When Samuel Guinn was born, his parents, Jennifer and Grant, expected at least one surprise. The couple from Jennings, Louisiana had chosen not to learn their third baby’s gender ahead of delivery. But soon after their beautiful boy’s birth, they got an unexpected surprise — he had a serious heart condition.
“Within an hour, the doctors noticed a heart murmur and transferred him to a larger hospital in Louisiana,” says Jennifer. “Once he was there, they diagnosed him with transposition of the great arteries (TGA) and a ventricular septal defect (VSD), but they couldn’t keep him stable, so they told us they wanted to send him to a bigger hospital in New Orleans.”
But Grant had done some research and found that the best pediatric hospital near them was in Texas. He asked to have Samuel transported there the next day. Once in Texas, Samuel was placed on a ventilator, and then on extracorporeal membrane oxygenation (ECMO) to stabilize him.
Fighting the odds
It was then that the Guinns received Samuel’s complete diagnosis. Samuel had double outlet right ventricle (DORV) with malposed great arteries, a VSD, pulmonary stenosis and a hypoplastic left ventricle. It was a lot for the couple to take in.
“He was on ECMO for 10 days, and had every hurdle thrown at him, but he was strong and managed to pull through,” says Jennifer. When he was able to have his first catheterization, the doctors recommended surgery for a single ventricle heart, meaning his heart would function as a single pumping ventricle instead of the normal two.
Finding a biventricular option
Before making a decision, Grant did more research. He found that the Complex Biventricular Repair Program at Boston Children’s Hospital offers the possibility of a two ventricle circulation, or a heart with two working ventricles, for some children with complex heart defects. The goal of the program is to take patients who would have traditionally gone through a single ventricle management plan, which can be associated with significant long-term problems, and devise a strategy to create a two ventricle circulation with a more normal blood flow pattern instead.
“After multiple discussions and research, we decided the biventricular repair would be best for Samuel, if it was possible. We sent all of his records to Boston for a second opinion, and they said they could help him,” says Jennifer.
The family flew to Boston and met with cardiologist Dr. Puja Banka and Dr. Pedro del Nido, chief of cardiac surgery. After a complete workup and catheterization, the team added straddling mitral valve to his list of diagnoses and del Nido confirmed that Samuel was a candidate for biventricular repair, but he wanted to wait a few months to perform the first surgery to give Samuel a chance to grow, given the complexity of the repair.
That November 2014, 10-month-old Samuel and his family returned to Boston for an 8-hour surgery that would begin his heart repair.
“We were warned that the first 24-48 hours after surgery was a critical time, so we were really nervous, but he did great,” says Jennifer.
A few days later, Samuel had another surgery to place a pulmonary artery band because of remaining VSDs and insert a pacemaker to help stabilize his heart rhythm. He did remarkably well once again and was able to go home shortly after.
Another unexpected surprise
All was going well until the following March, when Samuel suddenly went into heart failure. He was taken to Texas to be stabilized.
“Since the doctors in Texas didn’t perform Samuels’s biventricular repair and weren’t confident they could help him with another surgery, they recommended putting Samuel on the heart transplant list,” says Jennifer. “Because Dr. del Nido performed his first surgery, we were confident the Boston Children’s team knew Samuel’s anatomy best to make the next decision.
When they spoke to Dr. Banka, she explained their first option would be to repair whatever was going on inside his heart, and the second would be to do a single ventricle repair. A heart transplant would be their third option. “That sounded like a much better plan to us,” says Jennifer.
Once Samuel arrived in Boston, his team set about trying to figure out what had caused the heart failure. They discovered his left ventricle was getting extra blood flow from the remaining VSDs and leaking mitral and aortic valves.
Dr. del Nido operated on both the aortic and mitral valves, closed VSDs and was able to remove the pulmonary artery band. The family headed back to Louisiana and Samuel continued to thrive, doing his best to keep up with his two older siblings, McKenzie, 11, and Anthony, 8. Jennifer and Grant and their cardiologist in Texas, Dr. Buck Kyle, kept in close contact with the team in Boston to make decisions together about Samuel’s medications and care.
A planned trip and a peaceful recovery
In April of 2017, Samuel returned to Boston for an aortic valve repair. Over the previous year, his aortic valve had developed leakage again and his doctors wanted him to have the valve fixed before his symptoms worsened.
“This surgery and recovery felt less stressful than the previous three, and we felt at peace right after surgery, knowing he would recover quickly,” says Jennifer. Sure enough, they were all back home in Louisiana in less than two weeks.
Since then, Samuel has gained lots of weight, signed up for pre-kindergarten and joined a swim team. “He’s totally obsessed with swimming and water,” says Jennifer.
At his most recent follow-up appointment a few weeks ago, the Guinns received great news about Samuel’s heart.
“We walked out of Samuel’s appointment feeling blessed and reassured that his heart is the healthiest it’s been in his four years of life,” says Jennifer. “Our family is grateful to see Samuel continue to thrive and can’t thank the entire team at Boston Children’s enough for their huge, ongoing role in giving Samuel a brighter future.”
Learn more about the Complex Biventricular Repair Program.