A five-day-old baby with G6PD deficiency – a disorder caused by an abnormal X-chromosome gene, successfully underwent a complex open-heart surgery recently.
According to medical literature, till date, only two paediatric cardiac cases were associated with the disorder have been successfully treated.
Dr Vijay Agarwal, chief paediatric cardiac surgeon, at Fortis Hospital, said, “The child had turned blue due to lack of oxygen supply in his body. His tests showed that he had transposition of great arteries that required immediate surgery if we want to save him.”
Luckily, Dr Agarwal said the hospital where the child was born had done a battery of tests as a part of their routine which revealed that he was suffering from G6PD.
“It was a crucial piece of information that helped us in taking the right measures. In G6PD patients, you have to avoid certain medications that may lead to heavy bleeding and death.
Therefore, it was the biggest challenge in front us,” said Dr Agarwal.
Dr Agarwal added that apart from the G6PD disorder the child also had a single coronary artery instead of two. “Usually in open-heart surgery we need to cool the child below 30 degree Celsius to protect his organs. In his case, it was not possible as that would have led to severe bleeding because of his disorder,” said Dr Agarwal.
Taking help of medical literature, the team of doctors took measures for all possible risk associated with the surgery. The surgery lasted for seven hours. “We avoided the general drugs used in anaesthesia. We maintained the cooling temperature at 33 degree Celsius. Luckily, everything fell in place and the operation was successful. The child had an unusual combination of problems. Moreover, the child weighed only 2.4kg and had jaundice at birth. He has still managed to survive and we are happy that he is now fit to be discharged,” said Dr Agarwal. The team of doctors will be presenting the case in international conferences because of its uniqueness.
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