New, minimally invasive surgical procedures can help even people with severe congenital heart valve disease live a normal life.

In 2016, aortic surgeon Dr. Joseph Coselli and colleagues at Baylor College of Medicine and The Texas Heart Institute reported in The Texas Heart Institute Journal what was at the time a unique case. A 21-year-old woman who had already undergone several operations to address her complex congenital heart disease required replacement of her aortic valve—for the fourth time.

Her aortic root, the portion of the aorta that connects to the heart and that contains the aortic valve, had previously been replaced with an aortic root donated from a cadaver (a homograft root, with the donor’s aortic valve inside). Now, the homograft was failing, causing the valve to lose its shape and therefore not to open and close properly. This resulted in blood flowing both forward and backward through what should be a one-way valve, a condition called valve regurgitation.

Because repeated opening of the chest can cause complications, Dr. Coselli’s team decided to perform a transcatheter aortic valve replacement (TAVR), a technique originally developed for use in elderly patients with aortic stenosis (not regurgitation) for whom open surgery was inadvisable. Moreover, the surgeons inserted the new valve—a bioprosthetic made from preserved bovine myocardial tissue—inside the previous replacement valve. Thus, the operation was described as a “valve-in-valve” TAVR.

Six years later, at age 27, the patient returned for another aortic valve procedure. Her pre-existing aortic root homograft, which contained the previously placed bioprosthetic valve, had suddenly deteriorated during her pregnancy with her first child the previous year, deforming the valve and causing her aortic regurgitation to return. Now, she needed yet another replacement valve.

“Under other circumstances,” says Dr. Coselli, “we would have given the patient a mechanical prosthetic valve as part of an extensive procedure to replace the donated aortic root, because mechanical valves last much longer than bioprosthetic valves. But because mechanical valves pose a risk of blood clots, the patients have to take blood thinners for the rest of their lives. That would make childbirth dangerous, and this patient planned to have more children in the next few years. So after we discussed the options with her, the patient decided to receive another bioprosthetic valve on a temporary basis.”

In the latest issue of The Texas Heart Institute Journal, Dr. Coselli and colleagues describe this “valve-in-valve-in-valve” TAVR procedure. First, they inserted a catheter through the patient’s right carotid artery. This in itself was unusual; the TAVR catheter is most often inserted through the femoral artery in the leg and up through the abdominal and thoracic aorta. However, this patient had been born without the lower part of her abdominal aorta, making that route unusable.

The new bioprosthetic valve was then deployed inside the two previous valves. An echocardiogram done after the valve was placed showed that there was no regurgitation with the new valve, and the patient was discharged from the hospital two days later.

At the patient’s follow-up visit 14 months later, her new valve was still functioning well. Once she finishes having children, the patient plans to undergo a full aortic root replacement with a mechanical valve inside a prosthetic aortic root made of Dacron, which should provide a long-term solution to her aortic valve issues.

“Despite her severe congenital heart disease,” Dr. Coselli says, “this patient isn’t just surviving—she’s thriving. She has a career as a nurse, and she’s raising a family. This case is an example of how modern surgical technology and techniques can not just save lives but improve lives in people with severe valve disease.”

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Read Report

Hong JC, Orozco-Sevilla V, Diez JG, Coselli JS. Transcatheter aortic valve-in-valve-in-valve replacement in a young woman with transcatheter structural valve deterioration within a degenerated aortic root homograft. Tex Heart Inst J. 2023; 50 (2): e227874. https://doi.org/10.14503/THIJ-22-7874

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News Story By Stephen N. Palmer, PhD, ELS

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