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Capital doctors perform high-risk surgery to replace ball-size blood vessel

Marfan syndrome, a rare inherited disorder that affects connective tissue and organs such as the heart, eyes, blood vessels, and bones, Doctors say that the disease caused Devi her ascending aorta.

SNS | New Delhi |

Adding another feather to its cap doctors of capital successfully performed a rare surgery and saved a 23-year-old woman life by replacing a ball-sized blood vessel.

Earlier last week 23-year-old Devi (name changed) developed chest pain and was brought to Aakash Healthcare, Dwarka, little did she or her family realize the gravity of the situation. Doctors said that she was suffering from Marfan syndrome, a rare inherited disorder that affects connective tissue and organs such as the heart, eyes, blood vessels, and bones. Doctors say that the disease caused her ascending aorta (the main artery that carries blood away from the heart to the rest of the body) to become 6.5 cm-wide, i.e., double the normal width and could have ruptured anytime, causing her sudden death. Her aortic dissection (tear of the wall of the aorta) had spread from the heart till the right leg. However, doctors replaced the valve and a part of her aorta using an artificial graft that gave her a fresh lease of life.

Dr Abhay Kumar, Director, Dept of Cardiac Surgery, Aakash Healthcare, Dwarka, said, “The Marfan syndrome made the tissue of the aorta’s outer wall thinner, and there was a risk of rupture. Aortic dissection can be fatal as there is a 1 per cent risk of death with every passing hour. About 50 per cent of untreated patients of aortic dissection may die as quickly as in 2 days. If the diameter of the aorta is 4.5 cms or more, it is an indication of the ‘Bentall’ procedure. Considering her age and the proper functioning of all other organs, it was decided to be the right procedure for her. This is a complex and high-risk surgery and successful completion requires significant expertise and efficient teamwork. The surgery requires stopping the blood supply to the body completely and controlling blood supply to the brain to prevent paralysis for some time. We were very careful as we were aware how former New Zealand cricketer Chris Cairns recently suffered a stroke in the spine during surgery to correct his aortic dissection. Extracorporeal membrane oxygenation (ECMO) was used to pump oxygenated blood outside her body during the period of surgery and we completed the surgery successfully after 7 hours. Bentall was the only procedure that could have saved her life. She is doing fine and can lead a healthy, normal life.”

“Devi’s mother also had a similar heart problem and successfully underwent a Bentall surgery 15 years ago. Due to the genetic nature of Marfan syndrome, doctors advised her both brothers, who have tell-tale signs of the disease and are both in their 20s, to get tested. The test revealed that they too have already developed heart problems. Named after Professor Hugh Bentall, a British cardiologist, the Bentall procedure refers to an open-heart surgery that involves replacing the affected valve and aorta and reconnecting the coronary arteries. The process was invented in 1952 by Prof Bentall” said Dr. Amrish Kumar, Consultant, Dept of Cardiac Surgery, Aakash Healthcare, Dwarka.

“I never had any problem related to my heart so we were shocked to know that I had the risk of sudden death. But I am thankful to the doctors for their expert and prompt action that helped save my life. Besides, they anticipated that my brothers may also have a heart problem due to Marfan syndrome, which turned out to be true, and that saved their lives too. Doctors of Aakash Healthcare are no less than God to me and my family,” Devi said.