A surprise heart defect diagnosis in utero: Teddy’s story

By Elle Kennedy, mom of CHOC Children’s patient Teddy

When I got pregnant with my son Teddy, my husband and I planned to follow the route we took during my pregnancy with our daughter Charlotte—a midwife and practice we were already familiar with. In the years since our daughter had been born, that office had implemented a new rule—all babies must undergo a fetal echocardiogram, an ultrasound test during pregnancy to evaluate the heart of an unborn baby.

My pregnancy had been normal, so when I went in for this fetal echo around 23 weeks, I didn’t even have my husband Jeremy come with me. We were grateful for the precautionary test, but we didn’t expect for them to find anything abnormal.

What they found was anything but normal.

Teddy was diagnosed with anomalous pulmonary artery off the aorta. Typically, the blood is supposed to go through the heart and then into the lungs and then back through the other chamber of the heart. Teddy’s diagnosis meant that one of his lungs was attached in the wrong order. This lung was getting blood that was already oxygenated by the other lung, which can make the misaligned lung really sick. The lungs are not supposed to receive oxygenated blood; they’re supposed to oxygenate the blood.

Sometimes this condition isn’t caught in utero, and the baby seems healthy at birth, but a few months down the road they have breathing issues and the detective work to find a diagnosis begins. We were fortunate that this was diagnosed before Teddy was born, so we could be prepared.

Making a new plan

I quickly understood that the rest of my pregnancy would look different than my first, and that my birth plans would change. One of my first thoughts was, “I need to find a doctor who can deliver at St. Joseph Hospital in Orange, so that we can be right across the street from CHOC.”

We found an OB/GYN and were also referred to Dr. Nita Doshi, a pediatric cardiologist at CHOC who specializes in fetal cardiology.

In the weeks leading up to Teddy’s birth, we had a big meeting with Dr. Doshi; Dr. Richard Gates, Teddy’s cardiothoracic surgeon; many other CHOC doctors; my OB-GYN and the St. Joseph labor and delivery team. There were 15 people in the room with us, ready to share the game plan for Teddy’s birth and immediate care after he was born. Everybody went around the room, in order of what would happen to me and Teddy. It was like “OK, when you come in, here is going to be the birth team. Once he’s born, here is the team taking over. When he goes into surgery, here’s that team.” Everybody got to walk us through what they were going to do and what the stages of care would look like. That experience was amazing for us. It was really nice to be able to meet everyone ahead of time.

This was an entirely new experience for us. We had a lot of fear going into it. As a parent, you hear open heart surgery and it sounds so scary. Getting to talk to people on the team and learn about their area of expertise, and have them tell you what to expect, was a very relieving experience for us. They thought through every step of the journey.

After a lengthy but healthy delivery, Teddy was born weighing seven pounds, 14 ounces. After a quick check-up to ensure his heart was doing OK and he was breathing properly, we were able to spend 30 minutes with him before he needed to be taken to CHOC.

Teddy spent four days in the cardiac neonatal intensive care unit (NICU) at CHOC before undergoing open heart surgery.

During this two-and-a-half-hour surgery, Dr. Gates removed the right pulmonary artery from the aorta and transferred it to its proper position on the main pulmonary artery.

Caring for the whole family

After surgery, Teddy spent two weeks in the cardiovascular intensive care unit (CVICU). We would visit him every day, and then go home in the evenings to put our daughter to bed. We knew our son was in good hands, and we wanted to make this time as normal as possible for his big sister.

Although the hospital staff was there to care for Teddy, they helped us prepare our daughter for her first hospital visit, as well. When Teddy was first brought to the CVICU, he was hooked up to all kinds of machines and wires. That can be scary for siblings to see. They encouraged us to wait until he was closer to going home, so that it would be a more positive experience for Charlotte. We also knew that Teddy would return to this hospital many times for other procedures and check-ups, and we didn’t want Charlotte to think of a hospital as a scary place. When she did come in, it was amazing. She loved getting to see her baby brother, and all she wanted to do was hold him. The staff was so attentive to her. Even though this wasn’t the way we intended to welcome our son into the world, it turned out to be an amazing experience.

To thank the hospital staff, and to help Charlotte feel more involved in her brother’s care, we brought small felt hearts on safety pins for her to give out to his nurses and doctors. She walked around the unit handing them out and said, “Thank you for taking care of Teddy.” It’s a tradition we’ve continued at appointments to this day.

When Teddy was around 3 weeks old, we finally got to bring him home. His sister was so happy! He had a bandage on his chest so he couldn’t do tummy time right away, but other than that it was a normal routine.

