Isaiah Lunsford held the lumpy chunk of plastic against his bare chest, looked up at his mom and grinned.
In his hands, the 8-year-old boy cradled a replica of his heart. The swirling maze matched the wildly unusual anatomy of the organ that kept his blood pumping in his first years of life.
His heart has changed quite a bit since that model emerged from a 3-D printer. The scar running down the center of Isaiah’s chest bore witness to his recent open-heart surgery, performed at the Congenital Heart Center at Spectrum Health Helen DeVos Children’s Hospital.
As to the success of that operation―that was written all over Isaiah, as he laughed and squirmed during the doctor’s visit, eager to run and play.
“He’s doing excellent,” said Joseph Kuruvilla, DO, his primary pediatric cardiologist. “He has had an amazing result. As of now, he will have no restrictions as far as sports.”
The complex operation to repair Isaiah’s heart defects was unique.
Just like his heart.
Just like Isaiah.
From China to Michigan
The story of Isaiah’s remarkable heart follows a twisting path, one that includes a misdiagnosis, two operations, a trip across the world―and a happy new beginning.
It began in China, where Isaiah was born. He lived in an orphanage for his first five years and then with a foster family for two years.
We always had hope, but it ended up being better than what we hoped for.
Doctors detected heart defects soon after his birth. His heart sat on the right side of his chest, rather than the left. And while a healthy heart has two ventricles―the organ’s pumping chambers―his heart appeared to have only one. Tests showed no sign of the right ventricle―the one that sends blood to the lungs.
Just before his second birthday, Isaiah underwent surgery to place a shunt connecting the superior vena cava to the pulmonary artery. It didn’t cure the single-ventricle defect, but it helped―by routing blood to the lungs to pick up much-needed oxygen.
On July 4, 2016, at the age of 7, Isaiah met a new family―his forever family. Autumn and Dan Lunsford, of Zeeland, Michigan, went to China to adopt him.
“Adoption had been in our hearts for a long time,” Autumn said.
She and Dan, who had three children, began to think specifically about adopting a child with special needs after their youngest daughter, Ivy, was born four years ago with a bowel obstruction. She had surgery and spent her first three and half weeks in the neonatal intensive care unit at Helen DeVos Children’s Hospital. The experience deepened the Lunsfords’ compassion for orphans who face serious health challenges without parents to care for them.
“It really opened our hearts to saying we were open to a medical need like that,” Autumn said.
When they adopted Isaiah, they were told he had a heart condition that could mean a shorter lifespan, and potentially the need for a heart transplant someday.
They brought him home, and Isaiah quickly adapted to his new family. He played with his sisters, Naomi, 10, and Ivy, 4, and his brother, 7-year-old Roman.
“He’s like a mix of being really smart and thoughtful, and he has this other side of him that is super, super silly,” Autumn said. “He is a little helper. He’s just a really good kid.”
They communicated at first with the help of an online translator and lots of hand gestures. But in a few short months, Isaiah became fluent in English. He started kindergarten and excelled at reading in his new language.
“It’s incredible,” Autumn said. “He acclimated faster than I expected.”
A change in diagnosis
His energy level seemed surprisingly strong, given his medical condition. Autumn just noticed he would get tired at the end of the day. And his lips and fingertips showed a blue tinge, a sign of a low oxygen level in his blood.
As soon as Isaiah joined their family, the Lunsfords began to explore nationally known congenital heart centers to determine the next step in his medical care. They found what they needed close to home, at the congenital heart center at Helen DeVos Children’s Hospital in Grand Rapids.
He’s the most inquisitive kid I know. He knew the ins and outs of his medical condition.
Based on medical information provided by doctors in China and the US, the Lunsfords believed he would need a follow-up surgery to the one he underwent as an infant. The operation, called a Fontan procedure, would connect the inferior vena cava to the pulmonary artery. This would reroute the blood that returns to the heart from the body, sending it directly to the lungs for oxygenation.
As an orphan, Isaiah had not been eligible in China for that second surgery, Autumn said.
The operation would not be a permanent fix. When Isaiah reached his teen years or young adulthood, the limits in his ability to exercise, to play sports and keep up with other kids would become apparent.
And ultimately, the heart defect could lead to damage in other organs, especially the liver. But in the meantime, the operation could help.
“It’s palliative,” Autumn said. “It kind of helps to alleviate pressure on the (single) ventricle.”
