Open, endovascular, or hybrid repair of aortic arch disease: narrative review of diverse strategies with diverse options.

OBJECTIVES AND METHODS: The management of aortic arch disease is complex. Open surgical management continues to evolve, and the introduction of endovascular repair is revolutionizing aortic arch surgery. Although these innovative techniques have generated the opportunity for better outcomes in select patients, they have also introduced confusion and uncertainty regarding best practices. In New York, we have developed a collaborative group named the New York Aortic Consortium (NYAC) as a means of crosslinking knowledge and working together to better understand and treat aortic disease. In our meeting in May 2023, regional aortic experts and invited international experts discussed the contemporary management of aortic arch disease, differences in interpretation of the available literature, as well as the integration of endovascular technology into disease management. In this review article, we summarize the current state of aortic arch surgery. RESULTS: Approaches to aortic arch repair have evolved substantially, whether it be methods to reduce cerebral ischaemia, improve hemostasis, simplify future operations, or expand options for high-risk patients with endovascular approaches. However, the transverse aortic arch remains challenging to repair. Amongst our collaborative group of cardiac/aortic surgeons, we discovered a wide disparity in our practice patterns and management strategies of patients with aortic arch disease. CONCLUSIONS: It is important to build unique institutional expertise in the context of complex and evolving management of aortic arch disease with open surgery, endovascular repair, and hybrid approaches, tailored to the risk profiles and anatomical specifics of individual patients.

Impact of Non-Diameter Aortic Indices on Surgical Eligibility: Results from the TITAN: SvS Randomized Controlled Trial.

OBJECTIVES: Traditional criteria for intervention on an asymptomatic ascending aortic aneurysm has been a maximal aortic diameter of ⩾5.5cm. The 2022 ACC/AHA aortic guidelines adopted cross-sectional aortic area/height ratio, aortic size index (ASI) and aortic height index (AHI) as alternate parameters for surgical intervention. The objective of this study was to evaluate the impact of using these newer indices on patient eligibility for surgical intervention in a prospective, multicenter cohort with moderate sized ascending aortic aneurysms between 5.0-5.4 cm. METHODS: Patients enrolled from 2018 to 2023 in the randomization or registry arms of the multicenter trial, TITAN: SvS, were included in the study. Clinical data was captured prospectively in an online database. Imaging data were derived from a core CT lab. RESULTS: Among the 329 included patients, 20% were female. Mean age was 65.0 ± 11.6 years and mean maximal aortic diameter was 50.8 ±3.9 mm. In the one third of all patients (n=109) who met any one of the three criteria (i.e., ASI ⩾ 3.08 cm/m2, AHI ⩾ 3.21 cm/m or cross-sectional aortic area/height ⩾ 10 cm2/m), their mean maximal aortic diameter was 52.5 ±0.52 mm. Alternate criteria were most commonly met in females compared to males: 20% versus 2% for ASI (p<0.001), 39% versus 5% for AHI (p<0.001) and 39% versus 21% for cross-sectional aortic area/height (p=0.002), respectively. CONCLUSIONS: One third of patients in Titan:SvS would meet criteria for surgical intervention based on novel parameters vs. the classic definition of diameter⩾5.5cm. Surgical thresholds for ASI, AHI or cross-sectional aortic area/height ratio are more likely to be met in female patients compared to male patients.

Late Pulmonary Autograft Dilation: Can We Make a Good Operation Great? The Tailored Approach.

While it is the main viable option in the growing child and young adult, the Ross procedure has expanded its applicability to older patients, for whom long-term results are equivalent, if not superior, to prosthetic aortic valve replacement. Strategies aiming at mitigating long-term autograft failure from root enlargement and valve regurgitation have led some to advocate for root reinforcement with prosthetic graft material. On the contrary, we will discuss herein the rationale for a tailored approach to the Ross procedure; this strategy is aimed at maintaining the natural physiology and interplay between the various autograft components. Several technical maneuvers, including careful matching of aortic and autograft annuli and sino-tubular junction as well as external support by autologous aortic tissue maintain these physiologic relationships and the viability of the autograft, and could translate in a lower need for late reintervention because of dilation and/or valve regurgitation.

Hemiarch versus extended arch repair for acute type A dissection: Results from a multicenter national registry.

