Cardiac surgeon calls for screening in schools for Marfan Syndrome

On World Aortic Dissection Awareness Day SIMS brings together those treated for the syndrome

School screening programmes will help to pick up early conditions such as Marfan syndrome, said V.V. Bashi, director of the Institute of Cardiac and Aortic Diseases at SIMS Hospital, Vadapalani.

At a programme to raise awareness on aortic dissection, Dr. Bashi said aortic aneurysms could be caused by lifestyle changes resulting in diabetes and hypertension. But, there are a group of people with Marfan syndrome, a genetic condition, where the aorta is weak, resulting in aneurysms.

Annually, World Aortic Dissection Awareness Day is observed on September 19. With the European Association for Cardiothoracic Surgery (EACTS) and the US Society of Thoracic Surgeons (STS) recognising the aorta as an independent organ, there will be greater focus on treatment, developing new medicines and that would benefit patients, said Dr. Bashi, who has performed over 2,000 aortic surgeries, including for aortic aneurysm and conditions such as Marfan syndrome. SIMS hospital has treated so far 800 patients with aortic aneurysms.

Marfan syndrome children are exceptionally tall, with their hands extending below their knee (usually the hand span is only up to the thigh) and could suffer from skeletal deformities such as a curved spine, sunken or protruding chest or have eye problems. Such persons’ aorta is weak causing rupture of the vessel. Aorta is the main vessel from the heart that supplies blood to the vital organs in the body. The weakness could occur anywhere along the aorta requiring surgery. In some people, the aneurysm may be in multiple places, Dr. Bashi explained.

The surgery is complex involving stenting, grafting and even valve repair. One in 5,000 could have Marfan syndrome, he explained adding that a school health screening programme to detect the syndrome would help such children lead a normal life.

Dr. Bashi recalled that he recently met a patient who came with complaints of kidney failure, whereas the diagnosis showed that he had an aortic aneurysm. Dr. Bashi said the man was on dialysis after total failure of his kidneys. After the aneurysm was treated, he was able to pass urine, he added.

Patients with Marfan syndrome must be on lifelong surveillance. Surgery is recommended if the size of the lumen of the aorta, which should normally be 2.5 cm in diameter, doubles. According to Dr. Bashi, patients remain symptom-free until the aneurysm develops and becomes life-threatening. Such persons may continue to lead a normal life post-treatment, he said.

Published - September 19, 2024 09:20 pm IST