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Mosby items and derived items © 2012 Mosby, Inc., an imprint of Elsevier Inc. 1
Alterations of Cardiovascular Function in Children
Mosby items and derived items © 2012 Mosby, Inc., an imprint of Elsevier Inc. 2
Congenital Heart Defects
Major cause of death in the first year of life other than prematurity
Prenatal, environmental, and genetic risk factors: Maternal rubella or increased age, type 1
diabetes, alcoholism, PKU, drugs, and hypercalcemia
Antepartal bleeding Prematurity Chromosome aberrations
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Congenital Heart Disease (cont’d)
Heart defects Hemodynamic alterations
Right-to-left shunt, left-to-right shunt Status of tissue oxygenation
Cyanotic defects Acyanotic defects
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Obstructive Defects
Coarctation of the aorta Narrowing of the lumen of the aorta that
impedes blood flow Almost always in a juxtaductal position Manifestations:
• If severe decreased CO, acidosis, hypotension at birth
• If mild, no manifestations until find hypertension in upper extremities at older age
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Coarctation of the Aorta
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Obstructive Defects
Aortic stenosis Narrowing of the aortic outflow tract Caused by malformation or fusion of the cusps Causes increased workload on left ventricle
and left ventricular hypertrophy Various types Manifestations:
• Infant: if significant faint pulses, hypotension, tachycardia, and poor feeding
• Older children: may have complaints of exercise intolerance
• Risk for bacterial endocarditis
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Aortic Stenosis
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Obstructive Defects
Valvular aortic stenosis Malformed or fused cusps Progressive obstruction with episodes of
ischemia Strenuous activity limited
Subvalvular aortic stenosis Stricture caused by a fibrous ring below a valve Konno procedure used to correct
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Obstructive Defects (cont’d)
Pulmonic stenosis Narrowing of the pulmonary outflow tract Abnormal thickening of the valve leaflets Narrowing of the valve with resistance to flow
from right ventricle to pulmonary artery Right ventricular hypertrophy Pulmonary semilunar valve atresia Manifestations:
• If severe: cyanosis from right-to-left shunt through atrial septal defect; decreased CO
Mosby items and derived items © 2012 Mosby, Inc., an imprint of Elsevier Inc. 10
Pulmonic Stenosis
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Defects Increasing Pulmonary Blood Flow
Patent ductus arteriosus (PDA) Failure of the ductus arteriosus to close PDA allows blood to shunt from the aorta to
pulmonary artery causing left-to-right shunt Manifestations:
• Asymptomatic or pulmonary overcirculation (dyspnea fatigue, poor feeding)
Complications:• Risk for bacterial endocarditis
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Patent Ductus Arteriosus (PDA)
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Defects Increasing Pulmonary Blood Flow
Atrial septal defect Abnormal opening between the atria; blood
flows from left atria to right atria Three major types:
• Ostium primum defect• Ostium secundum defect• Sinus venosus defect
Manifestations:• Asymptomatic at early age• Pulmonary symptoms on exertion at later age
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Defects Increasing Pulmonary Blood Flow (cont’d)
Ventricular septal defect (VSD) Abnormal communication between the
ventricles Most common type of congenital heart lesion Two types Manifestations:
• May be asymptomatic• If severe: increased pulmonary blood flow from left-
to-right shunt; pulmonary hypertension Eisenmenger syndrome
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Defects Increasing Pulmonary Blood Flow (cont’d)
Atrioventricular canal defect (AVC) Results from nonfusion of the endocardial
cushions Demonstrates abnormalities in the atrial and
ventricular septa and atrioventricular valves Complete, partial, and transitional AVCs Manifestations
• Left-to-right shunt and pulmonary overcirculation
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Atrioventricular Canal Defect
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Defects Decreasing Pulmonary Blood Flow
Tetralogy of Fallot Syndrome represented by four defects:
• Ventricular septal defect (VSD)• Overriding aorta• Pulmonary valve stenosis• Right ventricle hypertrophy
Manifestations:• Acute cyanosis at birth or gradual cyanosis• Gradual clubbing, poor growth; Tet spells• If untreated, emboli, stroke, brain abscess, seizures
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Tetralogy of Fallot
