BLUE BABIES

CLINICAL SERVICES > SURGICAL CORRECTION > CYANOTIC DEFECTS (BLUE BABIES)

Tetralogy of Fallot

As the term tetralogy rightly indicates it is a collection of 4 defects; a large VSD, PS, right ventricular Hypertrophy (increase in the muscle mass of the right lower chamber) and over riding of the Aorta (instead of arising from the left lower chamber, the large vessel is centrally placed between both the left and right lower chambers). Hence both the pure blood from the left lower chamber and the impure blood from the right lower chamber enter the Aorta making the child blue. It is a relatively common defect and thanks to the advances in surgical techniques it can be completely corrected by open heart repair.

Defects

Normal Heart

The human heart has four chambers; the right upper chamber receives the impure blood from the body, which is then pumped into the lungs by the right lower chamber. The blood purified by the lungs now returns to the left upper chamber and is pumped to rest of the body by the left lower chamber. The vessel that carries the impure blood from the right side to the lungs is called the Pulmonary Artery and the vessel carrying the pure blood to the body from the left side is called the Aorta. Normally though blood moves freely from the upper to the lower chambers on the same side of the heart, there is no mixing between the right and the left sides.

CLINICAL SERVICES > SURGICAL CORRECTION > CYANOTIC DEFECTS
(BLUE BABIES)

Transposition of the Great Vessels (TGA):

In this condition the aorta arises from the right lower chamber thereby carrying the impure blood back to the body and the pulmonary artery arises from the left side carrying the purified blood back to the lungs. In order for these children to survive, there has to be some connection between the right and left sides(ASD,VSD,PDA). This allows some of the pure blood from the left to enter the right and hence be pumped to the body. These children are usually born very blue.The initial lifesaving procedure in these children is a balloon septostomy which is then followed by an arterial switch operation.

Defects

CLINICAL SERVICES > SURGICAL CORRECTION > CYANOTIC DEFECTS
(BLUE BABIES)

Total Anomalous Pulmonary Venous Return (TAPVR):

In this congenital anomaly the four veins which carry the pure blood from the lungs, instead of entering into the left upper chamber join together and continue to empty into the right upper chamber either directly or by emptying into one of the great veins (SVC or IVC). Usually in children with this abnormality there is an associated ASD so that the mixed pure and impure blood from the right upper chamber can flow to the left upper chamber through the ASD and get pumped to the rest of the body sustaining life. Surgical correction involves prompt implantation of these Pulmonary veins back into the left upper chamber. Urgency for the surgery will depend on the size of the ASD.

Defects

Normal Heart:

The human heart has four chambers; the right upper chamber receives the impure blood from the body, (via IVC and SVC) which is then pumped into the lungs by the right lower chamber. The blood purified by the lungs is now brought by four Pulmonary veins, two from each side, to the left upper chamber and is pumped to rest of the body by the left lower chamber.

CLINICAL SERVICES > SURGICAL CORRECTION > CYANOTIC DEFECTS
(BLUE BABIES)

DORV

In this condition both these great vessels i.e. the Aorta and the Pulmonary Artery arise from the right lower chamber. The left lower chamber having no outlet empties the pure blood through the VSD into the right lower chamber where it mixes with the impure blood and pumped out by both the aorta and pulmonary artery making the child blue, a highly complex disorder which needs early correction. The mouth of the VSD is surgically connected to the mouth of the Aorta arising from the right side by a tubular conduit, allowing the pure blood from the left lower chamber to directly flow into the Aorta without mixing with the impure blood of the right lower chamber.

Defects

CLINICAL SERVICES > SURGICAL CORRECTION > CYANOTIC DEFECTS
(BLUE BABIES)

Tricuspid Atresia

In this rare anomaly the valve between the right upper and lower chambers (Tricuspid) does not develop and is like a thin fibrous sheet, thereby not allowing the impure blood from the right upper chamber to enter the right lower chamber. The impure blood that collects in the right upper chamber crosses over to the left side through the ASD and enters the left lower chamber mixing with the pure blood of the left side. This mixed blood is pumped to the lungs (entering the pulmonary artery through the VSD) and the body, hence making the child blue. The right lower chamber is almost always underdeveloped and small in this condition. The survival of children with this disorder depends on the size of the ASD, size of the VSD and the size of the Pulmonary artery. Some infants may require immediate palliative surgery to sustain life while others can be directly taken up for corrective procedures like the “FONTAN” wherein the SVC & IVC ( vessels that bring the impure blood to the right upper chamber) are connected directly to the pulmonary artery diverting the impure blood straight into the lung.

Defects

Normal Heart

The human heart has four chambers; the right upper chamber receives the impure blood from the body, which then flows down to the muscular right lower chamber from where it is pumped to the lungs. The blood purified by the lungs now returns to the left upper chamber and is pumped to rest of the body by the left lower chamber. Hence, blood moves freely from the upper to the lower chambers in one direction only, because of the tricuspid valve on the right side and the mitral valve on the left side.

