Of all the symptoms that present with connective tissue diseases and affect the cardiovascular system, such as Marfan syndrome or Loeys-Dietz syndrome, it’s abundantly clear that the most serious is the effect on the aortic root. Without prophylactic surgery, this arterial section extending just a couple of inches out of the heart will expand over time and develop discrete weaknesses, eventually rupturing. For most patients with this condition, there are no obvious external signs of the danger lurking within—just a quiet knowledge, derived from periodic imaging studies, that this ticking time bomb will eventually need to be addressed.

Ever since the late 1960s, surgeons have resolved this issue with an operation called the Bentall procedure, which typically replaces the ascending aorta, root and valve with a composite graft. While lifesaving for Marfan patients, it comes with a host of drawbacks, explains Duke Cameron, director of the Division of Cardiac Surgery at The Johns Hopkins Hospital. In particular, the artificial valves that replace the aortic valve may wear out over time, leading to additional risky surgery. Or, the procedure may require patients to be on anti-coagulation therapy for the rest of their lives, which comes with its own inherent problems.

That’s why Cameron has made valve-sparing procedures—operations that allow patients to retain their own aortic valves while also mitigating their risk of an aortic aneurysm—a mainstay of his practice. Over the past two decades, he and his Johns Hopkins colleagues, including Luca Vricella, director of pediatric cardiac surgery, have performed more than 300 of these surgeries, with excellent long-term results.

Retaining the natural valve is a particularly attractive option for young patients, who typically have many years to live with the consequences of aortic repair, Cameron explains. “Keeping a patient’s own valves, with the hope that they’ll last a lifetime, can avoid the problems of artificial valves,” he says.

But youth adds an extra layer of challenge to this procedure, he notes. Because valve-sparing surgery was originally developed for adults, Cameron and his colleagues have worked to adapt the procedure for young patients, developing a set of guidelines to simplify the procedure and make it reliably reproducible in this population.

After ensuring that a patient’s valve is salvageable, the refined procedure involves assessing the size of the necessary graft that brings the natural valve’s leaflets into an optimal closed position. The procedure is further simplified from the original one, developed by Tirone David at the University of Toronto, by securing the graft with two or three sutures at the bottom of the root.

Cameron and his colleagues have further modified the procedure for patients with Loeys-Dietz syndrome, a condition first described at Johns Hopkins only a decade ago. As they gather more information about this condition, it has become apparent that blood vessels in these patients are significantly weaker than those of patients with Marfan syndrome and other connective tissue disorders. As a result, these patients tend to develop pseudoaneurysms at the suture points. Consequently, the surgeons have crafted a technique to reinforce the weakest points to help prevent this complication.

Cameron and his Johns Hopkins colleagues have trained surgeons in these valve-sparing techniques at meetings around the world and in Johns Hopkins’ own surgical suites, furthering their mission to help as many patients as possible through educating physicians.

“If you do 100 operations with great results, then you help 100 patients,” says Cameron. “But if you train 100 residents to do operations with great results, it’s a much broader and lasting impact. Being able to ease the burdens of the greatest number of patients, both here and elsewhere, is our ultimate goal.”