Teddy’s first angioplasty

Three months later, we came back to CHOC for an angioplasty. Scar tissue was building where they had stitched the artery in place, so the artery wasn’t growing properly. During this procedure, they made a small incision in an artery in Teddy’s thigh, inserted a small balloon and snaked it up to his heart, and slowly expanded the balloon in order to safely break up the scar tissue and gently stretch the artery. This time, we only had to spend one night in the hospital for observation.

Teddy had a follow-up angioplasty when he was three years old. He will need another angioplasty in another three to five years, depending on how his artery grows in relation to the rest of his body. Someday, he will have a stent (a tiny mesh tube) put in to permanently keep his artery open.

pre-op room with toys
Teddy playing with toys before an angioplasty,

Throughout this process, CHOC has been amazing for us because they’re willing to take our say into consideration. Our team always asks us, “Are you comfortable with this plan?” We have been involved in the decision-making progress every step of the way.

Teddy today

Teddy is smaller than the average 3-year-old boy. Personally, I think it’s a blessing that he’s growing a little slower than other kids his age. It’s giving his heart time to grow at a slower pace because the rest of his body isn’t growing as fast. Other than his smaller than average stature, he’s a healthy kid. He underwent occupational and physical therapy for a while to help him meet milestones, but he graduated from those therapies at 18 months and now he is hitting or exceeding all of his milestones.

These days, we see Dr. Doshi every six months for a check-up. Despite many appointments and procedures over the last few years, Teddy has no fear of the doctor. When he sees a Choco Bear logo on a billboard, he points and says, “Mom that’s where I went and had my operation. They took care of me there.”

If you look at him, you wouldn’t know he had a heart condition. Unless you see him shirtless and notice his scar, you would think he was just a typical kid. He talks well for his age, and he loves anything with wheels. He’s a very brave young boy, which we knew from day one.

bumblebee boy costume
After his last angioplasty, Teddy requested a photo shoot dressed as his favorite superhero. Bumblebee Boy.

A message to other moms

To any other moms with hospitalized babies, know that you are not alone. For me, the hardest part of this journey was not knowing anyone else whose baby was hospitalized after birth. Know that there are other parents out there who understand what you’re going through.

This journey could have been a scary experience for our family, but I never imagined it could actually be a positive experience. I thought we would just have to get through this, but Teddy has so much fun laughing and playing with the nurses who take care of him before and after procedures.

CHOC has done a really great job of thinking through everything from a patient perspective and a parent perspective. They try to create the best possible experience from start to finish for everyone involved. They make it a reassuring process for parents, a happy experience for siblings, and a safe experience for patients.

At CHOC, everyone’s goal is to make a child’s experience a positive one.

Learn more about the Heart Institute at CHOC Children's

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Meet Dr. Nita Doshi

CHOC Children’s wants its patients and families to get to know its specialists— today, meet Dr. Nita Doshi, a pediatric cardiologist with expertise in fetal cardiology.

nita-doshi-fetal-cardiology
Dr. Nita Doshi, a pediatric cardiologist at CHOC Children’s with expertise in fetal cardiology.

Q: What is your education and training?

A: I attended medical school at University of California, Irvine. I completed my pediatric residency internship, categorical pediatric residency, and pediatric cardiology fellowship at University of California at Los Angeles Mattel Children’s Hospital School of Medicine.

Q: What are your special clinical interests?

A: Fetal cardiology, non-invasive cardiology including infant and pediatric transthoracic echocardiography, transesophageal echocardiography, pediatric pulmonary hypertension, and pediatric heart failure.

Q: How long have you been on staff at CHOC?

A: Eight years.

Q: What are some new programs or developments within your specialty?

A: My team provides state-of-the-art ultrasound technology and comprehensive fetal cardiac imaging protocol for evaluation and diagnosis of fetal cardiac disease. We offer the only comprehensive fetal cardiology services in Orange County, including performing fetal echocardiograms on pregnant women. When necessary, we partner with families on prenatal palliative care planning for specialized cases. We also support continuity of care of prenatal patients with their primary obstetrical and perinatology teams when appropriate.

Q: What are your most common diagnoses?

A: Atrial septal defect, ventricular septal defect, atrioventricular canal defect, bicuspid aortic valve and aortic valve stenosis, coarctation of the aorta, pulmonary valve stenosis, tetralogy of Fallot, transposition of the great arteries, double outlet right ventricle, truncus arteriosus, pulmonary hypertension, and cardiomyopathy.

Q: What would you most like community/referring providers to know about you or your division at CHOC?

A:  I treat every patient and family as though they are my very own.  This connection is what has allowed to us create such an amazing network of heart families.  I am proud to serve as a member of a supremely talented and highly subspecialized team of cardiologists.

Q:  What inspires you most about the care being delivered here at CHOC?

A:  CHOC offers the ability for physicians such as me to serve a widely diverse patient population inside and outside of the county.  There is a unique and exceptional opportunity for one-on-one personalized care here at CHOC that doesn’t exist in other centers.  In this role, I am honored to learn from my patients and families every day, including aspects of their backgrounds, cultures, spectrum of disease, and their perspectives.  These are lessons that make me a stronger person and professional to support families in the future.

