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Pre by lect: ZAHID REHMAN
IPMS(KMU)
Congenital heart disease
Congenital heart defects are heart problems 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 with several
defects. Other types of congenital heart defects
are life-threatening, either immediately to the
newborn or over time.
congenital cardiac malformations resulting
from defective embryonic development
without obvious cause.
Between 3-8 weeks “gestation” all the fetal
heart structures are formed “organogenesis”
`
Majority of cases
of the congenital
heart diseases
are unknown
cause
Classification
Common acyanotic lesions
 Ventricular septal defects
 Atrial septal defects
 Atrio-ventricular septal defects
 Patent ductus arteriosus
 Truncus arteriosus
 Aortic stenosis
 Mitral stenosis/incompetence
 Coarctation of aorta
 Tricuspid regurgitation
Common Cyanotic Lesions
Tetralogy of fallot (TOF)
Transposition of great vessels
Truncus arteriiousus
Total anomalous pulmonary venous return
Hypoplastic left heart syndrome
Diagnosis
Early diagnosis of C.H.D mean better results.
40% of C.H.D diagnosed at 1st w of life.
50-60 % diagnosed at 1st two months .
Others are usually later during routine medical
examination.
Diagnosis depend on good clinical history +
good medical examination and investigations.
-History of dysnea on exertion.
-Shortness of breathing.
-Orthopnea.
-Lower limbs swelling
-Palpitation.
-Convulsion.
-All may be manifest of congestive heart failure.
Older child
Ventricular Septal defects(VSD)
 Sometimes called a hole in the heart, this defect
— the most common congenital heart defect —
occurs when the muscular wall (septum)
separating the bottom chambers of the heart
(right and left ventricles) doesn't 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. In the right
ventricle, the oxygen-rich blood mixes with blood
that doesn't have enough oxygen in it.
 Single most common congenital heart
malformation, accounting for almost 30% of all
CHD
 Defects can occur in both the membranous
portion of the septum (most common) and the
muscular portion
 Between 80% and 85% of all VSDs
 < 3 mm in diameter
 All close spontanously
 50% by 2 years
 90% by 6 years
 10% during school years
 Muscular close sooner than membranous
Ventricular septal defect can lead to
Heart failure
High blood pressure in the lungs (pulmonary
hypertension),
Infection of the heart (endocarditis)
Irregular heartbeats (arrhythmias)
Delayed growth.
Small holes may heal on their own or cause no
symptoms. Larger holes may require surgery to
stitch the hole closed or to cover the hole with a
patch.
Investigation
X-ray chest:
ECG
Echocardiography
The size and location can be assessed by
echocardiography. Main pulmonary artery
dilatation may be seen.
Atrial septal defect
 Atrial septal defect is a hole that occurs when the
muscular wall (septum) separating the top two
chambers of the heart (right and left atria) doesn't
close properly. This allows oxygen-rich blood from
the left atrium to flow into the right atrium, where
it mixes with blood that doesn't have adequate
oxygen in it. Blood from the left atrium should
normally flow into the left ventricle and on to the
aorta and the rest of the body.
 Minor cases may cause no symptoms and may
not require treatment. Larger defects may require
surgery or cardiac catheterization to repair the
hole.
A large ASD may be associated with growth failure
in infants and children. It.s more common in
women then in men.
Types :
There are three types of ASD
1. Ostium secundum ASD (75%)
Most common type and result from defect in the
region of the fossa ovalis . In this type there is
no involment of AV valve.
2. Ostium primum ASD (15%)
Ostium primum ASD result from absence of septum
primum (at the lower portion of the artrial septum.
In this type there is also involvement of AV valves.
3. Sinus venosus defect(10%)
Sinus venosus defect is in the upper part of the
septum near the entrance of the inferior venacava
and coronary sinus there for producing
communication between coronary sinus and left
atrium.
Clinical feature
Asymptomatic
Exertional dyspnea after 30 age
Pts are more prone to developed pulmonary
infection
Right ventricular failure with large defect and
prolonged pulmonary hypertension may develop
later in life.
 Enlargement of the right ventricle
 Enlargement of atrium
 Large pulmonary artery
 increased pulmonary vascularity is.
Patent ductus arteriosus(PDA)
 Before birth, a blood vessel called the ductus
arteriosus connects the pulmonary artery — the
artery carrying blood to the lungs — and the
aorta, the large artery that carries blood away
from the heart. Before a baby is born, the ductus
arteriosus allows blood to bypass the lungs
because the baby receives oxygen through the
placenta and umbilical cord. The ductus
arteriosus normally closes within soon after birth.
If the ductus arteriosus remains open (patent),
some blood that should flow through the body
goes to the lungs.
