Chennai: Doctors at MGM Healthcare performed a complex open-heart operation on a 14-year-old girl with two rare conditions that made surgery especially difficult.The valve between two chambers on the teen’s heart’s left side did not close properly leading to a condition called severe mitral regurgitation. “A valve works like a one-way door, keeping blood moving forward. In her case, blood leaked backwards, making the heart work harder and leaving her breathless and largely bedridden,” said paediatric transplant surgeon Dr R K R Noveen Davidson. She also had an obstruction in the pathway through which blood leaves the heart for the body, left ventricular outflow tract obstruction (LVOTO). In addition, her heart lay in the middle of her chest rather than to the left, a rare birth condition called mesocardia.The girl also has Morquio syndrome, an inherited disorder in which the body cannot break down certain sugars. These substances can build up and damage bones and organs. The condition can also make the neck unstable and narrow the airway, complicating anaesthesia, doctors said.During the open-heart surgery, surgeons used a carefully planned route through the upper chambers of the heart to reach the diseased mitral valve. “This was difficult to access because her heart lay in the center of the chest,” he said. Surgeons removed the damaged valve and implanted a 25-mm mechanical valve to restore one-way blood flow. The team also removed tissue causing the narrowing in the left ventricular outflow tract—the passage through which blood leaves the heart to reach the body. “She is now recovering well but will need close follow-up and may take three to six months to recover fully,” Dr Davidson added.