That was the path the Lunsfords expected to follow―until Dr. Kuruvilla examined Isaiah.
“Looking at his (echocardiogram), I was very skeptical of this being the diagnosis,” he said. “I realized this was a different anatomy from the one we thought it would be.”
He consulted his colleagues at the congenital heart center, including pediatric cardiologist Joseph Vettukattil, MD, an expert in advanced cardiac imaging and interventions.
“(Dr. Vettukattil) was instrumental in doing a cardiac catheterization and he performed a 3-D echocardiogram,” Dr. Kuruvilla said.
The tests revealed Isaiah did, in fact, have both right and left ventricles.
But his heart was tilted at an unusual angle. Rather than sitting side by side, the ventricles essentially were stacked one above the other. The condition, often called upstairs-downstairs ventricles, is “exceedingly rare,” Dr. Kuruvilla said.
Congenital heart defects affect about 1 percent of the population. Within that group, upstairs-downstairs ventricles account for a small percent.
Earlier tests did not detect it because the position of the right ventricle makes it difficult to see with traditional, two-dimensional echo imaging. But by using the more sophisticated 3-D echocardiogram―combined with a catheterization and a CT scan― the doctors could see Isaiah’s “right ventricle actually had adequate volume,” Dr. Kuruvilla said.
The imaging also revealed several other heart defects:
- Isaiah had a large hole in the septum―or wall―that divides the ventricles.
- A large hole also lay in the septum dividing the right and left atria―the heart’s collecting chambers.
- Both the heart’s major arteries connected to the right ventricle, a condition called double-outlet right ventricle. In a healthy heart, the aorta would connect to the left ventricle.
‘As complex as it can get’
“The cardiac anatomy was quite complicated,” Dr. Kuruvilla said.
After much analysis and discussion with his parents, Isaiah’s medical team decided his heart defects could be repaired surgically.
Dr. Vettukattil oversaw the creation of the 3-D heart model to guide Marcus Haw, MD, the pediatric congenital heart surgeon.
As far as complexity goes, I think this is as complex as it can get, for a complete repair. Not many other surgeons would have the operative skills and confidence to achieve that.
“For something like this, (the 3-D model) is very important,” Dr. Haw said. “It gives me a lot of confidence when I look inside and I know what I am going to do.”
It helped him navigate the anatomy of Isaiah’s heart. For example, knowing the tricuspid valve lay in an unusual spot, he made his incision to avoid that delicate structure.
“It is individual medicine and also precision medicine, in the sense that we are able to be more accurate,” he said.
Throughout the tests and discussions, Isaiah peppered his mom and dad with questions.
“He’s the most inquisitive kid I know,” Autumn said. “He knew the ins and outs of his medical condition.”
Although apprehensive about surgery, Isaiah “wasn’t super upset. He was kind of resolved that this was what we needed to do.”
The operation lasted eight hours, as Dr. Haw repaired the holes in the heart and rerouted the blood vessels.
“As far as complexity goes, I think this is as complex as it can get, for a complete repair,” Dr. Vettukattil said. “Not many other surgeons would have the operative skills and confidence to achieve that. There are not many people who would get this right.”
‘We have all been blessed’
Isaiah rebounded from the operation with a speed that impressed his parents and medical team.
His heart, though still on the wrong side of his body, now functions like other hearts―with four chambers collecting and pumping blood to the lungs and to the rest of his body.
“He’s doing great,” Autumn said.
Isaiah spent two weeks in the hospital and returned to school three weeks after surgery. He has limits on his activity for now―no climbing on monkey bars as he continues to heal. But his mom already sees results from an operation in Isaiah’s mouth and fingers, now a healthy shade of pink.
“We will have to monitor him,” Dr. Kuruvilla said. “But if his physiology remains the same as it is now, he really will be normal. I don’t predict he will have any restrictions.”
That change in outlook for Isaiah warms his heart.
“It reconfirms that this is my calling, as a pediatric cardiologist,” he said. “It really is tremendously rewarding to be involved in someone’s care like this.”
Autumn marvels at how much has happened in the past year―for Isaiah and for the Lunsford family.
“We have all been blessed and grown a lot through this experience,” she said.
“The picture looks so different today than it did back then,” she said. “We always had hope, but it ended up being better than what we hoped for.”
She predicts Isaiah, already fond of his doctors, will see them as heroes someday.
“I think his whole story has been a miracle,” she said. “I am just really thankful to be a part of it.”