Download 2 E MASANGA CONGENITAL HEART DISEASES

INTRODUCTION
• A 35 year old woman with transposition of the
great arteries repaired with a Mustard procedure
attends your clinic for annual follow-up.
• Her main concern, she tells you, is that her
recent life insurance application was refused
because of congenital heart disease.
• She is disturbed because at yearly clinic visits
she has always been reassured that she is
‘doing very well’.
• She asks you to write to her insurance company
on her behalf.
SCOPE OF THE PROBLEM
• Congenital heart disease (CHD) affects 8/1000 live
births.
• techniques for the diagnosis and management of CHD
over the last fifty years, expected survival into adulthood
has reached 85%.
• Adding to this number are an estimated 1600 cases with
complex or significant congenital heart lesions and many
more with simple lesions entering the adult age group
each year.
• These figures will continue to increase over time, leading
to an ever growing adult patient group seeking the
financial security of insurance coverage.
ANATOMY OF THE HEART
Atrial Septal Defect(ASD)
Ventricular Septal Defect(VSD)
Patent Ductus Arteriosus(PDA)
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What are the different types of congenital heart defects?
We can classify congenital heart defects into several categories in order to
better understand the problems the baby will experience. They include:
problems that cause too much blood to pass through the lungs
These defects allow oxygen-rich blood that should be traveling to the body to recirculate through the lungs, causing increased pressure and stress in the lungs.
problems that cause too little blood to pass through the lungs
These defects allow blood that has not been to the lungs to pick up oxygen (and,
therefore, is oxygen-poor) to travel to the body. The body does not receive enough
oxygen with these heart problems, and the baby will be cyanotic, or have a blue
coloring.
problems that cause too little blood to travel to the body
These defects are a result of underdeveloped chambers of the heart or blockages in
blood vessels that prevent the proper amount of blood from traveling to the body to
meet its needs.
Again, in some cases there will be a combination of several heart defects,
making for a more complex problem that can fall into several of these
categories.
• patent ductus arteriosus (PDA) - this defect,
which normally occurs during fetal life, short
circuits the normal pulmonary vascular system
and allows blood to mix between the pulmonary
artery and the aorta. Prior to birth, there is an
open passageway between the two blood
vessels, which closes soon after birth. When it
does not close, some blood returns to the lungs.
Patent ductus arteriosus is often seen in
premature infants.
• atrial septal defect (ASD) - in this
condition, there is an abnormal opening
between the two upper chambers of the
heart - the right and left atria - causing an
abnormal blood flow through the heart.
Some children may have no symptoms
and appear healthy. However, if the ASD is
large, permitting a large amount of blood
to pass through the right side, symptoms
will be noted.
• ventricular septal defect (VSD) - in this
condition, a hole in the ventricular septum (a
dividing wall between the two lower chambers of
the heart - the right and left ventricles) occurs.
Because of this opening, blood from the left
ventricle flows back into the right ventricle, due
to higher pressure in the left ventricle. This
causes an extra volume of blood to be pumped
into the lungs by the right ventricle, which can
create congestion in the lungs.
• atrioventricular canal (AVC or AV
canal) - atrioventricular canal is a complex
heart problem that involves several
abnormalities of structures inside the
heart, including atrial septal defect,
ventricular septal defect, and improperly
formed mitral and/or tricuspid valves.
• Some of the problems that cause too little blood to
pass through the lungs include the following:
• tricuspid atresia - in this condition, there is no tricuspid
valve, therefore, no blood flows from the right atrium to
the right ventricle. Tricuspid atresia defect is
characterized by the following:
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a small right ventricle
a large left ventricle
diminished pulmonary circulation
cyanosis - bluish color of the skin and mucous membranes
caused from a lack of oxygen.
• A surgical shunting procedure is often necessary to
increase the blood flow to the lungs.
• transposition of the great arteries (TGA) with this congenital heart defect, the positions of
the pulmonary artery and the aorta are reversed,
thus:
– the aorta originates from the right ventricle, so most of
the blood returning to the heart from the body is
pumped back out without first going to the lungs.
– the pulmonary artery originates from the left ventricle,
so that most of the blood returning from the lungs
goes back to the lungs again
• tetralogy of Fallot (TOF) - this condition is
characterized by the following four defects:
– an abnormal opening, or ventricular septal defect, that
allows blood to pass from the right ventricle to the left
ventricle without going through the lungs
– a narrowing (stenosis) at or just beneath the
pulmonary valve that partially blocks the flow of blood
from the right side of the heart to the lungs
– the right ventricle is more muscular than normal
– the aorta lies directly over the ventricular septal defect
• Tetralogy of Fallot results in cyanosis (bluish
color of the skin and mucous membranes
due to lack of oxygen).
• truncus arteriosus - the aorta and
pulmonary artery start as a single blood
vessel, which eventually divides and
becomes two separate arteries. Truncus
arteriosus occurs when the single great
vessel fails to separate completely, leaving
a connection between the aorta and
pulmonary artery.
• aortic stenosis (AS) - in this condition, the aortic valve
between the left ventricle and the aorta did not form
properly and is narrowed, making it difficult for the heart
to pump blood to the body. A normal valve has three
leaflets or cusps, but a stenotic valve may have only one
cusp (unicuspid) or two cusps (bicuspid).
Although aortic stenosis may not cause symptoms, it
may worsen over time, and surgery may be needed to
correct the blockage - or the valve may need to be
replaced with an artificial one.
Good
Intermediate
Uncertain or Poor
Atrial septal defect
Patent ductus arteriosus
Pulmonary stenosis
Ventricular septal defect
Coarctation of the aorta
Aortic stenosis
Tetralogy of Fallot
Transposition of the Great Arteries
—post Senning/Mustard procedure
Transposition of the Great Arteries
—post arterial switch procedure
Congenitally corrected transposition
Ebstein’s anomaly of tricuspid valve
Single ventricle physiology
Conclusion
• . With newer surgical and catheter
techniques, advancing medical therapy
and improved risk stratification, overall
prognosis and hence insurability will
continue to improve for adults with
congenital heart disease.