I. Half a Heart
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Nannette McDonagh was 20 weeks pregnant with her third child when she and her husband, Pat, went to the hospital for their first general ultrasound.
A blood test had prompted Nannette’s obstetrician to prescribe a Level 2 ultrasound. While assuring her the blood test was probably nothing to worry about, the obstetrician said the Level 2 ultrasound would offer a more detailed view of the baby’s anatomy.
The technician probed the outside of her belly with the wand of the ultrasound machine. Nannette watched the grainy images appear and dissolve and appear again on the screen. She had been through ultrasounds with her two girls but found it difficult to interpret what she was seeing on the screen.
“It’s a boy,” the technician said.
This was exciting news for the McDonaghs that July day in 2003. Their daughters would be getting a baby brother. Most of all, they were hoping for a healthy baby.
The technician kept probing and not saying much, taking what seemed to Nannette to be a long time. All of a sudden, without explanation, he put down the wand and left the room.
|At her 20-week ultrasound, Ian’s mom found out her unborn son had only half a heart. Keep reading to find out how his life was saved.
“I had a feeling something was up,” Nannette said.
A few minutes later, the technician returned with a doctor. They resumed the ultrasound.
“Your baby has a congenital heart condition called hypoplastic left heart syndrome,” the doctor said. “He will not be born here.”
At first, Nannette thought, Of course, he won’t be born here. They were almost 50 miles away from their home in McHenry, Illinois. The plan was he would be born in nearby Woodstock.
Then she thought - my baby has what?
“What is that?” Nannette asked.
“Basically, he only has half a heart,” the doctor said. “He will most likely not make it.”
II. Half a Heart and No Hole
Ian was born with hypoplastic left heart syndrome (HLHS), a rare heart defect where the left side of the heart is underdeveloped and cannot provide effective circulation. For babies born with HLHS to survive, a heart vessel called the ductus arteriosus (1), which normally closes shortly after birth, needs to remain open so that blood can pass from the baby’s heart to the body. To keep the vessel from closing, doctors typically treat the baby with a medication called prostaglandin E1. Babies with HLHS also need to have a hole between the upper chambers of the heart (2) to allow oxygen-rich blood to pass into the heart so it can be pumped to the rest of the body. Ian’s heart didn’t have this hole, so there was no way for oxygen-rich blood to travel from his lungs and through his heart. In order to save Ian’s live, Dr. Javois and his team would need to act quickly to create a hole in his heart.
Learn more about HLHS here.
This image is reprinted with permission from University of Michigan Congenital Heart Center.
Two weeks later, Nannette went to the Advocate Christ Medical Center in Oak Lawn, Illinois, to have a fetal echocardiogram, which is a specialized ultrasound of the baby’s heart.
The echocardiogram confirmed the baby had hypoplastic (“hypo” means underdeveloped) left heart syndrome, or HLHS.
After birth, in babies without a congenital heart defect, the right side of the heart pumps oxygen-poor blood from the heart to the lungs. The left side of the heart pumps oxygen-rich blood from the lungs to the rest of the body. In babies with HLHS, the left side of the heart does not develop properly - the mitral valve, left ventricle, aortic valve and the aorta are either too small or not formed at all. Birth defects of the heart happen in nearly 1 percent, or about 8 out of every 1,000 newborn babies.1 HLHS is among the rarest of them, affecting about one of every 4,344 babies born each year in the United States, according to the Centers for Disease Control and Prevention.2
Before birth when a baby is growing in a mother’s womb during pregnancy, there is natural hole between the heart’s upper chambers. For babies born with HLHS, the oxygen-rich blood from the lungs bypasses the poorly functioning left side of the heart through this small hole so that the right side of the heart can pump it out to the rest of the body. If the hole is too small, there is nowhere for the oxygen-rich blood to go. At that point, babies with HLHS develop trouble breathing, a weak pulse, and are ashen or bluish in skin color. Without emergency intervention, the condition is fatal.
The specialized ultrasound at Advocate showed Nannette’s baby did not have this hole in his heart, a condition called intact atrial septum. By not having this hole, once he was born - and detached from the life-supporting placenta - there would be no way for Nannette’s baby to get the oxygen-rich blood from his heart to the rest of the body.
