CHOC’s virtual pediatric lecture series continues with a lesson on cardiology.
This online discussion will be held Wednesday, July 28 from 12:30 to 1:30 p.m. and is designed for general practitioners, family practitioners and other healthcare providers.
Dr. Sanjay Sinha, pediatric cardiologist at CHOC, will discuss several topics, including:
Accurately diagnosing and refering patients with cardiac symptoms related to the current coronavirus pandemic.
Identifying new therapy and guidelines as they pertain to your patient populations.
This virtual lecture is part of a series provided by CHOC that aims to bring the latest, most relevant news to community providers. You can register here.
CHOC is accredited by the California Medical Association (CMA) to provide continuing medical education for physicians and has designated this live activity for a maximum of one AMA PRA Category 1 Credit™. Continuing Medical Education is also acceptable for meeting RN continuing education requirements, as long as the course is Category 1, and has been taken within the appropriate time frames.
Please contact CHOC Business Development at 714-509-4291 or BDINFO@choc.org with any questions.
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.
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.
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.
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.”
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.
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.
“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
“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.
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
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
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 as one of the first hospitals in America to use this device for those smallest and most vulnerable patients in the hospital.
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.