CLINICAL SERVICES > SURGICAL CORRECTION > CYANOTIC DEFECTS
(BLUE BABIES)

Common Atrium

The Atrial Septum is the wall separating both the upper chambers of the heart (The Atria). If this partition is completely absent due to faulty development, then both the upper chambers become one common big chamber where the impure blood from the right and the pure blood from the left thoroughly mix. This mixed blood flows into the lower chambers and is pumped to the body and lungs alike making the child blue. A partition between two chambers has to be surgically created to return the children to normalcy.

Defects

CLINICAL SERVICES > SURGICAL CORRECTION > CYANOTIC DEFECTS
(BLUE BABIES)

Truncus Arteriosus

As the fetus develops failure of these vessels to separate due to defective embryogenesis leaves a single large vessel called the Truncus Arteriosus supplying both the body and the lungs. This large vessel is placed centrally between both the lower chambers and there is always an associated VSD. The pure blood from the left lower chamber and the impure blood from the right lower chamber converge and flow into this vessel making the child blue. Open heart surgery aimed at separating both the vessels should be carried out as early in life as possible.

Defects

Normal heart

The human heart has four chambers; the right upper chamber receives the impure blood from the body, which is then pumped into the lungs by the right lower chamber. The blood purified by the lungs now returns to the left upper chamber and is pumped to rest of the body by the left lower chamber. The vessel that carries the impure blood from the right lower chamber to the lungs is called the Pulmonary Artery and the vessel carrying the pure blood to the entire body from the left lower chamber is called the Aorta. Early in the development of the heart, both these vessels i.e. the Aorta and the Pulmonary Artery were together as a single vessel.

CLINICAL SERVICES > SURGICAL CORRECTION > CYANOTIC DEFECTS
(BLUE BABIES)

Single Ventricle

If one of the two muscular lower chambers fails to develop then both the upper chambers open into the other well developed lower chamber. The Aorta and the Pulmonary Artery are also both attached to the same developed lower chamber. The pure and impure blood from the left and right upper chambers respectively mix in this common lower chamber ”The Single Ventricle” before being pumped to the body and lungs alike making the child blue. This is a very complex defect indeed which needs staged surgical correction.

Defects

Normal Heart

The human heart has four chambers; the right upper chamber receives the impure blood from the body, which then flows down to the muscular right lower chamber from where it is pumped to the lungs. The blood purified by the lungs now returns to the left upper chamber and is pumped to rest of the body by the left lower chamber. The vessel that carries the impure blood from the right lower chamber to the lungs is called the Pulmonary Artery and the vessel carrying the pure blood to the entire body from the left lower chamber is called the Aorta.

CLINICAL SERVICES > SURGICAL CORRECTION > CYANOTIC DEFECTS (BLUE BABIES)

Hypoplastic Left Heart Syndrome (HLHS)

In this rare disorder the entire left side of the heart fails to develop. Hence there is a thin underdeveloped Aorta, an imperforate sheet like mitral valve and an underdeveloped left lower chamber. The pure blood from the left upper chamber has to go to the right upper chamber through the ASD, then the right lower chamber, pulmonary artery and supply the entire body by entering the aorta through the PDA. Hence life can only be sustained if both these defects i.e. ASDand PDA are also present. A very complicated disorder indeed that requires staged palliative procedures just to sustain life.

Defects

CLINICAL SERVICES > SURGICAL CORRECTION > CYANOTIC DEFECTS (BLUE BABIES)

A.V.Canal Defects

This is indeed a very complex disorder in which there is a large gap in this muscular partition creating both an ASD and a VSD. Also the valves between the upper and lower chambers show varying degrees of are mal development rendering them incompetent and leaky. All this allowing mixing of the pure and impure blood and making the child eventually blue. Prompt surgical intervention is essential in treating this condition. Intracardiac repair can close the defective hole and correct the valvular deformity.

Defects

Normal Heart

The human heart has four chambers; the right upper chamber receives the impure blood from the body, which is then pumped into the lungs by the right lower chamber. The blood purified by the lungs now returns to the left upper chamber and is pumped to rest of the body by the left lower chamber. Hence, though blood moves freely between the upper and lower chambers on the same side of the heart, there is no mixing between the right and the left because of a muscular partition that separates both the sides.

CLINICAL SERVICES > SURGICAL CORRECTION > CYANOTIC DEFECTS (BLUE BABIES)

Pulmonary Atresia
In this uncommon condition the pulmonary valve does not develop and is like a thin fibrous sheet, thereby not allowing the impure blood from the right lower chamber to enter the lungs. Pulmonary Atresia is almost always associated with a large VSD and a PDA. The impure blood from the right lower chamber now enters the centrally placed Aorta along with the pure blood from the left lower chamber making the child blue. This mixed blood feeds the body and goes to the lungs through the PDA.

Defects

Normal Heart
The human heart has four chambers; the right upper chamber receives the impure blood from the body, which then flows down to the muscular right lower chamber from where it is pumped to the lungs. The blood purified by the lungs now returns to the left upper chamber and is pumped to rest of the body by the left lower chamber.The vessel that carries the pure blood to the body from the left side is called the Aorta and the vessel carrying the impure blood from the right side to the lungs is called the Pulmonary Artery. The Pulmonary valve is present in the very beginning of the Pulmonary Artery and its main function is to prevent the blood pumped into the vessel from flowing back into the heart.