Only two weeks after a groundbreaking heart operation at Boston Children’s Hospital, 11-year-old Clara Portnoy found herself standing in a sprinter’s stance on a grassy stretch during her school’s eagerly anticipated field day. Surrounded by supportive classmates and encouraging teachers, she was ready to take her place in that exhilarating moment.
Born with a condition characterized by underdeveloped structures on the left side of her heart, Clara had spent her entire childhood avoiding any rigorous exercise. It was understandable why she often expressed to her parents a heartfelt desire to partake in what she referred to as “normal kid things.” So, after her surgery, it was no wonder that she enthusiastically decided to run.
Captured in a poignant video by her father, Joe, Clara’s run showcased the determination and joy of a girl finally able to express herself freely. “She ran in front of the whole school,” Joe shared, beaming with pride as Clara sat shyly beside him, not quite ready to discuss her monumental achievement. “And it was truly awesome.”
A series of heart procedures began before birth
Clara’s jubilant run came after an especially tumultuous year, during which it appeared that her last surgical intervention was failing. She faced persistent fatigue, poor appetite, and overall malaise. Unsurprisingly, Clara missed almost the entirety of her school year as a result.
Clara underwent several significant heart and circulation procedures early in her life, beginning even before her birth. While still in the womb, she was diagnosed with severe aortic valve stenosis and hypoplastic left heart syndrome (HLHS), leading to improper development of her left-heart structures. To treat this, she underwent a fetal cardiac intervention, a complex procedure conducted by a skilled team led by Dr. Wayne Tworetzky, a renowned cardiologist and director of the Fetal Cardiology Program. This intervention aimed to halt the progression of her condition to HLHS.
However, despite this initial intervention, the left side of Clara’s heart was still insufficiently developed to manage the circulation demands of her body. After her birth, she was quickly transferred to the Cardiac Intensive Care Unit (CICU) at Boston Children’s, where just nine days later, she underwent the Norwood procedure—the first of three vital surgeries designed to help her survive with HLHS. Several months later, she received the Glenn procedure, followed by the Fontan surgery at the age of three. While the Fontan can be beneficial for some children, it may also result in adverse effects on the liver, bowels, and lungs in others.
Unfortunately, Clara experienced these negative repercussions firsthand. She began to suffer from decreased heart and valve function. After her family relocated to Colorado, her heart team in Denver, who had taken over her care, informed Clara’s parents, Joe and Joanna, that they had exhausted all treatment options. A heart transplant would be necessary to save her life.
A familiar heart team offers a better alternative
Eventually, Clara’s family discovered new hope in an unexpected place. Her Denver heart team discussed a promising alternative to a heart transplant. “They mentioned a groundbreaking surgery being performed by someone in the U.S. that could reconnect her heart structures differently and possibly prevent the need for a transplant,” Joe recalls with renewed optimism. “Joanna instantly asked, ‘Is it Ram Emani?’ They confirmed, ‘Yes.’ It was a relief knowing that it was, indeed, Dr. Ram Emani.”
How the reverse 1.5 ventricular repair helped Clara
The reverse 1.5 ventricular repair approach partially reversed Clara’s previous Fontan circulation to reintegrate her left ventricle into her body’s circulation but at a reduced workload. This technique allows her left ventricle to pump lower-body blood to her lungs, while her right ventricle takes on the heavier responsibility of distributing blood throughout the entire body.
By implementing this new circulation method, the pressure within Clara’s veins was lowered significantly. It enhanced her organs’ overall function and, critically, enabled her to significantly increase her cardiac output during physical activity, as vividly demonstrated when she joyfully sprinted at school.
Dr. Sitaram “Ram” Emani, an accomplished cardiac surgeon at Boston Children’s Hospital and director of the Complex Biventricular Repair Program, had previously performed Clara’s heart surgeries and was well-acquainted with both her and her family. The family had also built a trusting relationship with Dr. Tworetzky. He brought attention to the Denver heart team regarding the new surgical technique developed at the Benderson Family Heart Center: a reverse 1.5 ventricular repair. Later, Dr. Tworetzky informed Clara’s family that she could potentially be among the first patients worldwide to undergo this innovative procedure.
When Clara re-entered Boston Children’s Hospital in May, her left ventricle still lacked the capacity to handle a complete workload; however, her care team observed sufficient development to facilitate circulation. Thanks to her prior surgeries, she had become an ideal candidate for the reverse 1.5 ventricular repair. “This revolutionary circulation method can significantly enhance her blood flow,” Dr. Tworetzky remarked.
