Transcript Common types of congenital heart defects

Classification des
cardiopathies congénitales
• I - Physiopathologie :
• Chez le sujet normal, la petite et la grande
circulation sont en série et leurs débit sont
équivalent.
• Les régimes de pression sont réglés par
l’existence de résistances basses dans la
circulation pulmonaire et de résistances
élevées dans la circulation systémique y
conditionnant des pressions élevées.
• Dans les jours qui suivent la naissance ces
résistances de la petite circulation baissent
progressivement pendant que les shunts fœtaux
se ferment. On estime que
• RP/RS=0,5 entre 10 et 24h.
• RP/RS= 0,1 au 15ème jour , le même que chez
l’adulte.
• Cette baisse progressive des résistances
pulmonaires explique l’apparition fréquemment
retardée des souffles de CIV qui peuvent ne pas
être perçus dans les premiers jours de la vie.
• Chez le prématuré : le niveau des
résistances artériolaire pulmonaire est
moins élevé ( le développement
musculaire des artérioles se fait durant le
dernier trimestre. Cela explique le fait que
certains shunt G-Dte soient mal tolérés
précocement chez lui.
Congenital heart defects are
abnormalities that develop before
birth. They can occur in the heart's
chambers, valves or blood vessels.
A baby may be born with only one
defect or several that tend to occur
in combination. Of the dozens of
heart defects, some are mild and
may need minimal or no medical
treatment even through adulthood,
while others are life-threatening,
either immediately to the newborn
or over time. Here's a look at some
of the more common congenital
heart defects. Compare them to
the NORMAL HUMAN HEART,
SHOWN HERE
Congenital Heart Disease
Type of Defect
Mechanism
Ventricular Septal
Defect (VSD)
There is a hole within the membranous or muscular portions of the intraventricular septum
that produces a left-to-right shunt, more severe with larger defects
Atrial Septal Defect
(ASD)
A hole from a septum secundum or septum primum defect in the interatrial septum produces
a modest left-to-right shunt
Patent Ductus
Arteriosus (PDA)
The ductus arteriosus, which normally closes soon after birth, remains open, and a left-toright shunt develops
Tetralogy of Fallot
Pulmonic stenosis results in right ventricular hypertrophy and a right-to-left shunt across a
VSD, which also has an overriding aorta
Transposition of
Great Vessels
The aorta arises from the right ventricle and the pulmonic trunk from the left ventricle. A
VSD, or ASD with PDA, is needed for extrauterine survival. There is right-to-left shunting.
Truncus Arteriosus
There is incomplete separation of the aortic and pulmonary outflows, along with VSD, which
allows mixing of oxygenated and deoxygenated blood and right-to-left shunting
Hypoplastic Left
Heart Syndrome
There are varying degrees of hypoplasia or atresia of the aortic and mitral valves, along with
a small to absent left ventricular chamber
Coarctation of
Aorta
Either just proximal (infantile form) or just distal (adult form) to the ductus is a narrowing of
the aortic lumen, leading to outflow obstruction
Total Anomalous
Pulmonary Venous
Return (TAPVR)
The pulmonary veins do not directly connect to the left atrium, but drain into left innominate
vein, coronary sinus, or some other site, leading to possible mixing of blood and right-sided
overload
Ventricular septal defect
Sometimes called a hole in the heart, this
defect — the most common congenital
heart defect — occurs when the septum,
the muscular wall separating the right and
left ventricles, fails to fully form. The hole
allows oxygen-rich blood to leak from the
left ventricle into the right ventricle,
instead of moving into the aorta and on to
the body. Too much blood may flood the
lungs.
This defect can lead to heart failure,
excessive blood pressure in the lungs
(pulmonary hypertension), infections of
the heart (endocarditis), irregular heart
beats (arrhythmias) and delayed growth.
Small holes may heal on their own or
cause no symptoms. Larger holes may
require surgical repair by stiching
together or covering with a patch.
