Baby Elias Recovers After Glenn Procedure for Rare Heart Defect

Elias was born with double inlet left ventricle (DILV), a rare and serious congenital heart defect.

In a typical heart, the two upper chambers each empty into their own lower chamber. In DILV, both upper chambers connect to the same lower chamber. Oxygen-rich blood from the lungs and oxygen-poor blood from the body mix in that single pumping chamber, so the body often does not receive enough oxygen.

DILV is a single-ventricle condition. It cannot be made into a normal four-chamber heart. Treatment is staged reconstruction — a series of operations that reroute blood flow so the one working ventricle can support the body while the lungs receive blood by a different path. Many children need a first procedure in the newborn period, a second operation called the bidirectional Glenn (or hemi-Fontan) at about 3 to 6 months of age, and a later Fontan procedure.

Elias has already been through several heart procedures. He recently underwent the Glenn procedure, an important middle step. In that operation, the large vein that returns blood from the upper body is connected directly to the pulmonary arteries. Blood from the head and arms can then go to the lungs for oxygen without passing through the single ventricle, which reduces the workload on that chamber.

Recovery after Glenn is closely watched. Infants often spend days in intensive care on a breathing machine while the new circulation settles. Coming off the ventilator is a major milestone: it means the child is strong enough to breathe on his own after open-heart surgery.

Elias has now been successfully taken off the breathing machine and is recovering.

The Glenn is not the last operation. Most children with DILV still need the Fontan later, plus lifelong cardiology follow-up, medicines, and monitoring for complications. For now, the immediate news is simpler. A baby born with a heart that mixed two streams of blood has completed another stage of reconstruction — and is breathing on his own.