While Woody DelCorso was driving his car to work one day at age 20, his right lung suddenly collapsed for no apparent reason. It was the first clue that eventually led him to a diagnosis of Marfan syndrome. Now 39, DelCorso has had numerous surgeries at The Johns Hopkins Hospital to treat various symptoms of this condition, including valve-sparing aortic root replacement, installation of a pectus bar to correct his concave chest, emergency surgery to repair a dissected ascending aorta and another to repair his mitral valve.
“I know that I’m blessed to have had so many operations to save my life,” DelCorso says.
But his most extensive surgery to date, in April 2015, may have ended the need for future procedures to shore up his circulatory system. The operation was necessary to repair rapidly expanding thoracic abdominal aortic aneurysms. DelCorso had his descending aorta swapped with surgical graft, completing the replacement of his entire aorta.
“Now his risk for rupture is essentially zero,” says James Black, Johns Hopkins Medicine’s chief of the Division of Vascular Surgery and Endovascular Therapy, who performed DelCorso’s latest procedure alongside cardiac surgeon Duke Cameron and other colleagues. “There’s no more natural aorta left to develop aneurysms in the future.”
Such “root to boot” replacement takes place only at a handful of medical centers across the country with the expertise to care for patients with complex aortic disease like DelCorso’s, Black says. One of the riskiest aspects of such an extensive procedure, he explains, is the potential for paraplegia. Because the aorta supplies key circulation to vast swaths of the spinal cord in some patients, surgery to replace it could cut off the blood supply long enough to severely and permanently damage key nerves.
As a precaution before surgery, DelCorso, like others who undergo this procedure at Johns Hopkins, required an arteriogram performed by the hospital’s neuroradiology team. Imaging revealed that DelCorso was in the one-third of patients whose aorta predominantly supplies circulation to the spinal cord. Armed with this information, Black and other members of the surgical team planned the operation using techniques that would avoid interruption to the spinal cord’s blood flow. For additional protection, the surgeons implanted a spinal drain to reduce pressure in the spinal column in the days following the procedure. They also used deep hypothermia during the operation itself, a technique that has proven useful in a variety of other medical circumstances to protect tissue from damage by slowing its metabolism.
DelCorso’s procedure was ultimately successful, his entire aorta replaced with no subsequent damage to the spinal cord. “I would anticipate that he’ll have a completely normal life span, with no future risk of aortic rupture,” says Black.
For DelCorso and hundreds of other patients with complex aortic disease who seek help at The Johns Hopkins Hospital, adds Black, positive outcomes like these are a testament to the team approach and detailed protocols the hospital has developed to care for these patients, gathered through many years of experience.
“This is one of the most major operations that our hospital can provide, and we do it on a routine basis with a routine recovery for most patients,” Black says. “To be able to deliver this type of care to so many patients while building on a legacy of treating complex aortic disease is one of my favorite parts about practicing medicine here.”