MORRISTOWN, NJ (Morris County) – When you know a potentially fatal heart condition runs in your family, you worry that tragedy could strike at any moment. This was always in the back of Betulia M.’s mind — not just for herself, but for four of her five children who were born with Marfan syndrome.
One of Betulia’s greatest fears came true last October, when she arrived at Morristown Medical Center’s Emergency Department with intense pain and in critical condition due to a ruptured aorta. She needed immediate life-saving heart surgery.
Cardiac surgeon Christopher Magovern, MD, saved Betulia’s life that night. But it was a wake-up call for her 20-year-old identical twin sons, Pablo and Julio D., who had been seeing a pediatric cardiologist for Marfan since they were very young.
“Marfan syndrome is a disorder of the connective tissue, the tissue that holds everything together in the body,” said Benjamin van Boxtel, MD, surgical director at the Atlantic Aortic Center at Morristown Medical Center.
“It’s a genetic condition that affects about one in 5,000 people, which is fairly prevalent in terms of diseases, yet not widely familiar. It can affect every part of the body, from the eyes and spine to the heart.”
Standing over 6 feet tall, Pablo and Julio have long, lean bodies, thin lengthy digits, very loose joints, and chest wall defects — all stereotypical features of Marfan’s syndrome. They also both had an aortic root aneurysm, the most dangerous complication associated with Marfan. As the aortic aneurysms were growing, the aortic tissue was thinning, putting them at significant risk for a potentially fatal aortic rupture.
So, they decided to be proactive and undergo elective surgery.
Dr. van Boxtel explains that the gold standard surgery for young Marfan syndrome patients like Pablo and Julio, is a valve-sparing aortic root replacement. This approach allows patients to keep their own native aortic valve tissue, while removing the dangerous, thinned-out, dilated aortic root tissue. It also means there’s less chance they will need future surgery, while avoiding lifelong blood thinners associated with some prosthetic aortic valves.
“This a procedure we perform regularly, and with exceptional outcomes,” said Dr. van Boxtel, who specializes in the valve-sparing root procedure, along with other complex aortic reconstruction operations.
The brothers asked to be scheduled for surgery on the same day because they say they “do everything together”. Since Pablo was born first, he would go first, and Julio would follow.
“We helped take care of our mother after her surgery and we thought it would be easier for our family if we went through surgery and recovery together,” Julio said.
Operating on identical twins in one day was a first for Dr. van Boxtel and the entire cardiac surgical team and required very strict protocols to follow so each twin received identical care without any mixup.
“Our team put unique quality measures in place for this very unique situation,” said Dr. van Boxtel. “Not only did everything go perfectly in the operating room, but their recovery process was just as seamless. I work with a world-class team who make successful days like this possible: my fellow cardiac surgeons, cardiologists, anesthesiologists, intensivists, physician assistants, nurse practitioners, cardiopulmonary perfusionists, nurses, techs, pharmacists, dietitians, therapists and the list goes on and on!”
“I’m so grateful to my team and this hospital,” Dr. van Boxtel said. “Together we’ve been able to give Julio and Pablo the potential for a long, healthy lives.”
Dr. van Boxtel is especially pleased that the operation will allow Pablo and Julio to enjoy a relatively normal life. Pablo and Julio are especially pleased, too, but for different reasons.
“We do everything together in life, and surgery was no different,” said Pablo.
“Going through this experience with my brother and best friend has been surreal and I am beyond grateful. Now that it’s behind us, we’re trying to educate Spanish-speaking communities about Marfan syndrome and the importance of good health. Our goal is to help people understand this genetic disorder so they know what they can do to live a better life,” Julio said.