OBJECTIVE: We compared perioperative outcomes of patients with acute type A aortic dissection undergoing hemiarch (HA) versus extended arch (EA) repair with or without descending aortic intervention. METHODS: Nine hundred twenty-nine patients underwent acute type A aortic dissection repair (2002-2021, 9 centers) including open distal repair (HA) with or without additional EA repair. EA with intervention on the descending aorta (EAD) included elephant trunk, antegrade thoracic endovascular aortic replacement, or uncovered dissection stent. EA with no descending intervention (EAND), included unstented suture-only methods. Primary outcomes were in-hospital mortality, permanent neurologic deficit, computed tomography malperfusion resolution, and a composite. Multivariable logistic regression was also performed. RESULTS: Mean age was 66 ± 18 years, 30% (278 out of 929) were women, and HA was performed more frequently (75% [n = 695]) than EA (25% [n = 234]). EAD techniques included: dissection stent (39 out of 234 [17%]), thoracic endovascular aortic replacement (18 out of 234 [7.7%]), and elephant trunk (87 out of 234 [37%]). In-hospital mortality (EA: n = 49 [21%] and HA: n = 129 [19%]; P = .42), and neurological deficit (EA: n = 43 [18%] and HA: n = 121 [17%]; P = .74) were similar. EA was not independently associated with death (EA vs HA odds ratio, 1.09; 95% CI, 0.77-1.54; P = .63) or neurologic deficit (EA vs HA odds ratio, 0.85; 95% CI, 0.47-1.55; P = .59). Composite adverse events differed significantly (EA vs HA odds ratio, 1.47; 95% CI, 1.16-1.87; P = .001). Malperfusion resolved more frequently after EAD (EAD: n = 32 [80%], EAND: n = 18 [56%], HA: n = 71 [50%]; P = .004), although multivariable analysis was not significant (EAD vs HA odds ratio, 2.17; 95% CI, 0.83-5.66; P = .10). CONCLUSIONS: Extended arch interventions pose similar perioperative mortality and neurologic risks as Hemiarch. Descending aortic reinforcement may promote malperfusion restoration. Extended techniques should be approached with caution in acute dissection due to increased risk of adverse events.

Long-Term Clinical and Echocardiographic Outcomes Following the Ross Procedure: A Post Hoc Analysis of a Randomized Clinical Trial.

Importance: The Ross procedure as treatment for adults with aortic valve disease (AVD) has been the subject of renewed interest. Objective: To evaluate the long-term clinical and echocardiographic outcomes following the Ross procedure for the treatment of adults with AVD. Design, Setting, and Participants: This post hoc analysis of a randomized clinical trial included adult patients (age <69 years) who underwent a Ross procedure for the treatment of AVD, including those with active endocarditis, rheumatic AVD, decreased ejection fraction, and previous cardiac surgery. The trial, conducted from September 1, 1994, to May 31, 2001, compared homograft root replacement with the Ross procedure at a single center. Data after 2010 were collected retrospectively in November and December 2022. Exposure: Ross procedure. Main Outcomes and Measures: The primary end point was long-term survival among patients who underwent the Ross procedure compared with that in the age-, country of origin- and sex-matched general population. Secondary end points were freedom from any reintervention, autograft reintervention, or homograft reintervention and time-related valve function, autograft diameter, and functional status. Results: This study included 108 adults (92 [85%] male) with a median age of 38 years (range, 19-66 years). Median duration of clinical follow-up was 24.1 years (IQR, 22.6-26.1 years; 2488 patient-years), with 98% follow-up completeness. Of these patients, 9 (8%) had active endocarditis and 45 (42%) underwent reoperations. The main hemodynamic lesion was stenosis in 30 (28%) and regurgitation in 49 (45%). There was 1 perioperative death (0.9%). Twenty-five year survival was 83.0% (95% CI, 75.5%-91.2%), representing a relative survival of 99.1% (95% CI, 91.8%-100%) compared with the general population (83.7%). At 25 years, freedom from any reintervention was 71.1% (95% CI, 61.6%-82.0%); from autograft reintervention, 80.3% (95% CI, 71.9%-89.6%); and from homograft reintervention, 86.3% (95% CI, 79.0%-94.3%). Thirty-day mortality after the first Ross-related reintervention was 0% and after all Ross-related reinterventions was 3.8% (n = 1); 10-year survival after reoperation was 96.2% (95% CI, 89.0%-100%). Conclusions and Relevance: This study found that the Ross procedure provided excellent survival into the third decade postoperatively that was comparable to that in the general population. Long-term freedom from reintervention demonstrated that the Ross procedure may be a durable substitute into late adulthood, showing a delayed but progressive functional decline. Trial Registration: isrctn.org Identifier: ISRCTN03530985.

