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CYANOTIC HEART DEFECTS

1. TRANSPOSITION OF THE GREAT ARTERIES (TGA)


Transposition of the great vessels is a congenital heart defect in which the two major vessels that carry blood away from the heart -- the aorta and the pulmonary artery -- are switched (transposed).

In transposition of the great arteries, the aorta is connected to the right ventricle, and the pulmonary artery is connected to the left ventricle - the exact opposite of a normal heart's anatomy.
Oxygen-poor (blue) blood returns to the right atrium from the body, passes through the right atrium and ventricle, then goes into the misconnected aorta back to the body. Because of the low amount of oxygen provided to the body, TGA is a heart problem that is labeled "blue-baby syndrome."

CAUSES :
The cause of most congenital heart defects is unknown. Factors in the mother that may increase the risk of this condition include: Age over 40 Alcoholism Diabetes Poor nutrition during pregnancy (prenatal nutrition) Rubella or other viral illness during pregnancy

SYMPTOMS :
The new born becomes cyanotic in the transitional first day of life when the maternal source of oxygen (from the placenta) is removed Rapid breathing Labored Breathing Rapid Heart Rate Cool, Clammy Skin

DIAGNOSIS
Chest x-ray

Electrocardiogram (ECG or EKG)

Echocardiogram (echo)

Cardiac catheterization

TREATMENT
Baloon atrial septosomy as a part of cardiac catheterization is done. Within the first 1 to 2 weeks of age, transposition of the great arteries is surgically repaired. The procedure that accomplishes this is called a "switch," which roughly describes the surgical process. The operation is performed under general anesthesia, and involves the following: o The aorta is moved from the right ventricle to its normal position over the left ventricle. o The pulmonary artery is moved from the left ventricle to its normal position over the right ventricle. o The coronary arteries are moved so they will originate from the aorta and take oxygen-rich (red) blood to the heart muscle. o Other defects, such as atrial or ventricular septal defects or a patent ductus arteriosus, are commonly closed.

2. TETRALOGY OF FALLOT
Tetralogy of Fallot is classified as a cyanotic heart defect because the condition causes low oxygen levels in the blood. This leads to cyanosis (a bluish-purple color to the skin).

The classic form of tetralogy includes four related defects of the heart and its major blood vessels:

Ventricular septal defect (hole between the right and left ventricles) Narrowing of the pulmonary outflow tract (the valve and artery that connect the heart with the lungs) Overriding aorta (the artery that carries oxygen-rich blood to the body) that is shifted over the right ventricle and ventricular septal defect, instead of coming out only from the left ventricle A thickened muscular wall of the right ventricle (right ventricular hypertrophy)

Causes, Risk factors : Factors that increase the risk for this condition during pregnancy include: Alcoholism in the mother Diabetes Mother who is over 40 years old Poor nutrition during pregnancy Rubella or other viral illnesses during pregnancy

SYMPTOMS :
Cyanosis, which gets worse when the baby is upset. Clubbing of fingers Difficulty feeding (poor feeding habits) Failure to gain weight Passing out Poor development Squatting during episodes of cyanosis

DIAGNOSIS :
Chest x-ray Complete blood count (CBC) Echocardiogram Electrocardiogram (EKG) MRI of the heart (generally after surgery)

TREATMENT :
Surgery to repair tetralogy of Fallot is done when the infant is very young. Sometimes more than one surgery is needed. When more than one surgery is used, the first surgery is done to help increase blood flow to the lungs.

COMPLICATIONS :
Delayed growth and development Irregular heart rhythms (arrhythmias) Seizures during periods when there is not enough oxygen Death

3. TRUNCUS ARTERIOSUS
Truncus arteriosus is a rare type of congenital heart disease, in which a single blood vessel (truncus arteriosus) comes out of the right and left ventricles, instead of the normal two vessels (pulmonary artery and aorta).

If this condition is not treated, two problems occur: Too much blood circulation in the lungs may cause extra fluid to build up in and around them, making it difficult to breathe. The blood vessels to the lungs become permanently damaged. Over time, it becomes very hard for the heart to force blood to them. This is called pulmonary hypertension and it can be life-threatening.

SYMPTOMS : Bluish skin (cyanosis) Delayed growth or growth failure Fatigue Lethargy Poor feeding Rapid breathing (tachypnea) Shortness of breath (dyspnea) Widening of the finger tips (clubbing)

DIAGNOSIS
A murmur is usually heard when listening to the heart with a stethoscope. Tests include: o ECG o Echocardiogram o Heart catheterization (only needed in rare cases to help with the diagnosis or in planning a treatment strategy) o MRI of the heart o X-ray of the chest

TREATMENT
Surgery is needed to treat this condition. The surgery creates two separate arteries. Usually the truncal vessel is kept as the new aorta. A new pulmonary artery is created using tissue from another source. The branch pulmonary arteries are sewn to this new artery. The hole between the ventricles is closed.

COMPLICATIONS :
Heart failure High blood pressure in the lungs (pulmonary hypertension) with pulmonary obstructive lung disease

4. TOTAL ANOMALOUS PULMONARY VENOUS RETURN


Total anomalous pulmonary venous return is a congenital heart disease in which none of the four veins that take blood from the lungs to the heart is attached to the left atrium (left upper chamber of the heart).

CAUSE :

The cause of total anomalous pulmonary venous return (TAPVR) is unknown. In TAPVR, oxygen-rich blood returns from the lungs to the right atrium or to a vein flowing into the right atrium, instead of the left side of heart. In other words, blood simply circles to and from the lungs and never gets out to the body.

SYMPTOMS
The infant may appear very sick and may have the following symptoms: Bluish color of the skin (cyanosis) Frequent respiratory infections Lethargy Poor feeding Poor growth Rapid breathing Note: Sometimes, no symptoms may be present in infancy or early childhood.

DIAGNOSIS :
Cardiac catheterization can confirm the diagnosis by showing that the blood vessels are abnormally attached ECG shows enlargement of the ventricles (ventricular hypertrophy) Echocardiogram may show that the pulmonary vessels are attached MRI of the heart can show the connections between the pulmonary vessels X-ray of the chest shows a normal to small heart with fluid in the lungs

TREATMENT
Surgery to repair the problem is needed as soon as possible. In surgery, the pulmonary veins are connected to the left atrium and the defect between the right and left atrium is closed.

COMPLICATION :
Breathing difficulties Heart failure Irregular, fast heart rhythms (arrhythmias) Lung infections Pulmonary hypertension