Aim: The “2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease” provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes). Methods: A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate. Structure: Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.

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Background: Immediate open repair of acute type A aortic dissection (ATAAD) is traditionally recommended to prevent death from aortic rupture. However, organ failure due to malperfusion syndrome (MPS) might be the most imminent life-threatening problem for a subset of patients. Methods: From 1996–2017, among 597 ATAAD patients, 135 patients with MPS were treated with upfront endovascular reperfusion (fenestration/stenting) followed by delayed open repair (OR). We compared outcomes between the first and second decade, as well as observed mortalities with those expected with an “upfront OR for every patient” approach, determined using prognostic models from the literature (Verona, Leipzig-Halifax, Stockholm, Penn, and GERAADA models). Results: Overall, in-hospital mortality improved between the two decades (21.0% vs. 10.7%, p<0.001). In the second decade, for MPS patients initially treated with fenestration/stenting, mortality from aortic rupture decreased from 16% to 4% (p=0.05), the risk of dying from organ failure was 6.6 times higher than dying from aortic rupture (hazard ratio = 6.63, 95%CI 1.5–29, p=0.01), and 30-day mortality after OR for MPS patients was 3.7%. Compared to the expected mortalities with the “upfront OR for every patient” models, our observed 30-day and in-hospital mortalities (9% and 11%, respectively) of all ATAAD patients were significantly lower (p≤0.03). Conclusions: Immediate open repair is the strategy to prevent death from aortic rupture for the majority of ATAAD patients. However, relatively stable (no rupture, no tamponade) patients with MPS benefit from a staged approach: upfront endovascular reperfusion followed by open aortic repair at resolution of organ failure. Related to an abstract (“Malperfusion Syndrome Management in Acute Type A Aortic Dissection: Two-Decade Experience”) presented at the AHA Scientific Sessions 2017 (Anaheim, CA, Nov 2017).

Objective: To assess outcomes of endovascular reperfusion followed by delayed open aortic repair for stable patients with acute type A aortic dissection and mesenteric malperfusion syndrome (mesMPS).Methods: Among 602 patients with acute type A aortic dissection who presented to our center from 1996 to 2017, all 82 (14%) with mesMPS underwent upfront endovascular fenestration/stenting. Primary outcomes were in-hospital mortality and long-term survival. Patients with acute type A aortic dissection with no malperfusion syndrome of any organ (n ¼ 419) served as controls.Results: In-hospital mortality of all comers with mesMPS was 39%. After endovascular fenestration/stenting, 20 mesMPS patients (24%) died from organ failure and 11 patients (13%) died from aortic rupture before open aortic repair, 47 patients (58%) underwent aortic repair, and 4 patients (5%) survived without open repair. No patients died from aortic rupture during the second decade (2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016)(2017). The significant risk factors for death from organ failure after endovascular reperfusion were acute stroke (odds ratio, 23; 95% confidence interval, 4-144; P ¼ .0008), gross bowel necrosis at laparotomy (odds ratio, 7; 95% confidence interval, 1.4-34; P ¼ .016), and serum lactate 6 mmol/L (odds ratio, 13.5; 95% confidence interval, 2-97; P ¼ .0097). There was no significant difference in operative mortality (2.1% vs 7.5%; P ¼ .50) or long-term survival between patients with mesMPS who underwent open aortic repair after recovering from mesMPS and patients with no malperfusion syndrome.