 This defect can cause heart failure or an infection
of the heart (endocarditis). In infants, it can be
closed with medications. In older children and
adults, surgery is performed to close the vessel.
 Accounts for about 10% of all cases of CHD
 Higher incidence of PDA in infants born at high
altitudes (over 10,000 feet)
 More common in females
 Treatment
Medicaly treatment give NSAID which decrease the
prostaglandin.
Surgery :
PDA ligation proceduer.
Pulmonary valve stenosis
 In this condition, blood flow from one of the
heart's bottom chambers, the right ventricle, to
the pulmonary artery is slowed by narrowing at
the pulmonary valve. When there's narrowing
(stenosis), the right ventricle must pump harder to
get blood into the artery that carries blood to the
lungs (pulmonary artery). Pulmonary valve
stenosis may occur along with other defects, such
as thickening of the muscle of the right ventricle
below the valve.
 In many cases, pulmonary valve stenosis is mild
and doesn't require treatment.
But because it can cause
 heart failure
 arrhythmias
 enlargement of the right heart chambers
It may be necessary to correct the defect. In many
cases, a balloon (pulmonary valvuloplasty) opens
the narrowed valve. In some cases, especially in
people with other heart defects, surgery may be
necessary to replace the diseased valve with an
artificial valve.
Aortic stenosis
 Aortic stenosis is a defect that narrows or blocks
the aortic valve opening, making it difficult for the
heart to pump blood into the aorta — the main
artery leading away from the heart — on to the
rest of the body.
 The defect can cause the heart to get bigger
 left-sided heart failure
 abnormal heart rhythms (arrhythmias),
 infections of the heart (endocarditis)
 fainting.
Treatment:
Treatment includes surgery to repair or replace the
valve .
In young children, widening of the valve through a
surgical procedure called balloon valvuloplasty, in
which a balloon-like device widens the valve so
that blood can flow through.
Coarctation of the aorta
 This is a narrowing (coarctation) in a portion of
the heart's main artery (aorta). Coarctation forces
the heart to pump harder to get blood through the
aorta and on to the rest of the body.
 Coarctation of the aorta can cause several life-
threatening complications including
 severe high blood pressure
 a bulge in the aorta that can burst (aortic
aneurysm)
 infection of the heart (endocarditis)
 brain hemorrhage
stroke
heart failure
Treatment:
Repair is typically recommended before age 10,
either by surgically removing the affected portion
or widening it through a surgical procedure called
balloon angioplasty and placement of a mesh
tube that can hold the aorta open (stent).
Transposition of the great arteries
 With this defect, the positions of the aorta — the
main artery leading away from the heart — and
the pulmonary artery, which leads to the lungs,
are reversed (transposed). The aorta and
pulmonary artery are, together, sometimes
referred to as the great arteries. In transposition
of the great arteries, the aorta arises from the
right ventricle instead of the left ventricle and the
pulmonary artery arises from the left ventricle
instead of the right. This 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 allows oxygen-rich blood
to get to the body. Surgery to repair the condition
is usually necessary shortly after birth.
Tetralogy of Fallot
 This defect is a combination of four (tetralogy) heart
defects. The four defects typically are ventricular
septal defect (VSD), pulmonary valve 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 (fuh-LOE)
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, physical inability to
exercise, irregular heartbeats (arrhythmias),
delayed growth and development, and stroke.
Surgery to repair the defects is required early in
life.
Ebstein's anomaly
 This is a defect of the tricuspid valve, which controls
blood flow between the heart's right atrium, which is
an upper chamber of the heart, and the right
ventricle, a bottom chamber of the heart. The valve
is positioned lower than normal into the right
ventricle instead of remaining between the atrium
and the ventricle. The incorrectly formed ventricle is
too small and the atrium too large, and neither
functions properly. The valve often allows blood to
leak from the ventricle into the atrium. This defect
often occurs along with other heart defects.
 Some people have symptoms early in life,
including heart failure and life-threatening
irregular heartbeats (arrhythmias). Other people
may have no signs or symptoms until adulthood.
Treatment is with medications or with surgery.
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 of the defect allow extra blood to circulate to
the lungs, causing the heart to enlarge.
 The condition occurs most often in children 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.
Truncus arteriosus
 This is a defect in which the normally separate pulmonary
artery and aorta merge into one single large vessel (truncus)
arising from the two bottom chambers of the heart (right and
left ventricles). Many people who have this defect also have
a large ventricular septal defect, which turns the right and left
ventricles into a single chamber. This allows red oxygenated
blood and blue 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 life-threatening pulmonary
hypertension — high blood pressure in the lungs.
 Surgery is needed to close the septal defect with a patch and
to separate the pulmonary arteries from the trunk.
