Congenital defects of heart

1. ANOMALIES OF HEART

2. Congenital heart defect
Congenital heart defect (CHD) or congenital heart
anomaly is a defect in the structure of the heart and
great vessels that is present at birth. Many types of
heart defects exist, most of which either obstruct blood
flow in the heart or vessels near it, or cause blood to
flow through the heart in an abnormal pattern. Other
defects, such as long QT syndrome, affect the heart's
rhythm. Heart defects are among the most common
birth defects and are the leading cause of birth defect-related
deaths. Approximately 9 people in 1000 are born
with a congenital heart defect. Many defects do not
need treatment, but some complex congenital heart
defects require medication or surgery.

3. Causes
The cause of congenital heart disease may be
either genetic or environmental, but is usually
a combination of both.

4. Classification
A number of classification systems exist for
congenital heart defects. In 2000 the
International Congenital Heart Surgery
Nomenclature was developed to provide a
generic classification system.

5. Hypoplasia
Hypoplasia can affect the heart, typically resulting in the
underdevelopment of the right ventricle or the left ventricle. This
causes only one side of the heart to be capable of pumping blood
to the body and lungs effectively. Hypoplasia of the heart is rare
but is the most serious form of CHD. It is called hypoplastic left
heart syndrome when it affects the left side of the heart and
hypoplastic right heart syndrome when it affects the right side of
the heart. In both conditions, the presence of a patent ductus
arteriosus (and, when hypoplasia affects the right side of the heart,
a patent foramen ovale) is vital to the infant's ability to survive until
emergency heart surgery can be performed, since without these
pathways blood cannot circulate to the body (or lungs, depending
on which side of the heart is defective). Hypoplasia of the heart is
generally a cyanotic heart defect

6. Obstruction defects
Obstruction defects occur when heart valves,
arteries, or veins are abnormally narrow or
blocked. Common defects include pulmonic
stenosis, aortic stenosis, and coarctation of the
aorta, with other types such as bicuspid aortic
valve stenosis and subaortic stenosis being
comparatively rare. Any narrowing or blockage
can cause heart enlargement or hypertension.

7. Septal defects
The septum is a wall of tissue which separates the
left heart from the right heart. Defects in the
interatrial septum or the interventricular septum
allow blood to flow from the right side of the heart to
the left, reducing the heart's efficiency.Ventricular
septal defects are collectively the most common type
of CHD, although approximately 30% of adults have
a type of atrial septal defect called probe patent
foramen ovale.

8. Cyanotic defects
Cyanotic heart defects are called such because
they result in cyanosis, a bluish-grey
discoloration of the skin due to a lack of
oxygen in the body. Such defects include
persistent truncus arteriosus, total anomalous
pulmonary venous connection, tetralogy of
Fallot, transposition of the great vessels, and
tricuspid atresia.

9. Defects
1. Aortic stenosis
2. Atrial septal defect (ASD)
3. Atrioventricular septal defect (AVSD)
4. Bicuspid aortic valve
5. Dextrocardia
6. Double inlet left ventricle (DILV)
7. Double outlet right ventricle (DORV)
8. Ebstein's anomaly
9. Hypoplastic left heart syndrome (HLHS)
10.Hypoplastic right heart syndrome (HRHS)

10. 11. Mitral stenosis
12. Pulmonary atresia
13.Pulmonary stenosis
14.Transposition of the great vessels
15. dextro-Transposition of the great arteries (d-
TGA)
16. levo-Transposition of the great arteries (l-TGA)
17.Tricuspid atresia
18.Persistent truncus arteriosus
19.Ventricular septal defect (VSD)
20.Wolff-Parkinson-White syndrome (WPW)

11. Aortic valve stenosis
Aortic valve stenosis (AS) is a disease of the
heart valves in which the opening of the
aortic valve is narrowed. The aortic valve is
the valve located between the left ventricle of
the heart and the aorta, the largest artery in
the body, which carries the entire output of
blood to the systemic circulation.

13. Atrial septal defect
Atrial septal defect (ASD) is a form of a congenital heart
defect that lets blood flow between the normally
separated two upper chambers, the atria of the heart.
The atria are separated by a dividing wall, the interatrial
septum. If this septum is defective or absent, then
oxygen-rich blood can flow directly from the left side of
the heart to mix with the oxygen-poor blood in the right
side of the heart, or vice versa. This can lead to lower-than-
normal oxygen levels in the arterial blood that
supplies the brain, organs, and tissues.

15. Atrioventricular septal defect
Atrioventricular septal defect (AVSD) or
atrioventricular canal defect (AVCD), previously
known as "common atrioventricular canal"
(CAVC) or "endocardial cushion defect", is
characterized by a deficiency of the
atrioventricular septum of the heart. It is caused
by an abnormal or inadequate fusion of the
superior and inferior endocardial cushions with
the mid portion of the atrial septum and the
muscular portion of the ventricular septum.

17. Bicuspid aortic valve
A bicuspid aortic valve (BAV) is most commonly a
congenital condition of the aortic valve where two of the
aortic valvular leaflets fuse during development resulting
in a valve that is bicuspid instead of the normal tricuspid
configuration. Normally the only cardiac valve that is
bicuspid is the mitral valve (bicuspid valve) which is
situated between the left atrium and left ventricle.
Cardiac valves play a crucial role in ensuring the
unidirectional flow of blood from the atrium to the
ventricles, or the ventricle to the aorta or pulmonary
trunk.

