Synchronous Carotid Endarterectomy and Anaortic Off-Pump Coronary Artery Bypass Surgery.

BACKGROUND: There is ongoing debate regarding the optimal strategy and timing for the surgical management of patients with severe concomitant carotid and coronary artery disease. Anaortic off-pump coronary artery bypass (anOPCAB), which avoids aortic manipulation and cardiopulmonary bypass, has been shown to reduce the risk of perioperative stroke. We present the outcomes of a series of synchronous carotid endarterectomy (CEA) and anOPCAB. METHODS: A retrospective review was performed. The primary endpoint was stroke at 30 days post-operation. Secondary endpoints included transient ischaemic attack, myocardial infarction and mortality 30 days post-operation. RESULTS: From 2009 to 2016, 1,041 patients underwent anOPCAB with a 30-day stroke rate of 0.4%. The majority of patients had preoperative carotid-subclavian duplex ultrasound screening and 39 were identified with significant concomitant carotid disease who underwent synchronous CEA-anOPCAB. The mean age was 71±7.5 years. Nine patients (23.1%) had previous neurological events. Thirty (30) patients (76.9%) underwent an urgent operation. For CEA, a conventional longitudinal carotid endarterectomy with patch angioplasty was performed in all patients. For anOPCAB, total arterial revascularisation rate was performed in 84.6% and the mean number of distal anastomoses was 2.9±0.7. In the 30-day postoperative period, there was one stroke (2.63%), two deaths (5.26%), two transient ischaemic attacks (TIAs) (5.26%) and no myocardial infarction. Two patients experienced acute kidney injury (5.26%), one of which required haemodialysis (2.63%). Mean length of stay was 11.37±7.9 days. CONCLUSION: Synchronous CEA and anOPCAB is a safe and effective option for patients' severe concomitant disease. Preoperative carotid-subclavian ultrasound screening allows identification of these patients.

Novel Pressure-Regulated Deployment Strategy for Improving the Safety and Efficacy of Balloon-Expandable Transcatheter Aortic Valves.

OBJECTIVES: The authors propose a novel pressure-regulated method for balloon-expandable transcatheter heart valve (THV) deployment, aimed at optimizing prosthesis-annulus apposition while preventing significant tissue injury. BACKGROUND: The optimal method for balloon-expandable THV deployment remains debated. Current protocols are volume dependent, relying on under- and overfilling of the deployment apparatus. During deployment, the annular wall tension exerted by the expanding prosthesis is determined by maximal THV diameter and balloon pressure (Laplace's law). METHODS: Three hundred thirty consecutive patients with severe native aortic stenosis who underwent TAVR with SAPIEN 3 THVs were included. One hundred and six patients were considered at high risk for annular rupture. THVs were deployed until reaching a predetermined balloon pressure. Postdilatation was performed to reduce mild or greater angiographic paravalvular regurgitation (PVR). Using a biomechanical model, annular wall stress was estimated for each case and assessed against rates of postdilatation, mild or greater PVR on transthoracic echocardiography, new permanent pacemaker placement or left bundle branch block, and annular rupture. RESULTS: Patients with wall stress >3 MPa had reduced postdilatation rate (P < 0.001) and reduced final PVR (P = 0.014). Annular rupture occurred in 2 of 3 high-risk patients with wall stress >3.5 MPa (3.69 and 3.84 MPa); no rupture occurred in 95 high-risk patients with wall stress ≤3.5 MPa. We defined a single target deployment pressure per THV size to ensure deployment within target wall stress levels of 3 to 3.5 MPa: 6.25 atm for 23-mm THVs, 5.5 atm for 26-mm THVs, and 5 atm for 29-mm THVs. Patients within this target range (n = 136) had a 10.0% postdilatation rate, 12.7% mild PVR, and no moderate to severe PVR. The relationship between balloon filling volume and associated pressure and wall stress was inconsistent. CONCLUSIONS: Pressure-regulated THV deployment is a simple, reproducible, safe, and effective method, regardless of high-risk anatomical complexities.

Transcatheter Aortic Valve Implantation (TAVI) Versus Surgical Aortic Valve Replacement for Aortic Stenosis (SAVR): A Cost-Comparison Study.

