Cytogenetics and the evolution of medical genetics

Effectiveness Of ACE Inhibition In Patients With Marfan's Syndrome On β-blockade

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Effectiveness of ACE inhibition in patients with Marfan's syndrome on β-blockade. Nat Rev Cardiol 5, 68 (2008). Https://doi.Org/10.1038/ncpcardio1075

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Telangana Govt Hospital Doctors Perform Liver Transplantation On Patient Suffering From Marfan's And Rare Syndrome

Osmania General Hospital doctors from multiple specialisations coordinated to perform liver transplantation on a patient suffering from Marfan's syndrome and hepatopulmonary syndrome (HPS). The image is used for representative purposes only.Photo Credit: NAGARA GOPAL

In a medical breakthrough, doctors at Telangana government-run Osmania General Hospital (OGH) in Hyderabad have successfully performed a liver transplantion on a patient suffering from both Marfan's syndrome and very severe hepatopulmonary syndrome (HPS).

Breathing compromised

The 37-year-old patient was diagnosed with Marfan's syndrome in early adulthood. By then, he had reached a critical stage of HPS. His breathing became severely compromised due to low oxygen levels caused by advanced liver disease.

What are HPS and Marfan's syndrome?

Hepatopulmonary syndrome (HPS) is a rare and serious condition where liver disease leads to low oxygen levels and widening of blood vessels in the lungs, making it hard to breathe. Liver transplant is usually the only treatment. But in this case, the patient also had Marfan's syndrome, a genetic disorder that makes body tissues fragile and increases the risk of heart problems. These factors made the surgery much more risky, said CH Madhusudhan, a senior Surgical Gastroenterologist involved in the case.

A team of hepatologists, pulmonologists, cardiologists, and transplant surgeons at the hospital spent months evaluating the patient's condition. Following extensive discussions and reviews of global medical literature, the team concluded that liver transplantation remained the only viable treatment option despite the complex risks involved.

"While challenges such as vascular fragility were encountered during the surgery, the operation proceeded without major complications. In the days following the procedure, the patient's oxygen levels began to stabilise, and a marked improvement in respiratory function was observed. Continued follow-up has shown promising recovery and enhanced quality of life," Dr. Madhusudhan said.

"This is a milestone not only for our hospital but also for the global medical community. Successfully managing such a rare and high-risk case demonstrates the power of collaboration and innovation in modern medicine," the surgeon said.

Published - April 17, 2025 01:43 pm IST

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