'He Did Beat the Odds': Patient With Congenital Heart Defect Finds Care at Cook Children's From Infancy to Adulthood

Elmer Rios first came by ambulance to Cook Children’s as an infant in critical condition. Twenty years and six open-heart surgeries later, he still regularly visits Cook Children’s for checkups thanks to a pioneering program for adults born with heart defects.

Elmer’s pediatric cardiologist described him as the sickest of the sick when he arrived at Cook Children’s just days after he was born in 2002.

“He was cold. He was blue, and he wasn’t moving much, and he wasn’t making much urine. He was having difficulty breathing,” said Lisa Roten, M.D., who has treated Elmer through the years. “He was not getting adequate blood flow to his body, and so his organs were shutting down.”   

Tests revealed the cause: The left ventricle in his heart was undeveloped, a condition known as Hypoplastic Left Heart Syndrome (HLHS). The left ventricle is normally the heart’s stronger side, designed to pump oxygenated blood throughout the body. In HLHS, the left ventricle does not form appropriately.

Doctors worried what damage might have been done to Elmer’s brain, liver and kidneys from the poor circulation and near-fatal high acidity in his tissues. Immediate surgery could not be performed until his organ function improved.  Two weeks after birth, he was able to undergo his first surgery. 

These days Elmer lives with a pacemaker that regulates his heart rhythm. Like most people with HLHS, he tires easily and will ultimately require a heart transplant.

Even so, he stays busy accomplishing major milestones – graduation from Fort Worth’s O.D. Wyatt High School, enrollment at Tarrant County College, a full-time job at Amazon and purchase of a house.

“You never really know why somebody is the one who beats the odds,” Dr. Roten said. “It’s amazing. He’s somebody who’s gone through so much and shouldn’t have survived but did survive, and is making a good life for himself and his family.”

Elmer must take precautions to avoid heavy lifting and overexertion. Yet he occasionally forgets that he has a heart problem. “I just want to feel normal. I just want to be normal,” he said. 

In February to observe American Heart Month, we’re highlighting Elmer in recognition of the nearly 40,000 babies born in the United States each year with congenital heart defects. 

Elmer has shown resilience by achieving big goals in spite of in his lifelong health struggles. And he expresses gratitude for his ongoing care in the Adult Congenital Heart Disease program at Cook Children’s. This is his story.

How to Rebuild a Heart

According to the U.S. Centers for Disease Control and Prevention, about 1,025 children are born each year with HLHS, which affects the leftsided structures where most of the heart’s pumping workload takes place. 

Sometimes the abnormalities are detected in ultrasounds during pregnancy. In other cases the problem isn’t apparent until a few days after birth, when newborns with HLHS start showing a weak pulse, breathing difficulty and bluish or ashen skin due to lack of oxygen. Pulse oximetry screening and echocardiography can help make the diagnosis.

“Mom says that if I wouldn’t have gone to the hospital that same day, I would have died,” Elmer said, translating from Spanish for Brenda Rios.

Treatment for HLHS consists of surgery in three sequential stages that make the heart’s right side the main pumping chamber to the body:

1.     Norwood – Creates a new aorta, allowing the right ventricle to pump blood to the lungs and the rest of the body.

2.     Glenn – Creates a connection between the pulmonary artery and the superior vena cava, directing blue blood to the lungs.    

3.     Fontan – Another change in connections that redirects the Inferior vena cava blood flow, preventing the oxygen-rich and oxygen-poor blood from mixing together in the heart. 

Admitted to intensive care at Cook Children’s, Elmer was two-weeks old when he underwent the Norwood surgery, the first phase to rebuild his heart anatomy.  

Three months later he needed another surgery to open up an atrial septal defect – a hole in between the chambers of the heart – and for placement of a shunt. Elmer’s skin then turned even bluer, so a second shunt was added. When his heart rhythm did not return to normal, a pacemaker was put in.

“We usually think people who have that rocky of a course, they’re not going to be survivors,” Dr. Roten said. “We didn’t know what his long term brain function would be.”

