The stunning, unexpected recovery of two Tucson children from life-threatening heart failure will dramatically change the way such youngsters are treated — in Tucson and perhaps worldwide, experts at University Medical Center say.
Instead of resorting to heart transplants for these life-threatened children, doctors now believe many can recover full use of their natural hearts, using a mechanical heart-assist device to promote healing.
That is what happened in recent weeks for 16-month-old Tiana Lopez and 9-month-old Itxair Rodriguez at UMC, after each survived several weeks with the help of the child-sized assist device, the Berlin Heart.
Their recent, back-to-back recoveries — from heart damage that put them near death — have astonished their UMC doctors, who fully expected both children to need donor hearts.
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This experience may dramatically transform how doctors handle very young children with cardiomyopathy — severely weakened heart muscle, likely caused by a viral attack. No longer will heart transplants be the first priority.
"This could be a paradigm shift," said Dr. Jack G. Copeland, head of the University of Arizona's heart transplant team. Copeland implanted the Berlin Hearts in Tiana and Itxair and has pioneered the use of this device in children in the United States since 2000.
"We would now think first of recovery and second of transplant, and become more aggressive about using assist-device therapy for small children," he said, noting these recent recoveries are "exceptional."
"Previously, we would not have even considered such a thing possible."
Equally flabbergasted is Richard Smith, the engineer who supervises the use of all mechanical-heart devices, including Berlin Hearts, at UMC.
"Why would you cut the hearts out of little kids if you don't have to?" he asked. "We are looking at two children here who are now very near normal heart function. We're as stunned as anybody about this, and I have to say we don't fully understand it. But I think we've got a new therapy here."
However, there remains one major obstacle to making this happen, at UMC or anywhere in the United States. The Berlin Heart, manufactured in Germany, has never gone through U.S.-based clinical trials required for approval for use in this country.
Every time it has been used in the United States — on fewer than 50 children, including three at UMC — doctors have had to appeal to the Food and Drug Administration for permission, then have the device flown in from Germany — which can take up to a week. That can be too long for a dying child, Copeland has pointed out.
And after the device is used on each child, it must be returned to Germany.
But now, after these most recent success stories out of UMC, the push is on to get a "humanitarian-device exemption" for the Berlin Heart from the FDA. If that happens, UMC and other hospitals could buy Berlin Hearts and keep them in stock for immediate emergency use.
"We're pounding on them," Smith said. "We want this thing on the shelf here, as soon as possible."
However, the earliest that could happen is by the end of this year, said officials at Berlin Heart, in Germany. They are now enmeshed in the process of applying to the FDA for the humanitarian exemption.
"We are working to answer all their questions and go through the tests," said Dr. Johannes Mueller, the company's chief scientific officer.
"There's a lot of paperwork involved, and we expect to have it finalized by the fall. After that, the FDA needs three months to study it," he said. "Will we get approval? We're not 100 percent sure. It's not easy to deal with the FDA. But we're quite hopeful. The outcomes have been very good with the Berlin Heart."
In the United States, the device enjoys an 80 percent success rate at rescuing heart-stricken children. But most of them — 85-90 percent — so far have gone on to heart transplants, a number likely to drop significantly if Copeland's approach is widely accepted.
A total of 158 children have used the device — the longest survival nearly 15 months on it.
In the recent Tucson cases, Tiana Lopez spent 21 days on the device, while it pumped for her severely weakened and enlarged heart. She has returned home and is functioning as a normal toddler, her parents and doctors say.
While Tiana was still at UMC, Itxair was placed on the Berlin Heart, on March 6, and lived on it for 15 days, until it was removed last week. Both children had been slated for heart transplants but were taken off the waiting list when their own hearts began to regain strength.
Described as playful, happy, and eating well, Itxair was ready Wednesday to be moved out of intensive care and may be well enough to return home within a few days, Smith said.
The key to their wholly surprising recoveries was putting them on the Berlin Heart as soon as possible, rather than waiting until their hearts were all but gone, Copeland and Smith said.
The timing was not as speedy for Tiana, who had to wait several days to have the device shipped here. But because the Berlin Heart was at UMC when Itxair got sick, she was implanted immediately.
"With these two kids, we did not wait until the very last minute, when they were going over the edge. We pulled that string first and got the Berlin Heart here," Smith said. "That may explain their recovery.
"And that's why we want it in our closet, ready to go much earlier with these children."
"Why would you cut the hearts out of little kids if you don't have to?"
Richard Smith, Berlin Hearts engineer at UMC
