Catching a heart defect in utero: Marco’s story

Meagan and Dante Cipulli quickly settled on a name when they discovered their third baby would be a boy: Marco, which meant God of War.

And that name would become especially apt a few weeks later. When Meagan was about six months pregnant, the couple learned their baby had a congenital heart defect called tetralogy of Fallot and would need open-heart surgery soon after birth.

“Knowing my unborn baby would need open-heart surgery after birth was the scariest experience of my life,” Meagan says. “After receiving his diagnosis, we realized we couldn’t have picked a better name for our little heart warrior.”

Finding heart defects before birth

When a second ultrasound by a perinatologist confirmed that baby Marco’s aorta was enlarged, Meagan was referred to CHOC pediatric cardiologist Dr. Nita Doshi.

Dr. Doshi performed a fetal echocardiogram, which uses sound waves to create a picture of an unborn baby’s heart.

The evaluation confirmed that Marco had tetralogy of Fallot, a heart condition comprised of four related defects that cause inadequate amounts of blood to reach the lungs for oxygen, thus sending oxygen-deficient blood throughout the body.

“I was in complete shock,” Meagan says. “As a nurse, I knew exactly what tetralogy of Fallot was and that he would need open-heart surgery.”

Planning began immediately. With the help of Dr. Doshi, the Cipullis began researching hospitals, cardiologists and surgeons who could care for Marco when the time came.

CHOC emerged as the clear choice, and the Cipullis opted for Dr. Doshi to continue as Marco’s cardiologist and Dr. Richard Gates to perform the corrective surgery.

Organizing pre- post-birth care

Meagan moved her obstetric care to a physician aligned with St. Joseph Hospital so Marco could be transferred next door to CHOC’s neonatal intensive care unit (NICU) immediately upon birth.

During a perinatal conference, the Cipullis met with the obstetrical team at St. Joseph and CHOC’s neonatal team to discuss the baby’s birth and care.

“That allowed me to have all my questions answered and gave me peace of mind that all those related to our care were on the same page,” Meagan said. “I knew that I had made the right choice after meeting with the care team.”

The remaining weeks of Meagan’s pregnancy were an emotional roller coaster. They couple prepared their older sons as best they could for what was to come with their younger brother.

And while the family was scared to not understand the full extent of their baby’s medical needs, they felt assured knowing a plan was in place.

“Each day of my pregnancy after diagnosis was filled with worry and fear, but also gratitude and hope knowing we were fortunate enough to have Marco’s diagnosis in utero and we were able to plan for his care after birth,” Meagan says.

The Cipullis didn’t have to wait long for Marco. On May 16, 2017, Marco was born five weeks ahead of schedule. After a brief rest on his mother’s chest, Marco was moved to CHOC’s NICU, where he stayed for five days.

Surgery day

Marco was back at CHOC about three months later for surgery with Dr. Gates to repair his heart defects.

 

“At first it all seemed so surreal and somehow I was able to keep it all together until the moment they wheeled Marco into the operating room,” Meagan says. “While he was lying in the crib, he looked over his shoulder and gave me and his dad this smile and look like, ‘I got this, guys, don’t worry.’ I don’t think I have ever cried harder in my life.”

The surgery went well, and Marco spent five days recovering in CHOC’s cardiovascular intensive care unit.

Today, Marco is happy and healthy 9-month-old who loves to smile and laugh. He sees Dr. Doshi every four months for follow-up appointments, but otherwise requires no additional medication or therapy.

Many babies with tetralogy of Fallot will require additional surgeries as they age, but the Cipullis are hopeful that Marco’s early interventional measures will last for many years.

Feeling positive

Meantime, the Cipullis are enjoying every minute with their three boys, and are grateful for the care they received at CHOC after catching Marco’s condition early.

Meagan recommends that other families who find themselves in similar situations be vocal about their fears, but also stay positive about their baby’s future.

