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Innovative Surgery connects liver directly to the heart to save 21 year old from rare genetic disorder


Innovative Surgery connects liver directly to the heart to save 21 year old from rare genetic disorder

Gurgaon : Doctors at Fortis Memorial Research Institute (FMRI), Gurgaon gave a 21-year-old Iraqi woman another chance to live a full and happy life recently. The patient suffered from a rare genetic condition in which blood clots prevented the flow of blood into the liver and out flow from the liver to the heart. A team of doctors lead by Dr. Vivek Vij, Director, Liver Transplant, FMRI conducted an extremely complex lifesaving surgery, in a carefully crafted procedure on 28th of December, 2016.

“My parents sent me to India fearing relatives might say they didn’t try enough to save my life,”recalls 21-year-old Bnar Satar Mala, who till December 2016 had given up hope of survival. It all started somewhere in the year 2014,  with initial symptoms of turning pale, then yellow with severe pain shooting down the left shoulder to her arm soon developed into swelling in her limbs and abdomen. “I would feel nauseous at the mention of food, I could barely eat and often throw up soon after”. Doctors in Iran diagnosed her with a rare genetic disorder called Budd Chiari Syndrome, a condition where blood clots completely or partially blocks the blood flow to the liver in an individual. Very few cases of this syndrome have been reported worldwide so far. It is rare to the extent that many liver transplant surgeons might not have even got an opportunity to treat such a case. The blockage may occur anywhere from the small and large veins that carry blood from the liver (hepatic veins) to the inferior vena cava (that takes the blood back to the heart). The IVC drains fluid out of the liver too. Due to the blockage in the hepatic vein which obstructed the outflow, her liver was gradually “dying”. “No doctor wanted to take the risk of operating on me, at best, a stent was put inside my liver to drain the fluid out,” added Mala.

“By the time Bnar reached FMRI, her liver was completely black and shrunken, requiring urgent transplant in order to save her life. Her brother, 27-year-old Bzar, matched for a donor in Mala’s case. All necessary tests were done and Mala underwent the transplant on December 28th,” explained Dr Vij. “Her IVC was completely blocked and she required a lot of blood transfusion during the surgery. The affected liver was removed and a part of the liver was taken from her brother and transplanted in the patient. In this case, the challenge was to suture the liver “directly” to the heart as the patient’s native IVC was completely blocked. In order to suture the liver as close to the heart as possible, the heart had to be pulled down into the abdominal cavity. We decided not to open the recipient’s chest and instead pulled the heart down through a narrow gap made in the diaphragm separating the chest and the abdomen. By doing this, we significantly reduced the risk of any infection which could have occurred due to a large opening in the chest. Also, being a woman, her concern was the scar such an opening would have left on her chest,” added Dr Vij. The hepatic vein was sutured directly to the heart through this novel technique and the team made sure that they did all this avoiding sternotomy (cutting open the chest to reach the heart).

Doctors worked meticulously post-surgery to maintain a fine titration of her medicines to make sure of optimum anti-coagulation.

“FMRI has pioneered several complex surgeries in the past. This case was almost a lost cause when the patient reached us, however we gave it our best and were successful in our endeavour to save her life. Our efforts are proof of our need to provide quality health care services and medical aid to anyone across the globe who requires it,”said Dr. Simmardeep Singh Gill, Zonal Director, Fortis Memorial Research Institute.

Dr. Vivek Vij is a qualified liver transplant surgeon, having completed 500 liver transplants across the Fortis network. He has established safety procedures in accordance with the transplants and has been an active developer in the living donor surgery sector. He also founded the Liver Transplantation and Hepatobiliary Sciences at Fortis. He has the lowest complication rate when it comes to liver transplants, and has established very successful programmes with regard to liver transplants at Fortis Noida and Fortis Mohali. Overall, Along with his team, he has conducted, supervised and facilitated over 2,500 surgeries, majority of which have taken place at Fortis hospital, Noida, with a 100% donor rate and 95% patient rate.

Liver transplantation is currently in its golden period in India. The number of transplants being performed and the steady increase in new programmes that have emerged over the last decade is a testimony to it. A multipronged approach in developing infrastructure and the involvement of multidisciplinary teams in the management of transplant patients has had a major positive impact on the outcome and as a result a positive impetus to the growth of this specialty in India. To date, the majority of transplants performed in India are live donor liver transplants.

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supriya kashyap

supriya kashyap

Supriya Kashyap Joined Medical Dialogue as Reporter in 2015 . she covers all the medical specialty news in different medical categories. She also covers the Medical guidelines, Medical Journals, rare medical surgeries as well as all the updates in medical filed. She is a graduate from Delhi University. She can be contacted at supriya.kashyap@medicaldialogues.in Contact no. 011-43720751
Source: Press Release

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  1. A very informative write up.I had heard and read about this syndrome but got a chance to read this case report.