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Alterations of Cardiovascular
Function in Children
Chapter 24
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Congenital Heart Defects
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Major cause of death in the first year of life
other than prematurity
Prenatal, environmental, and genetic risk
factors
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Maternal rubella, type 1 diabetes, alcoholism,
PKU, and hypercalcemia
Drugs
Chromosome aberrations
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Congenital Heart Disease
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Heart defects
Hemodynamic alterations
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
Right-to-left shunt, left-to-right shunt
Status of tissue oxygenation
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Cyanotic defects
Acyanotic defects
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Obstructive Defects

Coarctation of the aorta
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
Narrowing of the lumen of the aorta that
impedes blood flow
Coarctation of the aorta is almost always in a
juxtaductal position, but it can occur anywhere
between the origin of the aortic arch and the
bifurcation of the aorta in the lower abdomen
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Coarctation of the Aorta
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Obstructive Defects

Aortic stenosis
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Narrowing of the aortic outflow tract
Caused by malformation or fusion of the cusps
Causes an increased workload on the left
ventricle
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Aortic Stenosis
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Obstructive Defects

Valvular aortic stenosis
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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

Pulmonic stenosis
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Narrowing of the pulmonary outflow tract
 Abnormal thickening of the valve leaflets
 Narrowing of the valve
 Pulmonary semilunar valve atresia
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Pulmonic Stenosis
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Defects Increasing Pulmonary
Blood Flow

Patent ductus arteriosus (PDA)
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
Failure of the ductus arteriosus to close
PDA allows blood to shunt from the pulmonary
artery to the aorta
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Patent Ductus Arteriosus (PDA)
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Defects Increasing Pulmonary
Blood Flow
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Atrial septal defect
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
Abnormal opening between the atria
Three major types
• Ostium primum defect
• Ostium secundum defect
• Sinus venosus defect
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Defects Increasing Pulmonary
Blood Flow

Ventricular septal defect (VSD)
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Abnormal communication between the
ventricles
 Most common type of congenital heart lesion
 Types
• Perimembranous VSD
• Muscular VSD
 Eisenmenger syndrome
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Defects Increasing Pulmonary
Blood Flow
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Atrioventricular canal defect (AVC)
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Results from nonfusion of the endocardial
cushions
Demonstrates abnormalities in the atrial and
ventricular septa and atrioventricular valves
Complete, partial, and transitional AVCs
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Atrioventricular Canal Defect
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Defects Decreasing Pulmonary
Blood Flow

Tetralogy of Fallot
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Syndrome represented by four defects
• Ventricular septal defect (VSD)
• Overriding aorta
• Pulmonary valve stenosis
• Right ventricle hypertrophy
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Tetralogy of Fallot
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Defects Decreasing Pulmonary
Blood Flow
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Tricuspid atresia
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Imperforate tricuspid valve
 Lack of communication between the right
atrium and right ventricle
 Additional defects
• Septal defect
• Hypoplastic or absent right ventricle
• Enlarged mitral valve and left ventricle
• Pulmonic stenosis
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Tricuspid Atresia
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Mixed Defects
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Transposition of the great arteries
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Aorta arises from the right ventricle and the
pulmonary artery arises from the left ventricle
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Mixed Defects
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Transposition of the great arteries
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Results in two separate, parallel circuits
• Unoxygenated blood circulates continuously through
the systemic circulation
• Oxygenated blood circulates continuously through
the pulmonary circulation

Extrauterine survival requires communication
between the two circuits
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Transposition of the Great
Arteries
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Mixed Defects
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Total anomalous pulmonary venous
connection (TAPVC)
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
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
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Truncus arteriosus
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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
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Hypoplastic left heart syndrome

Abnormal development of the left-sided cardiac
structures
• Obstruction to blood flow from the left ventricular
outflow tract

Under development of the left ventricle, aorta
and aortic arch, and mitral atresia or stenosis
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Hypoplastic Left Heart Syndrome
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Congestive Heart Failure
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Heart is not able to maintain cardiac output
at level that meets demands of body
Result from poor ventricular function
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Acquired Cardiovascular
Disorders
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Kawasaki disease
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
Also known as mucocutaneous lymph node
syndrome
Acute, self-limiting systemic vasculitis that may
result in cardiac sequelae
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Kawasaki Disease
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Stages
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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
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Kawasaki Disease

Stages
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Post convalescent: scarring of vessels,
thickening of tunica intima, calcification,
coronary artery stenosis
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Kawasaki Disease
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Diagnosis (5 of 6 major findings)
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Fever for 5 or more days (unresponsive to
antibiotics)
 Bilateral conjunctivitis without exudation
 Erythema of oral mucosa (strawberry tongue)
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Kawasaki Disease
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Diagnosis (5 of 6 major findings)
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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

Hypertension in children differs from adult
hypertension
• Often have an underlying disease

Renal disease or coarctation of the aorta
• A cause of the hypertension in children is almost
always found
• Children with hypertension are commonly
asymptomatic
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Acquired Cardiovascular
Disorders

Childhood obesity
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Multivariable and multidimensional
Risk factors
• Obesity, insulin resistance, diabetes, cardiovascular
disease
• Childhood nutrition, level of physical activity, and
engagement of sedentary activities (TV, computer
use, etc.)

Association with parental obesity
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