Only about 6 percent of babies with HLHS also have an intact atrial septum (although up to 20 percent may have a small or obstructed opening).3, 4 The blood flow through the hole in the heart appears to indirectly promote normal growth of the left side of the heart. As a result, it’s possible that in the case of Nannette’s baby, the absence of the hole played a part in the underdevelopment of the left side of the heart, said Alexander Javois, MD, FSCAI, a pediatric interventional cardiologist at Advocate Hope Children’s Hospital in Oak Lawn, Illinois.
Nannette’s prenatal cardiologist at Advocate, Bettina Cuneo, MD, referred Nannette to an out-of-state hospital that was developing an experimental procedure to open the atrial septum while the baby is still in the womb.5
“Okay, we’ll try it,” Nannette said. In early October, Nannette made the trip to Boston.
Nannette had not yet told her girls - Madison, 7, and Breanne, 9 - that their baby brother had a very serious condition. She decided it was time.
“We wanted to let them have their childhood, as much as possible,” Nannette said. “But since there was a possibility we would come home and not have a baby, we decided to tell them.”
The procedure performed in Boston would use ultrasound to help pediatric interventional cardiologists guide a catheter through Nannette’s abdomen, through the uterus and into the fetus. Through the catheter, a needle would be guided to the chamber wall of the baby’s heart to create a hole. Doctors had to hit a moving target - a grape-sized beating heart.
Although it had worked in some cases, the procedure was tricky, to say the least.
“They tried it, but it didn’t work,” Nannette said. “The baby was moving around a lot, and I think my blood pressure was getting a little high.”
They were back to square one. They would have to wait until the baby was born before they could begin to treat his heart.
The chances were not looking good, and they were about to get worse.
III. Not So Happy Halloween
It was Halloween. Nannette was at her daughters’ school, Riverwood Elementary in McHenry, taking photos for the school yearbook. She was also armed with a roll of toilet paper so her girls could play the “mummy game” in which the toilet paper was wrapped around them to make them into mummies.
Before anyone could say “Trick or Treat!” Nannette had quite a scare.
Her water broke.
She called her husband to come to pick her up and her mother to take care of the girls after school. On the way, they called Advocate to tell them what had happened and that they were on their way.
The 63-mile trip would normally take about 1½ hours. But traffic was backed up that day, especially on Interstate 290. If the baby came before they got to the hospital, he had little chance of survival.
“I had visions of a helicopter landing on the Tollway so they could fly me to the hospital,” Nannette said.
She never did go into labor that day. But her doctors weren’t taking any chances - Nannette would be staying at the hospital until Baby Ian was born.
IV. Planning for the Moment of Birth
Meanwhile, Dr. Javois was developing a plan.
He had reviewed the scientific literature on treating babies with HLHS and intact arterial septum, and the results were not promising. He found that the children were being delivered in the obstetrics suite, transferred to the Neonatal Intensive Care Unit (NICU), then transferred to the catheterization laboratory (cath lab) for the emergency procedure to create the hole in the heart. Very few survived. In most of the cases, the babies died during or soon after the procedure, or they died after a heart transplant because their condition was poor from the very start. One of the problems was the whole process took too long.
A new approach would be needed to "get the hole created faster," Dr. Javois said. The answer, he thought, was to deliver the baby in the cath lab in order to minimize the transport time. One team would set up for the delivery in one cath lab while another team would be waiting to perform the interventional procedure in a second cath lab next door.
“It raised a lot of eyebrows in the hospital because it had never been done before. But we knew that even seconds counted in Ian’s case,” Dr. Javois said.
Since they had never done this procedure (called an “atrial septostomy”) in this way before, Dr. Javois and his multi-disciplinary team walked through a mock procedure several times to help anticipate and address problems. The troubleshooting drill helped them understand what equipment they needed and anticipate the dynamics of the people in the room. They addressed procedural questions such as: How would the baby enter the room? Who would be putting in the breathing tube? Who would be placing the baby on the EKG monitor? Who would start the procedure? How would they get the catheters into the proper position?
“We wanted to make sure that not only things would move smoothly, but it would be very efficient,” Dr. Javois said. “We wanted everything to go as quickly as possible.”
Dr. Javois visited Nannette in the obstetrics ward, where he explained his plan to her. She seemed confused and scared. Ultimately, she would come to the conclusion it was worth the effort, and even if they couldn’t save her son, what the doctors would learn from the attempt could help someone else.
She did have one pressing concern.
“Can I kiss him before you do the procedure?” Nannette asked.
