Marfan’s Syndrome and Aortic Dissection
A very complex heart surgery lasting 8
hours helped save life if a 27 year old gentleman. According to Dr Pankaj Goel,
Director Heart And Lung Surgery at Ivy Hospital Amritsar, this gentleman
suffered from a disorder called Marfan’s syndrome. In this genetic disorder the
person has a lean tall stature and problems like joint dislocation, visual
impairment etc. The most serious problem , however, is that of aortic
dissection. It is caused by the weakness of the wall of body’s main artery- the
Aorta. Dissection of this artery leads to split in the wall and very high
chance of rupture and death. If not operated immediately the risk of death is
1%every hour? Only 20% survive 2 weeks.
This gentleman had
Marfan’s syndrome and developed sudden chest pain and uneasiness. Several years
ago his mother also succumbed to a similar problem on her way to Delhi as the
surgery was not done in Punjab . They were aware of the consequences and took
immediate medical advice. The diagnosis was confirmed on CT scan and within 30
min of reaching Ivy hospital he was in the operating room. Dr Goel and his team
performed a procedure called Bentalls operation and hemiarch replacement. In
this procedure the aorta along with the aortic valve are replaced and the leak is sealed. In order to do the
procedure the blood supply to brain has to be interrupted. The body is cooled to 18 degrees, circulation
stopped, the repair is done and circulation restarted. It is a race against
time Dr Goel added, everything has to be done perfect the first time. Delay can
lead to permanent brain damage. Only a few surgeons and hospitals have the
capability of managing such patients.
The family thanked Dr Goel and Ivy Hospital
for saving the life of their loved one. They had lost a family member to same
disease years ago due to non availability of surgery in Amritsar. Dr Goel
performed the first Aortic dissection surgery and Bentalls procedure of
Amritsar in 2008 and has performed over 50 such procedures since then.
#aorticdissection #marfanssyndrome #
#aorticdissection #marfanssyndrome #
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