Although her parents were warned she might not breathe when she was born, the moment Francesca Durkos came into this world, she let out a gutsy cry.
“It was music to our ears,” says her mom.
Michelle Carino Durkos was 40 weeks pregnant when she learned there was a tumor attached to her unborn daughter’s heart — a tumor so large that doctors near her home in Pensacola, Florida, were unsure if the baby would live.
“It was a shock, because at 20 weeks everything was normal,” says Michelle. “We had a wonderful ultrasound; we saw all four chambers.”
Yet, call it a mother’s intuition, Michelle knew something was wrong.
“The whole pregnancy I had this strange feeling. I didn’t want to upset her, so I’d sleep sitting up, as if she was fragile — as if she was in distress.”
Cardiac fibroma: A devastating diagnosis
Francesca’s tumor, a rapidly growing cardiac fibroma, is just one example of a group of benign congenital cardiac tumors that typically occur in children, and in most cases, are detected during in utero or infancy. But, because the heart is a vital organ, even benign tumors can be life threatening — interfering with the way the heart works, causing blockages and arrhythmias.
In Francesca’s case, her heart-sized tumor triggered ventricular tachycardia, an abnormal heart rhythm that originates in the ventricles and is often associated with cardiac arrest. “We didn’t know if our child would live,” says Francesca’s dad, Jon Durkos.
At Francesca’s five-month check-up, the tumor had grown and was making her heart work even harder. “The doctors told us, ‘The mass needs to go as soon as possible.’ It felt like we were hit by a truck.”
After meeting and speaking with multiple doctors and surgeons, the Durkoses decided to get a second opinion.
“No one had ever seen a tumor this size, so when I spoke with Dr. del Nido [chief of the Department of Cardiac Surgery] at Boston Children’s, the first thing I asked was, ‘Have you ever seen a cardiac tumor this big?’ He told me, he’d actually seen one even bigger. And then I asked, ‘Is the child still living?’ To hear, ‘Yes,’ was just unbelievable.”
In Boston, open-heart surgery offers hope
After arriving at Boston Children’s, Francesca underwent a series of preoperative evaluations. “Francesca’s tumor was massive in size,” says Francesca’s cardiologist Dr. Tal Geva, chief of the Department of Cardiology at Boston Children’s. “It not only compressed the left ventricle and distorted the mitral valve, causing leakage, but also compressed the left lung, left pulmonary artery and veins.”
During the complex and challenging procedure, the tumor was removed and the mitral valve repaired. Following her surgery, Francesca needed extracorporeal membrane oxygenation (ECMO) for two days to support her heart, because of the extensive surgery on the left side of her heart, required to remove the tumor. Seventeen days later, she was discharged from the hospital.
Four and moving forward
Four-year-old Francesca is running on the treadmill in the Heart Clinic. She’s performing her annual stress test at Boston Children’s, and she’s rising to the occasion. She’ll continue to be monitored regularly, but the prognosis is encouraging. Reoccurrence of cardiac fibromas is rare.
“Keep on going!” she exclaims.
Back in New York City, which is now home for the Durkos family, Francesca is making strides in school as well. She’s enrolled in classes for the gifted and talented. And, her mom says, she’s got a talent for singing too. “If I had known that almost five years later, she’d be thriving — it’s almost unreal.”
Francesca says she wants to be a swimming teacher when she grows up — for today at least. Yesterday, she wanted to be a singer, and the day before that, a mermaid.
The world is her ocean.
Explore bostonchildrens.org/fetalheart for more information and resources.