New lease of life for a neonate from Sudan after complex heart surgery

Hyderabad, February 10th, 2021: A baby boy, aged only 2 months that too from a distant place like Sudan came all along to KIMS hospitals, Secunderabad to get a very major and complex heart surgery. The baby, who's birth weight is only 2.8 kgs, had got a new lease of life after the surgery and went homeland with a beautiful smile on her lips.

Dr. Anil Kumar Dharmapuram, Senior Consultant Paediatric Cardiac Surgeon, KIMS Hospitals explains the situation and the treatment given to the baby boy.

"The Sudanese baby came to our hospital with heavy respiratory distress and feeding difficulty. He also developed bluish discolouration (cyanosis) and is also not gaining weight alongwith her age.  After the complete cardiac evaluation, our team of doctors found a complex cardiac defect called Taussig Bing Anomaly with hypoplastic aortic arch and severe coarctation of aorta. In this problem, the two major tubes coming out of the heart are placed in a reverse fashion, associated with a big hole (Ventricular Septal Defect / VSD) between the two major lower chambers of the heart. In addition, the major tube called aorta which takes blood to the body is small in the aortic arch region associated with a severe constriction called Coarctation.

This combination of these multiple problems is a rare lesion in the congenital heart defects. Because of this problem, the majority of blood from the heart is going to the lungs instead of the body. To add to the complexity of the lesion, there was a fourth major problem in combination called left ventricular outflow tract obstruction (LVOTO). This was due to an abnormal and additional tissue from the left sided valve in the heart (mitral valve). This tissue was protruding into the outflow tract of the major left sided chamber (left ventricle) causing obstruction to the flow of blood to the body through the aorta.

The entire combination of defects required a major and difficult surgery lasting as much as ten hours. In this surgery, an arterial switch had to be performed to correct the reverse fashion arrangement of the major tubes coming out of the heart along with enlargement of the entire aortic arch using appropriate patch material. In addition to this, the big defect (VSD) in the top end of the lower chambers had to be fixed with a big patch material to route the left ventricle blood to the aorta.

The most complex part of the surgical procedure was resection/excision and removal of the abnormal tissue corrected to the mitral valve causing obstruction in the LVOT. This tissue is called accessory mitral valve tissue. It is a very notorious and dangerous tissue to remove. The danger is because of the connection to the mitral valve and it resembles as if it is a part of the mitral valve. For a surgeon, it is a nightmare during surgery to decide the exact nature of this tissue and remove it by shaving off and careful cutting from the mitral valve. Any amount of judgement error of a few millimeters can cause damage and loss of the true mitral valve that can endanger the life of the boy and cause intraoperative death of the baby. This is because there is no substitute to replace the mitral valve if it is damaged by wrong cutting /removal.

The entire combination of surgical repair of this lesion is one of the most difficult and challenging surgery in the field of neonatal paediatric cardiac surgery. The combination of arterial switch operation and VSD repair is a fairly commonly performed surgery, but the addition of arch repair is uncommon. The fourth addition of excision of the abnormal tissue in the LVOT is even more uncommon and a rarely performed surgical challenge" explained Dr. Anil Kumar Dharmapuram.

After this surgery, the boy took some time to recover in the ICU. After complete recovery, the boy is happily playing in the lap of the mother and feeding well without respiratory distress and normal oxygen levels. The cardiac evaluation shows good repair in the heart with normal function and no structural abnormality in the left ventricle outflow tract.  This baby will grow normally like other babies and is ready to travel back to their country with their parents. The parents of the baby are very happy that the neonate got a new lease of life after a major and complex surgery.