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 to host NeoHeart: Cardiovascular Management of the Neonate Conference, March 27-29

Newborns with congenital heart disease are some of the most critical and fragile patients, says Dr. Amir Ashrafi, cardiac neonatologist at CHOC Children’s. To help address the needs of this complex patient population and their families, CHOC will once again be hosting NeoHeart: Cardiovascular Management of the Neonate. The conference will be held March 27-29, 2019 at the Hyatt Regency in Huntington Beach, and anticipated to attract over 650 attendees, from both across the country and internationally.

“It is imperative that physicians, nurses, and all front-line providers work closely together to give these babies the best chance at success” Dr. Ashrafi explains.

The dynamic, TED-style talk conference will emphasize cutting-edge science, innovations in medical care, controversies in management, as well as the importance of eliminating silos and creating an all-inclusive team which includes the families of neonatal patients. Physicians, physician’s assistants, nurses, nurse practitioners, fellows, residents, and other allied health professionals who specialize in neonatology, pediatric cardiology, pediatric intensive care, and cardiothoracic surgery are invited to attend.

Given the remarkable success of NeoHeart in previous years, Dr. Ashrafi, Dr. John Cleary, neonatologist at CHOC and colleagues from around the world launched the first international Neonatal Heart Society. The group works closely with other professional organizations including the American Academy of Pediatrics (AAP), World Congress of Cardiology, and Pediatric Cardiac Intensive Care Society to advocate for newborns with congenital heart disease and hemodynamic instabilities.

To register for NeoHeart: Cardiovascular Management of the Neonate, click here: www.choc.org/neoheart2019

This activity has been approved for AMA PRA Category 1 Credit TM

 

CHOC Surgical NICU Reduces Post-Op Hypothermia in Infants

Consistent, standardized efforts across several disciplines helped CHOC Children’s reduce rates of post-operative hypothermia in neonates by nearly 88 percent, results of a quality improvement project show.

Staff decreased the number of babies who returned to the Surgical Neonatal Intensive Care Unit with body temperatures below 36 degrees Celsius from 10.7 percent to 1.3 percent following surgeries between September 2014 and August 2015.

Due to high body surface area, infants undergoing surgery are at risk for hypothermia, especially premature infants with decreased subcutaneous and brown fat. Hypothermia-induced vasoconstriction can lead to impaired wound healing, surgical site infections, impaired coagulation and decreased drug metabolisms, which can collectively increase perioperative morbidity, said Dr. Irfan Ahmad, co-director of the unit.

Though CHOC’s baseline figure was well below the national average rate of 15.6 percent, reducing post-operative hypothermia rates wasmock-surgery-1 identified as an area for quality improvement for the Surgical NICU and staff set out to reduce rates by half, Dr. Ahmad said.

Involving a cross-disciplinary team including nurses, neonatologists, surgeons and anesthesiologists, the project tracked 76 patients. Because infants can be at risk for hypothermia before surgery, intra-operatively and post-operatively, their temperatures were tracked during each operative stage. Staff were then able to identify problem areas and make improvements over each quarter.

Dr. Ahmad attributed the success to consistently implementing measures such as ensuring patients wore hats and blankets while headed to the operating room; pre-warming transport isolettes before placing babies inside; and using intra-operative heating devices during procedures.

Dr. Ahmad presented this data earlier this month to a quality congress held by the Vermont Oxford Network, a nonprofit, voluntary collaboration of health care professionals dedicated to the quality and safety of medical care for newborns and their families.

CHOC established its Surgical NICU in October 2013, and remains one of a handful of hospitals nationwide to cohort infants needing and recovering from surgery in a dedicated space.

Surgical NICU patients receive care from a multidisciplimock-surgery-4nary team that includes neonatologists, surgeons and many other clinicians. The surgical NICU team cares for patients jointly, discussing the cases as a group and forming a treatment plan that often calls for the expertise of other specialties.

Patients and families are a key component of the surgical NICU care team, collaborating and partnering with clinicians on every stage of the patient’s care.

The Surgical NICU rounds out CHOC’s expansive suite of services for neonates, including a main NICU; the Small Baby Unit, where infants with extremely low birth weights receive coordinated care; the Neurocritical NICU, where babies with neurological problems are cohorted; and the Cardiac NICU, which provides comprehensive care for neonates with congenital heart defects.

Learn more about CHOC’s neonatal services.

CHOC Small Baby Unit Serves as Model at Conference

VON 1Dozens of representatives from neonatal intensive care units nationwide recently toured CHOC Children’s Small Baby Unit (SBU) and learned how to replicate the facility in their own hospitals as part of a conference held by the Vermont Oxford Network (VON).

About 50 attendees spent two days this month at CHOC, touring and attending workshops and roundtable discussions. Among the sessions was “Creating a Small Baby Program: The CHOC SBU Experience,” presented by Dr. Antoine Soliman, SBU director, and Mindy Morris, DNP, SBU program coordinator and nurse practitioner.

In that session, the pair defined key components and approaches of the program that help develop a team dedicated to the care of micro-preemies; identified strategies for staff engagement in developing tools and processes to standardize the care of babies with extremely low birth weights (ELBW); examined potential challenges and barriers to the development of an ELBW team, and devised possible solutions.

Morris also shared data accumulated by the unit since it opened in 2010, as well as outcome improvements for conditions that are common for this delicate patient population.

“Families as Team Members,” covered patient andSBU_tour_VON family-centered care, including how to enhance the family experience and further staff knowledge. In this session, former SBU parents shared their experience of being a part of the patient care team.

As part of the conference, SBU staff also offered insight into their roles and responsibilities within the unit, as well as the essential tools used by the team in standardizing care for the micro-premature infant.

Conference attendees also had time to devise ways that they could apply information gained from touring the SBU into their own NICU. They also had opportunities to ask questions and seek advice from SBU staff.

The visitors came from nine hospitals – adult and children’s – throughout the country, including Children’s Hospital at Providence (Alaska); Children’s Hospitals and Clinics of Minnesota; Helen DeVos Children’s Hospital (Grand Rapids, Mich.);  Stanford Children’s Health; and C.S. Mott Children’s Hospital (University of Michigan).

Founded in 1989, VON is a nonprofit, voluntary collaboration of health care professionals dedicated to the quality and safety of medical care for newborns and their families. VON comprises more than 900 NICUs worldwide.