Baby Emmanuel was born to bring joy to the world around him, his parents and family. But barely had the family held him in the crook of their arms than they saw him slipping out of their grasp as he was diagnosed with a rare heart defect. His parents carried him from one doctor to another, waited at clinics for a cure till the day he turned blue and they rushed him to Indraprastha Apollo Hospital in Delhi. There was only one way to save him, correct his defect through an open heart surgery. He was just six months old. Today, another month older, he is learning to flip and sit up as he probes the world.
What Emmanuel Lila Kamank, a Kenyan by birth, had was a rare cyanotic congenital heart defect known as Taussig-Bing anomaly. Explains Dr Muthu Jothi, Senior Consultant and Paediatric Cardiothoracic surgeon, “When Emmanuel came to Apollo, he was already cyanotic, which means that he had bluish discoloration of skin due to lack of oxygen in his blood. On examination, it was found that his respiratory rate was below normal at 20 breaths per minute. At the hospital, he suddenly underwent respiratory arrest. The attack was so severe that the child also had a cardiac arrest. His condition was pretty dire due to which he was put on ventilator and shifted to cardiac ICU.”
Dr Jothi elaborated the criticality of the baby’s condition: “The child was already diagnosed with Taussig-Bing anomaly, which is a malformation of the heart in which there is transposition of aorta to the right ventricle which in normal case should originate from the left ventricle. At the same time, his pulmonary artery was also wrongly positioned into the right ventricle. This is called double outlet right ventricle defect. Upon further investigation, we found out that there was also an interrupted aortic arch (block in the aorta). On top of that, the child had large subpulmonary ventricular septal defect (VSD), atrial septal defect (ASD), and Patent ductus arteriosus (PDA), wherein ductus arteriosus, the blood vessel that is open when the child is inside the mother’s womb, does not close. In addition to all this, he also had a single coronary origin giving rise to right and left coronary arteries. The right coronary had a part inside the wall of the aorta which we call as intramural coronary artery. This makes it very difficult technically to relocate the coronary arteries into the new aorta. Needless to say, it was very high risk case with limited chances of success even with surgery. We devised a treatment plan for the baby, but informed his family about the 50-60% surgical risk associated with it. The family consented to the high risk and we decided to go ahead with the procedure.”
On January 21, Dr Jothi and his team, consisting of Dr Manisha Chakrabarti, Senior Consultant, Paediatric cardiologistand Dr Reetesh Gupta, Senior Consultant, Paediatric intensivist, shared notes and attempted what would be a unique challenge to their established abilities. And they had to do it within a time limit or risk losing the baby forever. Explaining the complexity of the surgery, Dr. Muthu Jothi said, “The procedure was done under total circulatory arrest, which means that the entire blood in circulation in his body was drained into the heart lung machine. Before that we had to cool the child’s body to 16 degree Celsius. This is near freezing for the human body. We even put ice on the surface of his brain. Without circulation, we can keep the patient in this state for a maximum of 45 minutes. Beyond that, there’s the risk of injury to the brain, spinal cord, kidneys and other organs. The total time Emmanuel was on total circulatory arrest was 30 minutes. During this time we repaired the aortic arch interruption by tying off and disconnecting the PDA blood vessel and connecting it to the aortic arch.
“After doing this, we put the child again on the heart-lung machine and did the switch operation to put the blood vessels in their correct position. The intramural coronary artery was a very difficult condition where we had to mobilise this blood vessel and re-implant it into the new aorta. During this process if there’s an error of even half a millimetre, if it doesn’t go exactly where it needs to go, then the blood supply to the coronary artery could be reduced, which can cause a heart attack. This repair was followed by closure of ventricular septal defect (VSD) and atrial septal defect (ASD). It was a very long surgery lasting almost nine hours.”
The operation was successful and the baby was shifted to the ICU in a stable condition but with his chest still open. “The chest was kept open for 24 hours because there was a lot of swelling in the heart once we did the operation. Once the swelling came down and the child started passing urine post 12 hours after the operation, we closed the chest in the ICU. After this, child stayed for nearly a week on the ventilator,” Dr Muthu told us.
Baby Emmanuel slowly recovered and was discharged on the 17th day. For his parents, he was reborn that day. They thanked the doctors profusely, saying, “By the grace of God and the tireless efforts of Dr. Muthu Jothi and his team, Emmanuel is now well and has a long life ahead of him. Our prayers have been answered at Apollo and we cannot thank the doctors enough for their dedication. They kept us motivated throughout the treatment process.” We send our prayers to him in Kenya and hope he grows to be a fine young man. As we said, he was born to bring joy to the world.