2 weeks Ago
Chennai: Eight years ago when she was in her late 40s, Abirami* was told her breathlessness and discomfort were due to a leak in a valve that regulates blood flow from the heart to the rest of the body. The cause was Marfan syndrome , a a genetic disorder that weakens the aorta and increases the risk of life-threatening aortic aneurysms and dissections. "Aortic aneurysms, where the body's largest artery swells or bulges, are especially dangerous for those with Marfan syndrome, who are often younger than a typical patient.
If left untreated, this condition can be fatal," said Dr V V Bashi senior cardiac surgeon and director and senior consultant at Institute of Cardiac and Aortic Diseases, SIMS Hospital . She underwent the Bentall procedure, a complex operation where surgeons replaced the ascending aorta (the upper part of aorta) and the aortic valve. Between 2023 and 2024, her daughter and son came into the emergency room with the same problem.
In 2023, her 19-year-old daughter underwent the David procedure, also known as valve-sparing aortic root replacement, where surgeons repaired aortic root aneurysms. In less than six months, her brother, who was a year younger, underwent the Bentall procedure. "They have all recovered well, but may require constant monitoring to ensure they don't have another rupture," said consultant Dr Mohammed Idhrees.
Doctors have treated at least three other family clusters on World Aortic Dissection Awareness Day (Sept 19). This hereditary disorder affects one in 3,000 to 5,000 people and is often associated with tall stature, long limbs and a predisposition to serious aortic complications. Marfan syndrome affects approximately two lakh people in the United States.
"Given that India's population is roughly three times that of the US, it would be reasonable to estimate that at least 600,000 people in India could be living with this condition," Dr Bashi said. "It is important to diagnose these diseases and treat them to prevent complications, including death," he said. (*name changed).
