"Miracle Baby" In Illinois L Survives Rare Heart Procedures
Ian's introduction to the world began in early November when he was born in a heart catherization laboratory and then immediately taken to a second heart procedure room where a team of some 30 Christ Medical Center obstetricians, cardiologists, surgeons, nurses and technicians worked successfully to reverse an inoperable condition that would have caused his death in a matter of hours or less.
Baby Ian was born with an extremely rare combination of congenital heart disorders, known as hypoplastic left heart syndrome (HLHS), and an intact atrial septum. Infants with HLHS usually can survive for a few days to a few weeks after birth without surgery because a hole in the heart wall creates blood flow within the heart, allowing red oxygenated blood to get out to the body. However, in Ian's case, the hole in the atrial septum was missing, and the oxygen-carrying blood was sealed off completely and unable to get out to the body. Essentially, Ian had zero percent chance of survival.
To correct the intact atrial septum, Ian underwent an innovative non-surgical procedure -- this being one of only a handful performed in the United States. Dr Alexander Javois, an interventional pediatric cardiologist at Advocate Hope Children's Hospital and his team, performed an atrial septostomy, a procedure in which a searing wire was fed through the baby's umbilical cord vein leading to his heart and used to burn a hole in the wall between the heart's upper two chambers. The hole provided an escape hatch through which oxygen-carrying blood could flow from the heart to the baby's body.
"What makes this case so distinctive is that an atrial septostomy has never been successfully done using this type of equipment immediately at the time of delivery. The handful of previous attempts in this country have taken several hours to complete," said Javois, who completed the procedure in less than an hour. The procedure prevented life-threatening damage to the brain, lungs and heart.
"Our success was due to the well-organized multidisciplinary team of specialists and staff who simultaneously worked together to make this happen," stated Javois. "However, the work for our team did not end there."
"Only a few centers in the US can take on this type of technically challenging procedure," says Dr Audrey Marshall, a pediatric cardiologist at Children's Hospital in Boston, a forerunner in pediatric cardiology. "Children born with this rare combination of heart disorders possess the highest mortality rates, and time is critical in this type of demanding situation. What the physicians at Hope were able to pull off was terrific, and their strategic planning, teamwork and use of this smart technique worked and should be recognized."
After physicians cleared the first major hurdle, the next concern was Ian's lungs, which had to begin developing normally before surgeons could conduct the first of three critical open heart surgeries to correct his other congenital heart disorder, HLHS.
"This is by far one of the most risky and invasive procedures for a young child to go through," said Dr. Michel Ilbawi, director of pediatric heart surgery, Hope Children's Hospital, who led the surgical team in Ian's case. The surgery was successfully performed in early December.
Babies born with HLHS have a left ventricle that is severely underdeveloped. The anomaly prevents the heart from pumping sufficient blood through the body. If the problem is not repaired, the infant can survive for only a few days. HLHS is rare and represents two percent of all congenital heart defects.
Surgery for HLHS involves three procedures. The first, known as the Norwood procedure, must be done soon after the baby is born, preferably before the baby reaches 10 days of age. The Norwood procedure is designed to use the right ventricle (the right lower chamber) to replace the absent, or very small, non-functional left ventricle. In doing that, the right ventricle becomes the ventricle that pumps the blood to the body, instead of pumping the blood to the lungs as in normal hearts.
Blood is supplied to the lungs through a bypass inserted at the time of the procedure. The second surgery, usually done at two to six months of age, is intended to direct unoxygenated (blue blood) from the upper part of the body to the lungs (Glenn procedure). The third procedure directs the unoxygenated blood from the lower part of the body to the lungs (Fontan procedure).
"This is truly a miraculous outcome," said Javois. "We are extremely amazed and pleased with how rapidly the baby has responded to treatments. With so many odds working against him, little Ian never gave up. He is definitely a fighter."
"We could have never asked for a better Christmas present," said the
McDonaughs. "You couldn't ask for anything more precious and priceless."