Atrial septal defect
Similar to a ventricular septal defect,
this is a hole that occurs when the
septum separating the right and left
atria doesn't close properly. This allows
blood from the left atrium to flow into
the right atrium, instead of into the left
ventricle and on to the aorta and the
rest of the body.
The defect can cause several
complications, including arrhythmias,
heart failure, stroke and, in rare cases,
pulmonary hypertension. Minor cases
may cause no symptoms and may not
require treatment. Larger defects may
require surgical closure or cardiac
catheterization.
Patent ductus arteriosus
Before birth, a temporary blood vessel
called the ductus arteriosus connects the
pulmonary artery and the aorta. This
allows blood to bypass the lungs
because oxygen is delivered to the fetus
through the placenta and umbilical cord.
The temporary vessel normally closes
within a few hours or days of birth since
the lungs take over. If it remains open
(patent), some blood that should
circulate through the body is misdirected
to the lungs.
This defect can cause heart failure or
endocarditisse heart failure or
endocarditis. In infants, it can be closed
with medications. In older children and
adults, plugs, coils or surgery can be
used to close the vessel.
Pulmonary stenosis
In this condition, the flow of blood from
the right ventricle to the pulmonary artery
is obstructed by narrowing at the
pulmonary valve. When there's an
obstruction (stenosis), the right ventricle
must pump harder to get blood into the
pulmonary artery. The defect may occur
along with other defects, such as
thickening of the muscle of the right
ventricle immediately below the valve.
In many cases, pulmonary stenosis is
mild and doesn't require treatment. But
because it can cause heart failure,
arrhythmias or enlargement of the right
heart chambers, surgery may be
necessary to repair the stenosis or
replace the valve. Special balloons to
widen the valve (balloon valvuloplasty)
may also be used.
Tetralogy of Fallot
This defect is a combination of four
(tetralogy) congenital abnormalities.
The four defects typically are ventricular
septal defect (VSD), pulmonary stenosis,
a misplaced aorta and a thickened right
ventricular wall (right ventricular
hypertrophy). They usually result in an
insufficient amount of oxygenated blood
reaching the body.
Complications of tetralogy of Fallot (fuhLOE) include cyanosis — sometimes
called "blue baby syndrome," since the
lips, fingers and toes may have a bluish
tinge from lack of oxygen — as well as
poor eating, inability to tolerate exercise,
arrhythmias, delayed growth and
development, and stroke. Surgical repair
of the defects is required early in life.
Transposition of the great vessels
(arteries)
With this defect, the positions of
the aorta and the pulmonary artery
(the great arteries) are reversed
(transposed). The aorta arises from
the right ventricle instead of the left
and the pulmonary artery arises from
the left ventricle instead of the right.
This creates a circulatory pattern that
prevents nourishing oxygenated
blood from reaching the body.
This condition would quickly be fatal
to a newborn except it's generally
accompanied by another defect —
commonly a septal defect or patent
ductus arteriosus — that does allow
oxygen-rich blood to get to the body.
Surgical repair is usually necessary
shortly after birth.
Truncus arteriosus
This is a defect in which the normally
distinct pulmonary artery and aorta
merge into one single great vessel
(truncus) arising from the right and left
ventricles. In addition, there's usually a
large ventricular septal defect, essentially
turning the right and left ventricles into a
single chamber. This allows oxygenated
and unoxygenated blood to mix. Too
much blood may flow to the lungs,
flooding them and making it difficult to
breathe. It can also result in lifethreatening pulmonary hypertension.
Surgery is needed to close the septal
defect with a patch and to separate the
pulmonary arteries from the trunk. A
conduit is placed to connect the right
ventricle to the pulmonary artery.
Because the conduit doesn't grow with
the child, repeat surgery may be
necessary over time
Hypoplastic left heart syndrome
In this condition, the left side of the heart is
underdeveloped (hypoplastic), including
the aorta, aortic valve, left ventricle and
mitral valve. As a result, the body doesn't
receive enough oxygenated blood. In the
first few days after a baby is born, the
ductus arteriosus remains open (patent),
allowing normal circulation, so the baby
may seem fine initially. But when the
ductus arteriosus naturally closes, signs
and symptoms begin, including a bluish
cast to the skin from lack of oxygen,
difficulty breathing and poor feeding. This
condition may be accompanied by an atrial
septal defect.
