Collaborative effort involving milestone procedure saves premature baby with complex heart disease

Baby Hope looked into her mother’s eyes and gurgled.

Four days short of turning 9 months old, wearing a white onesie with the words “Best Gift Ever” on the front, she made more baby talk.

“You’re always a big chatterbox – what are you saying?” her mother, Elizabeth “Becca” Wyneken, said as she smiled and stared into Hope’s blue eyes.

Becca and Hope endured a lot to get to where they are now — a happy and very grateful mom and a relatively healthy 9-month-old baby girl whose light-brown hair is just starting to fill in.

The odds were stacked against Hope when she was born prematurely at 31 weeks and five days, weighing just 2 pounds, 3 ounces. Today, Hope is alive thanks to a team of doctors, nurses and others who cared for her throughout a four month stay on CHOC’s neonatal intensive care unit (NICU) and cardiovascular intensive care unit (CVICU).

Born with a complex heart disease, as well as only one kidney and defects on her right leg and foot, Hope’s cardiac neonatologist, Dr. Amir Ashrafi, pegged her chances of survival at between 20 and 30%.

When Becca first set eyes on Hope a couple of days after she was born, she was very concerned about her baby’s health.

“Don’t worry, Mom,” Dr. Ashrafi told Becca that day. “I think we can help her.”

It would take an extensive collaboration between highly regarded cardiovascular interventionalists, some of whom were consulted at hospitals as far away as London, to do so.

And it would involve a high-risk procedure never performed on a baby so small at CHOC.

Dr. Amir Ashrafi, neonatologist at CHOC

Grim news at 20-week scan

At 18 weeks pregnant, Becca, a teacher’s aide, went in for a checkup. Blood tests showed her baby had a high risk for Down syndrome and spina bifida.

Two weeks later, a scan of her baby’s anatomy revealed other potential problems.

Her baby had no kidneys, Becca was told. She appeared to have no bladder, no right leg, no lungs, issues with her bowels, and a heart defect.

“I don’t think I stopped crying for the rest of the day,” Becca recalls. “It was horrible.”

She couldn’t drive home from the clinic. A friend had to pick her up. That night, Becca had dinner with her mother and aunt.

Later, lying beside her mother, Becca cried.

“I can’t believe this is happening,” she said.

She felt a poke in her belly.

“Over and over again, when I got upset, she would poke me,” Becca said.

At that moment, she decided on a name for her baby.

“Hope,” Becca told her mother.

Second opinion reveals true complications

Becca got a second opinion about her unborn baby’s condition.

Her baby was missing a kidney and had a leg defect, she was told. Most seriously, Becca was told, she had a defect on her right ventricle, the chamber within the heart responsible for pumping oxygen-depleted blood to the lungs.

Hope’s aorta and pulmonary artery that carry blood away from the heart hadn’t developed properly. She had a hole in her heart as well as one in her left superior vena cava, a vein that helps circulate deoxygenated blood back to the heart. These holes caused blood to drain incorrectly; Hope would need a team of doctors to correct the blood flow.

“Being very small with complex heart disease, your options are very limited with what you can do and the timing of any procedures,” said Dr. Ahmad Ellini, Hope’s primary pediatric cardiologist.

There were lots of sleepless nights as Hope’s team of doctors and nurses monitored her closely. Becca was beside her nearly every night.

Dr. Ashrafi and Dr. Ellini consulted with two outside experts, San Francisco-based Dr. Mohan Reddy, who specializes in complex heart disease in small newborns, as well as renowned thoracic and cardiac surgeon Dr. Glen Van Arsdell of Ronald Reagan UCLA Medical Center on the best course of action.

The team of physicians determined that a stent needed to be inserted under a pulmonary artery that was becoming too narrow, making it hard for blood to flow through it. Such a procedure is risky, especially on a baby so small.

“In Hope’s case, the idea was if we could open up the area below the valve while not injuring the valve, that would be a home run,” said Dr. Sanjay Sinha, a CHOC pediatric cardiologist who put the stent in Hope’s heart. “Two things made this difficult: she was very small, and we had no stents this size.”

A vendor was able to secure the small stent needed a day before Hope’s surgery.

Assisting Dr. Sinha during the procedure was Dr. Michael Recto, medical director of CHOC’s Cardiac Catheterization Lab.

Observing the recently developed procedure, known as valve-sparing RVOT (right ventricular outflow tract) stent placement, were several cardiologists, from CHOC and other pediatric hospitals.

“In some patients, there is very little room for a stent. Hope had just enough room for the stent to be placed,” Dr. Sinha explained, “We knew we had the technical skills and ability to do this, but this had never been done before at CHOC on a baby this size.”