BACKGROUND: Comparative costing studies using real-world data stratified by patient case-mix, are valuable to decision makers for making reimbursement decisions of new interventions. This study evaluated real-world hospital admissions and short-term costs of transcatheter aortic valve implantation (TAVI) and isolated surgical aortic valve replacement (SAVR) for patients with aortic stenosis, stratified by the Society of Thoracic Surgeons (STS) risk scores. METHODS: Retrospective analysis of consecutive patients with a principal diagnosis of aortic stenosis who underwent isolated valve replacement at a single tertiary hospital, January 2012-December 2017. Patients were followed-up for 30 days post-procedure or until hospital discharge if index hospitalisation was greater than 30 days. Intensive care unit (ICU) and hospital length of stay (days), and costs in 2018 Australian dollars for the index procedure and 30-day follow-up were assessed. Multivariable generalised linear and two-part models with gamma distribution and log link function adjusting for Society of Thoracic Surgeons (STS) risk group and key sociodemographic characteristics were used. RESULTS: Of 488 patients, 61% males, median age 78 years (IQR 14 years), 221 (45%) received transcatheter aortic valve replacement (TAVI) and 267 (55%) received surgical aortic valve replacement (SAVR). STS risk scores were low (28%), intermediate (46%) and high (26%) for TAVI patients, and low (85%), intermediate (12%) and high (3%) for SAVR patients. When adjusted, TAVI length of stay was 57% shorter than SAVR (95% CI 31-83%, p<0.001) for intensive care unit (ICU) admission, and 64% shorter (95% CI 47-81%, p<0.001) for hospital admissions. TAVI costs were 13% lower than SAVR (95% CI 4-22%, p=0.005). CONCLUSION: This data suggests short-term health care costs are lower for patients with aortic stenosis undergoing TAVI than SAVR. A further roll-out of the TAVI program in hospitals across Australia may result in savings to the health system.

Five-Year Survival of Transcatheter Aortic Valve Implantation in High-Risk Patients.

BACKGROUND: Although transcatheter aortic valve implantation (TAVI) has become the standard treatment for severe aortic stenosis in high-risk patients in Australia, there is still limited data on long term survival. METHODS: All patients undergoing TAVI at a single tertiary institution between September 2009 and December 2015 were included. The primary outcome was survival, by linkage of patients with the National Death Index of the Australian Institute of Health and Welfare. Post-procedure data and echocardiographic measurements were retrospectively analysed for all patients. RESULTS: A total of 186 patients were included. It was a high-risk patient population (mean EuroSCORE 31.5±20.5, mean age 83.0±8.2 years). Valve prostheses used were Edwards SAPIEN (ES) (Edwards, Irvine, CA, USA) in 16.1%, Edwards SAPIEN XT (ESXT) in 74.2%, and Medtronic CoreValve (MCV) (Medtronic, Minneapolis, MN, USA) in 9.7%. Median survival time for the entire cohort was 68.2 months (95% Confidence Interval [CI]; Lower Limit [LL] 58.0 months, Upper Limit [UL] not defined). The 2- and 5-year estimates of survival were 85% (LL 80%, UL 90%) and 56% (LL 48%, UL 66%), respectively. There was no statistically significant difference in median survival between the ES and ESXT valves, or implantation approach. Survival was greater in patients with creatinine <200 µmol/L compared to >200 µmol/L (68.8 months [LL 61.4, UL n/a] vs 48.0 months [LL 25.5, UL n/a]). Over the study period, there was a statistically significant trend in increasing mean transvalvular gradient (ES: 1.66 mmHg/yr, p=0.0058; ESXT: 2.50 mmHg/yr, p≤0.001) and maximum velocity (ESXT: 0.16 m/s/yr, p=0.004) and decreasing valve area (ESXT: -0.07 cm2/yr, p<0.001). There was substantial attrition of patient echocardiographic follow-up (number of echocardiograms followed up at 5 years=6, number at risk=41). CONCLUSIONS: This study has demonstrated acceptable survival in a high-risk cohort of patients undergoing TAVI, with comparable results to larger international experiences. There was a trend for worsening haemodynamics that needs to be monitored. Future studies need to examine patient quality of life and the performance of newer generation prostheses.

Minimally-invasive versus transcatheter aortic valve implantation: systematic review with meta-analysis of propensity-matched studies.

