The chance of cure for the five-year-old Paridhi, who was surviving with the help of ventilator, was almost nil. She was suffering from nearly 50 percent shunt fraction due to advanced hepatopulmonary syndrome that leads to liver failure and also adversely affects lungs functioning.
“We have a long experience of operating milder cases of hepatopulmonary syndrome, but had never taken on a such an advanced case with nearly 50 percent shunt fraction,” Dr AS Soin , Medanta’s chief liver transplant surgeon, said. This is the rare liver transplant which was operated first time in India. The transplant was performed on Paridhi after her mother Mamta Sethi consented to donate 20 percent of her own liver.
Parents of Paridhi lost all hopes when doctors of Mumbai rejected her case as ‘far too advanced’ for a liver transplant. The condition of Paridhi, who lives in Borivali and studies in class 1, was very critical when she visited Medanta Hospital.
Dr Neelam Mohan, director of Children’s Liver diseases and transplantation, said, “Paridhi had jaundice by birth. She was also diagnosed Biliary Atresia when she was around two months old. Her health then deteriorated. She had life threatening liver infections with eight prolonged admissions in hospitals. When she came to us, she was deeply jaundiced and had marked growth failure. She also had severe HPS with oxygen saturation less than 70 percent causing breathlessness, necessitating continuous oxygen and had bluing and sounding nails typical of a low oxygen.”
Biliary Atresia is a condition where the bile duct is not formed by birth. Bile duct connects the liver to intestine. In this, liver gradually becomes cirrhotic and go in liver failure. She underwent Kasai surgery for biliary atreisa in Mumbai when she was two months old, where in the liver was connected to intestine.
“Unfortunately this surgery was not successful and her jaundice persisted and she developed cirrhosis. At one and-a-half years of age, she was also diagnosed with Kawasaki infection which affected her heart and her heart vessels got slightly dilated. She had 6 admissions thereafter for lung and liver infections. By 5 years, Paridhi became weak, got breathless — was not able to walk and would get easily tired. Besides growth failure, her finger nails and lips turned blue,” says Dr Neelam Mohan, director of Children’s Liver diseases and transplantation. The family contacted doctors of Medanta hospital for liver transplantation 3 months ago. Her Echo test (saline contrast test) showed that Paridhi had developed intra-pulmonary shunts that are the blood going from heart through pulmonary artery instead of going through lungs was getting into pulmonary veins which open in left side of the heart. This resulted in poorly oxygenated blood to left side of heart — which got circulated in body.
Doctors have faced lot of challenges before going for the major surgery because her PO2 (oxygen in blood) levels, which are normally near 100 percent, was only 48 percent and her shunt fraction on MAA scan was also 48 percent. This classified her as very severe hepatopulmonary syndrome (HPS).
“Intra-operatively include (a) we had to maintain O2. As her PO2 were not improving >60 percent, we gave her NO (Nitric Oxide) and kept ECMO which is lung bypass machine as standby. We ensured that surgery time was reduced to four and-a-half hours and the surgery would be with minimal blood loss and blood pressures were tightly controlled not allowing to fall much. We also monitored her heart though transesophageal echo during surgery,” says Dr A S Soin
Post-operative care is crucial for patient’s recovery but in this case, post operative was also a major challenge. The new liver would need good oxygenation. “We kept her on ventilator for four weeks along with Nitric oxide therapy for three weeks. We did tracheostomy to give above treatment. We had to maintain good balance of fluids, blood pressure, O2 saturation and platelets levels. Low platelets could lead to intracranial bleed, while high platelets with good coagulation could block the newly stitched small vessels of the liver,” says Dr Mohan.