Stories about: single ventricle heart

Can we improve neuropsychiatric outcomes in children with congenital heart disease?

Jane Newburger studies neurodevelopment in children with congenital heart defects
Jane Newburger, MD, has dedicated her career to helping children with heart defects reach their full potential.

About 1 out of 100 babies are born with a congenital heart defects. Thanks to medical and surgical advances, these children usually survive into adulthood, but they are often left with developmental, behavioral or learning challenges.

Children with “single-ventricle” defects — in which one of the heart’s two pumping chambers is too small or weak to function properly — are especially at risk for neurodevelopmental problems. “Single-ventricle physiology creates cerebrovascular hemodynamics that can reduce oxygen delivery to the brain,” explains Jane Newburger, MD, MPH, director of the Cardiac Neurodevelopmental Program at Boston Children’s Hospital.

How does this play out in adolescence? In three recent studies, Boston Children’s Heart Center collaborated with the departments of Neurology and Psychiatry to track neurodevelopmental outcomes after corrective Fontan operations. They evaluated preteens and teens as old as 19 — the longest follow-up to date.

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Using newborns’ own umbilical cords as shunts for heart surgery

umbilical-vein-pediatric-heart-surgery-single-ventricle

Cardiac surgery is reducing the use of plastic — starting with an operation for newborns who have life-threatening heart disease generally called single ventricle.

Single ventricle is so dangerous because it means only one of the heart’s two ventricles can adequately pump blood. Typically, affected infants undergo open-heart surgery to receive a Blalock shunt, which is a skinny tube made of PTFE — a synthetic polymer — that re-routes their blood flow to the lungs so enough oxygenated blood can get to their bodies. But when blood is exposed to foreign material, such as a plastic shunt, clots can form very easily.

This fall,a clinical trial at Boston Children’s Hospital will use patients’ own umbilical veins to create the shunt instead of plastic tubing.

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Building a whole heart for children born with half: Q+A with a cardiac surgeon

Could a new surgical approach help children like Lucas get the rest of their heart back?

Our pediatric heart surgeons are used to pushing the envelope. Last month we reported on a new kind of heart valve for children with mitral valve defects that can expand as they grow. Now the same team reports 10 years of experience trying to rebuild a lost half of the heart for children born with hypoplastic left heart syndrome (HLHS), a devastating, life-threatening defect.

The new strategy, called staged left ventricle recruitment (SLVR), seeks to harness a child’s native capacity for growth and healing to encourage the undersized left ventricle to grow, giving the child a fully functional heart.

I sat down with Sitaram M. Emani, MD—a cardiac surgeon in the Heart Center at Boston Children’s Hospital and lead author on the SLVR paper—to learn more. 

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