A very scary moment

After the surgery, Hope got seriously ill with a viral infection. At one point, Dr. Ashrafi said, her heart stopped but the team was able to revive her.

In cases like Hope’s, where a newborn’s state of health is fragile, members of her clinical team often must pivot in an instant, making their work schedules long and unpredictable.

Hope was at CHOC for four months before she was able to go home. After that, physicians at another hospital removed the stent, closed the hole in her heart, and corrected her left superior vena cava.

Dr. Ellini, who continues to see Hope at her check-ups, is very pleased with her progress.

“She basically has a normal circulation,” he said. “She needed a pacemaker. Overall, she’s doing great. She’s only on one medication and is gaining weight.”

In fact, she’s up to 13 pounds.

Baby Hope

Dr. Ellini said he’s proud of the extensive collaboration that was involved in Hope’s care at CHOC.

“We try to really foster a collaborative team approach in our interventional lab, and this is a great example of that,” he said. “Having a dedicated neonatal cardiac intensive team of physicians and nurses who are really experts in what they do really was paramount in making sure she did well.”

Becca can’t praise Hope’s team at CHOC enough. “They’re totally lifesavers,” she said. “It was a roller coaster — heartbreaking and exiting. I was pretty much afraid all the time, but they treat you like you are family.”

CHOC team using new device to close heart defect in tiny patients

Tiny premature babies who suffer from a common but potentially fatal opening in their hearts are now being treated with a new device by physicians at CHOC Children’s.

The team successfully completed the first procedures March 20 and 21 to close a patent ductus arteriosus (PDA), an opening between two blood vessels of the heart that has failed to close on its own. CHOC became one of the first hospitals to use the Abbott Amplatzer Piccolo™ Occluder since it was approved by the FDA in January.

The Abbott Amplatzer Piccolo™ Occluder is about the size of a pea.

“We’ve never had the capability of doing this here at CHOC,” says Dr. Amir Ashrafi, director of CHOC’s neonatal-cardiac intensive care. “While closing a duct in a catheterization lab is not a new technology, closing a duct in a cath lab in very small babies is a big deal. The fact that we are now going to be one of the centers that are doing this, that is a big deal.”

PDAs are among the most common heart defects in premature babies. The opening, also called a duct or channel, is present in all fetuses and plays a vital role in allowing oxygen-rich blood from the mother to circulate through the unborn child’s body. In most cases, it closes spontaneously after birth. But out of the 60,000 infants born prematurely each year, 1 in 5 (12,000) has a PDA severe enough to require urgent medical attention.

“What happens is that blood goes in the wrong direction, so instead of blood going to the body, it goes into the lungs, so now the lungs get flooded,” Ashrafi says.

Without treatment, a PDA can cause breathing difficulty and a variety of other problems.

“It affects their feeding, because they’re having such a hard time breathing,” says Dr. Gira Morchi, an interventional pediatric cardiologist at CHOC. “It’s a cascade effect. It can affect the GI tract, kidneys, and the brain.  Taking away the extra workload on the body allows for recovery.”

Abbott had previously developed the Amplatzer™ Duct Occluder to treat the same problem in larger pediatric patients. The new, smaller, device – measuring 3 mm by 2 mm – can be used in patients as young as 3 days old and weighing as little as 1.5 pounds, or 700 grams.

The procedure is performed through cardiac catheterization via a small incision made in the baby’s leg, near the groin area, to access a vein leading to the heart. A catheter is inserted, with the device inside. It’s the size of a small pea, and made of tightly woven metal mesh. Cameras and ultrasound guide the operator – in this case, Morchi – “correctly position the device” in the heart, she says. The device is deployed and placed in the opening, where it expands on its own. The device stays in the body, with tissue healing around it.

A cardiac catheterization lab at CHOC Children’s Hospital

The first patient was a girl from Fullerton, Calif., who was born at 28 weeks and weighed 1.1 kilograms. The second patient, a girl from Huntington Beach, was born at 25 weeks and weighed 800 grams. The procedures were conducted when the babies were 2.5kg and 2.4 kg, respectively. The device is approved for much smaller infants than those, but the CHOC team is being selective about its cases.

“We’ll just slowly work our way down,” Ashrafi says. 

Morchi herself has been doing such catheterizations for a decade. “We’re very comfortable with actually doing the procedure, so the real art here is to keep the babies stable while this procedure is happening,” she says. Conditions in the cath lab should closely match those in the NICU, including keeping the temperature warm. “We crank the heat up.”

Start to finish, the baby is in the room for 90 minutes to 2 hours, but the actual procedure only takes 20-30 minutes, she said.

Besides Morchi, the interventional cardiology team also included Dr. Sanjay Sinha and Dr. Mitch Recto.

