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Mid-aortic syndrome (MAS) is an uncommon condition characterized by severe narrowing of the abdominal aorta, usually involving visceral and renal arteries. Most patients are asymptomatic and typically present with incidental hypertension which might evolve into end-organ damage if untreated. Our aim was to review 8 new pediatric MAS cases. A retrospective observational study of all pediatric patients with MAS diagnosis (April 1992-November 2021) was conducted. Patients underwent systematic evaluation (medical and family history; 12-lead electrocardiogram; echocardiogram; angiography and/or computed tomography or magnetic resonance angiography). 8 pediatric patients with MAS were included. Median age at diagnosis was 2.6 [0.2-4.7] years; median follow-up time was 8.6 [6.6-10.0] years. 6/8 patients presented with incidental hypertension, 1/8 with heart murmur, and 1/8 with heart failure symptoms. All patients were on antihypertensive treatment. 1/8 patients underwent surgery and 7/8 an endovascular treatment. At the end of the study period, among the 6 patients that underwent a successful endovascular procedure, 2 achieved good blood pressure (BP) control, 2 acceptable BP control, 1 stage 1 hypertension and, another, stage 2 hypertension. There was 1 death during follow-up. BP monitoring in pediatric patients is crucial for early recognition of MAS. Treatment should be based on the individual clinical characteristics of patients with careful planning of surgical revascularisation, if possible, after adult growth is completed. Our study demonstrates that endovascular treatment might be a good alternative to surgery. Nevertheless, further trials with larger sample size and longer-term follow-up are required to determine the best treatment approach.

Instituto de Salud Carlos III (ISCIII), European Regional Development Fund/European Social Fund "A way to make Europe"/" Investing in your future" [PI18/0004, PI20/0320, PT17/0015/0043]; ISCIII; MCIN; Pro-CNIC Foundation; Severo Ochoa Centers of Excellence program [CEX2020-001041-S]; ISCIII [CM20/00101], Frutos F. de, Ochoa JP, Navarro-Peñalver M, Baas A, Bjerre JV, Zorio E, Méndez I, Lorca R, Verdonschot JAJ, García-Granja PE, Bilinska Z, Fatkin D, Fuentes-Cañamero ME, García-Pinilla JM, García-Álvarez MI, Girolami F, Barriales-Villa R, Díez-López C, Lopes LR, Wahbi K, García-Álvarez A, Rodríguez-Sánchez I, Rekondo-Olaetxea J, Rodríguez-Palomares JF, Gallego-Delgado M, Meder B, Kubanek M, Hansen FG, Restrepo-Córdoba MA, Palomino-Doza J, Ruiz-Guerrero L, Sarquella-Brugada G, Perez-Perez AJ, Bermúdez-Jiménez FJ, Ripoll-Vera T, Rasmussen TB, Jansen M, Sabater-Molina M, Elliot PM, Garcia-Pavia P; European Genetic Cardiomyopathies Initiative Investigators

Marfan syndrome is a rare connective tissue disorder secondary to mutations in the FBN1 gene, characterised by skeletal, ocular, and cardiovascular manifestations. We present an extensive cohort of paediatric patients with Marfan syndrome highlighting the vital importance of promptly referring paediatric first-degree relatives of Marfan syndrome paediatric patients to a tertiary hospital as our results confirm that they are at higher risk of aortic dilatation.

