Rare Heart Diseases That Affect Children From Birth

Rare Heart Diseases That Affect Children From Birth

Congenital Heart Defect is a condition, which includes a defect in the walls, valves, arteries, and veins of the heart in a newborn child. This imperfection in heart structure can possibly decrease the blood circulation or make it flow in the wrong direction or get completely blocked. This is a rare disease and approximately 9 out of 1000 kids are born every year, with poorly structured heart.

Types of well-known Congenital Heart Defect


• Coarctation of aorta


Aorta is the main and largest blood vessel that transports the blood from the heart to various parts of the body. In Coarctation of aorta, a section of aorta remains constricted or narrow, at birth. It becomes difficult for the heart to pump blood out through the aorta. This heart issue is found in people with a particular genetic disorder like Turner syndrome.

Symptoms


The curved part of aorta points upwards to the head and straight part points downwards. Coarctation of aorta can happen anywhere within the vessel. However, its tendency to occur at the top or aortic arc (C-shaped curve) is more. This makes it hard for blood to go out, so the blood pressure, before the narrowed segment is high, and after the constricted part, is low.


• Headaches from HBP (high blood pressure) 
• Camps in legs 
• Cold legs or feet 
• Abnormal heart sounds 
• Poor weight gain in babies because difficulty in feeding 
• Fainting or dizziness

Symptoms occur only, when the narrowing is severe in the aorta.

Treatment


The narrowed section is repaired, before the child's age turns 10. It can be repaired through balloon angioplasty or open-heart surgery.


• Hypoplastic Left Heart Syndrome (HLHS)


'Hypoplastic' means 'underdeveloped'. In HLHS the heart's left side that includes the left ventricle, aortic valve, mitral valve and aorta do not get developed in the womb. A healthy heart has two ventricles. Right ventricle pumps blood towards the lungs to get oxygenated, whereas the left ventricle pumps the oxygenated blood to the other parts of the body.

In HLHS, the left ventricle is very small to pump blood effectively. Usually, there is a hole in the wall that divides the right ventricle and left ventricle.

Symptoms


In the womb the fetus receives oxygen from the mother's circulation via placenta. In a couple of days, after birth the HLHS symptoms can be seen. This occurs because the placenta is disconnected and the newborn has to depend on its own heart for oxygenated blood circulation.

• Rapid heart beat 
• Rapid breathing 
• Cool, clammy and pale skin 
• Blue color lips and face 
• Weak pulses in arms and legs (because of poor circulation)

Treatment


Infant with HLHS, at birth will be given an IV (intravenous) to enhance blood flow. A series of heart surgeries are performed to reroute the blood in the right ventricle. The right side will perform the functions of both ventricles. Even if possible, a heart transplant surgery can be performed to provide the infant with a well-functioning heart.


• Patent Ductus Arteriosus


In the womb, ductus arteriosus allows the blood to evade the lungs of the baby. After birth as soon as the baby starts to breath, the responsibility of ductus arteriosus gets concluded and it shuts down automatically, in a few days. In certain cases, ductus arteriosus remains open and can cause abnormal blood circulation. This condition is called Patent Ductus Arteriosus. The cause of PDA is still unknown.

Symptoms


• Shortness of breath or rapid breathing 
• Sweating with playing or crying 
• Fast heart rate 
• Tire out easily 
• Poor eating & poor development


Treatment


PDA treatment focuses on closing ductus arteriosus to prevent complications. Closing of PDA is done either through medications, catheter, or surgery.

• Tetralogy of Fallot


A group of 4 heart defects at birth is called Tetralogy of Fallot. The flow of blood gets deteriorated, due to these defects causing a reduction in oxygen level.

The four defects are - 
1. Ventricular septal defect - There is a hole in the muscle wall between right and left side of the heart. 
2. Pulmonary valve stenosis - It is the narrowing of large blood vessel entrance. 
3. Over-riding aorta - Basically, aorta function starts from the left ventricle but in Tetralogy condition it begins from the center of the heart, just over the hole between two sides. 
4. Right ventricular hypertrophy - Due to the upper three heart defects the right side has to overwork, so the heart muscle on that side gets enlarged.

Symptoms


• Bluish tint on the lips, fingernails and skin 
• The tint darkens during activities like crying 
• Abnormal heart murmur 
• While feeding the baby gets tired easily, which hinders the growth 
• Fingertips have abnormal rounded shape

Treatment


Open heart surgery is applied to repair the defects of Tetralogy of Fallot. The pediatric cardiologists continue to check the child regularly, even after surgery. Sometimes, the child may need additional surgery, as they grow up because sudden complications may develop.

• Transposition of great arteries (TGA)


Basically, in normal condition the blood from heart goes to lungs to get oxygenated through pulmonary artery. After it comes back the aorta pumps the oxygenated blood through the body. In TGA, the blood comes to the heart but is pumped out directly without going towards the lungs for getting oxygenated.

Symptoms


• The baby has bluish colored skin 
• Rapid heart rate and breathing


Treatment


The baby receives intravenous medication, shortly after birth. If a hole is present in the hearts mid-wall then a hole will be made surgically. This allows the oxygen to mix with blood that is pumped out. Balloon atrial septostomy technique is used. Although this is a temporary treatment but the baby will require open-heart surgery called arterial switch operation within 4 weeks after birth, to correct the defect.