“I’m sorry, but no - there just isn’t enough time,” Dr. Javois said. “The moment he is born, he will start to die.”
V. Delivery in the Cath Lab
|When Ian finally arrived, he cried. It was a good sign, but his little body was gray and limp. To save him, said Dr. Javois, his care team had to make every second count. Nannette kissed her baby. He was baptized during the 10-step walk from the cath lab where he was born to the cath lab where 36 heart team members would work as fast as possible to save his life.
Every day Nannette saw Dr. Javois, she asked the same question: “Can I kiss him when he is born?” And every day, she would get the same answer: “No, there isn’t time.”
The morning of the delivery, she asked one last time.
“Can I kiss him?”
“We’ll make time. Yes, you can kiss him, but it has to be quick,” Dr. Javois said.
On Nov. 20, Ian McDonagh was delivered by cesarean section in the cath lab at Advocate Christ Medical Center. He surprised everyone by crying - they didn’t think he would be in good enough shape to do even that. Nannette kissed him on the head. As they brought him to the cath lab next door - a journey of some 10 feet - he was baptized on the way.
Nannette was a bit sleepy from the epidural anesthesia she had for the cesarean and didn’t remember much about how Ian looked right after delivery. He had not been cleaned up, so he was a bit bloody, she said. That was all she could remember.
Dr. Javois, however, remembers very well.
“Ian looked absolutely terrible,” he said. “He was just gray and limp and there was no motion.”
In the neighboring cath lab Ian was greeted by a team of 36 people. Besides the cath lab team consisting of Dr. Javois, nurses, and technologists, there were also cardiac surgeons, cardiac anesthesiologists, neonatologists, cardiologists who specialized in critical care, associated nurses and respiratory technicians. The large team was needed in case Ian did poorly. For instance, the surgeons were there if the baby needed to be put on a heart-lung bypass machine, while the other specialists were there to help with other potential problems.
“It's a real team approach,” Dr. Javois said.
As the lead interventional pediatric cardiologist, Dr. Javois performed the procedure to create the hole in Ian’s heart. Dr. Javois passed a small tube (catheter) through the umbilical cord, up into the heart, placing it against the wall of Ian’s heart. A wire was passed through the catheter and, using radio frequency energy, it was heated up to sear a hole through the wall. A progression of larger and larger balloons was passed over the wire to make larger the hole in Ian’s heart.
They were done before Ian was an hour old. Previous attempts at other institutions to perform a similar procedure had taken several hours to complete.
“Every second that passed without the hole in the heart, he would be in worse shape,” Dr. Javois said.
For Ian, it was so far, so good. But he faced three major surgeries, and a number of other corrective surgical procedures, to treat his HLHS. The first major surgery would be within two weeks, and it was the most dangerous one he would face.
VI. Home Coming
While Dr. Javois was busy with Ian, Nannette was intermittently sleeping.
“I remember waking up - my husband said I did it several times - and asking, ‘Is he okay? Is he alive?’ ” Nannette said.
Each time, her husband would respond: “Yeah, he’s holding his own, he’s doing okay.”
Ian made it through his birth day with flying colors, but he still had a long way to go. He was sent to the hospital’s NICU, where he was hooked up to a complex array of tubes and monitors.
His sisters, who were so anxious to see him, were too young to visit him in the NICU. All they could do was gaze at him through the window.
Since Ian was essentially missing the left half of his heart, his existing heart had to be reconfigured so that the right side could do the entire work of the heart on its own.
|Nannette monitored Ian’s progress by the number of tubes, wires, machines and monitors attached to his little body.
The first procedure, called the Norwood, had to be done within the first 10 days to two weeks of his life. Before he could undergo this procedure, his lungs needed time to recover.
Of the three major surgeries he was facing, this would be the most dangerous. The goal of the Norwood procedure was to create a “new” aorta and connect it to the normal right ventricle (the right lower chamber), which essentially replaced the function of Ian’s left ventricle. A bypass tube was placed from the right ventricle to the pulmonary arteries that take the oxygen-poor blood from the heart to the lungs. As a result, the right ventricle pumps the oxygen-rich blood to the body, although through the bypass it still also pumps oxygen-poor blood to the lungs.
The surgery was a success, and Ian was sent to recover to the adjacent Hope Children’s Hospital.
One way Nannette monitored Ian’s progress during his stay in the hospital was by the number of tubes, wires, machines and monitors attached to him.
