The aorta and aortic valve pose particular challenges for cardiovascular care. Subtle changes can eventually lead to significant problems, including ventricular scarring, heart failure, internal bleeding, stroke and organ damage. The presentations of these disease states are heterogeneous and conditions often overlap — it's not uncommon to find simultaneous aortopathy and valvulopathy.

Aortic disease also affects a wide population that includes both the older and frequently frailer patients typical of heart care and younger patients (under 65 or so) affected by congenital and genetic components. Among both groups, anatomy varies, including size and location of critical branch vessels. And aortic disease is longitudinal — even with treatment, patients may need further intervention in their lives, sometimes just years later.

Specialists in the renowned aorta program at Penn Medicine have spent decades working on these challenges, developing the comprehensive model others follow. But they have come to realize that more can — and should — be done. Their effort has evolved into the Penn Aorta Center, bringing together the range of disciplines crucial to effective care: top cardio-aortic surgeons and vascular surgeons as well as experts in medical cardiology, imaging, genetics and nursing.

"Our mission with the Penn Aorta Center is to have a more focused, 360-degree view around each patient," says Joseph Bavaria, MD, the cardiac surgeon who founded Penn's Thoracic Aortic Disease Program in 1996 and serves as the center's co-director. "We make decisions on medical management, we make decisions on endovascular treatment and we make decisions on open surgical treatment. It's about the total management of the aortic valve and the aorta."

The center will operate from a specially designed space within the new 1.5 million square foot Penn Medicine Pavilion — operating suites will contain the most advanced imaging and true hybrid functionality for open surgeries, endovascular approaches and combinations — as well as at Penn Presbyterian Medical Center.

The program's expansion provides referring physicians an unparalleled resource and opportunity for partnership. In the works are new clinical programs, extended patient support and enhanced research efforts, as well as a larger goal — truly transforming the way care for the aorta and aortic valve is conceived and delivered.

New Vision for Aortic Care from a National Leader

Already running the busiest aorta program in Greater Philadelphia (by caseload) and one of North America's four largest, Penn sees a deep variety of aortic disease, including complex presentations. High volume means experience in the full range of worthwhile interventions, with the ability to safely step in sooner when warranted.

Members of the Penn aorta team publish globally influential research (more than 550 articles to date), with contributions that have helped change guidelines for earlier intervention in aortic insufficiency and Marfan syndrome. They also present at conferences worldwide and hold leadership positions with the Society of Thoracic Surgeons.

In addition to expertise, clinical excellence and leading outcomes, Penn is known for innovation. The team continues to perform aortic root operations which can repair and preserve a patient's own heart valve, reduce Type A dissection mortality through a trauma-oriented approach and employ branched endovascular stent-grafts (customized when needed) to make procedures less invasive.

With the center, the Penn team will further enhance its approach to comprehensive aorta care. It will continue to:

• Make procedures increasingly safer, more effective and longer-lasting
• Ensure patients get the appropriate procedure, with a deep understanding of timing (not too early because of surgical risk, and not too late because of irreversible harm and reduced options)
• Take a nuanced view of when to intervene, moving beyond just aortic diameter to consider age, body size, valve configuration, family history and any underlying genetic predisposition or connective tissue disorder
• Use advanced imaging and interpreting expertise, not just for confirming diagnosis but to also carefully watch the valve and aorta before surgery and after
• Offer access to novel stents, stent-grafts and additional devices others don't, through standard clinical trials we lead, special FDA exemption and unique early-feasibility industry partnerships
• Conduct leading genetics research and provide full testing and counseling support (the next step: directly embedding geneticists and genetic counselors in each clinic)
• Consider lifetime needs and quality of life, rather than just the challenge of the moment

Penn already offers surveillance for nearly 2,000 patients a year — patients who had an initial intervention for a bicuspid aortic valve, aortic dissection or aortic aneurysm or may eventually need one. The transition to the Penn Aorta Center, and the resources it entails, means that support becomes a clinical reality for every patient. It's an unprecedented, holistic approach that looks beyond just the original disease.