BACKGROUND: Minimally invasive aortic valve replacement (MiAVR) and transcatheter aortic valve implantation (TAVI) provide aortic valve replacement (AVR) by less invasive methods than conventional surgical AVR, by avoiding complete sternotomy. This study directly compares and analyses the available evidence for early outcomes between these two AVR methods. METHODS: Electronic databases were searched from inception until August 2019 for studies comparing MiAVR to TAVI, according to predefined search criteria. Propensity-matched studies with sufficient data were included in a meta-analysis. RESULTS: Eight studies with 9,744 patients were included in the quantitative analysis. Analysis of risk-matched patients showed no difference in early mortality (RR 0.76, 95% CI, 0.37-1.54, P=0.44). MiAVR had a signal towards lower rate of postoperative stroke, although this did not reach statistical significance (OR 0.42, 95% CI, 0.13-1.29, P=0.13). MiAVR had significantly lower rates of new pacemaker (PPM) requirement (OR 0.29, 95% CI, 0.16-0.52, P<0.0001) and postoperative aortic insufficiency (AI) or paravalvular leak (PVL) (OR 0.05, 95% CI, 0.01-0.20, P<0.0001) compared to TAVI, (OR 0.42, 95% CI, 0.13-1.29, P=0.13), while acute kidney injury (AKI) was higher in MiAVR compared to TAVI (11.1% vs. 5.2%, OR 2.28, 95% CI, 1.25-4.16, P=0.007). CONCLUSIONS: In patients of equivalent surgical risk scores, MiAVR may be performed with lower rates of postoperative PPM requirement and AI/PVL, higher rates of AKI and no statistical difference in postoperative stroke or short-term mortality, compared to TAVI. Further prospective trials are needed to validate these results.

Minimally invasive surgical approaches to left main and left anterior descending coronary artery revascularization are superior compared to first- and second-generation drug-eluting stents: a network meta-analysis.

OBJECTIVES: There are a number of minimally invasive approaches to revascularization of coronary artery disease that involve the left main or proximal left anterior descending artery; however, studies to date provide mixed results. METHODS: A Bayesian network meta-analysis was performed to compare early and late postoperative outcomes between percutaneous coronary intervention with first- and second-generation drug-eluting stents (DESs), off-pump coronary artery bypass and minimally invasive direct coronary artery bypass (MIDCAB) in patients with involvement of left main or left anterior descending disease. RESULTS: A total of 37 studies with 31 728 patients were included in the analysis. There were no significant differences in early mortality rates, strokes or myocardial infarctions (MIs). The long-term all-cause mortality rate was equivalent between the groups. Patients who had off-pump coronary artery bypass had fewer late MI compared with those who had first-generation DES (DES1) [odds ratio (OR) 0.38, 95% confidence interval (CI) 0.20-0.72] and MIDCAB (OR 0.41, 95% CI 0.17-0.97) and reduced late target vessel revascularization compared with DES1 (OR 0.17, 95% CI 0.09-0.32) and second-generation DES (DES2) (OR 0.32, 95% CI 0.14-0.72). The rate of late major adverse cardiac events was lower with off-pump coronary artery bypass compared with that with DES1 (OR 0.33, 95% CI 0.26-0.43) and DES2 (OR 0.62, 95% CI 0.45-0.90). The rate of late major adverse cardiac events with MIDCAB was lower than that with DES1 (OR 0.43, 95% CI 0.31-0.62) as was that with DES2 compared with DES1 (OR 0.53, 95% CI 0.39-0.70). CONCLUSIONS: Surgical approaches to left main or proximal left anterior descending disease remain superior to first- or second-generation DES in terms of long-term freedom from MI and target vessel revascularization as well as improved overall long-term survival. Conflicting rates of late MI and target vessel revascularization in patients who underwent MIDCAB suggest disease in alternate vessels that may best be approached via hybrid techniques.

Extracorporeal membrane oxygenation support in refractory perioperative anaphylactic shock to rocuronium: a report of two cases.

In recent years, extracorporeal membrane oxygenation has become increasingly common in the treatment of in-hospital cardiac arrest in non-cardiac surgery patients. This includes cardiac arrest secondary to perioperative anaphylactic shock refractory to standard advanced life support protocols, which is a rare but catastrophic event associated with significant mortality. Neuromuscular blocking drugs are most commonly implicated in perioperative anaphylaxis, with rocuronium playing a major role. In this article, we report two cases of young and otherwise fit and well patients who experienced a perioperative arrest secondary to rocuronium anaphylaxis before elective surgery; both patients did not respond to conventional advanced life support, but survived neurologically intact after institution of urgent veno-arterial extracorporeal membrane oxygenation.

Dual inflow, total-arterial, anaortic, off-pump coronary artery bypass grafting: how to do it.

