Histology of a Marfan aorta 4.5 years after personalized external aortic root support

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.

Mechano-biological adaptation of the pulmonary artery exposed to systemic conditions

“…These results are in line with our previous studies [27][28][29][30] . In contrast, a patient study examining the capacity of the macroporous material to withstand further dilatation of a weakened vessel wall does not mention atrophy of the SMC 31 . However, the latter was a study of the aortic root of a single Marfan patient, which has a low number of SMC to start with.…”

Effect of personalized external aortic root support on aortic root motion and distension in Marfan syndrome patients

“…Histology of the artery after implantation of the mesh used in PEARS surgery was investigated in a sheep model [10] and also in the autopsy study of one of the patients who had sudden cardiac death (the death was not related to aortic pathology as the exostent and the aorta were found intact in the autopsy without any evidence of aortic dissection, but there were findings of dilated cardiomyopathy) [18]. Histologic evidence from both of these studies showed that the mesh is fully incorporated in the adventitia with collagen fibers filling the interstices of the macroporous mesh.…”

Personalised External Aortic Root Support (PEARS) Compared with Alternatives for People with Life-Threatening Genetically Determined Aneurysms of the Aortic Root

“…After an average of five months it was similarly incorporated with the connective tissues growing through the interstices of the mesh [ 41 ]. Third, one of the PEARS patients died in his bed at home 4.5 years after PEARS surgery [ 42 ]. There was no aortic dissection and the aortic valve was only minimally regurgitant as it had been throughout.…”