Amazing Joy: Seeking Care as an Adult Congenital Heart Patient at Children’s Hospital New Orleans

Joy Soublet has been a walking miracle all her life. When she was just one month old, doctors discovered that she had a rare congenital heart condition impacting the structure and function of her heart. Known as double outlet right ventricle, her heart’s two major arteries—the pulmonary artery and the aorta—both connected to the right ventricle. Typically, the aorta connects to the left ventricle, which is responsible for pumping oxygen-rich blood into the body. Joy also had a ventricular septal defect, which is a hole in the wall that separates the lower two chambers of the heart.

In most cases, babies undergo surgeries for these heart defects at a very young age. But Joy was unique. Despite the structural problems inside her chest, she was a healthy kid who thrived during her childhood and adolescence—so much so that her doctors continued to delay the open-heart surgery she would eventually need. But the summer after she graduated high school, at 18 years old, Joy finally underwent the surgery to replace her pulmonary valve.

Fast forward to 35 years later, and Joy’s valve was still going strong—or so she thought. But in September of last year, she began experiencing concerning symptoms, including shortness of breath and extreme fatigue. First, she blamed it on her thyroid levels being off. Then, she began wondering if she was entering menopause.

“I remember the weekend of our church picnic, I was carrying two bags filled with jars of pickles up the steps to our church, and when I reached the top, I had to sit down and catch my breath,” Joy recalled.

Joy brushed it off at the time, but a month later, she found herself struggling to keep up during a birthday weekend trip to Alabama and Tennessee. Her friends kept checking on her to see if she was OK, but she smiled her friendly smile and reassured them that everything was fine.

“I’m usually a ball of energy and the life of the party,” Joy said. “It’s not like me to be quiet or to feel the need to sleep in the middle of the day.”

The next month, Joy flew to Texas to spend Thanksgiving with her best friend—who could immediately tell Joy wasn’t herself. Still, the two had a nice holiday together, and that Sunday, Joy headed to the airport to fly home.

At the airport, however, Joy could barely get through the TSA line.

“I remember asking if there was any shorter way because I could barely breathe,” Joy said. “Then all of a sudden, I started throwing up.”

Joy knew then that something was wrong. She managed to get on the plane and make it home, and she promised herself she would go to the emergency room the next day. In the morning, however, she had so little energy that she was unable to get out of bed. Finally, on Tuesday, she forced herself to get dressed and drive straight to the emergency room at University Medical Center (UMC) New Orleans.

Joy spent the next two days undergoing tests. She told the specialists there about her heart condition from her childhood, and that her established cardiologist was Dr. Scott Macicek, a Pediatric and Adult Congenital Cardiologist and Electrophysiologist and the Chief Experience Officer at Children’s Hospital New Orleans.

It was quickly determined that Joy’s valve was failing, and for the second time in her life she would need to undergo open-heart surgery—and she would have it at Children’s Hospital New Orleans.

Children’s hospitals are often the best place for adults with congenital heart conditions to be treated because they typically have teams with specialized knowledge and experience in congenital heart disease, a condition present from birth. Since these conditions are diagnosed and often treated starting in childhood, pediatric cardiologists and cardiac surgeons at children’s hospitals are highly skilled in dealing with the complexities of these conditions.

The Heart Center at Children’s Hospital New Orleans is the leading heart care provider in Louisiana and the Gulf South for adults with congenital heart disease. The program offers adult congenital heart services focused on providing extensive care to both teenagers and adults with congenital heart disease, regardless of their age or the severity of their condition. The team is known for their high standards and comprehensive care approach, and they also maintain a multidisciplinary partnership with LCMC adult hospitals, granting patients access to specialized adult congenital heart care.

Joy was transferred to Children’s Hospital New Orleans, and on Monday, December 4 at 7 a.m., Dr. Frank Pigula, Chief of Pediatric Cardiothoracic Surgery and Co-Director of The Heart Center at Children’s Hospital New Orleans, performed Joy’s surgery.

“Joy had previously undergone surgical interventions, including VSD repair, a right ventricle to pulmonary artery conduit, and the placement of a bioprosthetic pulmonic valve,” Dr. Pigula said. “But with her recent history of arrhythmias, specifically non-sustained atrial tachycardia and ventricular tachycardia, we performed an atrial flutter ablation and VT induction. Unfortunately, Joy had also developed severe right ventricular outflow obstruction due to a pseudoaneurysm, so we needed to do a pulmonary valve replacement procedure to improve cardiac function and alleviate the obstruction in the right ventricular outflow tract."

The surgery went perfectly, and even more, Joy bewildered her doctors, nurses, and the entire care team with how quickly she recovered.

“Everyone kept coming in and saying how amazing I was, so I started using that word, too,” Joy said. “When they asked me how I was feeling, I would just say, ‘Amazing.’”

Joy said she will forever be thankful for the compassionate staff at Children’s Hospital New Orleans for supporting her recovery, and especially for the congenital heart specialists whose expert care repaired her heart.

“If you must have heart surgery for a congenital heart condition, Children’s Hospital was a great place to be,” Joy said. “The nurses and CNAs and everyone were so attentive and helpful, and I knew I was in the best hands.”

Joy continues to go to Children’s Hospital New Orleans for monitoring and follow-up care, and when she does, she makes a point to visit the inpatient nurses and care team, who she now considers friends.

“I’m back home and continuing to recover, but I truly feel like myself again,” Joy said. "At the time, I was unaware of the severity of my condition. My valve was severely blocked, reducing blood flow to the size of a pinhole. I'm deeply grateful and feel blessed to be here and to be able to share my story.”

To learn more about adult congenital heart services available to Children’s Hospital New Orleans, visit https://www.chnola.org/services/heart-center/adult-congenital-heart-services/.