Coronary surgery performed on an arrested heart, using one internal mammary artery and a saphenous vein carries two main potential drawbacks: the known failure rate of vein grafts and the relatively high rate of neurologic injury. To address these concerns, we describe a technique that achieves complete revascularization without manipulating the ascending aorta (anaortic, off-pump) and utilizing total arterial grafts. All patients undergo thorough preoperative investigation, including bilateral carotid, vertebral and subclavian artery Duplex ultrasounds. A pulmonary artery catheter, transoesophageal echocardiography, and point-of-care coagulation testing are used in each case. The left and right internal mammary arteries and non-dominant radial artery are harvested using a fully skeletonised technique. Wide bilateral extrapleural retrothymic tunnels are developed and the pericardium is opened widely to facilitate cardiac positioning. A tandem graft is constructed with the right internal mammary artery (RIMA) in situ and radial artery using an end-to-end anastomosis. This graft is brought into the pericardium and through the transverse sinus in order to graft the lateral and inferior walls with multiple sequential distal anastomoses. The left internal mammary artery (LIMA) in situ is used to graft the anterior wall. Four main cardiac positions (high and low lateral walls, inferior and anterior walls) are obtained using a combination of off-pump stabilizer positioning, alternate tension on pericardial 'heart-strings', table tilting and folded wet sponges. All distal anastomoses are performed using silastic intracoronary shunts and an off-pump myocardial stabilizer. All grafts are checked using transit-flow time measurements. Milrinone is continued overnight and dual antiplatelet therapy is continued for 3 months postoperatively.

Robotically Assisted Minimally Invasive Off-Pump Coronary Artery Bypass Surgery in a Patient With Permanent Tracheostomy.

BACKGROUND: Patients with a permanent tracheostomy requiring coronary surgery represent a unique challenge, being at increased risk of sternal wound complications, mediastinitis and stoma necrosis. Several techniques have been described including manubrium sparing sternotomy, thoracoscopic internal mammary harvest and hybrid revascularisation. METHODS: We report a case of robotic assisted (daVinci®Xi™ Surgical System) total arterial off-pump revascularisation in a patient with previous laryngectomy and permanent tracheostomy. The main advantage of this approach was to minimise the risk of postoperative sternal complication and mediastinatis, whilst still providing the prognostic benefit of total arterial grafting and the neurological advantage of the aorta no-touch technique.

Management of aortic regurgitation and bilateral carotid occlusion in severe Takayasu arteritis.

We present a patient with Takayasu arteritis and severe aortic valve regurgitation and bilateral carotid artery occlusions, who underwent aortic valve replacement and aorto-bicarotid bypass. The management of the cardiovascular manifestations of Takayasu arteritis is reviewed.

Long-term prognosis and cost-effectiveness of left ventricular assist device as bridge to transplantation: A systematic review.

BACKGROUND: This systematic review aimed to evaluate the clinical outcomes and cost-effectiveness of left ventricular assist devices (LVADs) used as bridge to transplantation (BTT), compared to orthotopic heart transplantation (OHT) without a bridge. METHOD: Systematic searches were performed in electronic databases with available data extracted from text and digitized figures. Meta-analysis of short and long-term term post-transplantation outcomes was performed with summation of cost-effectiveness analyses. RESULTS: Twenty studies reported clinical outcomes of 4575 patients (1083 LVAD BTT and 3492 OHT). Five studies reported cost-effectiveness data on 837 patients (339 VAD BTT and 498 OHT). There was no difference in long-term post-transplantation survival (HR 1.24, 95% CI 1.00-1.54), acute rejection (HR 1.10, 95% CI 0.93-1.30), or chronic rejection and cardiac allograft vasculopathy (HR 0.99, 95% CI 0.73-1.36). No differences were found in 30-day post-operative mortality (OR 0.91, 95% CI 0.42-2.00), stroke (OR 1.64, 95% CI 0.43-6.27), renal failure (OR 1.43, 95% CI 0.58-3.54), bleeding (OR 1.56, 95% CI 0.78-3.13), or infection (OR 2.44, 95% CI 0.81-7.38). Three of the five studies demonstrated incremental cost-effectiveness ratios below the acceptable maximum threshold. The total cost of VAD BTT ranged from $316,078 to $1,025,500, and OHT ranged from $179,051 to $802,200. CONCLUSION: LVADs used as BTT did not significantly alter post-transplantation long-term survival, rejection, and post-operative morbidity. LVAD BTT may be cost-effective, particularly in medium and high-risk patients with expected prolonged waiting times, renal dysfunction, and young patients.