Treatment options for this life-threatening
condition are a heart transplant or a
multistage surgical procedure done during
the first few years of life.
Coarctation of the aorta
This is a narrowing
(coarctation), or constriction,
in a portion of the aorta.
Coarctation forces the heart to
pump harder to get blood
through the aorta and on to the
rest of the body.
This defect can cause several
life-threatening complications,
including severe hypertension,
aortic aneurysm, dissection or
rupture, endocarditis, brain
hemorrhage, stroke, heart
failure and premature coronary
artery disease. Repair is
typically recommended before
age 10, either by surgically
removing the affected portion or
widening it through balloon
angioplasty and placement of a
stent.
Aortic stenosis
This is a defect that narrows
or obstructs the aortic valve
opening, making it difficult for
the heart to pump blood into
the aorta. Mild cases may not
have symptoms initially, but they
can worsen over time.
The defect can cause heart
enlargement, left-sided heart
failure, arrhythmias, endocarditis
and fainting. Treatment includes
surgical repair or replacement of
the valve or, in young children,
widening through balloon
valvuloplasty.
If there's an extra conduction
pathway, the electrical signal
may arrive at the ventricles too
soon. This condition is called
Wolff-Parkinson-White syndrome
(WPW). It's in a category of
electrical abnormalities called
"pre-excitation syndromes."
Ebstein's anomaly
This is a defect of the tricuspid valve, which
controls blood flow between the heart's right
atrium and right ventricle. The valve is
positioned lower than normal into the
ventricle instead of remaining between the
atrium and the ventricle. Consequently, the
ventricle is too small and the atrium too
large, and neither functions properly. The
valve is also malformed, often allowing
blood to leak from the ventricle into the
atrium. This defect often occurs along with
other heart defects, including patent
foramen ovale, atrial septal defect or WolffParkinson-White syndrome.
Severe cases are life-threatening. Milder
cases may have no signs or symptoms until
adulthood. Treatment is with medications or
with surgery to repair or replace the
tricuspid valve, as well as treatment of
associated conditions.
Atrioventricular canal defect
This is a combination of defects,
including a large hole in the center of
the heart and a single common valve
instead of the separate tricuspid and
mitral valves. Also called atrioventricular
septal defect, this defect is classified by
whether it's only partial, involving only
the upper chambers of the heart,
or complete, in which blood can travel
freely among all four chambers of the
heart. Both forms allow extra blood
to circulate to the lungs, causing the
heart to enlarge.
The condition is often associated with
Down syndrome. Infants may also have
trouble breathing and not grow well.
Surgery is often done in infancy to close
the hole and reconstruct the valves.
• II- Classification :
• On distingue 3 classes de cardiopathies
congénitales
– cardiopathies avec shunt G-dte
– cardiopathies cyanogènes
– cardiopathies par obstacle isolé
•
Cardiopathies par shunts Gauche-dte.
En raison du régime de pressions plus
élevées dans la circulation systémique
que dans la circulation pulmonaire, un
défaut de cloisonnement du cœur ou la
persistance d’une communication
anormale entre les 2 circulations suffit à
la création d’un shunt G-dte.
• La communication peut siéger soit à
l’étage auriculaire ou veineux soit à l’étage
ventriculaire ou artériel. Le shunt gauchedroite a pour conséquences une
augmentation du débit pulmonaire qui
dépend de la taille de la communication.
• Dans les shunts importants cet hyper débit
peut s’accompagner d’une augmentation
de la pression pulmonaire dite de débit.
Cette hypertension pulmonaire et cet
hyper débit sont responsables de la
défaillance cardiaque.
