Imagine - Three of a Kind

The joint efforts of a trio of doctors spanning institutions, specialties and even subspecialties brought a pioneering fetal cardiac procedure to Southern California for the first time.

I cannot believe this.

It was all an exasperated Ramen Chmait, MD, could think. His patient, 28-year-old Sandra Barraza, lay on an operating table at CHA Hollywood Presbyterian Medical Center in her sixth month of pregnancy, fastened in for a surgery intended to open up her baby’s critically narrow aortic valve. It had taken a consummate amount of diligence, trust, coordination and expertise to lead her there. This would be the first time the procedure, called a fetal aortic valvuloplasty, was performed by doctors from the Institute for Maternal-Fetal Health (IMFH), a partnership of physicians across Children’s Hospital Los Angeles and the University of Southern California. In fact, this would be the first time the procedure was done anywhere in Southern California.

Barely a year earlier, the trio of doctors who would work to pass a needle through Barraza’s womb into a fetal heart the size of a walnut would not have been available to her. Frank Ing, MD, an interventional cardiologist whose job it would be to widen the impaired valve with a balloon, only came to Children’s Hospital in September 2012. The director of the hospital’s Cardiac Catheterization Laboratory, he had done the procedure multiple times, but not here. Chmait, an obstetrician who specializes in fetal surgeries, had also participated in the procedure, but again, never at Children’s Hospital -- and never with Ing.

The third player was pediatric cardiologist Jay Pruetz, MD, an expert in the use of ultrasound imagery to identify fetal heart disease. Today his images would guide Chmait’s needle into the uterus, through the fetal chest wall and toward the left ventricle. Ing would then run a wire through the needle, ride a balloon along the wire until it reached the aortic valve, and inflate it.

Painstaking run-throughs had made it so that places, positions and responsibilities were so familiar, the threat of any glitch was virtually removed.

And then this happened.

Barraza’s baby had been sedated, but right after the sedation was administered, the fetus turned onto its stomach. Now Chmait had no access to the heart, with a fetus rendered still by the anesthetic. Just minutes earlier it had been on its back, in perfect position.

“I remember thinking,” Chmait says, “I cannot believe this.”

Risk vs. Reward

Pruetz had already waited more than five years for this day, and now he would have to wait a little longer. Unlike Ing and Chmait, Pruetz, who came on staff at Children’s Hospital in 2008, had never participated in the procedure before. CHLA’s director of Fetal Cardiology, he had diagnosed critical aortic stenosis numerous times, but always had to refer the patients elsewhere for treatment. The hospital simply didn’t have the people to execute it.

“But when Dr. Ing joined us, and we knew we had Dr. Chmait, we realized that now was the moment,” says Pruetz. “We had the people with the expertise and the skills to make this not only doable, but likely successful.”

Even recently, Pruetz had seen several patients who were considered for the procedure. The first three dropped out. The fourth was Barraza, a Sylmar, Calif., resident having her first child with Joseph Molina; the two were to be married a month after the surgery. After making the diagnosis, Pruetz sent Barraza to Ing and Chmait.

“I didn’t get it at first,” she says. So Ing played her a video of the procedure, and Chmait showed her a slide show of the heart. The risk of moving forward was explained: If she went into labor during the surgery, the baby wouldn’t survive.

There was virtually equal risk in not doing it. With critical aortic stenosis, the aortic valve is so pinched, blood can’t pump adequately out to the body and gets trapped in the left ventricle. Untreated, the ventricle would become so damaged that Barraza’s baby would be born with hypoplastic (meaning underdeveloped) left heart syndrome, or HLHS, with only the right ventricle capable of pumping blood. Aortic valvuloplasty opens the valve and allows the baby the opportunity to have a normal, two-ventricle circulation.

But there was no guarantee of reward, or even favorable odds of it. Even when the procedure succeeds in stretching the valve, both Ing and Pruetz note that the desired outcome of two functioning ventricles is seen in less than half of cases. Ing says, “What we haven’t figured out is: Will the heart grow the way we want it to grow?”

Rehearsal Time

As director of the Los Angeles Fetal Therapy program, a component of the Institute for Maternal-Fetal Health, Chmait does about 130 fetal surgeries a year. He was confident the experience of the IMFH team would see Barraza through.