Coronary Artery Bypass Grafting With and Without Manipulation of the Ascending Aorta: A Network Meta-Analysis.

BACKGROUND: Coronary artery bypass grafting (CABG) remains the standard of treatment for 3-vessel and left main coronary disease, but is associated with an increased risk of post-operative stroke compared to percutaneous coronary intervention. It has been suggested that CABG techniques that eliminate cardiopulmonary bypass and reduce aortic manipulation may reduce the incidence of post-operative stroke. OBJECTIVES: A network meta-analysis was performed to compare post-operative outcomes between all CABG techniques, including anaortic off-pump CABG (anOPCABG), off-pump with the clampless Heartstring device (OPCABG-HS), off-pump with a partial clamp (OPCABG-PC), and traditional on-pump CABG with aortic cross-clamping. METHODS: A systematic search of 6 electronic databases was performed to identify all publications reporting the outcomes of the included operations. Studies reporting the primary endpoint, 30-day post-operative stroke rate, were included in a Bayesian network meta-analysis. RESULTS: There were 13 included studies with 37,720 patients. At baseline, anOPCABG patients had higher previous stroke than did the OPCABG-PC (7.4% vs. 6.5%; p = 0.02) and CABG (7.4% vs. 3.2%; p = 0.001) patients. AnOPCABG was the most effective treatment for decreasing the risk of post-operative stroke (-78% vs. CABG, 95% confidence interval [CI]: 0.14 to 0.33; -66% vs. OPCABG-PC, 95% CI: 0.22 to 0.52; -52% vs. OPCABG-HS, 95% CI: 0.27 to 0.86), mortality (-50% vs. CABG, 95% CI: 0.35 to 0.70; -40% vs. OPCABG-HS, 95% CI: 0.38 to 0.94), renal failure (-53% vs. CABG, 95% CI: 0.31 to 0.68), bleeding complications (-48% vs. OPCABG-HS, 95% CI: 0.31 to 0.87; -36% vs. CABG, 95% CI: 0.42 to 0.95), atrial fibrillation (-34% vs. OPCABG-HS, 95% CI: 0.49 to 0.89; -29% vs. CABG, 95% CI: 0.55 to 0.87; -20% vs. OPCABG-PC, 95% CI: 0.68 to 0.97), and shortening the length of intensive care unit stay (-13.3 h; 95% CI: -19.32 to -7.26; p < 0.0001). CONCLUSIONS: Avoidance of aortic manipulation in anOPCABG may decrease the risk of post-operative stroke, especially in patients with higher stroke risk. In addition, the elimination of cardiopulmonary bypass may reduce the risk of short-term mortality, renal failure, atrial fibrillation, bleeding, and length of intensive care unit stay.

Intraoperative tremor in surgeons and trainees.

OBJECTIVES: Tremor may be expected to interfere with the performance of fine motor tasks such as surgery. While tremor is readily quantified in inactive subjects, it is more challenging to measure tremor as the subjects perform complex tasks. The objective of this work was to quantify tremor during the performance of a realistic simulated surgery. METHODS: Our novel surgical simulator incorporates a force sensor that allows identification and quantification of the intraoperative effects of tremor on the manipulandum. We have collected preliminary data from trainees and experienced surgeons carrying out multiple simulated anastomoses on silicone vessels, mimicking a procedure such as distal coronary anastomosis. We calculated transient and overall tremor intensity, and tested for a hypothesized 'learning effect'. RESULTS: Several of the recordings of intraoperative force data manifested distinctive features corresponding to substantial oscillation in the range of 8-12 Hz. We attribute this to enhanced physiological tremor. These early results indicate a significant reduction in the transmission of surgeon's tremor to the operative field from the first attempt to later attempts (P = 0.039, standardized effect size = 0.91), which may be associated with increasing confidence. CONCLUSIONS: This new method does not just quantify tremor, but quantifies the transmission of tremor to a manipulandum in the operative field during high-fidelity simulated coronary surgery. This may be used to assess and provide feedback on the performance of trainees and experienced surgeons, along with other fields in which fine motor skills are of vital importance.

The Aortic Root Replacement Procedure: 12-year Experience from the Australian and New Zealand Society of Cardiac and Thoracic Surgeons Database.

