New technique to save kids born with 'half a heart'

It uses the existing standard single-ventricle treatment for HLHS and additional procedures to spur the body's capacity for healing and growth and encourage the small left ventricle in these children to grow and function.

HLHS is a rare but severe congenital heart defect in which the left ventricle, the chamber of the heart that pumps blood out into the body, does not develop properly. The majority of children born with HLHS undergo a series of three procedures, together known as single ventricle palliation (SVP), culminating in the Fontan procedure.

Surgeons use the sequence to reconstruct a child's heart so that it can function with only a single working ventricle, but only about half of patients treated in this way survive to adulthood.

"Many of the children who reach adulthood eventually require a heart transplant because their Fontan circulation doesn't work effectively or causes additional complications," according to researcher Sitaram M Emani.

"Children have an enormous growth and healing potential," Emani said in a statement.
 
"We realised that rather than give up on the left ventricle and commit a child to single ventricle circulation for life, we could leverage that growth potential in a staged approach that would promote growth in the left ventricle and gradually recruit it back into operation," Emani added.

In their report in the Journal of the American College of Cardiology, the authors document their experiences developing and refining the SLVR approach between 2001 and 2010.

In that time, 34 children were treated using the approach, 12 of whom were successfully converted to complete,
two-ventricle circulation.

In the short term, survival rates among SLVR-treated patients have been slightly better than a comparable group of children who underwent SVP.

In addition, patients who underwent SLVR also displayed significant growth in the valves and chambers on the left side
of the heart, even if surgeons could not get the left ventricle to function completely, demonstrating that the strategy can effectively rehabilitate the heart and successfully support its growth and function.

"We believe the SLVR strategy could revolutionise the paediatric cardiology field's approach to treatment of HLHS,"
Emani said.