Introduction Marfan syndrome (MFS) is a connective tissue disorder related to FBN1 mutations. The main cardiac feature of this syndrome is the enlargement and the dissection of the ascending aorta. Close monitoring of the aortic root diameters and its preventive replacement has allowed improving life expectancy of these patients in the last decades. However, there is paucity of data about the outcome of aortic dissection affecting the descending aorta and its markers of poor evolution. Purpose To characterize patients with MFS and dissection involving the descending aorta. To compare baseline characteristics and the clinical outcome between patients with isolated type B dissection (Group B) and patients with type A dissection with residual B dissection (Group A). Methods Retrospective longitudinal analysis of patients that attended our Marfan unit between 2001 and 2021 with an aortic event involving the descending aorta. Clinical data were collected from electronic health records. Results From the global cohort of adults with Marfan syndrome seen at our centre, we identified a total of 37 adults (11.5% of the overall cohort) that had an acute aortic event with involvement of the descending aorta. 20 (54.1%) were type A aortic events and 17 (45.9%) were isolated type B dissections. Cardiovascular risk factors were similarly distributed in both groups but there was a significant higher percentage of women in Group B (35.0% vs 76.5%, p=0.012) (Figure 1). Acute management of the aortic event implied surgery in 17 (85.0%) patients in Group A and in 5 (29.4%) patients in Group B (p=0.001). In Group B, interventions were 3 TEVAR and 2 open surgeries. 2 (5.4%) patient died during the acute phase, 1 (5.0%) in Group A and 1 (5.9%) in Group B (p=ns).The remaining 35 patients (19 in Group A, 16 in Group B) were followed-up for 10.4±6.7 years. 10 patients died during follow-up (6 in Group A, 4 in Group B). In Group A, 2 patients died of sudden death, 2 patients died in the perioperative setting of aortic surgery, and 2 patients died of an aortic rupture. In Group B, 3 patients died in the perioperative setting of aortic surgery and 1 of an intracranial hemorrhage. The proportions of aortic events during follow-up (death, distal redissection, proximal dissection, distal intervention or aortic root surgery) in both groups are described in Figure 2. Median survival free of reintervention or death (non-related to initial event) was 7.5 years in the overall cohort; 8.2 years in Group A and 2.2 years in Group B (log rank p=0.04) (Figure 2). Conclusions Patients with Marfan syndrome and aortic dissection involving the descending aorta have a poor clinical outcome, with high rates of reintervention, redissection or death. Although Group B required less often surgery during the acute phase, worst survival free of reintervention or death was observed during follow-up. Funding Acknowledgement Type of funding sources: None.

Aortic aneurysm; Bicuspid aortic valve; Computed tomography Aneurisma aòrtic; Vàlvula aòrtica bicúspide; Tomografia computada Aneurisma aórtico; Válvula aórtica bicúspide; Tomografía computada Multimodality imaging is the basis of the diagnosis, follow-up, and surgical management of bicuspid aortic valve (BAV) patients. Transthoracic echocardiography (TTE) is used in our clinical routine practice as a first line imaging for BAV diagnosis, valvular phenotyping and function, measurement of thoracic aorta, exclusion of other aortic malformations, and for the assessment of complications such are infective endocarditis and aortic. Nevertheless, TTE is less useful if we want to assess accurately other aortic segments such as mid-distal ascending aorta, where computed tomography (CT) and magnetic resonance (CMR) could improve the precision of aorta size measurement by multiplanar reconstructions. A major advantage of CT is its superior spatial resolution, which affords a better definition of valve morphology and calcification, accuracy, and reproducibility of ascending aorta size, and allows for coronary artery assessment. Moreover, CMR offers the opportunity of being able to evaluate aortic functional properties and blood flow patterns. In this setting, new developed sequences such as 4D-flow may provide new parameters to predict events during follow up. The integration of all multimodality information facilitates a comprehensive evaluation of morphologic and dynamic features, stratification of the risk, and therapy guidance of this cohort of patients.

