A Family's Journey With Congenital Heart Disease

Kiley Beam and Isaiah Covert of Shirleysburg, Pennsylvania, knew what it was like to have a child with a congenital heart disease. Aiden, their son, was born with tetralogy of Fallot, a serious condition that involves several heart defects. However, when Kiley became pregnant again, the couple was told their second child should be healthy.

Instead, when Beam was still 24 weeks pregnant with their daughter, they received the stunning news. “When I was checked, she had the same thing — but it was worse,” she says. Genetic testing of each parent did not reveal any abnormalities to explain why both children developed this condition.

[Read: Best Children’s Hospitals: Honor Roll and Overview.]

Heart-Lung Connection

Heart and lung function are closely intertwined. The upper chambers of the heart, the right and left atria, receive blood from the body and lungs. The lower chambers of the heart, the right and left ventricles, pump blood to the lungs and body.

A system of veins and arteries connect the heart to the lungs and body. The pulmonary arteries connect the right ventricle to the lungs. The aorta connects the left ventricle to the body.

Four heart valves — the mitral, tricuspid, aortic and pulmonary valves — allow blood to flow in the proper direction. The atrial septum and ventricular septum act as walls, separating the right and left sides of the heart.

In a normally functioning heart, blue (oxygen-depleted) blood passes into the right ventricle, which pumps it out to the lungs. Blood collects oxygen in the lungs and turns red. It then travels into the left side of the heart and down into the left ventricle, which pumps the oxygen-rich blood out though the aorta and into the body. Once used by your organs and tissues, your veins return the deoxygenated blood to your heart.

Multiple Heart Defects

In tetralogy of Fallot, babies are born with multiple heart defects that disrupt normal blood flow and oxygen delivery:

— Ventricular septal defect, or VSD, is an abnormal hole in the wall separating the lower heart chambers.

— A defective pulmonary valve allows blood to leak backwards, allowing oxgenated and deoxygenated blood to mix and impeding blood from moving forward.

— Right ventricle thickening results from the heart having to pump against resistance.

— Overriding aorta means the aorta — the major artery that carries blood away from the heart — is malpositioned, directly over the VSD.

As a result of these defects, pressure is too high in the right side of the heart. Oxygen-poor blood is pumped out into the body through the VSD. The child’s skin is blue-tinged, instead of the typical pink tone that comes from healthy circulation.

Gracie Covert had tetralogy of Fallot with pulmonary atresia and a ventricular septal defect — the most severe form. “The pulmonary valve doesn’t develop at all, so there’s no flow going into the pulmonary arteries,” explains Dr. John Cheatham, professor emeritus at the Ohio State University College of Medicine and an interventional cardiologist in The Heart Center at Nationwide Children’s Hospital in Columbus, Ohio.

Pulmonary arteries are typically very underdeveloped in this condition, Cheatham says. They’re extremely small with many areas of narrowing, or stenosis. To compensate, the baby’s body develops extra little blood vessels, called collaterals, to supply blood flow to the lungs. Oftentimes, these babies need a surgical shunt to improve pulmonary blood flow.

[See: 11 Questions You Should Ask Your Cardiologist During Your First Visit.]

Early Days

On Nov. 2, 2010, Gracie was born at Penn State Children’s Hospital in Hershey, Pennsylvania. Beam had an emergency cesarean section in a packed operating room, with neonatal and cardiology specialists at the ready. Her newborn daughter was immediately whisked away to intensive care.

“She actually did very well,” Beam recalls. “She needed some oxygen, but she was able to come home with me.” There, the family had a brief respite before the first of a number of surgeries to repair Gracie’s heart was performed.

At 2 months old, Gracie had her first open-heart surgery. It was scary, Beam says. The procedure lasted for hours and Gracie was struggling in the intensive care unit afterward. “They came out and got us in the waiting room, and told us they felt we needed to come back and be there,” says Beam, who describes standing helplessly watching while the medical team performed a whirlwind of emergency measures to save their daughter. Thankfully, their efforts worked and she recovered.