BACKGROUND: The aortic root replacement procedure (ARR), is often considered the gold standard in the management of aortic root and ascending aorta aneurysms. Our aim was to review the Australian experience with this procedure to ascertain early and late outcomes of mortality and morbidity. METHODS: We reviewed the Australian and New Zealand Society of Cardiac and Thoracic Surgeons' (ANZSCTS) database for patients undergoing ARR. Preoperative, intraoperative and postoperative variables were analysed. Multiple regression was performed to determine independent predictors of 30-day mortality and permanent stroke, and predictors of late death. Survival estimates were obtained by cross-linking the ANZSCTS database with the Australian Institute of Health and Welfare's National Death Index database. RESULTS: Between January 2001 and December 2011, 954 patients underwent ARR with a mean age of 56±15.2 years. The overall 30-day mortality was 5.9% (n=56) with a permanent stroke rate of 2.3% (n=21). The elective surgery mortality was 3.6%. Long-term survival was estimated as 84.4% and 68.7% at 5 and 10 years, respectively. CONCLUSIONS: Aortic root replacement surgery reveals acceptable early mortality, low postoperative stroke rates, and acceptable long-term survival.

Cardiac Magnetic Resonance Imaging Predictors of Short-Term Outcomes after High Risk Coronary Surgery.

BACKGROUND: The euroSCORE II is a widely used pre-coronary artery bypass graft surgery (CAGS) risk score, but its predictive power lacks the specificity to predict outcomes in high-risk patients (16) and left ventricular (LV) dysfunction (<40%) based on 2D-echocardiography who underwent CAGS and in whom CMRI (1.5T) was performed preoperatively were retrospectively studied. Cardiac magnetic resonance imaging parameters were assessed in patients who either had complications immediately post-surgery (n=35), six weeks post-surgery (n=20) or were uncomplicated. RESULTS: The average age of patients recruited was 69±5 years with high euroSCORE II (22±4) and low 2D-echocardiography LV ejection fraction (38%±2%). Cardiac magnetic resonance imaging results demonstrated that those with immediate complications had higher LV scar/infarct burden as a proportion of LV mass (17±3% vs 10±3%; p=0.04) with lower circumferential relaxation index (2.5±0.46 vs 2.8±0.56; p=0.05) compared to those with no complications. Early mortality from surgery was 17% (n=9) and was associated with lower RV stroke volume (55±12 vs 68±18; p=0.03) and higher LV infarct scar/burden (18±2% vs 10±2%, p=0.04). Cardiac magnetic resonance imaging showed patients with complications at six weeks post-surgery had higher LV scar/infarct burden (14.5±2% vs 6.8±2%, p=0.03) compared to those without complications. CONCLUSION: Cardiac magnetic resonance imaging preoperative LV and RV parameters are valuable in assessing the likelihood of successful outcomes from CAGS in high-risk patients with LV dysfunction.

Nonsyndromic Thoracic Aortic Aneurysm and Dissection: Outcomes With Marfan Syndrome Versus Bicuspid Aortic Valve Aneurysm.

BACKGROUND: Genetic aortopathy (GA) underlies thoracic aortic aneurysms (TAA) in younger adults. Comparative survival and predictors of outcomes in nonsyndromic TAA (NS-TAA) are incompletely defined compared to Marfan syndrome (MFS) and bicuspid aortic valve (BAV). OBJECTIVES: The study sought to compare survival and clinical outcomes for individuals with NS-TAA, MFS, and BAV. METHODS: From 1988 to 2014, all patients presenting with GA 16 to 60 years of age were enrolled in a prospective study of clinical outcomes. Risk factors for death and aortic dissection were identified by Cox proportional hazards modeling and a mortality risk score developed. RESULTS: Diagnosis of GA was made for 760 patients (age 36.9 ± 13.6 years, 26.8% female; NS-TAA, n = 311; MFS, n = 221; BAV, n = 228). MFS patients were younger than NS-TAA and BAV. Presentation with aortic dissection was more common for NS-TAA than MFS or BAV. The 687 patients surviving >30 days after presentation were followed for a median of 7 years. Calculated 10-year mortality was 7.8% for NS-TAA, 8.7% for MFS, and 3.5% for BAV (NS-TAA and MFS vs. BAV p <0.05). Factors associated with all-cause mortality were MFS (p = 0.04), age at presentation, and family history of dissection. CONCLUSIONS: Clinical outcomes for MFS and NS-TAA are similar but worse than BAV. Independent predictors of mortality, including family history of aortic dissection and age, can be included in an Aortopathy Mortality Risk Score to predict survival. Management of NS-TAA, including surgical intervention, should be similar to that of MFS.