AimsMid-aortic syndrome (MAS) is an uncommon condition characterized by severe narrowing of the abdominal aorta, usually involving visceral and renal arteries. Most patients are asymptomatic and typically present with incidental hypertension which might evolve into end-organ damage if untreated. Our aim was to review 8 new paediatric MAS cases.MethodsA retrospective observational study of all paediatric patients with MAS diagnosis (April 1992-Novembre 2021) was conducted. Patients underwent systematic evaluation (medical and family history; 12-lead electrocardiogram; echocardiogram; angiography and/or computed tomography or magnetic resonance angiography).Results8 paediatric patients with MAS were included. Median age at diagnosis 2.6 [0.2–4.7] years; median follow-up time 8.6 [6.6–10.0] years. 6/8 patients presented with incidental hypertension, 1/8 with heart murmur and 1/8 with heart failure symptoms.All patients were on antihypertensive treatment. 1/8 patients underwent surgery and 7/8 an endovascular treatment. At the end of the study period, among the 6 patients that underwent a successful endovascular procedure, 2 achieved good blood pressure (BP) control, 2 acceptable BP control, 1 stage 1 hypertension and, another, stage 2 hypertension. There was 1 death during follow-up.ConclusionBP monitoring in paediatric patients is crucial for early recognition of MAS. Treatment should be based on the individual clinical characteristics of patients with careful planning of surgical revascularisation, if possible, after adult growth is completed. Our study demonstrates that endovascular treatment might be a good alternative to surgery. Nevertheless, further trials with larger sample size and longer term follow-up are required to determine the best treatment approach.

Funding Acknowledgements Type of funding sources: None. Background Mitral annular disjunction (MAD) is a structural abnormality of the mitral annular fibrosus characterized by a separation between the atrial wall-mitral valve junction, and the left ventricular attachment (1,2). It has been associated with mitral valve prolapse (MVP). Limited data is available regarding the impact of the presence of MVP on the evolution of mitral regurgitation in these syndromic entities. Purpose To evaluate the prevalence of MAD, PMV, and the combination of both in patients with syndromic hereditary thoracic aortic disease (HTAD) including Marfan (MFS), Loeys-Dietz (LDS), and vascular Ehlers-Danlos syndromes (vEDS), and its relationship with mitral regurgitation (MR) severity and the need for mitral surgery at the follow-up. Methods Adult patients with syndromic HTAD seen at our specialized unit were retrospectively included. The presence of MAD, MVP, and significant MR at the first echocardiogram were evaluated. Electronic medical records were reviewed to register the need for mitral surgery. The last echocardiogram available was also assessed to evaluate MR progression. Results A total of 295 patients were included (235 MFS, 42 LDS, and 18 vEDS). The mean age at baseline was 39.0+-14.4 and 52.9% were female. MAD was present in 87 (37.0%) of MFS, 6 (14.3 %) of LDS and was not present in vEDS (p< 0.001). MVP was found in 105 (44.7%) of MFS, 6 (14.3%) of LDS and 0 in vEDS (p< 0.001). In MFS, MAD was significantly associated with MVP (p= A second echocardiogram was available in 220 patients at > = 1 year (mean 4.1 +- 1.4 years). Overall, 25 (11.4%) presented significant progression of MR, 0 in the isolated MAD group, 13 (19.4%) in the MAD/MVP group and 6 (20.0%) of the isolated MVP (p = 0.007). After a mean clinical follow-up of 7.5 +- 3.2yrs, 10 patients required mitral surgery (6 prostheses, 4 valvuloplasties). After adjustment for the presence of MVP and time of follow-up, MAD was not associated with progression of MR (p= 0.529) but MVP was (OR 5.2 IC 1.70 - 15.93). Similarly, MVP (OR 5.6 CI 1.23 - 25.86) but not MAD (P= 0.096) was associated with the need for mitral surgery ., Conclusions The prevalence of MAD in syndromic HTAD is high, especially in Marfan syndrome, and absent in vEDS. In this retrospective observational study, the presence of MVP but not MAD was associated with mitral regurgitation evolution and the need for mitral surgery. Abstract Table 1 Abstract Figure 1

