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

Treasure, Tom; Pepper, John

doi:10.3390/diseases3010002

Cited by 12 publications

(6 citation statements)

References 46 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Upon development, his surgeon successfully installed the aortic support, and the clinical advantage was clear. To date, the novel treatment has been successfully applied to more than 40 patients in the period from 2004 to 2014 [25]. …”

Section: Discussionmentioning

confidence: 99%

Innovation by patients with rare diseases and chronic needs

Oliveira

Zejnilović

Canhão

et al. 2015

Orphanet J Rare Dis

View full text Add to dashboard Cite

We provide the first empirical exploration of disease-related innovation by patients and their caregivers. Our aims were to explore to what degree do patients develop innovative solutions; how many of these are unique developments; and do these solutions have positive perceived impact on the patients’ overall quality of life? In addition, we explored the factors associated with patient innovation development, and sharing of the solutions that the patients developed.MethodsWe administered a questionnaire via telephone interviewing to a sample of 500 rare disease patients and caregivers. The solutions reported were pre-screened by the authors for their fit with the self-developed innovation aim of the study. All the reported solutions were then validated for their novelty by two medical professionals. Logistic regression models were used to test the relationships between our key variables, patient innovation and solution sharing.Results263 (53%) of our survey respondents reported developing and using a solution to improve management of their diseases. An initial screening removed 81 (16%) solutions for being an obvious misfit to the self-developed innovation aim of the study. This lowered the sample of potentially innovative solutions to 182 (36%). Assessment of novelty and usefulness of the solutions, conducted by two medical evaluators, confirmed that 40 solutions (8%) were indeed novel, while the remaining 142 (28%) were already known to medicine. The likelihood of patient innovation increased as the education level increased (OR 2, p < 0.05), and as their perception of limitations imposed by their disease increased (OR 1.3, p < 0.05). 55 individuals diffused their solutions to some degree, with 50 of these sharing via direct diffusion to other patients. There is a positive relationship between the impact of a solution on the respondents’ overall quality of life and likelihood of solution sharing.ConclusionsGiven that hundreds of millions of people worldwide are afflicted by rare diseases, patient and their caregivers can be a tremendous source of innovation for many who are similarly afflicted. Our findings suggest that many patients could be greatly assisted by improved diffusion of known solutions and best practices to and among patients and their caregivers.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-015-0257-2) contains supplementary material, which is available to authorized users.

show abstract

Section: Discussionmentioning

confidence: 99%

Innovation by patients with rare diseases and chronic needs

Oliveira

Zejnilović

Canhão

et al. 2015

Orphanet J Rare Dis

View full text Add to dashboard Cite

show abstract

“…The possibility of a pre-emptive operation might be considered for other congenitally determined root aneurysms, some of which are included in our experience [3]. The aorta may not yet be at a size at which root replacement is mandatory by established criteria, but may nevertheless be a cause for serious future concern [4]. PEARS may be considered for these patients because it spares the aortic valve, conserves the architecture of the aortic valve support, but presents no obstacle to further surgery.…”

Section: Introductionmentioning

confidence: 99%

Personalized external aortic root support: a review of the current status

Treasure

Petrou

Rosendahl

et al. 2016

Eur J Cardiothorac Surg

Self Cite

View full text Add to dashboard Cite

Personalized external aortic root support (PEARS) is an emerging technology. It is a pre-emptive operation to halt aortic root expansion and maintain aortic valve function in Marfan syndrome and is also applicable to aortic root aneurysms of other aetiologies. To fully evaluate PEARS, awareness of all those who advise these patients is necessary to ensure that patients are fully informed of the alternative operations, to carefully build experience, to ensure safety and quality and to monitor outcomes. Herein, we present a summary of published methods and outcomes and the arrangements in place for fuller evaluation.

show abstract

“…Tal Golesworthy, a process engineer, diagnosed with Marfan syndrome in 1992, is commonly referred to as a man who fixed his heart, for developing an external aortic root support to prevent its further dilatation (Treasure and Pepper 2015). Marfan syndrome is a rare inherited disorder that affects the aorta.…”

mentioning

confidence: 99%

“…In 2011, the External Aortic Root Support Project was the winner in the Medical & Healthcare category of the Engineer's Technology and Innovation Awards; in 2015, Golesworthy won one of the Patient Innovation Awards from our project. By the end of 2015, 56 patients had received the aortic support ExoVasc (Treasure and Pepper 2015).…”

mentioning

confidence: 99%