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Defects Decreasing Pulmonary Blood Flow
Tricuspid atresia Imperforate tricuspid valve Lack of communication between the right
atrium and right ventricle Additional defects:
• Atrial septal defect• Hypoplastic or absent right ventricle• Enlarged mitral valve and left ventricle• Pulmonic stenosis
Manifestations:• In newborn, cyanosis, tachycardia, dyspnea, poor
feeding• In older child, signs of chronic hypoxemia
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Tricuspid Atresia
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Mixed Defects
Transposition of the great arteries Aorta arises from the right ventricle and the
pulmonary artery arises from the left ventricle Results in two separate, parallel circuits
• Unoxygenated blood circulates continuously through the systemic circulation
• Oxygenated blood circulates continuously through the pulmonary circulation
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Mixed Defects (cont’d)
Transposition of the great arteries (cont’d) Extrauterine survival requires communication
between the two circuits Manifestation:
Depends on size and associated defects
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Transposition of the Great Arteries
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Mixed Defects
Total anomalous pulmonary venous connection (TAPVC) Pulmonary veins connect to the right side of
the heart, directly or indirectly through one or more systemic veins that drain into the right atrium
Classified by point of attachment• Supracardiac• Cardiac• Infracardiac
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Total Anomalous Pulmonary Venous Connection (TAPVC)
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Mixed Defects
Truncus arteriosus Failure of the embryonic artery and the truncus
arteriosus to divide into the pulmonary artery and the aorta
The trunk straddles an always present VSD Types I, II, and III
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Truncus Arteriosus
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Obstructive Defects
Hypoplastic left heart syndrome Abnormal development of the left-sided cardiac
structures• Obstruction to blood flow from the left ventricular
outflow tract Underdevelopment of the left ventricle, aorta
and aortic arch; mitral atresia or stenosis; coarctation of the aorta
Manifestations occur early in newborn (cyanosis, tachypnea, decreased CO)
Fatal in early life if untreated
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Hypoplastic Left Heart Syndrome
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Heart Failure
Heart is not able to maintain cardiac output at level that meets demands of body
Result from poor ventricular function Complication of many congenital heart
defects
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Acquired Cardiovascular Disorders
Kawasaki disease Also known as mucocutaneous lymph node
syndrome Acute, self-limiting systemic vasculitis that may
result in cardiac sequelae Usually occurs in children under 5 years of age
and in winter or spring
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Kawasaki Disease
Stages: Acute: capillaries, venules, arterioles, and the
heart become inflamed Subacute: inflammation of larger vessels;
coronary aneurysms appear Convalescent: medium-sized arteries begin
granulation process; small vessel inflammation decreases
Post-convalescent: scarring of vessels, thickening of tunica intima, calcification, coronary artery stenosis
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Kawasaki Disease (cont’d)
Diagnosis (five of six major findings) Fever for 5 or more days (unresponsive to
antibiotics) Bilateral conjunctivitis without exudation Erythema of oral mucosa (strawberry tongue) Changes in the extremities, such as peripheral
edema and erythema with desquamation of palms and soles
Polymorphous rash Cervical lymphadenopathy
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Acquired Cardiovascular Disorders
Systemic hypertension In children defined as systolic and diastolic
pressure that is over the 95th percentile for age and gender on at least three occasions
• Hypertension in children differs from adults: Often has an underlying disease Renal disease or coarctation of aorta A cause of the hypertension in children is almost
always found Children commonly asymptomatic Seeing increased incidence of primary hypertension in
older children related to obesity
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Acquired Cardiovascular Disorders (cont’d)
Childhood obesity Multivariable and multidimensional Risks:
• Insulin resistance, diabetes, cardiovascular disease• Childhood nutrition, level of physical activity, and
engagement of sedentary activities (TV, computer use, etc.)
Association with parental obesity Epidemic in the USA