Developing Better Devices

Mother Amy Ramer and daughter MacKenzie, patient, child, atrial septal defect

Amy Ramer and daughter
MacKenzie have similar heart
defects — but MacKenzie's
recovery was easier, thanks
to a new device tested at
Seattle Children's.

When Amy Ramer's first child was born in July 2002, it took only two days for a troubling heart murmur to turn into a certain diagnosis: Little MacKenzie had an atrial septal defect — a hole in the upper wall of her heart.

After several trips to consult with specialists at Madigan Army Medical Center near Seattle, "We were told that the hole would probably close up on its own," recalls Ramer, an Army nurse who was stationed in Alaska with her husband, Cory, when MacKenzie was born.

After Ramer's discharge, the family moved back to Helena, Montana, where pediatric cardiologist Dr. Bruce Hardy began to monitor MacKenzie's condition — the quarter-inch hole hadn't closed, but the little girl was growing and developing just fine.

But a near tragedy in early 2005 made Dr. Hardy insist that MacKenzie's heart be fixed sooner rather than later: Ramer had a stroke brought on by a heart condition similar to her daughter's.

"I started having migraines at about the age of 7, but no one ever connected them to a heart defect," recalls Ramer. "I lived 28 years before finding out the hard way."

Ramer's surgical recovery was difficult. The stiff, wire device implanted to close the hole made her heart beat irregularly and she needed medications. She worried that her precious 3-year-old would suffer the same fate.

Lucky for the Ramers, Hardy referred them to Children's interventional cardiologist Dr. Thomas Jones.

When the couple arrived at Jones' office in September 2005, he immediately offered MacKenzie a few toys. The normally shy girl slid off her mom's lap and played at his feet. "MacKenzie trusted him right off," explains Ramer. "After we got done talking, we knew without a doubt that he was the one to fix MacKenzie's heart."

Jones gave the couple two options: Use the FDA-approved device put in Ramer's own heart or be part of a clinical trial for an experimental device — the Helex Septal Occluder — which featured a Gore-Tex membrane rather than the usual wire mesh construction. The Ramers chose the Helex because Jones had successfully implanted the device in nearly 50 children in addition to having participated in its design and initial testing.

"Before the surgery, we thought MacKenzie had a lot of energy," says Ramer. "A day after she had the device implanted, she was bouncing off the walls. We jokingly asked Dr. Jones if he would take it out to save us from chasing her around!"

Six months after her surgery, MacKenzie had grown an inch and gained 10 pounds; she never needed any medication. In fall 2006, the FDA approved the Helex based on results of the clinical trials in which Jones was the lead author.