Thank you

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Congenital heart disease

  • 1. Pre by lect: ZAHID REHMAN IPMS(KMU) Congenital heart disease
  • 2. Congenital heart defects are heart problems 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 with several defects. Other types of congenital heart defects are life-threatening, either immediately to the newborn or over time.
  • 3. congenital cardiac malformations resulting from defective embryonic development without obvious cause. Between 3-8 weeks “gestation” all the fetal heart structures are formed “organogenesis” `
  • 4.
  • 5. Majority of cases of the congenital heart diseases are unknown cause
  • 6. Classification Common acyanotic lesions  Ventricular septal defects  Atrial septal defects  Atrio-ventricular septal defects  Patent ductus arteriosus  Truncus arteriosus  Aortic stenosis  Mitral stenosis/incompetence  Coarctation of aorta  Tricuspid regurgitation
  • 7. Common Cyanotic Lesions Tetralogy of fallot (TOF) Transposition of great vessels Truncus arteriiousus Total anomalous pulmonary venous return Hypoplastic left heart syndrome
  • 8. Diagnosis Early diagnosis of C.H.D mean better results. 40% of C.H.D diagnosed at 1st w of life. 50-60 % diagnosed at 1st two months . Others are usually later during routine medical examination. Diagnosis depend on good clinical history + good medical examination and investigations.
  • 9. -History of dysnea on exertion. -Shortness of breathing. -Orthopnea. -Lower limbs swelling -Palpitation. -Convulsion. -All may be manifest of congestive heart failure. Older child
  • 10. Ventricular Septal defects(VSD)  Sometimes called a hole in the heart, this defect — the most common congenital heart defect — occurs when the muscular wall (septum) separating the bottom chambers of the heart (right and left ventricles) doesn't 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. In the right ventricle, the oxygen-rich blood mixes with blood that doesn't have enough oxygen in it.
  • 11.  Single most common congenital heart malformation, accounting for almost 30% of all CHD  Defects can occur in both the membranous portion of the septum (most common) and the muscular portion  Between 80% and 85% of all VSDs  < 3 mm in diameter  All close spontanously  50% by 2 years  90% by 6 years  10% during school years  Muscular close sooner than membranous
  • 12. Ventricular septal defect can lead to Heart failure High blood pressure in the lungs (pulmonary hypertension), Infection of the heart (endocarditis) Irregular heartbeats (arrhythmias) Delayed growth. Small holes may heal on their own or cause no symptoms. Larger holes may require surgery to stitch the hole closed or to cover the hole with a patch.
  • 13.
  • 14. Investigation X-ray chest: ECG Echocardiography The size and location can be assessed by echocardiography. Main pulmonary artery dilatation may be seen.
  • 15. Atrial septal defect  Atrial septal defect is a hole that occurs when the muscular wall (septum) separating the top two chambers of the heart (right and left atria) doesn't close properly. This allows oxygen-rich blood from the left atrium to flow into the right atrium, where it mixes with blood that doesn't have adequate oxygen in it. Blood from the left atrium should normally flow into the left ventricle and on to the aorta and the rest of the body.  Minor cases may cause no symptoms and may not require treatment. Larger defects may require surgery or cardiac catheterization to repair the hole.
  • 16.
  • 17. A large ASD may be associated with growth failure in infants and children. It.s more common in women then in men. Types : There are three types of ASD 1. Ostium secundum ASD (75%) Most common type and result from defect in the region of the fossa ovalis . In this type there is no involment of AV valve.
  • 18. 2. Ostium primum ASD (15%) Ostium primum ASD result from absence of septum primum (at the lower portion of the artrial septum. In this type there is also involvement of AV valves. 3. Sinus venosus defect(10%) Sinus venosus defect is in the upper part of the septum near the entrance of the inferior venacava and coronary sinus there for producing communication between coronary sinus and left atrium.
  • 19. Clinical feature Asymptomatic Exertional dyspnea after 30 age Pts are more prone to developed pulmonary infection Right ventricular failure with large defect and prolonged pulmonary hypertension may develop later in life.
  • 20.  Enlargement of the right ventricle  Enlargement of atrium  Large pulmonary artery  increased pulmonary vascularity is.
  • 21. Patent ductus arteriosus(PDA)  Before birth, a blood vessel called the ductus arteriosus connects the pulmonary artery — the artery carrying blood to the lungs — and the aorta, the large artery that carries blood away from the heart. Before a baby is born, the ductus arteriosus allows blood to bypass the lungs because the baby receives oxygen through the placenta and umbilical cord. The ductus arteriosus normally closes within soon after birth. If the ductus arteriosus remains open (patent), some blood that should flow through the body goes to the lungs.
  • 22.  This defect can cause heart failure or an infection of the heart (endocarditis). In infants, it can be closed with medications. In older children and adults, surgery is performed to close the vessel.  Accounts for about 10% of all cases of CHD  Higher incidence of PDA in infants born at high altitudes (over 10,000 feet)  More common in females
  • 23.