“It’s true that this particular intervention was new to Children’s Hospital Los Angeles,” he says, “but the people who were going to do the procedure had done it before.” Chmait’s experience had come at St. Joseph’s Women’s Hospital in Tampa, Fla., assisting Ruben Quintero, MD, considered a pioneer in fetal surgery. 

Molina, the baby’s father, saw the advantages of going first. “I felt more confident they would go the extra mile to make sure that everything went right, and better than expected,” he says. “You don’t want your grand opening to go bad.”

Ing, too, said as much: “If you mess up the first time, it’s going to take a long time to get to the next case.”

To guard against that, Ing called on a best practice he had employed in the past. He sank a grape into a Jell-O mold, which then obscured the small fruit from view. Could Chmait next plunge a needle into the Jell-O and find the grape using only Pruetz’s ultrasound guidance?

“It’s not an exact model, but it replicates an idea that you have a tiny little shape you have to enter without seeing it,” Ing says. “Prove to me you can get the needle there based on this image.”

“I was literally imaging the grape,” Pruetz says. “I’m telling Dr. Chmait, ‘This is where you are, you’re at the grape.’”

Chmait found the mark.

But the dry runs served a larger purpose than target practice. They also gave Chmait and Ing the chance to sync the placement of their hands. During the procedure, the two men would be working one on top of the other, Chmait first passing the needle toward the aortic valve, Ing then inserting the wire. Ing wanted to balance his hand on the flat, stable part of Chmait’s hand -- the soft patch between the thumb and forefinger -- as he advanced the wire.

“Just like any basketball team, you put players together, they have to learn to work with each other,” Ing says. “It’s just trying to figure out each other’s preferences, knowing what the steps would be.”

Start to Finish

For 45 minutes, Chmait manipulated Barraza’s abdomen as if he were kneading dough, but to no avail. The baby wouldn’t budge. So he got resourceful. He drew back the needle from the tube that enclosed it and then used the blunted instrument to turn the fetus over. “I actually used the side of it to get the baby to flip in the right direction,” he says.

The procedure could now begin, and the benefits of all the rehearsing became apparent. Steered by Pruetz’s images, Chmait routed his needle to the baby’s left ventricle. Ing inserted the wire, got the balloon into position and inflated it, tearing open the leaflets of the aortic valve. The needle, wire and balloon were promptly removed. Chmait and Ing were in and out in 15 minutes without complication.

“When you have a needle in the fetal heart,” Pruetz says, “the longer you’re there, the more the risk.”

Curiously, fetal aortic valvuloplasty falls under the category of minimally invasive. That’s a clinical designation, to be sure. Symbolically, there was nothing minimal about the imprint it left, particularly on Pruetz, who had waited years for the opportunity to make it available to his patients.

“It was a first for our institution,” he says, “and it was a first for me and many other people in the room.”

Defining Success

In the aftermath, one question prevailed: Did it work? That won’t likely be answerable for some time. All three doctors make a point of separating technical and clinical success.

“There was a valve that was tight, and we opened it,” Chmait says. “It’s pumping blood forward, unlike before the procedure. So we were technically successful, 100 percent. There’s no question.”

But the problems with the baby’s heart didn’t end with the aortic valve. The mitral valve -- which leads into the left ventricle, while the aortic valve leads out of it -- was also diseased. That had been a concern of Pruetz’s throughout.

“It’s thick and immobile and just not opening up well,” he says. But no action could be taken because no intervention exists for the mitral valve. Pruetz suspects it may be “the Achilles’ heel of this lesion.”

Ing contends that even if it turns out the procedure was unable to avert hypoplastic left heart syndrome, that wouldn’t automatically negate the work the doctors did. “There are questions about whether we can bring these patients to two ventricles,” he says. “Some do get there, but even if they don’t, are they still better off? Is their survival better? We need time to figure that out.”

No one, though, would dispute the success of the collaboration, across expertise, across institutions, across the professional histories of the three physicians. “We all fought for this baby,” Chmait says, “different people with very different backgrounds. We all had to come together.”

Looking forward, Pruetz says Children’s Hospital is now positioned as a leader in fetal cardiology. “Building this type of intervention program gives us the ability to push the field forward.

“The truth is, this is a relatively new science, so we don’t really know the long-term ramifications of doing this procedure. It’s to be continued, right?”

He didn’t say whether he meant the hospital or the baby. Either way, it was true for both.