ANOMALIES OF HEART

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.

Causes

The cause of congenital heart disease may be

either genetic or environmental, but is usually

a combination of both.

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.

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

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.

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.

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.

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)

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)

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.

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.

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.

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.

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.

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.

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.

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.

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.

Hypoplastic right heart syndrome

Hypoplastic right

heart syndrome is a

congenital heart

defect in which the

right atrium and right

ventricle are

underdeveloped.

Mitral valve stenosis

Mitral stenosis is a valvular heart disease

characterized by the narrowing of the orifice of

the mitral valve of the heart.

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.

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.

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.

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.

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.

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.

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.

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.

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.