“My husband and I each night would talk about what we were feeling that day,” she recalls. “At first, it was more about our fears and worries, but eventually each day we would talk more about our excitement and joy to meet our little warrior.”

Learn more about CHOC’s fetal cardiology services.

Meet Dr. Anjalee Warrier Galion

CHOC Children’s wants its referring physicians to get to know its specialists. Today, meet Dr. Anjalee Galion, a pediatric neurologist and sleep specialist.

CHOC Children's

Q: What is your education and training?
A:  I attended the University of Medicine and Dentistry of New Jersey- New Jersey Medical School, and completed my residency in pediatrics at University of San Francisco, Fresno.  During my academic year, I worked for Walter Reed Army Institute of Research to help identify a vaccine for malaria. My second day was September 11, 2011, and it was an amazing, humbling, and scary experience to be a part of the military for this day. My first fellowship was in pediatric neurology at the University of California, Irvine (UC Irvine), and my second fellowship was in sleep medicine at the University of California Los Angeles- Cedars Sinai Sleep Medicine Fellowship.

Q: What are your administrative appointments?
A: Assistant clinical professor at UC Irvine, assistant program director for the UC Irvine child neurology residency program, chair of the junior faculty leadership council, and co-chair of the sleep workgroup for the National Autism Treatment Network.

Q: What are your special clinical interests?
A: Sleep disorders in children with neurologic diseases such as epilepsy and autism, as well as sleep and cognition.

Q: Are you involved in any current research?

A: Evaluation of efficacy of specific sedative hypnotics in children with Autism spectrum disorder, and Identification of sleep architecture and pathology in children with epilepsy.

Q: How long have you been on staff at CHOC?
A: Four years.

Q: What are some new programs or developments within your specialty?
A: CHOC is one of the few, if only, hospitals in the country doing combined long-term video EEG as well as polysomnography (sleep study).  This allows us a very unique opportunity to look at the brain activity and pathology in sleep. Multidisciplinary sleep clinics involving psychology and pulmonology are also unique. Also, identification and treatment of a variety of pediatric sleep disorders including all types of insomnia, narcolepsy and parasomnias, such as sleepwalking/sleep talking/night terrors.

Q: What are your most common diagnoses?
A: Insomnia, narcolepsy, sleepwalking (or somnambulism).

Q: What would you most like community/referring providers to know about you or your division at CHOC?
A: We treat all types of sleep-related disorders and are providing state-of-the-art care for children with sleep disorders. It is estimated that more than 30 percent of children have sleep-related disorders, and improvement in sleep is essential for learning and cognition. Research suggests improved sleep supports optimal athletic performance as well. If there is any concern for a sleep-related disorder we are happy to help evaluate these children.

Q:  What inspires you most about the care being delivered here at CHOC?
A: We treat every child with the highest level of care and the physicians genuinely care for the patients and our community.

Q: Why did you decide to become a doctor?
A: I had been interested in neuroscience since I was a Howard Hughes fellow at the University of Maryland, having done work in spinal cord regeneration. I heard a talk in my first year of medical school about pediatric neurology and haven’t looked back since.

Q: If you weren’t a physician, what would you be and why?
A: If I was not a physician I would mostly likely be a PhD working in the field of neurobiology and sleep medicine. Both my parents were PhDs and I grew up hearing about fascinating advances in the world of science, so I have been drawn to science and research from a young age. For quite a few years I was strongly considering becoming a professional flute player. I was fortunate enough to travel through Italy with my youth symphony and performed around the country in orchestras, but science drew me in by the time I was in college.

Q: What are your hobbies/interests outside of work?
A: I enjoy hosting parties and events for family and friends. Our family enjoys traveling and spending time together.

Q: What have you learned from your patients?
A: The best part about working with pediatric patients is that you are constantly reminded to enjoy life and be grateful for every day. The smallest things can make a child happy, like playing with a light-up toy or seeing bubbles, and it is a great reminder to take pleasure in all the simple things around us every day.