• La maladie obstructive artériolaire
pulmonaire est le risque des shunts G-Dte
importants ; C’est une réduction du calibre
artériolaire pulmonaire. Les lésions sont
d’abord réversibles (épaississement
musculaire) puis irréversibles( fibrose)
•
•
•
•
•
•
•
•
•
Cardiopathies avec défaut septal : shunt gauchedroite
CIA avec ou sans SS. RVPA
CAV
CIV
CA
Fistule ou fenêtre aorto-pulmonaire
Fistule artère coronaire cavités droites.
Naissance anormale de la coronaire gauche à partir de
l’AP
Rupture du sinus de Valsalva dans les cavités droites
•
•
VDDI
VGDI
•
•
Les cardiopathies cyanogènes : La
cyanose est un symptôme grave.
Physiopathologie : pour qu’il y ait
cyanose il est nécessaire que du sang
désaturé venant des veines caves passe
dans l’aorte.
• Ces shunts droite-gauche peuvent se faire
systématiquement dans trois
circonstances
– obstacle pulmonaire + défaut septal : T4F,
T3F, Ebstein, CIA, Eisenmenger, atrésie tric.
Toutes ces cardiopathies ont en commun une
réduction du débit pulmonaire.
– TGV
– Mélange total des 2 circulations / RVPAT, VU,
TA commun
• Les obstacles isolés : peuvent siéger sur
la voie G ou droite. Ils ont en commun une
augmentation de la post charge avec
hyper pression en amont de la sténose .
•
Cardiopathies par obstacle :
–
•
•
•
•
Cœur triatrial
RM congénital
Rao SS val ; val ; supraval
Coarcatation de l’aorte
–
•
•
•
Droit
Maladie d’Ebstein
RP infund ; orificiel ; des branches
HTAP primitive
–
•
•
Gauche :
Mixte
Hypoplasie artérielle multiple
Syndrome de Williams et Beuren
–
•
•
•
•
•
•
•
Cardiopathies avec mélange des 2 circulations :
RVPAT
Oreillette unique
Hypoplasie du VG et atrésie mitrale
VU
Tronc artériel commun
Veine cave anormalement abouchée dans
l’OG
Fistule artério-veineuse pulmonaire
Congenital heart defects are
abnormalities that develop before
birth. They can occur in the heart's
chambers, valves or blood vessels.
A baby may be born with only one
defect or several that tend to occur
in combination. Of the dozens of
heart defects, some are mild and
may need minimal or no medical
treatment even through adulthood,
while others are life-threatening,
either immediately to the newborn
or over time. Here's a look at some
of the more common congenital
heart defects. Compare them to
the NORMAL HUMAN HEART,
SHOWN HERE
Congenital Heart Disease
Type of Defect
Mechanism
Ventricular Septal
Defect (VSD)
There is a hole within the membranous or muscular portions of the intraventricular septum
that produces a left-to-right shunt, more severe with larger defects
Atrial Septal Defect
(ASD)
A hole from a septum secundum or septum primum defect in the interatrial septum produces
a modest left-to-right shunt
Patent Ductus
Arteriosus (PDA)
The ductus arteriosus, which normally closes soon after birth, remains open, and a left-toright shunt develops
Tetralogy of Fallot
Pulmonic stenosis results in right ventricular hypertrophy and a right-to-left shunt across a
VSD, which also has an overriding aorta
Transposition of
Great Vessels
The aorta arises from the right ventricle and the pulmonic trunk from the left ventricle. A
VSD, or ASD with PDA, is needed for extrauterine survival. There is right-to-left shunting.