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines.

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines.

The Ross Procedure in Adolescence and Beyond: Are There Still Contraindications?

The Ross procedure is an excellent operation to treat children and adults with aortic valve disease. Compared to prosthetic aortic valve replacement, it provides important clinical benefits in terms of survival, hemodynamics, freedom from valve-related complications, and durability, especially in women of childbearing age. However, the Ross procedure is a longer and technically more challenging operation. As a result, the choice of procedure should be driven by patient anatomy and clinical characteristics. This highlights the importance of concentrating care in Ross reference centers where surgical expertise and experience are present to ensure patient safety and long-term effectiveness of the operation. This manuscript reviews the major and relative contraindications to the Ross procedure.

Formal consensus study on surgery to replace the aortic valve in adults aged 18-60 years.

OBJECTIVE: There is uncertainty about surgical procedures for adult patients aged 18-60 years undergoing aortic valve replacement (AVR). Options include conventional AVR (mechanical, mAVR; tissue, tAVR), the pulmonary autograft (Ross) and aortic valve neocuspidisation (Ozaki). Transcatheter treatment may be an option for selected patients. We used formal consensus methodology to make recommendations about the suitability of each procedure. METHODS: A working group, supported by a patient advisory group, developed a list of clinical scenarios across seven domains (anatomy, presentation, cardiac/non-cardiac comorbidities, concurrent treatments, lifestyle, preferences). A consensus group of 12 clinicians rated the appropriateness of each surgical procedure for each scenario on a 9-point Likert scale on two separate occasions (before and after a 1-day meeting). RESULTS: There was a consensus that each procedure was appropriate (A) or inappropriate (I) for all clinical scenarios as follows: mAVR: total 76% (57% A, 19% I); tAVR: total 68% (68% A, 0% I); Ross: total 66% (39% A, 27% I); Ozaki: total 31% (3% A, 28% I). The remainder of percentages to 100% reflects the degree of uncertainty. There was a consensus that transcatheter aortic valve implantation is appropriate for 5 of 68 (7%) of all clinical scenarios (including frailty, prohibitive surgical risk and very limited life span). CONCLUSIONS: Evidence-based expert opinion emerging from a formal consensus process indicates that besides conventional AVR options, there is a high degree of certainty about the suitability of the Ross procedure in patients aged 18-60 years. Future clinical guidelines should include the option of the Ross procedure in aortic prosthetic valve selection.

Matched comparison between external aortic root support and valve-sparing root replacement.

OBJECTIVES: Differences in indication and technique make a randomised comparison between valve-sparing root replacement (VSRR) and personalised external aortic root support (PEARS) challenging. We performed a propensity score (PS)-matched comparison of PEARS and VSRR for syndromic root aneurysm. METHODS: Patients in the PEARS 200 Database and Aortic Valve Insufficiency and ascending aorta Aneurysm InternATiOnal Registry (undergoing VSRR) with connective tissue disease operated electively for root aneurysm <60 mm with aortic regurgitation (AR) <1/4 were included. Using a PS analysis, 80 patients in each cohort were matched. Survival, freedom from reintervention and from AR ≥2/4 were estimated using a Kaplan-Meier analysis. RESULTS: Median follow-up was 25 and 55 months for 159 PEARS and 142 VSRR patients. Seven (4.4%) patients undergoing PEARS required an intervention for coronary injury or impingement, resulting in one death (0.6%). After VSRR, there were no early deaths, 10 (7%) reinterventions for bleeding and 1 coronary intervention. Survival for matched cohorts at 5 years was similar (PEARS 98% vs VSRR 99%, p=0.99). There was no difference in freedom from valve or ascending aortic/arch reintervention between matched groups. Freedom from AR ≥2/4 at 5 years in the matched cohorts was 97% for PEARS vs 92% for VSRR (p=0.55). There were no type A dissections. CONCLUSIONS: VSRR and PEARS offer favourable mid-term survival, freedom from reintervention and preservation of valve function. Both treatments deserve their place in the surgical repertoire, depending on a patient's disease stage. This study is limited by its retrospective nature and different follow-ups in both cohorts.

Structural dysregulation of the pulmonary autograft was associated with a greater density of p16INK4A-vascular smooth muscle cells.