Following her first surgery, Gracie underwent multiple heart catheterizations to help open the tiny arteries leading from her heart to her lungs and allow them to grow.

Keeping Tiny Blood Vessels Open

During Gracie’s first open-heart procedure in Pennsylvania, the surgeon sewed one end of the aorta directly into her diminutive pulmonary arteries to provide a more reliable way for blood to enter the pulmonary arteries, Cheatham explains. The hope was that the tiny arteries would grow with time. The problem was that Gracie’s arteries had narrowed in many areas.

In a follow-up procedure, the surgeon placed a tiny tube, or conduit, that led directly from the right ventricle to the pulmonary artery. With this conduit in place, cardiologists would ideally be able to perform more multiple catheterization procedures to improve Gracie’s circulation.

The second heart surgery, when Gracie was 10 months old, went well. However, another hurdle arose at her next catheterization procedure. Heart block — a rhythm problem in which the heart beats too slowly — almost led to Gracie having a tiny pacemaker put in. Fortunately, doctors were able to treat the problem and avoid the pacemaker implant.

Following her third heart surgery at 14 months old, Gracie had a massive seizure. “It caused (temporary) brain damage,” Beam says. “When she woke up, she was like a newborn baby. She had to learn everything over again,” including how to walk.

The situation began to seem hopeless. Catheterization procedures to keep Gracie’s tiny blood vessels open were no longer effective, Beam says. “Her oxygen levels were dropping,” she says. “They couldn’t help her anymore.”

Gracie was getting weaker and weaker. “She couldn’t walk 20 feet from the couch to the bathroom,” Beam says. “She would be so exhausted she would nap for an hour.”

Double-Transplant Option

Before she reached the age of 2, Gracie’s worsening heart condition was posing multiple treatment challenges. The possibility of another episode of heart block during catheterization procedures made pulmonary arterial treatment too challenging. The blood pressure in the right side of her heart was abnormally high, so that her heart muscle had to work much harder. The hole between her ventricles could not be repaired under these circumstances.

At this point, the discussion turned to the possibility of an infant heart-lung transplant. “It’s a big deal,” Cheatham says. “Centers that can perform this complex procedure are few and donors are scarce. Also, it’s a long process with immunosuppression. So centers are very picky with who they accept.”

With immunosuppression, transplant recipients must take multiple drugs to prevent their immune system from rejecting donor organs. As a result, the body’s ability to fight infection is weakened, posing another health threat.

Finding a Hospital

The family began seeking evaluations for a heart and lung transplant, starting in-state with two Pennsylvania children’s hospitals and then branching out to one in Massachusetts. Cardiologists and surgeons all agreed that Gracie wasn’t a good candidate for this major, high-risk surgery in her current state of health.

“We were seeing her get sicker and we couldn’t just sit there and watch it,” Beam says. Eventually, she says, their insurer refused to pay for referrals. “We had exhausted everything,” she says.

That’s when the local community rallied around the family. A big benefit ride with motorcycle bikers raised $10,000 so the family could afford an attorney. He helped them with Medicaid coverage issues and their search for the care Gracie needed.

The family and lawyer made a massive push to reach out to any hospital that might be able to help. They pored over the Best Children’s Hospitals for Cardiology & Heart Surgery rankings to identify pediatric medical centers across the country that perform the double heart-lung transplant.

With the lawyer’s help, they created comprehensive packets. These included Gracie’s medical records, 8-by-10 pictures of her, family pictures, letters from her doctors and letters from the family.

“If we could hit a doctor in the heart and make him realize, ‘Oh, this is a kid, not a medical record,’ that was our goal,” Beam says. “Somebody out there could help her. Somebody will.”

They sent Gracie’s packets to 25 major transplant facilities in the United States, as well to facilities in South Africa. “Hospitals in South Africa were willing to take her if we could get her there,” Beam says. A single U.S. hospital — Nationwide Children’s in Columbus, Ohio — agreed to take Gracie’s high-risk case.

[See: 10 Heart Health Breakthroughs.]