Truncus Arteriosus
There is incomplete separation of the aortic and pulmonary outflows, along with VSD, which
allows mixing of oxygenated and deoxygenated blood and right-to-left shunting
Hypoplastic Left
Heart Syndrome
There are varying degrees of hypoplasia or atresia of the aortic and mitral valves, along with
a small to absent left ventricular chamber
Coarctation of
Aorta
Either just proximal (infantile form) or just distal (adult form) to the ductus is a narrowing of
the aortic lumen, leading to outflow obstruction
Total Anomalous
Pulmonary Venous
Return (TAPVR)
The pulmonary veins do not directly connect to the left atrium, but drain into left innominate
vein, coronary sinus, or some other site, leading to possible mixing of blood and right-sided
overload
Ventricular septal defect
Sometimes called a hole in the heart, this
defect — the most common congenital
heart defect — occurs when the septum,
the muscular wall separating the right and
left ventricles, fails to fully form. The hole
allows oxygen-rich blood to leak from the
left ventricle into the right ventricle,
instead of moving into the aorta and on to
the body. Too much blood may flood the
lungs.
This defect can lead to heart failure,
excessive blood pressure in the lungs
(pulmonary hypertension), infections of
the heart (endocarditis), irregular heart
beats (arrhythmias) and delayed growth.
Small holes may heal on their own or
cause no symptoms. Larger holes may
require surgical repair by stiching
together or covering with a patch.
Atrial septal defect
Similar to a ventricular septal defect,
this is a hole that occurs when the
septum separating the right and left
atria doesn't close properly. This allows
blood from the left atrium to flow into
the right atrium, instead of into the left
ventricle and on to the aorta and the
rest of the body.
The defect can cause several
complications, including arrhythmias,
heart failure, stroke and, in rare cases,
pulmonary hypertension. Minor cases
may cause no symptoms and may not
require treatment. Larger defects may
require surgical closure or cardiac
catheterization.
Patent ductus arteriosus
Before birth, a temporary blood vessel
called the ductus arteriosus connects the
pulmonary artery and the aorta. This
allows blood to bypass the lungs
because oxygen is delivered to the fetus
through the placenta and umbilical cord.
The temporary vessel normally closes
within a few hours or days of birth since
the lungs take over. If it remains open
(patent), some blood that should
circulate through the body is misdirected
to the lungs.
This defect can cause heart failure or
endocarditisse heart failure or
endocarditis. In infants, it can be closed
with medications. In older children and
adults, plugs, coils or surgery can be
used to close the vessel.
Pulmonary stenosis
In this condition, the flow of blood from
the right ventricle to the pulmonary artery
is obstructed by narrowing at the
pulmonary valve. When there's an
obstruction (stenosis), the right ventricle
must pump harder to get blood into the
pulmonary artery. The defect may occur
along with other defects, such as
thickening of the muscle of the right
ventricle immediately below the valve.
In many cases, pulmonary stenosis is
mild and doesn't require treatment. But
because it can cause heart failure,
arrhythmias or enlargement of the right
heart chambers, surgery may be
necessary to repair the stenosis or
replace the valve. Special balloons to
widen the valve (balloon valvuloplasty)
may also be used.
Tetralogy of Fallot
This defect is a combination of four
(tetralogy) congenital abnormalities.
The four defects typically are ventricular
septal defect (VSD), pulmonary stenosis,
a misplaced aorta and a thickened right
ventricular wall (right ventricular
hypertrophy). They usually result in an
insufficient amount of oxygenated blood
reaching the body.
Complications of tetralogy of Fallot (fuhLOE) include cyanosis — sometimes
called "blue baby syndrome," since the
lips, fingers and toes may have a bluish
tinge from lack of oxygen — as well as
poor eating, inability to tolerate exercise,
arrhythmias, delayed growth and
development, and stroke. Surgical repair
of the defects is required early in life.
Transposition of the great vessels
(arteries)
With this defect, the positions of
the aorta and the pulmonary artery
(the great arteries) are reversed
(transposed). The aorta arises from
the right ventricle instead of the left
and the pulmonary artery arises from
the left ventricle instead of the right.
This creates a circulatory pattern that
prevents nourishing oxygenated
blood from reaching the body.
This condition would quickly be fatal
to a newborn except it's generally
accompanied by another defect —
commonly a septal defect or patent
ductus arteriosus — that does allow
oxygen-rich blood to get to the body.