The present study tested the hypothesis that a senescent phenotype of vascular smooth muscle cells (VSMCs) may represent the seminal event linked to maladaptive pulmonary autograft remodeling of a small number of patients that underwent the Ross procedure. The diameter of the pulmonary autograft (47±4 mm) of three male patients was significantly greater compared to the pulmonary artery (26±1 mm) excised from bicuspid aortic valve (BAV) patients. The pulmonary autograft was associated with a neointimal region and the adjacent medial region was significantly thinner compared to the pulmonary artery of BAV patients. Structural dysregulation was evident as elastin content of the medial region was significantly reduced in the pulmonary autograft compared to the pulmonary artery of BAV patients. By contrast, collagen content of the medial region of the pulmonary autograft and the pulmonary artery of BAV patients was not significantly different. Reduced medial elastin content of the pulmonary autograft was associated with increased protein levels of matrix metalloproteinase-9. The latter phenotype was not attributed to a robust inflammatory response as the percentage of Mac-2(+)-infiltrating monocytes/macrophages was similar between groups. A senescent phenotype was identified as protein levels of the cell cycle inhibitor p27kip1 were upregulated and the density of p16INK4A/non-muscle myosin IIB(+)-VSMCs was significantly greater in the pulmonary autograft compared to the pulmonary artery of BAV patients. Thus, senescent VSMCs may represent the predominant cellular source of increased matrix metalloproteinase-9 protein expression translating to maladaptive pulmonary autograft remodeling characterized by elastin degradation, medial thinning and neointimal formation.

Ross Procedure Versus Mechanical Versus Bioprosthetic Aortic Valve Replacement: A Network Meta-Analysis.

Background The Ross operation appears to restore normal survival in young and middle-aged adults with aortic valve disease. However, there are limited data comparing it with conventional aortic valve replacement. Herein, we compared outcomes of the Ross procedure with mechanical and bioprosthetic aortic valve replacement (M-AVR and B-AVR, respectively). Methods and Results MEDLINE and EMBASE were searched through March 2022 to identify randomized controlled trials and propensity score-matched studies that investigated outcomes of patients aged ≥16 years undergoing the Ross procedure, M-AVR, or B-AVR. The systematic literature search identified 2 randomized controlled trials and 8 propensity score-matched studies involving a total of 4812 patients (Ross: n=1991; M-AVR: n=2019; and B-AVR: n=802). All-cause mortality was significantly lower in the Ross procedure group compared with M-AVR (hazard ratio [HR] [95% CI], 0.58 [0.35-0.97]; P=0.035) and B-AVR (HR [95% CI], 0.32 [0.18-0.59]; P<0.001) groups. The reintervention rate was lower after the Ross procedure and M-AVR compared with B-AVR, whereas it was higher after the Ross procedure compared with M-AVR. Major bleeding rate was lower after the Ross procedure compared with M-AVR. Long-term stroke rate was lower following the Ross procedure compared with M-AVR and B-AVR. The rate of endocarditis was also lower after the Ross procedure compared with B-AVR. Conclusions Improved long-term outcomes of the Ross procedure are demonstrated compared with conventional M-AVR and B-AVR options. These results highlight a need to enhance the recognition of the Ross procedure and revisit current guidelines on the optimal valve substitute for young and middle-aged patients.

Growth Differentiation Factor-15 as a Predictor of Functional Capacity, Frailty, and Ventricular Dysfunction in Patients With Aortic Stenosis and Preserved Left Ventricular Ejection Fraction.

In aortic stenosis (AS), left ventricular (LV) remodeling often occurs before symptom onset, and early intervention may be beneficial. Risk stratification remains challenging and identification of biomarkers may be useful. We evaluated the association between growth differentiation factor-15 (GDF-15) and soluble suppression of tumorigenicity 2 (sST2) and known markers of poor prognosis in AS. Baseline plasma GDF-15 and sST2 levels were measured in 70 patients with moderate-severe AS (aortic valve area <1.5 cm2) and preserved LV ejection fraction (>45%). Patients were categorized into "low GDF-15" versus "high GDF-15" and "low sST2" versus "high sST2" groups. Groups were compared for differences in cardiovascular risk factors, 6-minute walk test, 5 m gait speed, cognitive function (Montreal Cognitive Assessment), and echocardiographic parameters. Overall, 44% of patients were deemed asymptomatic by New York Heart Association class, 61% had severe AS (aortic valve area <1 cm2) and all patients had preserved LV ejection fraction. GDF-15 levels were not predictive of AS severity. However, high GDF-15 (>1,050 pg/ml) was associated with LV dysfunction as shown by lower indexed stroke volume (p <0.01), worse LV global longitudinal strain (p = 0.04), greater mean E/e' (p = 0.02) and indexed left atrial volume (p <0.01). It was also associated with decreased functional capacity with shorter 6-minute walk test (p = 0.01) and slower 5 m gait speed (p = 0.02). Associations between sST2 levels and markers of poor prognosis were less compelling. In this study of patients with moderate to severe AS, elevated GDF-15 levels are associated with impaired functional capacity, poorer performance on fragility testing, and LV dysfunction. In conclusion, GDF-15 may integrate these markers of adverse outcomes into a single biomarker of poor prognosis.