Full of life, full of joy — a new beginning for Clara
Thrilled to witness Clara’s remarkable improvement, Joe and Joanna couldn’t be happier. “Now she has a pink face and vibrant lips—she looks full of life,” Joe proudly exclaimed. “Her life is utterly transformed. She wakes up in the morning, walks a quarter-mile to the bus stop, finishes a full day of school, and walks back home. Then she eagerly goes outside to play with her friends. Seeing her progress from being told she needed a heart transplant to knowing we may only need to address her heart valve in the future is just incredible.”
Clara’s newfound energy propels her to engage in joyful activities with her sisters, 10-year-old Eleanor and 8-year-old Poppy. From dancing to climbing trees, indulging in creative writing, and relishing the music of Taylor Swift, her life is now brimming with possibility.
“Even during her toughest times, we felt an overwhelming gratitude for her existence,” Joe reflected. “While she grappled with health issues, we focused on the joy of having her here, healthy in heart, with all her fingers and toes intact. Now that she’s thriving, we have even more to celebrate.”
Learn more about the Complex Biventricular Repair Program or request a second opinion.
Clara’s Heartwarming Journey: From Heart Struggles to Joyful Runs
Let’s talk about little Clara Portnoy, shall we? Just 11 years young, and already a contender for the title of "Most Inspiring Kid of the Year." After a serious heart surgery at Boston Children’s Hospital—kind of like having your heart ‘remodeled’—she strutted onto the field at her school’s field day ready to tackle life head-on. And, oh yes, she ran like Usain Bolt in a race against time. Those of you who stayed in pajamas all day after minor surgeries, take note!
Clara was born with hypoplastic left heart syndrome—sounds like something Harry Potter would have faced down in a duel, but unfortunately, it’s a real-life conundrum where the left side of the heart doesn’t develop properly. Instead of casting spells, she had to endure surgical spells—starting before she was even out! She has gone through a series of heart procedures that would make even an Olympic athlete’s training schedule look like a walk in the park.
Surgery Before Birth? Now That’s a Plot Twist!
Imagine being diagnosed in the womb—bet that wasn’t a conversation her parents were expecting over dinner. But that began Clara’s saga of surgeries that started before she could even properly pronounce “healthcare.” And here we are complaining about a paper cut!
Again, her life wasn’t all rainbows and unicorns. After years of dodging vigorous playtime like it was a game of dodgeball—the psychological horror for any child—she finally decided to let loose. One minute you’re missing out on school because of health issues, and the next, you’re out there sprinting like a gazelle on the savannah! Her dad captured it all on video—a spark of joy in the chaos of life, if I ever saw one!
Meet the Heart Heroes
Of course, we can’t overlook the real heroes in this tale—her medical team! Dr. Wayne Tworetzky and the magical wizards at the Benderson Family Heart Center performed surgical enchantments on her heart. After some “you might need a heart transplant” chats, Clara’s parents were introduced to an alternative: the reverse 1.5 ventricular repair—a mouthful, I know, but it’s like upgrading from a bicycle to a sleek electric scooter in the race of life.
This procedure didn’t just spark her heart to work better; it allowed Clara to rediscover her zest for life. As Dr. Tworetzky put it, it “substantially improves her blood flow”—which I interpret as giving her the fuel to run amok with her sisters, Eleanor and Poppy, once again.
The Joy of Being Alive
And let’s talk about the modern miracle of Clara’s life post-surgery. She’s a new child—seriously, has anyone seen a 180-degree transformation like this? She now has ‘the pink’—you know, that vibrant color that means you’re no longer grey and lifeless but full of pizzazz! She’s getting herself up, walking to the bus stop, and managing to survive the school day like a pro (with friends, dancing, and Taylor Swift!).
Her father, Joe, encapsulated the transformation beautifully, stating, “She now looks full of life.” Well, Joe, if “life” could be bottled and sold, Clara would be the best-selling product of the year!
Remember, this incredible woman is beating every odd with style—dancing, climbing trees, and scribbling her heart out. If we’re taking notes here, the takeaways are: be grateful for the little things, celebrate the big moments, and for goodness’ sake, don’t let anyone dull your sparkle—not even a surgical team!
So here’s to Clara Portnoy—the symbol of resilience and that vibrant spirit we all should aspire to. Looking forward to more of those joyous runs and likely a career as a motivational speaker in her teenage years! Watch out, world—Clara’s coming, and she’s bringing her heart with her, one sprint at a time!
