About 8-10 babies out of every thousand born, have some form of congenital heart defect, some simple and some complex and life-threatening.
Out of this, 5-7% of the patients have transposition of great arteries (TGA) which is a serious of heart disease wherein the baby is very blue at birth itself and requires immediate treatment mostly by one month of age.
In a normal heart, one large tube called the pulmonary artery carries all the blood from the right side (which is low in oxygen content) to the lungs where it derives its oxygen and flows back to the left side of the heart. From the left side, another large tube called the aorta arises that carries all the blood rich in oxygen content to the body for further use. Normally, there is no communication between the left and right side of the heart to allow any kind of mixing between this deoxygenated (blood with less oxygen) and oxygenated (blood rich in oxygen) blood.
In TGA, the left and right-sided large tubes get interchanged so that the pulmonary artery arises from the left side and the aorta arises from the right side. In such a situation, the deoxygenated blood instead of going to the lungs for purification returns back to the body and oxygenated blood from the left side recirculates to the lungs. Such babies are born with a small hole between the upper two chambers of the heart through which some amount of oxygenated and deoxygenated blood mixes and hence the body receives a very minimal amount of oxygen enough to maintain survival. However, such a small hole becomes insufficient very soon after birth.
Normally, such babies undergo a major heart surgery called “arterial switch operation” at 3-4 weeks of life wherein the large blood carrying tubes from the right and left side are cut and put back in position. Some babies who have missed this train undergo another type of surgery for normalizing the blood circulation called “Sennings operation” at 5-6 months of age.
However for babies to remain alive till this stage, a life-saving intervention called “Balloon Atrial Septostomy (BAS)” is performed during the first month of life.
In this procedure, a balloon catheter is taken inside the heart through a big vein of the leg and a hole is created between the upper two chambers of the heart enabling good amount of oxygen to reach the body. This reduces the blueness of the baby and allows for survival until he/she receives a final open-heart surgery.
The procedure is life-saving but carries a higher risk than usual interventions since it is performed on a newborn baby whose other organ systems are immature, veins and arteries are small and most importantly, such babies are almost always very sick with very low oxygen in the body. Giving general anaesthesia to such deeply blue newborn babies also carries a higher risk.
This procedure is generally recommended for babies between 1-2 weeks of life. However, in suitable patients, we perform this procedure up to 2 months of age since a lot of babies are presented very late in sick condition. They require emergency stabilization before going for a major open-heart operation.
To date, we have performed around 7 such procedures in babies with TGA with the lowest weight being 2 kgs. Age of these children varies from 4 days to 2 ½ months.
Awareness and early detection of this heart disease preferably in intrauterine life itself through foetal echocardiography will yield better results and save a lot of babies with this life-threatening heart disease offering useful citizens for the nation.
This was a 2 months old boy, weight: 3.25kg named Master Badal. Diagnosed as TGA, IVS just 3 days prior. Basal’s saturation was 40% on room air. Was taken for emergency BAS at 6 pm. The pinhole opening between the upper chambers (PFO) was enlarged up to 5mm (ASD). After the procedure, the saturation increased to 75%. He was discharged on the 2nd day and is planned for a corrective surgery at about 6 months of age. Till then we will follow the child every month.
Dr. Sumanta Shekhar Padhi (MD, DM, PFD), Consultant, Cardiology – Adult, MMI Narayana Multispeciality Hospital, Raipur
