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A rare ‘hole-in-the-heart’ cardiac surgery was performed at Poona Hospital on Thursday on a 44-year-old man suffering from Scimitar Syndrome. This syndrome is seen in 1 in 100,000 live births and is usually present, detected and treated in infancy. What made this case rarer was the adult presentation and the hole in the heart with the severe pulmonary hypertension.
Dr Vijay Natarajan, who led the cardiac team that performed the surgery, told Pune Newsline that complicating factors were the large hole in the heart and the greatly raised pressure on the lung. Few cases have been reported in world medical literature and fewer still in India.
“The 44 year old man had virtually no symptoms. He was diagnosed as having an atrial septal defect (ASD) with severe pulmonary hypertension — which means he had a large hole in the heart, between the two upper chambers in the heart with a consequent very high pressure on the lung circulation.”
However, what made his condition rare was that he was subsequently diagnosed as having a large vein from the entire right lung going, not to the left atrium as it normally should but to the inferior vena cava (this is a large vein on the right side of the heart which collects impure blood from the entire lower half of the body. “His right lung was smaller than normal with an abnormal blood supply. This combination of anomalies is known as the Scimitar Syndrome,” Natarajan explained. (Scimitar is an ancient Turkish sword nad has given its name to this medical condition because the X-ray appearance of this condition mimics that sword).
The four-hour-long surgery was challenging because it involved a middle-aged adult who had this condition since birth. It involved separating this vein through the right chest, rerouting it to the left side of the heart with closure of the large hole in the heart. Sources said the patient is doing fine after the surgery, was taken off the ventilator within 24 hours and will shifted to the ward within 48 hours.