A World 
of Heart: Piper’s congenital heart defect

Organization: Service: Story Topics:
Pediatrics
piper-heart-defect

At two months, Piper Riness appeared healthy, but to her parent’s surprise, 
she was born with a congenital heart defect. 

Tracey and Clayton Riness love Saturday afternoons. With the chaos of the week behind them, this is their time to create memories and family traditions with their three children.

Piper was the newest addition to their growing household. She was perfect in 
every way, and seemed every bit as healthy as her two older siblings. At first,
 she was feeding well, sleeping like any newborn does and her development had
 textbook timing.

Then at Piper’s two-month checkup, her pediatrician, Peggy Tse, M.D., listened to her heart and, for the first time, detected a murmur. She referred the couple to pediatric cardiologist Ahmad Ellini, M.D., who noticed that Piper’s breathing was a bit labored. Dr. Ellini performed an echocardiogram, a type of ultrasound that showed pictures of her heart. The results revealed a serious abnormality in the structure and blood flow of Piper’s heart.

JUST IN TIME


Piper was born with a rare congenital heart defect called Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA). The aorta is the major artery that carries oxygen-rich blood throughout the body. In a normal heart, both the right and the left coronary arteries originate from the aorta, and supply the heart with oxygenated blood. In Piper’s heart, her left coronary artery branched off from the pulmonary artery instead of the aorta.

“The pulmonary artery is responsible for transporting oxygen-poor blood from the heart to the lungs,” explains Shaun Setty, M.D., medical director, Pediatric & Adult Congenital Cardiac Surgery Program, Miller Children’s & Women’s Hospital 
Long Beach. “In ALCAPA, when the left coronary artery stems from the pulmonary artery, it is unable to supply the left side of the heart with enough oxygen. The left ventricle becomes scarred and weak, while the left heart valve begins to leak. This causes serious issues for the patient as the function of the heart cannot keep up with the body’s needs, and may be life threatening at this point.”

Though it was detected early, Piper’s condition had already caused considerable damage. Both the upper and lower chambers of her heart were dilated from continuous strain, and the valve between them was leaking severely. Piper’s lower left chamber also was scarred from a lack of oxygen.

Because the mortality rate for ALCAPA is 90 percent in the first year, Piper was immediately admitted late that night to Miller Children’s, and scheduled for surgery the very next morning with Dr. Setty.

“We were shocked; floored; completely in a daze, because it all happened so fast,” says Tracey. “We wanted the best care for our Piper, and that’s why we chose 
Dr. Setty and Miller Children’s.”

AN OPEN HEART


Congenital heart defects are the most common type of birth defect. Some are simple, requiring only medication or no treatment at all. Others, however, are complex, where surgery and special medical care is the only option. With prompt medical diagnosis and corrective surgery where needed, children live happy, full and active lives.

Dr. Setty and his team navigated Piper safely through the surgery, successfully relocating the misplaced coronary artery without having to add artificial tissue or
 a graft.

Following surgery, Piper remained in the Pediatric Intensive Care Unit (PICU), where she was monitored around the clock. Her chest cavity remained open to allow for proper cardiac function and for the swelling to decrease. After two days, Dr. Setty closed her chest cavity, and two days later she was off the ventilator.
 She went home eight days after coming into the hospital.

UNIFIED CARE

The Centers for Disease Control and Prevention estimates that approximately 40,000 infants are born with a heart defect each year.

The Pediatric Heart Center at Miller Children’s is one of only a few programs in Southern California to offer comprehensive congenital cardiac care to patients of all ages. With advanced, highly specialized prenatal diagnostics, the heart specialists at Miller Children’s are able to detect many types of congenital heart problems while the baby is still in the mother’s womb.

“When a congenital heart defect is found before birth, outcomes may be improved,” says Dr. Setty. “The parents and the care team can develop an extensive treatment plan, and be fully prepared so there are as few surprises as possible.”

He adds, “Even with corrective surgery, patients with congenital heart defects need life-long care and monitoring. They might need additional procedures, a change in medication or new diagnostics. No two cases are ever alike, and that’s why it’s important to have a highly trained, highly specialized team that sees children or adults with these conditions every day.”

Dr. Setty and the multi-disciplinary pediatric cardiac care team partner with the MemorialCare Heart & Vascular Institute at Long Beach Memorial to treat virtually every type of congenital heart defect at any age. Patients have access to the latest imaging technology, cutting-edge surgical intervention and compassionate care teams from their time at Miller Children’s to when they transition to adult care at Long Beach Memorial. Steps apart, Miller Children’s and Long Beach Memorial physicians are able to collaborate regularly on patient cases, providing a unique continuity of care into adulthood.

“Part of the same team that’s taking care of the babies, takes care of the same patients as adults. Having a children’s hospital on the same campus as an adult hospital provides patients with the best of both worlds over their lifetime.”

Despite everything she’s been through, Piper is a happy baby. It’s been three months since her surgery, and she continues to hit all of her milestones. 

“Unless you see that beautiful scar on her chest, you wouldn’t even know she’s had surgery,” says Tracey. “She smiles every day, but more importantly, she makes all of us smile every day. Thanks to Dr. Setty and Miller Children’s, our family is whole.”

Featured physician:

Shaun P. Setty, MD

Helen E. Hoag Endowed Chair in Pediatric CardioVascular Surgery; Medical Director, Pediatric and Adult Congenital Cardiac Surgery at both Long Beach Memorial and Miller Children's & Women's Hospital Long Beach
Specialties:
  • Cardiothoracic / Thoracic Surgery