02 Aug 2012, BioSpectrum Bureau , BioSpectrum
Bangalore: Chennai-based MIOT Hospitals successful performed a surgery on a 14-year-old boy from Mumbai, who was suffering from several disorders including the breast bone pushing the whole heart to left side of the chest thereby compressing the lungs, a hole in the heart, a bulge in the heart's biggest blood vessel and a leaky valve.
All these disorders are features of Noonan's syndrome, which is an autosomal congenital disorder considered to be a type of dwarfism. The typical features of this syndrome also include, webbed neck and a flat nasal bridge. It occurs one-in-1,000 child births.
The boy was diagnosed to have a large hole in the heart (ASD) as a child at the age of four and was also suffering from progressive breathlessness. He was further diagnosed to have a large aneurysm of the aorta with a leak in the aortic valve, while he underwent treatment for the hole in the heart.
This combination of aneurysm of the aorta with leak in the aortic valve and a hole in the heart has not been reported before. The young boy in addition had a chest wall abnormality called pectus excavatum in which the breast bone has gone very deep and this has pushed the heart completely to the left side of the chest.
The boy was referred to MIOT Hospitals for surgery after he was having breathlessness and was unable to sleep properly. Normally, open heart surgery is performed by cutting the breast bone because the heart is pushed behind. Done this way, the operation is easy to perform. However, in case of this young boy, since the heart was totally on the left side of the chest, it was impossible to do this operation through the midline. There is not a single case report of this combination of disease in the world.
Dr V V Bashi, chairman, Center for Thoracic and Cardio Vascular Care, MIOT Hospitals, "To our knowledge doing a Bentall's operation and closing the hole (ASD) by opening the left side of the chest in this type of congenital abnormality in a child has not been reported in the world before."
The surgical team was headed by Dr VV Bashi assisted by Dr Vijith K Cherian, Dr Kannan R Nair, Dr Harilal and Dr Gopal. The anaesthetic team was headed by Dr Aju Jacob and assisted by Dr Jyotsana and Dr Ajith. Furthermore, a dedicated team of perfusionists technicians and skilled nurses where involved in the procedure.