"We are trying to take one of the world's best models of aortic care and make it more sophisticated and comprehensive than we have ever been able to in the past," says cardiac surgeon Nimesh Desai, MD, the center's other co-director. "We are looking not just at the current problem that the patient has, but actually trying to understand all the elements that we are going to need to support that patient and their family through their lifetime of aortic disease."

Supplementing medical and surgical care, patients will have access to psychological support under the center — crucial for conditions like aortic dissection that can still pose a risk after initial treatment. The team will also work with neurologists to monitor the impact of interventions on the brain, extending the focus beyond operative and postoperative stroke to consider a range of potential long-term changes and how to prevent them.

Meeting a Growing and Significant Need for Aortic Disease Care

Estimates place the number of enlarged aortas in the U.S. in the hundreds of thousands, a figure likely to increase as improved imaging leads to more incidental findings (the most common way aortic disease gets discovered) and a large segment of the population transitions into old age.

Not all these aortas will need an intervention, but they may still benefit from evaluation and possible surveillance at a multidisciplinary center. Experts can watch for possible progression and, should that occur, work to prevent future rupture or dissection. (Ruptures and dissections affect 30,000 Americans a year)

Enlarged aortas may also warrant aortic valve examination, given the interconnected nature of the two structures and the frequency of valvulopathy: Up to 30% of the population has some degree of aortic insufficiency and up to 2% a bicuspid aortic valve. Left ventricular decline can result from these valve conditions, as well as aortic remodeling.

In addition to lifetime disease management, the Penn Aorta Center will meet these needs by continuing to improve interventions. Penn's cardiac and vascular surgeons are:

• Using investigational stent-grafts, often branched, to extend endovascular treatment into the ascending aorta, arch and thoracoabdominal segment
• Accounting for the fact that downstream complications often follow Type A dissection repair, by preparing for later stent-grafting or potentially avoiding reintervention altogether
• Extending valve-sparing procedures into more complicated anatomy, with the ability to repair both tricuspid and bicuspid aortic valves
• Leading the reinvigoration of the Ross procedure, when they cannot save the aortic valve

"That's what makes Penn such a special place," says cardiac surgeon Wilson Szeto, MD, who will oversee the center's efforts at Penn Presbyterian Medical Center. "We're continuing to challenge ourselves to be better. We're continuing to push the boundaries to improve the care of our patients, while asking the question of how do we embrace technology and innovation in doing so."

Conducting Groundbreaking Research to Inform Aortic Care

A final key element for the Penn Aorta Center is research, both in immediate clinical application (outcomes and more than 15 clinical trials) and translational work. As part of the program's transformation, we're hiring additional researchers to partner with physician-investigators.

Many questions remain about how aortic disease forms and progresses, unknowns the team plans to address. The center will feature a new aortic tissue bank, to determine how aortic-related genetic changes manifest and how care might need further adjustment.

The Penn aorta team also has years of data from interventions and surveillance, a rich resource for addressing shortcomings in national and international treatment guidelines. Given the limitations inherent in the field to date, those guidelines often lack strong backing data, Dr. Bavaria says.

The analyses will potentially provide the field with a greater understanding of risk and a more nuanced stratification, to determine at what point to step in for each patient, and with what care. "We are just at the tip of the iceberg in terms of what we can do," Dr. Desai says.

Refer a Patient to the Penn Aorta Center

To make a referral to the Penn Aorta Center, call 877-937-7366.

Whether you're in Greater Philadelphia or elsewhere in the country, the Penn Aorta Center welcomes inquiries. The team is equipped to handle complex needs, including reinterventions after previous surgeries. Other reasons to inquire about possible referral include:

• Aortic insufficiency
• Bicuspid aortic valve or related repair/replacement
• Diagnosed connective tissue disorder
• Dissected aorta
• Enlarged thoracic aorta (4 centimeters or more)
• Family history of thoracic aortic aneurysm or dissection or known, related mutation
• Repaired congenital heart defect