obstruction to outflow from the RV at
    the pulmonary valve
  • Fused or absent pulmonary valve commissures
  • Pulmonary valve cusps are thickened
  • RV pressure must increase in order to deliver
    cardiac output beyond the obstruction

103
thickened pulmonary valve
104
Hemodynamics of Valvar PS
  • to maintain cardiac output, the RV pressure must
    increase enough to overcome the stenosis
  • increased RV pressure results in RV hypertrophy
    and decreased RV compliance
  • decreased RV compliance results in a need for
    higher RA pressures to pump blood into R
  • increased RA pressure may result in R gt L
    shunting across a patent foramen ovale if present
    - cyanosis may occur

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106
Clinical Picture Valvar PS
  • History
  • usually asymptomatic with a murmur
  • neonates may present with cyanosis (R gt L
    shunting at atrial level)
  • fatigue with exercise if severe stenosis
  • May be associated with Noonans syndrome

107
Noonan syndrome
  • Disorder is distinct from Turner syndrome
  • Female male
  • Occurs in 1/1,000-2,000
  • Clinical features overlap with other syndromes

108
Examination Valvar PS
  • murmur systolic ejection murmur at upper left
    sternal border
  • turbulent blood crossing the stenotic pulmonary
    valve
  • systolic ejection click

109
CXR - Valvar PS
  • usually normal
  • poststenotic dilatation of main and left
    pulmonary artery
  • neonate with cyanosis and severe pulmonary
    stenosis - decreased pulmonary blood flow
  • if severe there may be RV enlargement

110
CXR Normal heart size Dilated MPA post
stenotic dilation
111
Natural History and Management - Valvar PS
  • mild - does not usually progress
  • moderate and severe - may be progressive
  • balloon angioplasty has replaced surgical
    valvotomy in most instances

112
Angiogram of Valvar PS
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