6-day-old with rare heart defect struggling to survive

“It’s a miracle and challenge for doctors. Every extra day he survives gives us an opportunity to deal with the rare disease and we are learning a lot from the case. We are doing our best to ensure the child lives,” stated A.K. Bisoi, a cardiologist and additional professor at the Cardiothoracic and Vascular Surgery Department of AIIMS.

The baby of Chandar Majhi, a labourer from Parihar in Bihar is suffering from an ailment known as thoracic ectopia cordis, a malformation that can be detected within 3-5 weeks of pregnancy through ultrasound and foetal echo cardiogram. The survival rate in these cases is extremely poor.

"My son was born on August 26 in Krishnadevi Deviprasad Kejriwal Maternity Hospital through a caesarean. My wife Vibha didn't get any ultrasound done in the first three months of her pregnancy and we found out about some abnormality only in the last month as she started complaining of pain.

“But I wasn't told the child would have a defect. Doctors in Muzaffarpur were amazed to see how my baby survived. He is a fighter and the only way to save him was to bring him to AIIMS,'' said Chandar.

The different stages of treatment
Besides the many complexities involved in the case, the infant showed added complications of dehydration and acute infection as a consequence of the 20 hours of train journey he had endured.

In the first phase of treatment, his condition was closely monitored and his heart covered with a synthetic membrane. Next, the infected blood from his body was successfully replaced with fresh sterile blood. A series of tests were conducted which showed the child was immune-deficient.

To carry out the procedure, his chest wall needs to be reconstructed in order to make space to accommodate his heart, said Dr Bisoi. “In subsequent stages, we will put the heart back in its natural location. We have to see how the baby responds to each stage of treatment,” he added.

Doctors skeptical about type of surgery
Since the infant has poor immunity, the doctors are skeptical whether he will be able to survive multiple stages of surgery. Although nothing has been finalized yet, the doctors are contemplating a one-go operation to ease the heart in the chest cavity.

“The baby is alright and we will perform a second exchange blood transfusion on the baby Wednesday to rule out any infection. We may do a cardiac surgery upon him Thursday,” stated Bisoi.

If the doctors opt for single surgery, the child will be put on a heart-and-lung machine and his blood will be cooled down to 18 degree Celsius.

"He will be in total circulatory arrest state for 30 minutes, in which we will fix his heart. But it is a very risky procedure. That is why our team will evaluate the two surgeries and then take a decision,'' explained Dr Bisoi.