Hypoplastic Left Heart Syndrome and Heterotaxy Syndrome: Brendan’s Story

Brendan's mom, Beth, was 20 weeks pregnant with him when a routine prenatal ultrasound revealed her unborn baby had two rare and severe birth defects: hypoplastic left heart syndrome and heterotaxy syndrome.

Fast forward 18 years and Beth's son, Brendan, is now thriving. His family decided to share Brendan's story as a way of repaying all the families who shared their stories and helped them find hope during their darkest days.

Rare birth defects

Hypoplastic left heart syndrome (HLHS) is a congenital birth defect that affects the left side of the heart, leaving it under-developed and unable to pump oxygenated blood through the body. The left ventricle of the heart is too small in patients with HLHS, leaving the right ventricle to do all of the work. HLHS typically requires three reconstructive surgeries to redirect the blood flow: the Norwood, Glenn and Fontan procedures. Heterotaxy syndrome is a condition in which some of the organs of the body are not located in their correct positions and some organs have not formed normally. Some children with heterotaxy syndrome have complicated congenital heart disease and need heart surgery. Some need abdominal surgery and some do not have a spleen.

Brendan was born in a hospital near the family's home in Arizona and spent four days in the neonatal intensive care unit (NICU) while doctors assessed his condition and determined next steps. Doctors determined Brendan would not need the first of a traditional trio of reconstructive heart surgeries for HLHS. The family was able to bring him home to grow stronger until his first surgery at 3 months old.

Reconstructing Brendan’s heart

Brendan had his first surgery, the Glenn procedure, to redirect the flow of blood return from the upper half of his body directly to the lungs. He also needed an additional procedure to connect the veins from his lungs back to his heart. Though he needed supplemental oxygen for the first two months after his operation, Brendan recovered quickly. He grew into an active boy and showed no signs of illness. It made the idea of the final reconstructive surgery, performed when he was 3 years old, difficult to imagine for his family.

“I remember waiting in the waiting room before he was going in to get the Fontan," says Beth. "He was running around — a completely active kid. We were thinking ‘Why are we doing this? 'It didn't seem right."

Brendan's Fontan operation was initially successful, but his condition soon took a turn for the worse. Every time doctors tried to close the incision on Brendan's chest, his heart would stop. His surgeon, Michael Teodori, MD, decided to keep the incision open for four days while Brendan’s condition stabilized. That decision may have saved the little boy’s life.

Brendan’s road to recovery was long and hard. At 3 years old, he had to relearn how to walk and use his hands. “He came back to us a little bit more every day,” says his mom.

Relocating to Philadelphia and finding CHOP

Brendan's family is originally from the Philadelphia area and was planning to return after Brendan’s surgery, but they hadn’t anticipated the complications Brendan would experience, or the length of his recovery. Their surgeon, Dr. Teodori, whom the family was close to in Arizona, had done his residency at CHOP. He recommended the family contact Meryl S. Cohen, MD, a pediatric cardiologist at Children's Hospital of Philadelphia (CHOP) for follow-up care and ongoing monitoring.

“We felt an immediate connection to Dr. Cohen. She was calm, and she reassured us. She was amazing from the start.”

Brendan was 4 years old when he began seeing Dr. Cohen. From their first meeting, Dr. Cohen impressed his family by talking directly to Brendan about his condition. The precocious preschooler responded by asking questions his mother never suspected he had.

“I think I tried to shield Brendan a lot, but when we started with Dr. Cohen, it was almost like it was the beginning of him understanding that he was different," Beth says. "It was important for him to build a relationship with his doctor.”

Dr. Cohen encouraged Brendan and his family to become part of CHOP’s FOntan Rehabilitation, Wellness, Activity and Resilience Development (FORWARD) program. The family was thrilled with the program that provided specialized care, evaluations and ongoing monitoring to patients like Brendan, with single-ventricle heart defects and Fontan circulation. The multidisciplinary program helps children and teens build resilience to improve the quality and duration of their lives.

A long recovery

After his Fontan surgery, Brendan experienced many developmental, academic, speech and fine motor skill delays. He overcame them all with help from CHOP doctors, therapists, and outside tutors and therapists.

As Brendan got older, it became difficult for him to accept the physical limitations he had because of his condition and treatment. He wasn't allowed to play football, paintball or other high-impact sports.

“It has not always been easy," Brendan says. "I was frequently mad at my parents for not letting me do things because of my heart defect — things like playing football or going on the school trip to Great Adventure … [it was] devastating.”

When Brendan’s parents encouraged him to try swimming, something clicked. It was the perfect sport for him. “Whenever we go to CHOP for his exercise test, the doctors say he’s in the most amazing shape and to never stop swimming!” Beth says.

Brendan continues to be treated for heterotaxy syndrome. In Brendan’s case, this means he does not have a working spleen, an organ that plays an important role in the immune system. As a result, Brendan is more susceptible to infections and must take an antibiotic daily. Brendan’s doctors also monitor his liver function during annual visits to CHOP.

Finding inspiration

While visiting colleges on the West Coast, Brendan and his family reconnected with Dr. Teodori, whom they hadn’t seen in 12 years.

“It was an amazing experience because I don’t think Brendan truly knew what the surgery meant for him,” explains Beth. “Dr. Teodori told him: ‘I always considered you like one of my kids. I worked my whole life to be able to help kids.’”

At a swim meet late last year, Brendan also experienced what it’s like to be a source of inspiration to others. A man approached him and his family to ask about the scars on his chest. As Brendan’s mother explained his condition and successful treatment, the man began to cry. His nephew had just been born with HLHS and his family was worried about his future.

Adds Brendan:

“He was overcome with joy to see a fellow HLHS [patient] enjoying life and thriving. He asked permission to video my next race to share with his family. I have been thinking about that man a lot recently and how my story could make a difference.”

Bright future

Today, Brendan is in his senior year of high school in Philadelphia. Through the school, he volunteers for the Travis Manion Foundation, an organization that helps military and Gold Star families. A die-hard Eagles and Sixers fan, he’s interested in studying sports medicine or sports management.

“We’ve been so lucky with CHOP,” says Beth. “His doctors. His surgeon [in Arizona] learned everything from CHOP. At CHOP, they help the whole person: mentally, emotionally, they’ve helped us in every way. I never knew that was possible.”