World Heart Day: Supporting Patients with Single-Ventricle Heart Defects and Their Families

Posted by Jodi McCaffrey on Sep 29, 2022 7:00:00 AM
6 minute read

Jaime and Steve Moreno were thrilled to learn that Jaime was pregnant with their second child. The couple had undergone in vitro fertilization several times to conceive a sibling for big brother Blake and were down to their last embryo when they got the good news. Then, during Jaime’s 18-week anatomy scan, the Morenos learned that their baby had a rare congenital heart defect called hypoplastic left heart syndrome (HLHS).

“We had never heard of HLHS before,” Jaime explained. “The diagnosis was like hearing a foreign language. We were in shock. We walked back to the car and just lost it emotionally.”

A Closer Look at Single-Ventricle Heart Conditions

The human heart has four chambers: a left and right atrium and a left and right ventricle. HLHS is a birth defect that occurs when the left side of a baby’s heart doesn’t form as it should. Because the left ventricle is smaller than normal, it can’t pump enough blood to the body. HLHS is one of several conditions that lead to single-ventricle heart disease. Other single-ventricle conditions include tricuspid atresia, pulmonary atresia and some forms of double outlet right ventricle, atrioventricular canal defect and Ebstein’s anomaly.

“We don’t completely understand what causes single-ventricle heart defects,” said pediatric cardiologist Eileen Stewart, M.D., of Pediatric and Congenital Cardiology Associates of Texas. “We believe they are caused by a combination of genetic and environmental factors during the early stages of embryo growth. Most are diagnosed prenatally, so we can begin working with families before the baby is born.”

Single-ventricle defects are some of the most complex heart problems and usually require at least one surgery and ongoing cardiac care. The Morenos were referred to Dr. Stewart, the director of the Single Ventricle Program at Dell Children’s Medical Center in Austin. She also runs the hospital’s interstage monitoring program, called IMPACT (Interdisciplinary Monitoring Program for Ambulatory Cardiac Care of Central Texas).

IMPACT Program Supports Patients and Parents

IMPACT follows patients and families throughout their interstage journey, bridging the transition from inpatient to outpatient care. It helps families better understand their child’s condition, navigate the multiple surgeries that are required and learn how to care for their children between and after surgeries. Families in the program meet with the team to discuss each procedure and learn the milestones along the path to recovery. They are also matched with a social worker who supports their emotional and financial needs.

Parents receive workbooks and can use stickers to mark an “education passport” as they advance through the program. Passport milestones include “We can explain our baby’s heart surgery,” “We know how and when to give our baby’s medications” and “We know red-flag signs and our baby’s warning signs.”

After the child is born, a social worker checks in with IMPACT families weekly to address things like postpartum depression and anxiety over having a sick child. If needed, a psychologist can provide additional support. Speech and physical therapists, skilled in caring for infants with complex heart disease, assist with feeding skills and motor development throughout the infant’s journey. Before discharge, parents “room in” with their babies to ensure they understand how to care for their complex newborn, administer medications and identify concerning symptoms and signs.

“IMPACT is a multidisciplinary effort among cardiology, cardiovascular surgery, therapy, psychosocial services and nutrition,” said Dr. Stewart. “We provide 360-degree, holistic care to medically fragile, complex children and their families.”

Infants typically graduate from the program and go home once they are clinically stable after their second surgery, usually around 6 months of age. The program recently celebrated its 50th graduate.

“Years ago, when families faced complex conditions like these, it was often an isolating experience. They didn’t have support,” Dr. Stewart said. “Now, when parents receive a concerning diagnosis, it is my hope that this program makes them feel like they have someone going through this journey with them.”

Chase’s Story

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Dr. Stewart monitored Jaime and her baby throughout her pregnancy. The Morenos welcomed Chase to the world on Oct. 6, 2021, and pediatric congenital cardiothoracic surgeon Carlos Mery, M.D., performed his first surgery five days later with input from Dr. Stewart. During the open-heart surgery, called the Norwood procedure, a new, larger aorta is created, a shunt is placed to deliver blood flow to the lungs and an atrial connection is enlarged to encourage the mixing of blood throughout the heart.

“Dr. Stewart has been with us from the beginning, looking at his heart throughout the gestational period,” Jaime explained. “We had many conversations with the care team about Chase’s condition, but nothing truly prepares you mentally and emotionally for surgery. We are lucky to have the single-ventricle expert on our team.”

Between his first and second surgeries, Chase experienced reflux issues. Luckily, according to Jaime, his care team was available 24/7 to field questions and offer advice.

“The biggest benefit of the IMPACT program for us was the rooming-in period, which allowed us to get used to monitoring his condition before taking him home after his first surgery,” she said. “They made sure that we felt comfortable and were equipped to care for him — everything from administering his medications, teaching us the warning signs to look for and how to manage his feeds using his nasogastric tube. The IMPACT team became our mental and emotional safety net — an extended family, really — since we had to isolate between surgeries due to COVID-19.”

Chase underwent a second surgery — the Glenn operation — in March 2022. He was weaned off a feeding tube this summer and is now enjoying being a normal baby, Jaime said. In a few years, he will need a third and hopefully final surgery, called the Fontan procedure.

“He’s still a little on the small side, but we’re on the right path,” she said. “We’re enjoying watching him grow and develop.”

To help other parents on the same journey, Jaime is working with Dr. Stewart as a local representative from Dell to the National Pediatric Cardiology Quality Improvement Collaborative to create an even stronger support community for parents of single-ventricle patients.

“There are many congenital heart defects, but HLHS is very unique,” Jaime explained. “So many things can go wrong before, during and after surgery. IMPACT provides parents with a great foundation of knowledge, but we’ll always have questions. I want to help build a support system of parents who have been where we are.”

Topics: Pediatric Cardiology, Pediatric