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.

Know the Risks of Sunken Chest

Pectus excavatum, or sunken chest, is the most common congenital chest wall abnormality in children. Although some medical providers may think that the abnormality is purely a cosmetic problem, the limited chest cavity space can displace the heart as well as limit lung capacity, says Dr. Mustafa Kabeer, a pediatric surgeon at CHOC Children’s. Fifteen percent of patients can experience arrhythmia or mitral valve regurgitation, wherein the heart valves allow blood to leak back into the heart, as a result of the inward compression from the sternum.

Dr. Mustafa Kabeer, pediatric surgeon at CHOC Children’s
Dr. Mustafa Kabeer, pediatric surgeon at CHOC Children’s

Although the cause is unknown, 40 percent of patients report a family history of pectus excavatum, and 40 percent of cases occur in tandem with scoliosis, says Dr. Kabeer.

Parents may notice an indentation in their child’s chest wall either when they are first born, or closer to puberty, when changes in the chest wall can become more pronounced.

Dr. Kabeer urges medical providers to carefully evaluate symptoms, as they can be subtle and often go unnoticed. If a patient is older than 10 years with pectus deformities, he asks medical providers to look for signs of shortness of breath, difficulty breathing during exercise, unexplained dizziness, occasional chest pain or progressing changes in chest wall appearance.  Some patients notice they are not able to keep up with their peers. If they show even mild symptoms that were not previously recognized, they should be referred to the pectus excavatum team at CHOC, he explains.

The multidisciplinary team is comprised of experts in pediatric pulmonology, cardiology and surgery. The team performs various testing to examine heart and lung function, before and after surgery that expands the chest wall. By using the latest techniques in minimally invasive surgery that dramatically reduces the appearance of incisions, along with recent improvements in pain management, patients are able to return home and get back to their daily activities sooner than ever before.

“We have a comprehensive team ready to evaluate these patients because chances are high that they have some compromise on the cardiopulmonary system given the compression of the sternum. This is not generally noticeable until they are in some kind of increased activity at which point the increased demand from the body has difficulty being met by the heart and lungs,” Dr. Kabeer says.

“These patients should be referred to pediatric surgeons because we are trained in the minimally invasive procedure and should be the first line approach to this problem; there is no need to put a child through a more invasive operation that is not needed,” he adds.

Dr. Kabeer recommends the following referral guidelines:

  • Age < 10 years can be monitored and symptoms and severity of deformity based only on exam should be logged. No studies are necessary at this time unless symptoms are severe at which point, they should be referred to the pectus excavatum team at CHOC, but workup is not necessary prior to referral.
  • Age >10 years with very mild pectus deformities and with no symptoms can be referred or observed.
  • Age >10 years with pectus deformities that are moderate or severe with or without symptoms and mild deformity with symptoms should be referred to the pectus excavatum team at CHOC. No imaging or workup is necessary until seen by the pediatric surgeon.

To contact Dr. Kabeer, or to refer a patient, please call 714-364-4050.

Learn more about CHOC’s surgical services.

CHOC Specialist Appointed to CalOptima Board

A CHOC Children’s specialist has been appointed to CalOptima’s board of directors.

Dr. Paul Yost, chair of CHOC’s anesthesiology division, was sworn in Aug. 4.

Paul Yost, M.D.

He was appointed by the Orange County Board of Supervisors as one of six new members to guide the agency that administers health insurance programs for low-income children, adults, seniors and people with disabilities in Orange County.

“I am honored to join the CalOptima Board of Directors,” Dr. Yost said. “This role presents an exciting opportunity to serve our community’s children and families in a new way.”

He attended medical school at Baylor College of Medicine in Houston. Dr. Yost performed his anesthesiology residency at UCLA and his pediatrics residency at the University of Miami.

Dr. Yost’s CalOptima seat is reserved for a physician who is affiliated with a contracted health network. His term ends in August 2020.