• The kid was diagnosed with Chronic Liver Disease due to a rare congenital disorder along with a rare lung condition

Bengaluru, March 30, 2022:  10-year Bangladeshi boy got a new lease of life by undergoing successful liver transplant at Fortis Hospital, Bannerghatta Road. The boy was diagnosed with hepato-pulmonary syndrome, a rare condition that affects the lungs of people with advanced liver disease. The multidisciplinary team of doctors was led by Dr Mahesh Gopasetty- Senior Consultant- HPB and Transplant Surgery, Dr Ravindra Nidoni – Consultant- Liver Transplant Surgeon, Dr Prasanna K S- Consultant – Gastroenterology & Hepatology, Dr Yogesh Gupta- Head PICU, Dr Murali Chakravarthy- Director- Anesthesiology at Fortis Hospital, Bannerghatta Road.

Back in Bangladesh, Patient was diagnosed with difficulty in breathing and was advised to undergo Pulmonary Arteriovenous Malformation (PAVM) correction (a condition that affects blood flow between the heart and the lungs), therefore the patient’s family decided to visit Bangalore. 

 As the patient had persistent low oxygen saturation, he was put on oxygen support for the past 16 months. In Bangalore, the family visited several hospitals, before coming to Fortis Hospital, Bannerghatta Road where they met Dr Mahesh Gopasetty- Senior Consultant – HPB and Transplant Surgery.

At Fortis Hospital, the patient was examined thoroughly and was diagnosed with Chronic Liver Disease secondary to congenital intrahepatic portosystemic shunt– type 4 with severe hepato-pulmonary syndrome, patient was advised to undergo a liver transplant surgery. 

Congenital intrahepatic portosystemic shunts (IPSS) are abnormal vascular communications within the liver between branches of the portal vein and the hepatic veins

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Considering the deteriorating condition of the boy, an urgent transplant was needed. However, being a diabetic patient, the boy’s father was unfit for the donation and the mother was pregnant. In that scenario, in order to save the life of the boy, his aunt came forward to donate part of her liver.

Dr Mahesh Gopasetty- Senior Consultant – HPB and Transplant Surgery, Fortis Hospital, Bannerghatta Road, said, “The challenges which we anticipated were only oxygen support, however anaesthetists were very well prepared and were kept on standby. With the help of our hepato biliary and paediatric critical team, we were able to extubate him from the ventilator after 3 days post the transplant. The paediatric critical care team led by Dr. Yogesh Gupta played a crucial role in managing the oxygen level of the boy for continuous 3 months. 

Dr Prasanna K S- Consultant – Gastroenterology & Hepatology, “The boy was referred to us for a liver transplant, however prior to liver transplant we did a complete evaluation and we found out that he had congenital intrahepatic portosystemic venous shunt (CIPSS), which is very rare condition, nearly about 1 in 10 lakh people will be diagnosed with this condition which has caused his oxygen deficit.”

Dr Yogesh Gupta- Head – Pediatric Intensive Care Unit, explained, “We expected his post-operative period to be difficult and he would need a long ventilator support due to underlying Hepato- pulmonary syndrome. He was shifted to PMICU, and within 72 hours, we were able to take him off ventilator and put him on high flow nasal oxygen. He remained stable, he was shifted to the ward, and with the help of multi-disciplinary team of doctors, he was managed well and we could prevent any kind of infection and complications which usually arise post transplantation. The boy was off oxygen support within 30 days post operatively which typically takes about 6 months in a case of hepato-pulmonary syndrome.

Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population.

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