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Published on: May 21, 2017
Histology of a Marfan aorta 4.5 years after personalized external aortic root support
John Pepper1, Martin Goddard2, Raad Mohiaddin3
1National Institute of Health Research (NIHR) Cardiovascular Biomedical Research Unit (BRU), Royal Brompton Hospital, London, UK.
Abstract:
In 2008, a 26-year old man had personalized external aortic root support (PEARS) with a macroporous mesh. He was the 16th of 46 patients to have this operation. He had a typical Marfan habitus. His mother died of this disease as did his brother, with an aortic dissection. The patient himself died suddenly 4.5 years after his PEARS operation. At autopsy, there was no blood in the pericardium. The coronary orifices and proximal arteries were normal. His bicuspid aortic valve was minimally regurgitant as it was prior to operation and remained throughout follow-up. Macroscopically the implanted mesh was embedded in the adventitia and not separable from the aortic wall. Microscopically it was fully incorporated with collagen fibres as has been seen in our animal studies. The unsupported aortic arch showed some focal fragmentation of elastic fibres and a mild increase in mucopolysaccharides consistent with Marfan syndrome. These appearances were not present in the supported aortic root, which had the histological appearance of a normal aorta. He was the first patient to die with an implant. The histological appearances suggest the possibility that the incorporated support of the aortic root allowed recovery of the microstructure of the media.
Insights
Personalized external aortic root support (PEARS) in a Marfan syndrome patient showed excellent histological integration. The aortic root histology suggested potential microstructural recovery post-PEARS.
Area of Science:
- Cardiovascular Surgery
- Medical Engineering
- Histopathology
Background:
- Marfan syndrome is a genetic disorder affecting connective tissue, predisposing individuals to aortic root dilation and dissection.
- Personalized External Aortic Root Support (PEARS) is a surgical technique using a macroporous mesh to reinforce the aortic root.
- This case involves the 16th patient to undergo PEARS, a 26-year-old male with a family history of Marfan syndrome and aortic dissection.
Observation:
- The patient died suddenly 4.5 years after PEARS implantation.
- Autopsy revealed no pericardial effusion or abnormalities in coronary arteries.
- The implanted mesh was macroscopically integrated into the aortic adventitia and microscopically incorporated with collagen fibers.
Findings:
- The unsupported aortic arch exhibited histological features consistent with Marfan syndrome, including elastic fiber fragmentation.
- The PEARS-supported aortic root showed normal aortic histology, without signs of Marfan syndrome.
- This is the first reported death of a patient with a PEARS implant, providing a unique opportunity for histological analysis.
Implications:
- Histological findings suggest that PEARS may facilitate the recovery of the aortic media's microstructure.
- The study provides crucial long-term histological data on the integration and effect of PEARS in a Marfan syndrome patient.
- Further research into the long-term microstructural effects of PEARS on the aorta is warranted.
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