Finding Her Rhythm

Watching Briana Aguilar run around the playroom on 6 East, you would never know she had a first-ever procedure to implant a child-sized defibrillator system only five days earlier. She plays with Monopoly money, saying she will use it to “go to Chuck E. Cheese” and smiles like a pro when a camera is held up. But it was a very different scene on June 8 when the 2-year-old was rushed to the hospital in her hometown of Manassas, Virginia, with her heart beating out of control and her skin turning blue.

“I looked up at the monitor and her heart rate was 248 beats per minute,” says her mother Lanette. “I knew something was seriously wrong.”
Unfortunately for Briana, such scenes were becoming all too common. She was diagnosed in utero with holes in her heart, known as atrial and ventricular septal defects, and a narrowing of her aorta, called a coarctation. Doctors in Miami, where Lanette and her husband Ruben lived at the time, repaired the defects after Briana’s birth, but may have inadvertently made the problem worse. Scar tissue from the procedure grew so thick that it blocked her aorta, triggering the first episode when little Briana became lethargic and turned blue.

After having the aorta un-blocked and moving to Virginia, Briana and her parents began a game of wait-and-see: wait until Briana’s next episode and see which hospital could help. The family traveled to three hospitals when the problem occurred on June 8. Doctors at Manassas Hospital had her airlifted to Georgetown Medical Center in Washington, DC, where things only got worse. Briana’s heart rate topped out at 300 beats per minute, she went into cardiac arrest and had to be cardioverted with shock paddles in an attempt to get her heart back into normal rhythm.
Briana was stabilized and airlifted to Fairfax Hospital the next day, but her primary care physician suggested that Children’s might be able to help, so Lanette and Ruben drove to Boston with hopes of finding a long-term solution.

Luckily, they found exactly what they were looking for. John Triedman, MD, and Charles Berul, MD, both associates in Cardiology, knew that Briana would need a defibrillator that could send a shock to her heart when it began to go into life-threatening tachycardia (uncontrolled beating), but also knew she was too small for many implantable devices.
“The wires of most defibrillators are too big for young children,” says Triedman. “They can damage the vessels surrounding the heart or the heart itself, making the problem worse. We thought this procedure was perfect for her.”

The procedure he’s referring to, implanting the leads of a small defibrillator under the skin of the chest wall near her heart, was an experimental procedure that had only been tested on animals. But Berul thinks it holds great promise. “Since it allows for minimally-invasive implantation without the need for large shocking wires through the veins or patches surrounding the heart, it might be the optimal procedure for small children requiring defibrillators.”

The results so far have exceeded their expectations. Joseph Forbess, MD, assistant in Cardiovascular Surgery, performed the procedure on July 26 and Briana was released only five days later, looking for all the world like a child with a happy and healthy life ahead of her.
As her parents loaded the car to head home for the first time in more than two months, Briana’s father, Ruben, reflected on the care that he and his family have received at Children’s. “We’ve seen a lot of hospitals, especially in the last couple of months, and there was a different feeling from the day we walked in here. The communication was unbelievable. They tell you the things they can do, but are honest about the things they can’t, and that’s very reassuring.”
Briana’s mother, who worries that none of the doctors back home will be able to handle her daughter’s complicated case, put it a different way. “If we need to move closer to Boston to get her the care she needs, we will.”