3-year-old suffers from a rare combination of reversed body organs & heart defect

Master Krishna seems like any other active three-year-old. His physical and mental growth is on par with others his age. But he suffered from an extremely rare combination of reversed body organs along with a rare congenital heart defect known as Transposition of Great Arteries (TGA).

Situs Inversus or inversion of the body organ with TGA is extremely rare and a child with TGA living beyond one year is even rarer. Only about 10% of children with TGA survive beyond one year and these are the ones who need to be operated upon as their chances of survival are good. Cardiac specialists at Narayana Multispeciality Hospital, Jaipur treated the patient successfully, in what is reported to be the first such case in Rajasthan.

In Situs Inversus the internal organs in the chest & abdomen are on the opposite side of their normal position. The heart is situated on the right side instead of the left and the liver on the left side instead of the right. In the patient’s case, this was accompanied by a rare heart defect known as Transposition of the Great Arteries (TGA) in which the two major blood vessels coming out of the heart (aorta & pulmonary artery) were also in reversed positions. TGA is a result of abnormal development of the fetal heart during the first 8 weeks of pregnancy.

“While the reversed body organs did not pose any problem to the child as they were functioning effectively even in the reversed positions, the congenital heart defect was leading to breathing difficulties, bluish skin tone & poor weight gain. In such cases, treatment is usually aimed at helping symptoms of a related complication like the heart defect. Surgery to correct the organs’ positioning is usually not recommended” said Dr. Prashant Mahawar, Consultant – Pediatric Cardiology who diagnosed the condition.

Due to the heart defect, the oxygen-rich blood from the lungs was flowing back to the lungs (instead of the body) and oxygen-poor blood from the body was flowing back to the body (instead of the lungs). The two major blood vessels coming out of the heart were in reversed positions and connected to the wrong chambers. Children like Krishna look blue because insufficiently oxygenated blood is circulating in their bodies.

When the patient came to us, he was already three years old. Older children require a technically advanced type of surgery known as the Senning Procedure in which multiple incisions are made at the atria level (blood collecting point of the heart). The atria is sutured & folded in such a way that two tunnels like structures are created within the heart, one over another, which ultimately helps direct the oxygen-rich blood to the body” said Dr. CP Srivastava, HOD & Chief Consultant – Cardiac surgery at Narayana Multispeciality Hospital, Jaipur who performed the complex surgery. Because every structure was in the revere direction, the technique was more difficult added Dr. Srivastava.

The other team members included Dr. Pradeep Goyal, HOD & Sr. Consultant – Anesthesia & Critical Care, and Cardiac Surgeons Dr. Sunil Sharma, Dr. Abhinav Singh and Dr. Amit Sharan. The patient recovered well and was discharged from the hospital within a few days.

“Such cases present unique challenges to the surgeon at the operation table as complex re-construction of specific portions of the heart is involved. It is important that complex cases such as these are performed at specialized centers where the clinical & support team has a lot of experience, as the period of 2-3 days post-surgery is also very crucial for the patient’s recovery” said Dr. Mala Airun, Zonal Clinical Director at Narayana Multispeciality Hospital, Jaipur