A multicentre, propensity score matched analysis comparing a valve-sparing approach to valve replacement in aortic root aneurysm: Insight from the AVIATOR database.

OBJECTIVES: Our goal was to evaluate the outcome of valve-sparing root replacement (VSRR) and to compare the outcomes to those of patients having composite valve-graft conduit aortic root replacement (CVG-ARR) in a cohort of patients with aortic root aneurysm ± valve insufficiency, without valvular stenosis. Although valve-sparing procedures are preferable in young patients, there is a lack of comparative data in comparable patients. METHODS: The VSRR procedures were performed in 2005 patients, and 218 patients underwent a CVG-ARR procedure. Exclusion criteria included aortic dissection, endocarditis and valvular stenosis. Propensity score matching (3:1 ratio) was applied to compare VSRR (reimplantation 33% and remodelling 67%) and CVG-ARR. RESULTS: We matched 218 patients with CVG-ARR to 654 patients with VSRR (median age, 56.0; median follow-up was 4 years in both groups; interquartile range 1-5 years). Early mortality was 1.1% of those who had VSRR versus 2.3% in those who had CVG-ARR. Survival was 95.4% [95% confidence interval (CI) 94-97%] at 5 years in patients who had VSRR versus 85.4% (95% CI 82-92%) in those who had CVG-ARR; P = 0.002. Freedom from reintervention at 5 years was 96.8% (95% CI 95-98%) with VSRR and 95.4% (95% CI 91-99%) with CVG-ARR (P = 0.98). Additionally, there were more thromboembolic, endocarditis and bleeding events in the patients who had CVG-ARR (P = 0.02). CONCLUSIONS: This multicentre study shows excellent results after valve-sparing root replacement in patients with an ascending aortic aneurysm with or without valve insufficiency. Compared to composite valve-graft aortic root replacement, survival is better and valve-related events are fewer. Consequently, valve-sparing procedures should be considered whenever a durable repair is feasible. We advocate a valve-sparing strategy even in more complex cases when performed in experienced centres.

Structural abnormalities after aortic root replacement with stentless xenograft.

OBJECTIVE: In complex and high-risk aortic root disease, the porcine Freestyle stentless bioprosthesis (Medtronic Inc, Minneapolis, Minn) is an important surgical treatment option. We aimed to determine prevalence and clinical effect of structural and functional abnormalities after full-root Freestyle implantation. METHODS: Our cross-sectional 2-center study combined with clinical follow-up included 253 patients with full-root Freestyle bioprostheses implanted from 1999 to 2017. Patients underwent transthoracic echocardiography (TTE) and contrast-enhanced, electrocardiogram-gated 4-dimensional cardiac computed tomography (4DCT) at median age 70 (interquartile range, 62-75) years. After 4DCT, clinical follow-up continued throughout 2018. Median follow-up was 3.3 years before 4DCT and 1.4 years after. RESULTS: We identified abnormalities in 46% of patients, including pseudoaneurysms (n = 32; 13%), moderate or severe coronary ostial stenosis (n = 54; 21%), and moderate-severe leaflet thickening or reduced leaflet motion (n = 51; 20%). TTE only identified 1 patient with pseudoaneurysm. After 4DCT, the unadjusted hazard ratio for surgical reintervention among patients with abnormal 4DCT was 4.2 (95% confidence interval, 1.2-15.3), in all, 10% required a reintervention. 4DCT abnormalities were associated with a statistically nonsignificant increased risk of death, stroke, or myocardial infarction (hazard ratio obtained using Cox proportional hazards regression analysis, 2.4; 95% confidence interval, 0.7-7.6). In all, 4.0% died, 3.6% had a myocardial infarction, and 2.0% had a stroke. CONCLUSIONS: Structural and functional abnormalities of the aortic root are frequent after Freestyle implantation and TTE appears to be insufficient for follow-up. Abnormalities might be associated with increased risk of reintervention and potentially adverse clinical outcomes. Longer follow-up and larger study populations are needed to further clarify the clinical implications of abnormalities identified with 4DCT.