Position Statement
Canadian Cardiovascular Society Position Statement on the Management of Thoracic Aortic Disease

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Abstract

This Canadian Cardiovascular Society position statement aims to provide succinct perspectives on key issues in the management of thoracic aortic disease (TAD). This document is not a comprehensive overview of TAD and important elements of the epidemiology, presentation, diagnosis, and management of acute aortic syndromes are deliberately not discussed; readers are referred to the 2010 guidelines published by the American Heart Association, American College of Cardiology, American Association for Thoracic Surgery, and other stakeholders. Rather, this document is a practical guide for clinicians managing adult patients with TAD. Topics covered include size thresholds for surgical intervention, emerging therapies, imaging modalities, medical and lifestyle management, and genetics of TAD. The primary panel consisted of experts from a variety of disciplines that are essential for comprehensive management of TAD patients. The methodology involved a focused literature review with an emphasis on updates since 2010 and the use of Grading of Recommendations Assessment, Development, and Evaluation methodology to arrive at specific recommendations. The final document then underwent review by a secondary panel. This document aims to provide recommendations for most patients and situations. However, the ultimate judgement regarding the management of any individual patients should be made by their health care team.

Résumé

Cet énoncé de position de la Société canadienne de cardiologie a pour but de donner des perspectives succinctes sur les aspects clés de la prise en charge de la maladie de l’aorte thoracique (MAT). Ce document n’est pas un aperçu complet de la MAT, et les éléments importants de l’épidémiologie, du tableau clinique, du diagnostic et de la prise en charge des syndromes aortiques aigus ne sont délibérément pas discutés; les lecteurs sont invités à consulter les lignes directrices de 2010 publiées par l’American Heart Association, l’American College of Cardiology, l’American Association for Thoracic Surgery et les autres parties prenantes. Ce document est plutôt un guide pratique pour les cliniciens prenant en charge des patients adultes atteints d’une MAT. Les thèmes couverts incluent les seuils de l’ampleur de l’intervention chirurgicale, les nouveaux traitements, les modalités d’imagerie, la prise en charge médicale et du mode de vie, et la génétique de la MAT. Le premier panel comprenait des experts de diverses disciplines qui sont essentielles à la prise en charge complète des patients atteints d’une MAT. La méthodologie exigeait une revue de littérature ciblée s’appuyant sur les mises à jour depuis 2010 et l’utilisation de la méthodologie GRACE (Grading of Recommendations Assessment, Development, and Evaluation) pour parvenir à des recommandations précises. Le document final était ensuite passé en revue par un second panel. Ce document a pour but de fournir des recommandations pour la plupart des patients et la plupart des situations. Cependant, la décision définitive concernant la prise en charge de chaque patient devrait être prise par l’équipe soignante.

Section snippets

Size thresholds for elective thoracic aortic intervention

Thoracic aortic aneurysms are largely asymptomatic until a sudden and catastrophic event, including aortic rupture or dissection, occurs, and is rapidly fatal in a large proportion of patients.1, 2 Elective intervention on the thoracic aorta carries a much lower risk of mortality and morbidity, and prophylactic aortic surgery can be life-saving.

The decision to perform aortic intervention is a balance between risks of natural history of the disease vs the risk of the surgical intervention

Antihypertensive therapy

The rationale for antihypertensive therapy is based on mechanistic and animal studies,55, 56 and observational reports linking aortic dissection with hypertension.57, 58 Randomized controlled trials of antihypertensive therapies have not included patients with TAD per se or reported consistently on aortic end points.59, 60 Although summaries of antihypertensive trials support treatment of hypertension when present, they do not offer specific guidance on the management of patients with thoracic

Screening for Family Members of Patients With Genetic Aortopathy

Key features of common genetic aortopathies are described in Table 4.

Knowledge Gaps

Most of the epidemiology and natural history of TAD is based on: (1) surgical series from selected populations; (2) retrospective cohorts of acute aortic syndromes; (3) single-centre studies of patients with inherited or degenerative forms of TAD; and (4) extrapolation from non-TAD patients, leaving important knowledge gaps in this patient population.

Future research should be focused on these key knowledge gaps in the pathophysiology, natural history, and treatment of patients with TAD,

Multidisciplinary Care and Quality Indicators

Comprehensive management of TAD spans multiple disciplines including but not limited to cardiac surgery, vascular surgery, cardiology, genetics, imaging, and adult congenital heart disease. Therefore, care for these patients is best provided in such a multidisciplinary environment and clinics are currently emerging across major cardiac centres in Canada. These might also be an important source of critical natural history data on thoracic aortic pathologies, and facilitate prospective clinical

Acknowledgements

The authors thank the members of the secondary panel for their thorough review of the manuscript draft and insightful comments that helped shape the final document.

Secondary panel: Hal Dietz, MD (McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University, Baltimore, MD), Francois Dagenais, MD (Division of Cardiac Surgery, Laval University, Québec City, Québec, Canada), Christopher Fiendel, MD (Division of Cardiac Surgery, University Health Network, Toronto, Ontario, Canada),

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    The disclosure information of the authors and reviewers is available from the CCS on the following websites: www.ccs.ca and/or www.ccsguidelineprograms.ca.

    This statement was developed following a thorough consideration of medical literature and the best available evidence and clinical experience. It represents the consensus of a Canadian panel comprised of multidisciplinary experts on this topic with a mandate to formulate disease-specific recommendations. These recommendations are aimed to provide a reasonable and practical approach to care for specialists and allied health professionals obliged with the duty of bestowing optimal care to patients and families, and can be subject to change as scientific knowledge and technology advance and as practice patterns evolve. The statement is not intended to be a substitute for physicians using their individual judgement in managing clinical care in consultation with the patient, with appropriate regard to all the individual circumstances of the patient, diagnostic and treatment options available and available resources. Adherence to these recommendations will not necessarily produce successful outcomes in every case.

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