“I knew he was getting better when stuff started to disappear,” she said. That was capped on the day they were able to completely wean Ian off of the ventilator that helped him breathe.
More than a month after he was born and two days before Christmas, it was time for Ian to go home.
As they were getting ready to bring Ian home, a host of reporters and camera crews from Chicago area media assembled at the hospital. The family did interviews in the hospital room. Some of the media reports would dub Ian the “miracle baby.”
But the biggest surprise was waiting for Ian’s two sisters at home.
“It was quite a surprise for the girls. We didn’t tell them he was coming home,” Nannette said.
VII. White Coat Syndrome
What a surprise! Ian’s sisters got the best Christmas present ever. Their baby brother came home just in time for the holiday.
|After the crisis on his Birth Day, Ian faced a series of procedures and surgeries and significant time in the hospital.
Ian needed much more care than the typical newborn. Babies off to a rough start due to their heart disease can often have problems with eating. Ian had a continuous feeding tube that allowed him - and his parents - to sleep through the night, since he would never wake up hungry, Nannette said. But things went well and by March he was off the feeding tube completely.
In June of 2004, Ian underwent the second major surgery, the bidirectional Glenn procedure, to directly send oxygen-poor venous blood from the upper part of the body to the lungs to be re-oxygenated, reducing the work the right ventricle has to do. This rerouting would allow the previously placed surgical shunt to be removed.
“That went wonderfully - it didn’t slow him down at all,” Nannette said. Ian was back home in three days.
Things weren’t quite so wonderful in Ian’s eyes. It was about this time that Ian had developed a strong case of “white coat syndrome” - he had had enough unpleasant experiences with doctors that he hated the sight of their white coats.
“All he had to do was see someone in a white coat or scrubs, and he would just start screaming,” Nannette said.
Visiting Dr. Javois to do periodic echocardiograms to check his heart was a nightmare, Nannette said
“It took three of us to hold him still,” she said.
Dr. Javois admits: “He hated me!”
In October 2005, just before his second birthday, Ian had the last of the three major surgical procedures to restructure his heart. Called the Fontan, this procedure directs the oxygen-poor blood from the lower part of the body directly to the lungs (remember, the previous bidirectional Glenn procedure did this for the upper body). Now oxygen-rich and oxygen-poor blood would no longer mix in Ian’s heart and his oxygen levels would be nearly normal.
Ian spent 10 days in the hospital.
“It hurt so much when he walked that he had to relearn how to walk again,” Nannette said.
But, for the most part, getting through that final major surgery was a relief for the whole family. Despite reassurances from Dr. Javois that Ian was “very strong, except for his heart,” the McDonaghs were on pins and needles for the first two years of Ian’s life.
“We just tried to live the best we could each day,” Nannette said.
In Dr. Javois’ eyes, however, Nannette was a strong and fearless source of energy and support for Ian.
“She's been a rock, really, for him throughout all his life,” Dr. Javois said.
Although Ian would face other non-invasive procedures, life settled down for Ian and his family. Soon Ian even stopped shrieking every time he saw a white coat, even lying still for his echocardiogram when he saw Dr. Javois.
But would he be able to lead a normal life?
VIII. Ian Today
|Ian’s family has been a strong and fearless source of support for Ian since before he was born.
Today, Ian is a typical 11-year old boy. He loves to play with his friends. He loves to play outside. He loves video games, Legos, making stop-action animations and playing the drums.
“If you saw him walking down the street you would never know he had anything wrong with his heart,” Nannette said.
He is a little smaller than your average 11-year-old. When his friends start running around at recess, Ian sometimes can’t keep up.
“He’s pretty energetic,” Nannette said. “He gets winded, but when he does, he just stops.”
Dr. Javois discourages him from playing contact sports. Fortunately, Ian does not appear to have a strong desire to play sports. Ian’s older sisters were into the theater and music, not sports, and Ian seems to have followed suit.
One day, when Ian was playing Little League baseball, he asked his dad how many scenes were left. That is when they realized Ian was not cut out for sports.
“We had to explain that plays had scenes, but baseball games had innings,” Nannette said.
To be sure, Ian likes football games - but more for the marching band performance at halftime than the game. Ian wants to be in band when he gets older, but his lungs aren’t strong enough to play a wind instrument. So he took up the drums.
“I like the drums because you don't have to blow,” Ian said.