Elmer underwent the Glenn surgery at age eight months. When the Fontan surgery was performed at age 3, his original pacemaker was replaced. At age 6, another surgery was needed to close a hole in his heart that had been deliberately left to aid in circulation. Each of the six bypasses brought the risk of strokes and brain complications, Dr. Roten pointed out. His pacemaker lead was replaced in 2014 and 2018. 

“Elmer is inspirational to the HLHS family because he did beat the odds, and he was one of the worst ones out there,” she said.  

Following the series of surgeries, many babies born with left heart defects regain a normal skin color. But it’s not a cure …. HLHS patients must receive follow-up cardiology care for the rest of their lives because of the risk of heart failure and other complications, such as slow growth and developmental delays. 

In the 40 years since the first successful Norwood procedure, the population of adults with HLHS is small but growing, according to a study cited by the American Heart Association.

Dr. Roten said Elmer will require a heart transplant ultimately. He comes for checkups every six months and will never outgrow the Adult Congenital Heart Disease program at Cook Children’s. She expressed admiration for how hard he has worked at school and at jobs despite low physical stamina.

“He’s had to grow up fast. His tomorrows are never guaranteed,” Dr. Roten said. “Most adolescents have that ‘I’m going to live forever’ attitude. Elmer’s never thought that about himself. But that has not changed his desire to go on with life.”

Warrior with a Kind Heart

In Elmer’s earliest memory of Cook Children’s, he is 4 or 5 years old and having blood drawn. As a child he played soccer by himself – not on a team – in order to protect the pacemaker. 

By his teen years he focused on academics with a special interest in science. Maybe, he thought, some day he could be a pediatric cardiologist. In high school Elmer took dual credit college courses and simultaneously worked evenings at a grocery store.   

Now he takes classes at TCC two days a week while working full time at an Amazon fulfilment center, where he drives a forklift and operates other machinery to lessen the physical aspects of the job. He gets fatigued, he admits, especially when it’s hot. While Elmer doesn’t want his heart defect to be a barrier or excuse, he acknowledges that it isn’t easy dealing with HLHS.   

His advice to others with the same condition? Never give up.

“Don’t let a heart defect stop you from accomplishing whatever you want to do when you grow up. Don’t let other kids or anybody else see you in a different way,” he said. “Whatever goals you have, just keep going at it, no matter what. Because one day you’ll look back and say ‘Wow, I’ve been through this journey. I’ve been through so much.’”

Elmer thinks his friends would describe him as happy and energetic. When he’s not working or studying, he plays chess. In 2021 he bought a three-bedroom house in Fort Worth, where he lives with his mother, stepfather and two younger siblings. 

He was accepted to his dream school, Texas Christian University, but missed out on the scholarship he needed to afford it. That disappointment aside, he’s instead considering the University of Texas at Arlington after he finishes his basics at TCC.

His mother remembers her fear when infant Elmer was fighting for his life and there were no guarantees he would make it. Brenda believes her “warrior” son has a unique purpose in life.   

“She always tells me ‘You have a big heart, even though your heart is undeveloped and it’s pretty weak. You always have a big heart,’” he said.

Elmer remembers following Dr. Roten around her office when he was a little boy. He remembers his surgeon Vincent Tam, M.D., urging him to get straight As in school. 

At one point he belonged to a support group called Mended Little Hearts, where he answered questions from younger patients. He career shadowed Dr. Roten and Dr. Tam because he hopes some day to work for Cook Children’s, the place he calls his second home. Helping others appeals to him.

“Cook Children’s is like half of my life. I grew up there. I met so many awesome staff and doctors and nurses and practitioners,” Elmer said.

Transitioning from pediatric care to the Adult Congenital Heart Disease program was easy, he said. The program is one of only a few formal programs nationwide to offer inpatient and outpatient care for teens and adults who were born with heart defects.

For now, Elmer takes aspirin and a medication to control his blood pressure. He tries to avoid overtaxing his body. Brenda still urges him to be careful with his pacemaker. But he doesn’t want a heart transplant just yet.  

“I think it’s better for me to just stay with the heart I have unless it’s really required for me to get a transplant. Then I would consider it,” he said, laughing. “But until then, I’m pretty happy with my own heart.”