Q: Why did you decide to become a doctor?

A:  My parents are exceptional role models.  They remind me to this day that when I was eight years old, I informed them I wanted to take care of babies and their hearts.  They are still not quite sure from where the specific inspiration stemmed (perhaps because they are also in the health profession), but they said that I was determined to follow through on this goal, and I did.

Q: What are your hobbies/interests outside of work?

A:  My two year old daughter and my husband are my life.  We play, cook, travel, and explore every aspect of the outside world together.  We pride ourselves on learning every trade possible that enhances our lives.  We have remodeled our entire home with our own bare hands, cook our meals from scratch, and thoroughly enjoy every aspect of creative design (event planning and decorating and all things arts and crafts).

Q: What have you learned from your patients, or what is the funniest thing a patient has ever told you?

A:  One of the funniest moments was when my 4 year old patient being wheeled into the operating room to undergo a heart transplant asked me if they could put “mac n’ cheese” in her IV.

Learn more about fetal cardiology services at CHOC Children's

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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 means God of War.

That name would become especially fitting 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 Children’s pediatric cardiologist Dr. Nita Doshi.

choc-childrens-patient-heart-defect

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, then 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, director of cardiothoracic surgery as well as CHOC’s surgeon-in-chief, to perform the corrective surgery.

Organizing pre-, post-birth care

Meagan moved her obstetric care to a physician aligned with St. Joseph Hospital so that when Marco was born, he could be immediately transferred next door to CHOC’s neonatal intensive care unit (NICU).

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

“That conference allowed me to have all my questions answered. It 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. The couple prepared their older sons as best they could for what was to come with their younger brother.

Not understanding the full extent of their baby’s medical needs, the Cipullis’ were terrified. However, they felt at ease knowing a plan was in place.

“Each day of my pregnancy after diagnosis was filled with worry and fear. But they were also filled with gratitude and hope knowing we were fortunate enough to have Marco’s diagnosis in utero and 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 the next five days before going home.

Surgery day

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

marco-at-choc-after-surgery-for-heart-defect

“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 a 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.

choc-childrens-patient-with-heart-defect-and-family

Many babies with Tetralogy of Fallot will require additional surgeries as they age. The Cipullis however 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. They are grateful for the care they received at CHOC after catching Marco’s condition early.

choc-childrens=patient-with-heart-defect-and-brothers

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 the Heart Institute at CHOC Children's

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Heart Disease Medications for Children

 By Melody Sun, clinical pharmacist, & Dr. Nita Doshi, fetal cardiologist at CHOC Children’s

About eight in every 1,000 babies in the US are born with a heart defect. In some cases there are known risks such as pregestational or first trimester maternal diabetes, a previous child with congenital heart disease, mother or father with history of congenital heart disease, certain genetic syndromes, or certain prenatal medication exposures. Many cases, however, do not have a known reason. The heart is formed by week 12 of pregnancy, but sometimes, the usual developmental stages of the heart do not occur. Your prenatal care team may refer you to a fetal cardiologist for additional testing and monitoring. Sometimes, the defect does not significantly impact the quality of life, and the child can grow into adulthood without restrictions. It is when the defect causes severe consequences that the child may require surgery and/or long-term medications.

With advancing imaging technology, the majority of significant congenital heart defects may be detected during pregnancy. The state of California’s newborn screening program mandates newborn pulse oximetry screening for detection of congenital heart disease. If diagnosed with significant congenital heart disease, young patients may exhibit the following warning signs and symptoms:

  • Difficulty with feeding and/or poor weight gain
  • Difficulty with breathing
  • Turning blue inside or around the mouth, or in certain areas of the face
  • Cool arms and legs
  • Tiring quickly with exertion or activity

In severe cases of significant congenital heart disease, medical, minimally-invasive (catheterization-based), or surgical therapy may be considered by your child’s cardiology team. Medications may be started before and after the surgery to improve heart and lung function.

Examples of medications that may be recommended to treat a patient with heart disease include:

Medication Intended Use
Aspirin Decreases the risk of clot formation
Furosemide (Lasix), chlorothiazide (Diuril) Reduces the heart’s workload by decreasing the amount of blood volume the heart has to handle
Spironolactone (Aldactone) Helps counteract the loss of potassium from diuretics and may help support heart function
Sildenafil Reduces elevated pressure in the lung arteries
Captopril/enalapril/lisinopril Reduces the resistance and heart’s afterload to improve the heart’s function or to help reduce certain types of valve leakage
Propranolol, Digoxin, Sotatol, Amiodarone Treats rhythm disturbances due to abnormal electrical signals through the heart cells

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