Ian has undergone numerous procedures to improve his heart and lung functions. In 2012, he received a stent to enlarge the blood vessel to his left lung. He will need to have this stent stretched open more as he grows.
Ian takes it all in stride as well as an 11-year-old can.
“When I was born, the left side of my heart wasn't there and the hole in my heart wasn't there,” Ian said. “So the doctor had to open the hole so the blood could go through. It was serious because if they didn't do that, the blood wouldn't go through and I would die.”
His case still gets attention. There was a flurry of stories on television and in newspapers and magazines about him just after he was born. Then came Grey’s Anatomy. The producers of the popular television show had learned about Ian from an old magazine account they found on the Internet. They decided to incorporate a version of Ian’s story into an episode that aired April 25, 2013, with “Dr. Yang” playing Dr. Javois’ role. The Grey’s Anatomy episode spawned a new batch of media stories on Ian.
At 11, Ian has plans for a future making movies. He’s a natural in front of and behind the camera. The SecondsCount team can’t wait to “like” his YouTube channel.
Ian still sees Dr. Javois every six months for a heart check-up.
“It's never going to be perfect or normal, but it's better than we thought it could ever be,” Dr. Javois said. “To watch Ian run around like the crazy little guy that he is is incredibly rewarding.”
Since Ian has gotten over his “white coat syndrome,” he and Dr. Javois have become good friends. Ian seems to like the visits, and they enjoy talking with each other, Dr. Javois said.
“He teases a lot and he's also serious,” Ian said.
Dr. Javois and his team wrote a study of Ian’s case that was published in a peer-reviewed scientific journal.6 Since the case was published, this approach of delivering the HLHS baby without a heart hole in the cath lab for immediate intervention is being done internationally. In fact, Dr. Javois gets regular calls from interventional cardiologists from around the world who want to talk through the process.
“They ask how to get all the specialists involved in the delivery. How do I do it? Exactly what's the conduct in the cath lab?” Dr. Javois said.
As this approach gains popularity, the prognosis for babies like Ian has improved. But there are still babies, especially those who were not diagnosed before birth, who don't make it out of the delivery room, Dr. Javois said.
Meanwhile, Ian aspires to be a film maker. He has already made several stop-motion videos, in which objects - Legos, for instance - are moved from frame to frame so that they appear to be in motion when the frames run in continuous sequence.
“I want to have my own YouTube channel with lots of stop-motion videos and soon, hopefully, my own show on TV,” Ian said. Someday, perhaps, Ian could make a movie about his life.
After all, Ian is lucky to be alive and he knows it. He said if he ever has a baby with the same condition he had, “I'm going straight to Dr. Javois.”
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1 Centers for Disease Control and Prevention. Congenital Heart Defects: Data and Statistics. Page last reviewed: July 9, 2014. Page last updated: July 9, 2014 http://www.cdc.gov/ncbddd/heartdefects/data.html
2 Centers for Disease Control and Prevention. Congenital Heart Defects: Facts about Hypoplastic Left Heart Syndrome. Page last reviewed: June 17, 2014. Page last updated: June 17, 2014 http://www.cdc.gov/ncbddd/heartdefects/hlhs.html
3 Vlahos AP, Lock JE, McElhinney DB, van der Velde ME. Hypoplastic Left Heart Syndrome With Intact or HighlyRestrictive Atrial Septum Outcome After Neonatal Transcatheter Atrial Septostomy. Circulation. 2004; 109: 2326-2330.
4 Divanovi A, Hor K, Cnota J, Hirsch R, Kinsel-Ziter M, Michelfelder E. Prediction and perinatal management of severely restrictive atrial septum in fetuses with critical left heart obstruction: Clinical experience using pulmonary venous Doppler analysis. J Thorac Cardiovasc Surg. 2011 Apr;141(4):988-94.
5 Marshall AC, et al. Creation of an Atrial Septal Defect In Utero for Fetuses With Hypoplastic Left Heart Syndrome and Intact or Highly Restrictive Atrial Septum. Circulation (2004); 110: 253-258. http://circ.ahajournals.org/content/110/3/253.full.pdf+html
6 Javois AJ, Van Bergen AH, Cuneo BF, Husayni TS. Novel approach to the newborn with hypoplastic left heart syndrome and intact atrial septum. Catheter Cardiovasc Interv. 2005 Oct;66(2):268-72.