  • 24.
  • 25.  Treatment Medicaly treatment give NSAID which decrease the prostaglandin. Surgery : PDA ligation proceduer.
  • 26. Pulmonary valve stenosis  In this condition, blood flow from one of the heart's bottom chambers, the right ventricle, to the pulmonary artery is slowed by narrowing at the pulmonary valve. When there's narrowing (stenosis), the right ventricle must pump harder to get blood into the artery that carries blood to the lungs (pulmonary artery). Pulmonary valve stenosis may occur along with other defects, such as thickening of the muscle of the right ventricle below the valve.
  • 27.  In many cases, pulmonary valve stenosis is mild and doesn't require treatment. But because it can cause  heart failure  arrhythmias  enlargement of the right heart chambers It may be necessary to correct the defect. In many cases, a balloon (pulmonary valvuloplasty) opens the narrowed valve. In some cases, especially in people with other heart defects, surgery may be necessary to replace the diseased valve with an artificial valve.
  • 28.
  • 29. Aortic stenosis  Aortic stenosis is a defect that narrows or blocks the aortic valve opening, making it difficult for the heart to pump blood into the aorta — the main artery leading away from the heart — on to the rest of the body.  The defect can cause the heart to get bigger  left-sided heart failure  abnormal heart rhythms (arrhythmias),  infections of the heart (endocarditis)  fainting.
  • 30. Treatment: Treatment includes surgery to repair or replace the valve . In young children, widening of the valve through a surgical procedure called balloon valvuloplasty, in which a balloon-like device widens the valve so that blood can flow through.
  • 31.
  • 32. Coarctation of the aorta  This is a narrowing (coarctation) in a portion of the heart's main artery (aorta). Coarctation forces the heart to pump harder to get blood through the aorta and on to the rest of the body.  Coarctation of the aorta can cause several life- threatening complications including  severe high blood pressure  a bulge in the aorta that can burst (aortic aneurysm)  infection of the heart (endocarditis)  brain hemorrhage
  • 33. stroke heart failure Treatment: Repair is typically recommended before age 10, either by surgically removing the affected portion or widening it through a surgical procedure called balloon angioplasty and placement of a mesh tube that can hold the aorta open (stent).
  • 34.
  • 35. Transposition of the great arteries  With this defect, the positions of the aorta — the main artery leading away from the heart — and the pulmonary artery, which leads to the lungs, are reversed (transposed). The aorta and pulmonary artery are, together, sometimes referred to as the great arteries. In transposition of the great arteries, the aorta arises from the right ventricle instead of the left ventricle and the pulmonary artery arises from the left ventricle instead of the right. This prevents nourishing oxygenated blood from reaching the body.
  • 36. 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 allows oxygen-rich blood to get to the body. Surgery to repair the condition is usually necessary shortly after birth.
  • 37.
  • 38. Tetralogy of Fallot  This defect is a combination of four (tetralogy) heart defects. The four defects typically are ventricular septal defect (VSD), pulmonary valve 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 (fuh-LOE) include cyanosis — sometimes called "blue baby syndrome," since the lips, fingers and toes may have a bluish tinge from lack of oxygen.
  • 39. as well as poor eating, physical inability to exercise, irregular heartbeats (arrhythmias), delayed growth and development, and stroke. Surgery to repair the defects is required early in life.
  • 40.
  • 41. Ebstein's anomaly  This is a defect of the tricuspid valve, which controls blood flow between the heart's right atrium, which is an upper chamber of the heart, and the right ventricle, a bottom chamber of the heart. The valve is positioned lower than normal into the right ventricle instead of remaining between the atrium and the ventricle. The incorrectly formed ventricle is too small and the atrium too large, and neither functions properly. The valve often allows blood to leak from the ventricle into the atrium. This defect often occurs along with other heart defects.
  • 42.  Some people have symptoms early in life, including heart failure and life-threatening irregular heartbeats (arrhythmias). Other people may have no signs or symptoms until adulthood. Treatment is with medications or with surgery.
  • 43.
  • 44. 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 of the defect allow extra blood to circulate to the lungs, causing the heart to enlarge.  The condition occurs most often in children 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.
  • 45.
  • 46. Truncus arteriosus  This is a defect in which the normally separate pulmonary artery and aorta merge into one single large vessel (truncus) arising from the two bottom chambers of the heart (right and left ventricles). Many people who have this defect also have a large ventricular septal defect, which turns the right and left ventricles into a single chamber. This allows red oxygenated blood and blue 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 life-threatening pulmonary hypertension — high blood pressure in the lungs.  Surgery is needed to close the septal defect with a patch and to separate the pulmonary arteries from the trunk.
  • 47.