19. Dextrocardia
Dextrocardia is a congenital defect in which the
apex of the heart is situated on the right side of
the body. There are two main types of
dextrocardia: dextrocardia of embryonic arrest
(also known as isolated dextrocardia[citation
needed]) and dextrocardia situs inversus.

21. Double inlet left ventricle
A double inlet left ventricle (DILV) or
"single ventricle", is a congenital heart
defect appearing in 5 in 100,000 newborns,
where both the left atrium and the right
atrium feed into the left ventricle. The right
ventricle is hypoplastic or doesn't exist.

23. Double outlet right ventricle
Double outlet right ventricle (DORV) is a form of
congenital heart disease where both of the great
arteries connect (in whole or in part) to the right
ventricle (RV). In some cases it is found that this
occurs on the left side of the heart rather than the
right side.

25. Ebstein's anomaly
Ebstein's anomaly is a congenital heart
defect in which the septal and posterior
leaflets of the tricuspid valve are
displaced towards the apex of the right
ventricle of the heart.

27. Hypoplastic left heart syndrome
Hypoplastic left heart syndrome (HLHS) is
a rare congenital heart defect in which the
left ventricle of the heart is severely
underdeveloped.

29. Hypoplastic right heart syndrome
Hypoplastic right
heart syndrome is a
congenital heart
defect in which the
right atrium and right
ventricle are
underdeveloped.

30. Mitral valve stenosis
Mitral stenosis is a valvular heart disease
characterized by the narrowing of the orifice of
the mitral valve of the heart.

31. Pulmonary atresia
Pulmonary atresia is a congenital malformation
of the pulmonary valve in which the valve orifice
fails to develop. The valve is completely closed
thereby obstructing the outflow of blood from
the heart to the lungs. The pulmonary valve is
located on the right side of the heart between
the right ventricle and pulmonary artery.

33. Pulmonic stenosis
Pulmonic stenosis, also known as pulmonary
stenosis, is a dynamic or fixed obstruction of
flow from the right ventricle of the heart to the
pulmonary artery. It is usually first diagnosed in
childhood. Pulmonic stenosis is usually due to
isolated valvular obstruction (Pulmonary valve
stenosis), but may be due to subvalvular or
supravalvular obstruction. It may occur in
association with more complicated congenital
heart disorders.

35. dextro-Transposition of the great arteries
Dextro-Transposition of the great arteries
(d-Transposition of the great arteries,
dextro-TGA, or d-TGA), sometimes also
referred to as complete transposition of the
great arteries, is a birth defect in the large
arteries of the heart. The primary arteries
(the aorta and the pulmonary artery) are
transposed.

37. levo-Transposition of the great arteries
levo-Transposition of the great arteries (L-Transposition
of the great arteries, levo-TGA, or l-TGA), also
commonly referred to as congenitally corrected
transposition of the great arteries (CC-TGA), is an
acyanotic congenital heart defect (CHD) in which the
primary arteries (the aorta and the pulmonary artery) are
transposed, with the aorta anterior and to the left of the
pulmonary artery; the morphological left and right
ventricles are also transposed.

39. Total Anomalous Pulmonary Venous
Return (TAPVR)
The pulmonary veins are the four blood vessels (two
on each side) that return oxygen-rich blood from the
lungs to the left atrium of the heart.
Total anomalous pulmonary venous return (TAPVR)
is a rare congenital malformation in which all four
pulmonary veins do not connect normally to the left
atrium. Instead the four pulmonary veins drain
abnormally to the right atrium by way of an abnormal
(anomalous) connection.

41. Tricuspid atresia
Tricuspid atresia is a form of congenital heart
disease whereby there is a complete absence
of the tricuspid valve. Therefore, there is an
absence of right atrioventricular connection.
This leads to a hypoplastic (undersized) or
absent right ventricle. This defect is contracted
during prenatal development, when the heart
does not finish developing. It causes the heart
to be unable to properly oxygenate the rest of
the blood in the body.

43. Persistent truncus arteriosus
Persistent truncus arteriosus (or patent truncus
arteriosus), also known as Common arterial
trunk, is a rare form of congenital heart disease
that presents at birth. In this condition, the
embryological structure known as the truncus
arteriosus fails to properly divide into the
pulmonary trunk and aorta. This results in one
arterial trunk arising from the heart and
providing mixed blood to the coronary arteries,
pulmonary arteries, and systemic circulation.

45. Ventricular septal defect
A ventricular septal defect (VSD) is a defect
in the ventricular septum, the wall dividing
the left and right ventricles of the heart.
The ventricular septum consists of an
inferior muscular and superior membranous
portion and is extensively innervated with
conducting cardiomyocytes.

47. Wolff–Parkinson–White syndrome
Wolff–Parkinson–White syndrome (WPW) is one of
several disorders of the conduction system of the heart
that are commonly referred to as pre-excitation
syndromes. WPW is caused by the presence of an
abnormal accessory electrical conduction pathway
between the atria and the ventricles. Electrical signals
traveling down this abnormal pathway (known as the
bundle of Kent) may stimulate the ventricles to contract
prematurely, resulting in a unique type of supraventricular
tachycardia referred to as an atrioventricular reciprocating
tachycardia.