How do you know if you’re making the right medical decision for your child?
Nine years ago, Rosamaria Rand and her family faced this difficult question. While pregnant, Rosamaria learned that her daughter, Alexa, had a severe heart defect known as hypoplastic left heart syndrome (HLHS), where half the heart fails to develop properly. Doctors told the Rands that most children born with HLHS go through a process known as single ventricle palliation or “SVP”— a series of three surgeries to reconstruct the heart so it can function with a single working ventricle. They also let the Rands know that only about 50 percent of patients treated this way survive to adulthood.
Alexa had her initial procedure in utero (before she was born) at Boston Children’s. At this time, her parents learned about an alternative treatment method to SVP that can help patients with HLHS. Under development at Boston Children’s, this relatively new approach held the promise of helping children born with HLHS avoid long-term complications and improve their overall health.
Called the “staged left ventricle recruitment” (SLVR) strategy, this method had been in development at Boston Children’s since 2001. It uses the standard single-ventricle treatment for HLHS, but incorporates other procedures to reconstruct the heart, leaving the patient with two working ventricles, not just one. According to the doctors who developed SLVR, the new method is more intense and invasive than SVP, but it could help patients avoid future hospitalizations, additional surgeries or a heart transplant.
As the Rands were deciding whether Alexa would be treated with the standard SVP process or the newer SLVR approach, they sought guidance from their local doctors on Long Island and Wayne Tworetzky, MD, who they met while at Boston Children’s. “We took everything in, and we listened to both sides.” Rosamaria says. “But we always wanted to know what Wayne thought.”
Tworetzky and others explained what could go right—and what could go wrong—with both approaches. While they were excited about the possibilities SLVR presented, Rosamaria said knowing the procedure was so new lent its own set of worries. “It was scary,” she remembers. “Not something we took lightly.”
After consultations with the doctors, much family support and “a lot of praying,” the Rands opted for the new SLVR technique for Alexa’s care.
It was “sketchy at the beginning,” Rosamaria reports, as Alexa faced complications. However, in the past few years Alexa has been “doing great.” Now nine-years-old, Alexa is doing well in school and has shown a strong interest in music. “She joined the chorus,” a proud Rosamaria reports, and is writing songs and starting to learn the cello.
“She’s a go-getter,” Rosamaria says. “She wants to try to do everything.”
A recent report by members of the Boston Children’s Cardiac Surgery and Cardiology, suggests Alexa may not be alone in benefiting from this exciting new medical technique. Published in the Journal of the American College of Cardiology, the study follows the recovery of 34 children with the SLVR approach between 2001 and 2010.
The study, led by cardiac surgeon Sitaram M. Emani, MD, shows that children treated with the SLVR approach had as good, if not better, survival rates compared to children treated with SVP. In addition, the valves and chambers of SLVR-treated children grew a great deal. They were even able to successfully convert 12 of the 34 patients to complete, two-ventricle circulation.
According to Emani, “We think that children treated with SLVR will have fewer complications as they grow, better survival outcomes and less need for a transplant, thereby keeping long-term medical expenses down.” Looking ahead, Emani says, “As we gain experience, we hope to reduce both the amount of time in surgery and the number of surgeries.”
With SLVR, the future looks brighter for children like Alexa Rand who were born with HLHS. Parents of children with serious heart conditions now have another option to consider, an option that has shown great promise for reducing multiple procedures and improving overall health. Still, a parent’s decision to choose the right treatment for their child will never be easy.
“It was not a simple decision,” said Rosamaria Rand of choosing SLVR for her daughter, “but we didn’t want to look back and think we didn’t do whatever we could.”