Intricate Procedures

At Nationwide Children’s, specialists — cardiologists, surgeons, anesthesiologists and others — considered Gracie’s case with all its challenges in 2013.

They agreed that her circulation needed to improve before she could tolerate a heart-lung transplant. Cheatham evaluated her condition in the hospital’s hybrid catheterization lab — a combination of a cath lab and operating room. The goal was to gradually enlarge and repair her pulmonary arteries in a series of delicate procedures.

First, however, the team had to find the reason for her heart block and dangerously low heart rhythm during her most recent catheterization procedure. They determined that a large catheter wire had put pressure on the area of the heart that serves as its internal pacemaker.

“There’s no way to get to the pulmonary arteries unless you go through this area,” Cheatham says. They solved this by putting in a temporary pacing catheter with each catheterization procedure they performed, to maintain a normal heart rhythm and allow repairs to continue.

“She had four more caths,” Beam says. “And then, in 2014, she was able to have her fourth open-heart surgery — the one that all the other doctors said she’d never live to get.”

With Gracie under anesthesia, Dr. Mark Galantowicz, chief of cardiothoracic surgery, replaced the original conduit with a larger one and patched the hole between Gracie’s right and left ventricles, which couldn’t be permanently closed until the pressure in her right ventricle was lowered. Cheatham then inserted a small metal tube called a stent in her tiny pulmonary arteries during the surgery.

The team used balloon angioplasty and stents to gradually enlarge pulmonary artery sections. “When you’re doing pulmonary rehab, you’re always talking small success stories,” Cheatham says.

This work went on through March 2015. Gracie’s mother reported that she was becoming more active and her skin was looking pinker. Eventually, the pressure in the right side of her heart became lower than pressure on the left side — as it should be.

The catheterization treatments were successful and Gracie’s health started to turn around. The need for double transplant became less urgent, and it was only reserved as a last resort.

Gracie Today

Fortunately, a transplant wasn’t needed. However, the future holds more heart procedures for Gracie, as it does for most children born with congenital heart conditions.

These days, Beam is more likely to be cheering on her daughter’s baseball team than sitting in a hospital waiting room. “Gracie’s 9 and in the third grade,” her mother says. “She goes to public school. She does gymnastics. She’s so happy, bopping down the halls at school — she’s just so happy to be alive.”

If Your Child Has a Heart Defect

Nearly 1% of children born in the U.S. each year have congenital heart defects, making CHDs the most common type of birth defect, according to the Centers for Disease Control and Prevention. If your child is diagnosed with a complex heart condition, there’s a lot to take in. Amy Basken, director of programs for the Pediatric Congenital Heart Association, offers some suggestions for parents:

— First, “The most important thing is to just take a deep breath and know you’re not alone,” Basken says.

— You’ll need to ask many questions about your child’s condition and treatment. The PCHA website offers educational resources and tools for questions to ask your doctor at different stages.

— Look at how many highly complex procedures a hospital has performed to treat your child’s condition. Evidence shows that higher-volume hospitals have better outcomes.

— Consider your individual situation. “Obviously, surgical outcomes and quality are of utmost importance at a hospital,” Basken says. “But it’s also important to think about your own well-being. And: Can you travel? Can you afford to leave your local center? Or can you consider the highest quality of care in your area?”

— Keep all of your child’s medical records together. “Remember the medical records are your own,” Basken says. “You can request them from the hospital and have copies in hand as you’re working through this.”

— Talk to other parents facing similar situations. “It’s psychologically important to have the support and encouragement you need,” Basken says. However, she emphasizes, it’s crucial to keep in mind that every child’s case is unique, and someone else’s experience may be very different than yours.

“Keep advocating for yourself and your child,” Basken says. “You are the voice of your child. Continue to ask questions until you’re comfortable with the answers. It’s OK to get a second opinion and know that you’re doing the very best you can with the information you have.”

More from U.S. News

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12 Medical Emergencies You Need to Address Right Away

The Honor Roll of Best Children’s Hospitals 2019-20

A Family’s Journey With Congenital Heart Disease originally appeared on usnews.com

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