Editor's Choice - A European Delphi Consensus on the Management of Abdominal Aortic Aneurysms in Patients with Heritable Aortic Diseases

Abstract

Objective: Abdominal aortic aneurysm (AAA) in a patient with an underlying heritable aortic disease (HAD) is rare, and evidence based recommendations for its management are lacking. This study aimed to generate a consensus from multidisciplinary specialists on the diagnosis, treatment, and surveillance of AAA associated with HAD and to define topics of interest for future research.

Methods: A Delphi consensus was designed involving European multidisciplinary specialists and reported using the ACcurate COnsensus Reporting Document (ACCORD) reporting guideline. Four rounds were carried out by email: the first two rounds to agree on the content of the questionnaire and the final two rounds to reach an agreement. Questions focused on the composition of the team, imaging, clinical suspicion, genetic testing, medical management, indications for surgical repair, surgical technique, surveillance, exercise, and quality of life. The level of agreement for each question was graded using a Likert type scale and classified into four categories: A (very strong), B (strong), C (fair), and D (no agreement).

Results: A total of 35 experts from 18 different hospitals and 10 different countries participated in the study: 19 vascular surgeons, 11 cardiologists, four geneticists, and one cardiac surgeon. Of the 97 statements of the final questionnaire, 16 were graded A (16%), 23 B (24%), five C (5%), 44 D (45%) and nine (9%) were invalid. The experts highlighted the need to develop personalised therapies for each pathology, as well as international multicentre databases to collect data on the natural course of AAA patients with HAD.

Conclusion: This Delphi process led to consensus on several aspects of care for patients with AAA and underlying HAD, providing guidance to manage them in a more standardised fashion. The agreements reached focus on multidisciplinary aortic teams, imaging and genetic diagnosis, treatment, and follow up. Furthermore, several topics for future research were identified.

Keywords: Abdominal aortic aneurysm; Connective tissue diseases; Delphi technique; Ehlers–Danlos syndrome; Loeys–Dietz syndrome; Marfan syndrome.