A Practical Guide to Clinical Management of Thoracic Aortic Disease

Physicians and surgeons faced with patients with thoracic aortic disease (TAD) need to determine the underlying diagnosis to facilitate decisions regarding appropriate investigations as well as which other specialists to involve, when to start medical therapy, when to refer for surgery, and how to plan follow-up and family screening. Increased understanding of conditions predisposing to thoracic aortic aneurysm (TAA) provides the opportunity for more personalized care. However, given advances in the genetics of TAD, clinicians are now faced with an expanded and often confusing list of associated differential diagnoses. We present a practical guide to managing patients with TAD based on current knowledge and guidelines. Important "flags" on history taking and "tips" to diagnosis on physical examination along with what investigations to order and what referrals to request are discussed. Need for medical therapy, indications for surgical repair, and planning long-term follow-up of TAD are determined by age, the underlying TAD diagnosis, previous vascular history in the patient or other family members (or both), aortic dimensions and growth rate, and any coexisting cardiovascular disease. Although medications may slow the progression of TAA, effective aortic surveillance and timely elective surgical repair remain the mainstays of prevention of acute aortic complications. Emergent repair of acute aortic dissection carries a far worse prognosis. Taking a practical approach to the management of TAD allows for standardized assessment and implementation of current best practice clinical guidelines. Ongoing discovery of new genes, better medical therapies, and innovative surgical techniques necessitate constantly adapting knowledge and integrating it into everyday clinical practice.