1 in 5000 live births can sometimes present with a condition called Hypoplastic Left Heart Syndrome. They might present as early as immediately after birth. They require a complex 3 staged procedure which our pediatric cardiac surgical team is very well experienced with.
Stage I is a palliative procedure, i.e., Norwood procedure which is done by very few surgeons in India. Our cardiac surgical team has had a legacy of great outcomes in this procedure.
Stage II and Stage III includes Bidirectional Glenn procedure and Fontan procedure respectively done at appropriate stages of life.
These procedures are used to treat hypoplastic left heart syndrome, a group of defects in which the left ventricle is very small or absent.
Norwood 1 is a palliative operation – a fix, not a cure – performed in newborns as an emergency procedure using cardiopulmonary bypass pump. It converts the functionally single right ventricle to act as the left ventricle while the pulmonary trunk is surgically joined with the tiny aorta to form a large new aorta, using a modified Damus-Kaye-Stansel procedure. The aortic arch is reconstructed as well, if necessary. The pulmonary artery branches are detached and connected to the new aorta by a small plastic tube, a procedure known as a modified Blalock-Taussig anastomosis. Norwood 1 enables an infant to grow to 4 to 10 months of age, when the second stage palliative operation can be undertaken.
Norwood 2 converts the Blalock-Taussig anastomosis to a bi-directional Glenn or a Hemi-Fontan anastomosis in preparation for the eventual Fontan procedure, which is the corrective repair. The Glenn and the Hemi-Fontan, a version of the Glenn, consist of connecting both pulmonary artery branches to the superior vena cava, enabling the blue blood to reach the pulmonary circulation directly without having to pass through the heart chambers. At a suitable age, around 4-5 years, the child becomes eligible for the Fontan operation, which eliminates cyanosis by directing the inferior vena cava to the pulmonary artery and partitioning the two atria.