Surgical repair is usually necessary
shortly after birth.
Truncus arteriosus
This is a defect in which the normally
distinct pulmonary artery and aorta
merge into one single great vessel
(truncus) arising from the right and left
ventricles. In addition, there's usually a
large ventricular septal defect, essentially
turning the right and left ventricles into a
single chamber. This allows oxygenated
and unoxygenated blood to mix. Too
much blood may flow to the lungs,
flooding them and making it difficult to
breathe. It can also result in lifethreatening pulmonary hypertension.
Surgery is needed to close the septal
defect with a patch and to separate the
pulmonary arteries from the trunk. A
conduit is placed to connect the right
ventricle to the pulmonary artery.
Because the conduit doesn't grow with
the child, repeat surgery may be
necessary over time
Hypoplastic left heart syndrome
In this condition, the left side of the heart is
underdeveloped (hypoplastic), including
the aorta, aortic valve, left ventricle and
mitral valve. As a result, the body doesn't
receive enough oxygenated blood. In the
first few days after a baby is born, the
ductus arteriosus remains open (patent),
allowing normal circulation, so the baby
may seem fine initially. But when the
ductus arteriosus naturally closes, signs
and symptoms begin, including a bluish
cast to the skin from lack of oxygen,
difficulty breathing and poor feeding. This
condition may be accompanied by an atrial
septal defect.
Treatment options for this life-threatening
condition are a heart transplant or a
multistage surgical procedure done during
the first few years of life.
Coarctation of the aorta
This is a narrowing
(coarctation), or constriction,
in a portion of the aorta.
Coarctation forces the heart to
pump harder to get blood
through the aorta and on to the
rest of the body.
This defect can cause several
life-threatening complications,
including severe hypertension,
aortic aneurysm, dissection or
rupture, endocarditis, brain
hemorrhage, stroke, heart
failure and premature coronary
artery disease. Repair is
typically recommended before
age 10, either by surgically
removing the affected portion or
widening it through balloon
angioplasty and placement of a
stent.
Aortic stenosis
This is a defect that narrows
or obstructs the aortic valve
opening, making it difficult for
the heart to pump blood into
the aorta. Mild cases may not
have symptoms initially, but they
can worsen over time.
The defect can cause heart
enlargement, left-sided heart
failure, arrhythmias, endocarditis
and fainting. Treatment includes
surgical repair or replacement of
the valve or, in young children,
widening through balloon
valvuloplasty.
If there's an extra conduction
pathway, the electrical signal
may arrive at the ventricles too
soon. This condition is called
Wolff-Parkinson-White syndrome
(WPW). It's in a category of
electrical abnormalities called
"pre-excitation syndromes."
Ebstein's anomaly
This is a defect of the tricuspid valve, which
controls blood flow between the heart's right
atrium and right ventricle. The valve is
positioned lower than normal into the
ventricle instead of remaining between the
atrium and the ventricle. Consequently, the
ventricle is too small and the atrium too
large, and neither functions properly. The
valve is also malformed, often allowing
blood to leak from the ventricle into the
atrium. This defect often occurs along with
other heart defects, including patent
foramen ovale, atrial septal defect or WolffParkinson-White syndrome.
Severe cases are life-threatening. Milder
cases may have no signs or symptoms until
adulthood. Treatment is with medications or
with surgery to repair or replace the
tricuspid valve, as well as treatment of
associated conditions.
Atrioventricular canal defect
This is a combination of defects,
including a large hole in the center of
the heart and a single common valve
instead of the separate tricuspid and
mitral valves. Also called atrioventricular
septal defect, this defect is classified by
whether it's only partial, involving only
the upper chambers of the heart,
or complete, in which blood can travel
freely among all four chambers of the
heart. Both forms allow extra blood
to circulate to the lungs, causing the
heart to enlarge.
The condition is often associated with
Down syndrome. Infants may also have
trouble breathing and not grow well.
Surgery is often done in infancy to close
the hole and reconstruct the valves.