A closer look at the Abbott Amplatzer Piccolo™ Occluder

The achievement was the result of a 2-year collaboration between CHOC and UC Irvine. Credit also goes to Dr. Evan Zahn of Cedars Sinai Medical Center in Los Angeles, who was an early adopter of the procedure and was lead investigator in the FDA approval study for the Abbott device. The trial included 50 patients at eight facilities in the U.S.

Upon FDA approval of this device, Abbot Vascular recognized CHOC Children’s as one of the first hospitals in America to use this device for those smallest and most vulnerable patients in the hospital. 

“This device offers a new era in treating PDAs, and was successful at CHOC in great part due to a strong effort of collaboration between the cardiologist and the neonatologists,” says Dr. Sinha, a CHOC/UCI pediatric cardiologist.

In the Spotlight: Michael Recto, M.D.

As an internationally-recognized expert in interventional pediatric cardiology, and division chief of cardiology, CHOC Children’s Specialists, Dr. Michael Recto’s goal is to provide world-class cardiac care. He treats children with serious congenital heart defects, and performs both diagnostic and interventional cardiac catheterization procedures.

Dr. Michael Recto

Prior to coming to CHOC, Dr. Recto served as both chief of pediatric cardiology and director of cardiac catheterization at Tulane Medical Center in New Orleans. Previously, he was chief of pediatric cardiology and director of inpatient transplant services at Kosair Children’s Hospital in Louisville.

Dr. Recto takes great pride in having worked throughout his career with some of the top cardiologists in the field. When he joined the CHOC Children’s Heart Institute in 2013, he witnessed the same level of talent and knew instantly he was in the right place, he says.

“Everyone on the CHOC team is an expert in their field. We have experts in echocardiography (fetal, transthoracic and transesophageal echo), cardiac MRI, electrophysiology and cardiac intensive care. I am proud to be part of such a talented team,” he says.

Dr. Recto enjoys spending time in CHOC’s state-of-the-art cardiac catheterization laboratorities, where he is able to diagnose problems and if needed, perform an intervention and help a patient right on the spot, he explains.

Dr. Recto is board certified in pediatrics and pediatric cardiology. He attended medical school at University of the Philippines College of Medicine, followed by a pediatric internship and residency at New York University Medical Center in New York City. He completed a pediatric cardiology fellowship at Mount Sinai Medical Center in New York City, and a pediatric interventional cardiology senior fellowship at Texas Children’s Hospital, Baylor College of Medicine in Houston, under the tutelage of Dr. Charles E. Mullins, known as the Father of Modern Interventional Pediatric Cardiology.

In addition, Dr. Recto is a fellow of the American College of Cardiology (ACC) and a fellow of the Society for Cardiac Angiography and Interventions (SCAI), among other professional organizations. He has co-authored numerous articles in publications such as Pediatrics, Pediatric Cardiology, and Journal of the American College of Cardiology, to name a few.

Long before Dr. Recto was treating serious heart conditions, however, he thought of becoming an engineer or architect.  His mother asked if he had ever considered a career in medicine. Although unsure about this career path, he decided to give it a try. After his first semester as a pre-med student, Dr. Recto felt that he had never studied as much in his life, he says jokingly, and decided he better continue the hard work he had started. He was eventually accepted to the University of the Philippines College of Medicine, where only a small number of students are accepted every year.  The young doctor was first exposed to pediatric patients during his rotating internship at the Philippine General Hospital, where patients with some of the most complex clinical problems are sent for care.  That experience solidified his passion for pediatrics.

Today, Dr. Recto’s approach to delivering care is to treat his patients and their families the same way he would like his family to be treated. He has learned a lot from his patients and their families along the way, and is still surprised at the touching moments he experiences on a daily basis.

“I had a patient just the other day with an atrial septal defect and I explained to this child’s family that this particular hole between the two atria was going to be hard to close. The patient would possibly require open-heart surgery,” Dr. Recto says. “The patient’s father looked at me and said, ‘We have a lot of faith in you.’ I was indeed able to close the defect in the cath lab. When I came out of the procedure and told the entire family the good news, they stood up and applauded and the father gave me a big hug. I was not expecting that. A moment like that is one of the best things you can experience. It was truly gratifying and humbling.”

When Dr. Recto is not caring for patients at CHOC, he enjoys spending time with his wife, a pediatric emergency medicine physician at CHOC, and their three grown children. He and his wife enjoy eating out and traveling.  Dr. Recto is also an avid tennis player.

Dr. Recto is open to questions from community physicians, and encourages physicians to call him or use Pingmd. To contact him, or to refer a patient, please call 714-509-3939.