With an intense interest in Marfan syndrome, physician-researcher Victor McKusick put Johns Hopkins on the map decades ago as a haven for patients with genetic aortic diseases. Today, that focus has expanded to encompass the myriad of conditions that affect the aorta.
“One of our biggest strengths is our team approach,” says Harry “Hal” Dietz, who’s often the first member of the care team that patients see.
Dietz, a pediatric cardiologist who switched careers to genetics early in his training to better help patients with Marfan syndrome, has made numerous discoveries in inherited aortic diseases. These include identifying the gene responsible for Marfan syndrome and co-discovering the related Loeys-Dietz syndrome. He sees patients of all ages with inherited aortic disease, counseling them on their conditions and risks at the Dana and Albert “Cubby” Broccoli Center for Aortic Diseases.
He says being able to care for both pediatric and adult patients is particularly helpful for those with genetic diseases that affect the aorta. “When different family members see different providers based on their ages, they may get different points of view and different recommendations,” he says. “Being able to accommodate all members of the same family gets everyone on the same page.”
Another longtime member of the team is Elliot Fishman, director of diagnostic imaging and body CT. Over the past three decades, Fishman and his colleagues have been developing better imaging techniques for aortic conditions. These include three-dimensional CT, an imaging modality now in widespread use across the country.
Fishman’s group provides results from imaging studies using methods that physicians can access remotely, including on tablets or cellphones. “In radiology, we’re in the business of providing information that doctors and surgeons can use to decide on the next step or follow up after procedures to make sure recovery continues in a positive direction.”
Such information provides the impetus for surgeons such as Duke Cameron, Luca Vricella, Bruce Perler and James Black to move forward with procedures to treat aortic aneurysms with minimally invasive stent grafts or open surgery, or to watch and wait if observation is more appropriate. “Many patients come here because they know we’re somewhat conservative and careful about operating,” Cameron says. “We’ve followed some of our patients for more than 10 years before we eventually move ahead. You don’t typically get that type of long-term monitoring from surgeons.”
Cameron, who, with Vricella and colleagues in pediatric and adult cardiac surgery, has built one of the largest valve-sparing aortic root replacement case series in the country, focuses on aortic repairs close to the heart. For abdominal aortic aneurysms, vascular surgeons including Perler and Black take the lead.
“Fixing an aortic aneurysm is truly a lifesaving intervention,” Perler says. “It’s extremely gratifying to be part of a team that works together to benefit our patients so tremendously.”
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