Hypoplastic Left Heart Syndrome and Heart Transplant: Hudson's Story

Published on

By the time Hudson was 4 ½ years old, he'd survived three open heart surgeries, two brain hemorrhages and a blood clot in his brain. But, he'd been in heart failure since he was 3, and his weakened heart simply couldn't supply enough blood to his body. He needed a heart transplant — and soon — to survive. 

Hudson smiling Both pediatricians, Hudson's parents knew how serious their son's condition was and that he'd likely be waiting on the heart transplant list for a significant amount of time before a suitable donor organ would be available.

Due to Hudson's worsening clinical status and need for closer monitoring, in January 2018 he and his mom Tori moved into Children's Hospital of Philadelphia (CHOP), where he'd been treated since he was 2 years old.

In June, Hudson's family received the call they were waiting for: Doctors had a heart for Hudson and would be operating that day. Thirteen days after his successful heart transplant, Hudson went home with his parents and siblings. Today, 5-year-old Hudson runs circles around his parents and loves attending pre-kindergarten.

Early diagnosis of heart condition

Tori and her husband Tad were living in Ohio when they first learned that Hudson's heart wasn't normal at their 20-week prenatal ultrasound. Doctors suspected the baby had hypoplastic left heart syndrome (HLHS), a severe congenital defect in which the left side of the heart is underdeveloped. Treatment for HLHS would require staged reconstructive heart surgery, where surgeons perform a series of surgical procedures over several years.

Hudson was born May 9, 2013, at a hospital in Cincinnati, OH, and immediately transferred to a nearby children's hospital for cardiac care. Doctors confirmed Hudson had HLHS, and five days later he had his first open heart surgery. Hudson had his second reconstructive heart surgery at 5 months old.

For the next 18 months, Hudson was regularly monitored by his cardiologist to determine optimal timing for the third reconstructive surgery. As Hudson grew, doctors discovered another problem: He had tricuspid regurgitation, a disorder in which a heart valve doesn't close completely, causing blood to flow backward from the right ventricle, Hudson's only pumping chamber.

Tori and Tad were planning to move to Pennsylvania for work, and after consulting with Hudson's doctors in Ohio and his new cardiac specialists at Children's Hospital of Philadelphia (CHOP), they decided to proceed with repair of the tricuspid valve during Hudson's planned third reconstructive heart surgery, which would be his first surgery at CHOP.

Heart surgery at CHOP

In May 2015, Thomas Spray, MD, then Chief of Cardiothoracic Surgery at CHOP, performed open heart surgery on Hudson. A few days later, Hudson returned home to Hummelstown, PA, with his family.

"Hudson was doing great for a while," Tori says. "He was really active and playful."

But in January 2016, Hudson got sick with what his family originally suspected was a cold. When it didn't go away, they went to their local hospital.

Doctors performed an echocardiogram and determined Hudson was in heart failure. His heart could no longer pump enough blood to meet the needs of his body. Heart failure is a progressive condition that can cause severe swelling, difficulty breathing, fatigue and poor growth.

Hudson was transferred to CHOP where his primary cardiologist, Chitra Ravishankar, MD, and the heart failure team, took charge of his care. The team prescribed medications to make his heart work more efficiently and beat more regularly. Hudson was able to go home with a continuous intravenous medication that helped his heart pump more efficiently. Tori and Tad learned how to care for Hudson's peripherally inserted central catheter (PICC) line and medication, and Hudson was discharged.

Listed for heart transplant

By March 2016, it was clear the medications weren't helping enough. Hudson would need a new heart. He was listed for a heart transplant on the United Network of Organ Sharing (UNOS).

"We expected it would take a while on the transplant list, but we wanted to be ready when the call came that he had a heart offer," Tori says.

Hudson needed frequent monitoring, medications and testing at CHOP. Though he tired more easily than before, he was able to still do many of the things he enjoyed like playing with toy cars and watching car repair shows.

Neurological issues

In November 2017, Hudson experienced weakness in his right side and his speech became slurred. "We were worried he was having a stroke," Tori says. Doctors at CHOP discovered Hudson had a blood clot in his brain.

"Thankfully they found it quickly and were able to treat it," Tori says. Doctors inserted a catheter into Hudson's groin, threaded it up into his brain and removed the clot. Afterwards, Hudson seemed fine and there was no evidence of permanent brain injury. After a week in the hospital, he returned home.

But four days later, Hudson woke up in the middle of the night with a headache and vomited. His family brought him directly to CHOP where clinicians performed a CT scan of Hudson's brain and learned he'd had a subarachnoid hemorrhage. A few days later, while still in the hospital, Hudson had a seizure. This time, doctors determined it was an intracerebral hemorrhage (bleeding within the brain).

"Everyone was really concerned," Tori says. "With heart failure, the next option is generally a VAD (ventricle assist device) but to use it, Hudson's blood had to be anticoagulated. For him, this wasn't an option.

"We needed to get him a heart — and soon."

Moving into CHOP

After discussing their options with CHOP's Heart Failure and Transplant Program team, Hudson's family decided they needed to take action. The only way for him to move up the transplant list was if he was hospitalized; he certainly was sick enough.

So, in January 2018, Hudson and Tori moved into CHOP. Hudson lived on a cardiac unit where he received specialized cardiac care and monitoring, as well as support services like physical therapy, occupational therapy, cardiac rehabilitation, music and art therapy.

Tori took a leave of absence from her job and essentially spent all her time at Hudson's side. Tad visited on weekends, but remained at home with the couple's other children, Julian, 8, and Vivian, 2.

Heart transplant

On June 13 at 5:20 a.m., one of the transplant cardiologists, Kimberly Y. Lin, MD, called Tori with news: "We have a heart."

At 2 p.m. Hudson was wheeled into surgery. Cardiothoracic Surgeon Stephanie Fuller, MD, performed the complex heart transplant in six hours. The family was updated every hour during the procedure, ensuring the family knew what was happening at each step during surgery.

Two days after surgery, Hudson was standing up and walking. On June 26, he went home.

Hudson's energy quickly returned once we were back home. Now, he runs circles around us.

Doing well and planning for the future

Four months after his heart transplantation, Hudson is doing well. He has had no signs of organ rejection and his clinical team is working to gradually wean him off some of his medications. Hudson began pre-kindergarten in September and is really enjoying being around kids his own age, Tori says.

"After the transplant, he had to stay pretty isolated for a few months to keep the risk of infection down, but now he can do a lot more," Tori says.

Some of Hudson's favorite things to do involve cars and things that go fast. He loves playing with cars, helping his dad and a neighbor fix cars, and watching all sorts of car races, auto repair shows, and shows that transform older junk cars into hotrods.

"Hudson can also run really fast … and he's super excited about that," Tori says. "Before his transplant, he couldn't always keep up with his friends because of his heart condition. Now he can and sometimes he's even faster than his friends — which he loves."

Hudson may only be 5 years old, but he has big plans for his future: "I want to be a race car driver or a rock star."