Background Heart transplant (HT) remains the last treatment option for patients with non-obstructive hypertrophic cardiomyopathy (HCM) who develop end-stage heart failure (HF). Early identification of patients who may require a HT in the future is crucial in order to advise them, establish the appropriate follow-up and determine the appropriate time to include them in the waiting list. Objectives Our study sought to find predictive factors related with requiring HT during follow-up in patients with HCM. Methods Consecutive patients with HCM referred to a HCM monographic clinic from 2018 to 2020 (HCM controls) and transplanted patients due to HCM in the same tertiary HT hospital since 2003 (cases) were included. Baseline (on the date of HCM diagnosis) and longitudinal data regarding clinical, genetic, ECG and echocardiographic variables were retrospectively evaluated. Follow-up was registered from HCM diagnosis to HT (in cases) or last medical check up (controls). Results A total of 157 patients (24 HCM-HT cases and 133 HCM controls) were included (45±19 yo; 57% male). At the time of MCH diagnosis (Table), cases were significantly younger than controls, were more frequently symptomatic and showed significantly higher BNP levels and more advanced diastolic dysfunction (larger left atrium, higher E/A ratio and lower e'); also, HCM-HT reported more family history and had higher proportion of pathogenic mutations (being MYH7 the most frequently involved). Left ventricular (LV) systolic function was slightly reduced in HCM-HT cases. In contrast, HCM controls were more frequently diagnosed by casual findings or family screening and had more LV outflow tract obstruction at first medical evaluation. LV maximal wall thickness (MWT) did not differ between groups. During a median follow-up since HCM diagnosis of 6.2 years (median follow-up of 8.9 and 7.1 years in cases and controls, respectively), HCM-HT cases presented a higher incidence of sustained ventricular tachycardia or ICD therapy (HR=4.0; CI95%:1.6–10.0 p=0.03) and HF admissions (HR=3.9; CI95%:1.8–8.1 p Conclusions The presence of symptoms in a young non-obstructive HCM patient, along with family history and a pathogenic mutation, should advice clinicians a closer follow-up and early transfer to a HT referral center, especially if associated with diastolic dysfunction and high BNP values. Funding Acknowledgement Type of funding sources: None. Table 1

Background Mitral annular disjunction (MAD) is a structural abnormality of the mitral annular fibrosus characterized by a separation between the atrial wall-mitral valve junction, and the left ventricular attachment (1). It has been associated with mitral valve prolapse (MVP) (2) but also, with arrhythmias and sudden cardiac death (SCD) (3). There is no evidence of its prevalence and clinical significance in patients with syndromic hereditary aortopathies. Purpose To evaluate the prevalence of MAD, PMV, and the combination of both in patients with syndromic hereditary thoracic aortic disease (HTAD) including Marfan (MFS), Loeys-Dietz (LDS) and vascular Ehlers-Danlos syndromes (vEDS), and its relationship with arrhythmias, SCD, mitral regurgitation (MR) severity and the need for mitral surgery at the follow-up. Methods Adult patients with syndromic HTAD seen at our specialized unit were retrospectively included. The presence of MAD, MVP, and significant MR at first echocardiogram were evaluated. Electronic medical records were reviewed to register the occurrence of arrhythmic events and the need of mitral surgery. Last echocardiogram available was also assessed to evaluate MR progression. Results A total of 295 patients were included (235 MFS, 42 LDS and 18 vEDS). Mean age at baseline was 39.0±14.4 and 52.9% were female. MAD was present in 87 (37.0%) of MFS, 6 (14.3%) of LDS and was not present in vEDS (p In MFS, the presence of MAD was significantly associated with MVP (p≤0.001) (Table 1). However, 14 (6.0%) of patients had isolated MAD (Table 2). At baseline, significant MR was observed in 18 (24.7%) of patients with concurrent MAD and MVP and was not present in patients with isolated MAD (Table 2). MVP (OR 16.85 IC 4.43 – 64.07) but not MAD (p=0.607), was associated with significant MR in the multivariate analysis. A second echocardiogram was available in 220 patients at ≥1 year (mean 4.1±1.4 years). Overall, 25 (11.4%) presented significant progression of MR, 0 in the isolated MAD group, 13 (19.4%) in the MAD/MVP group and 6 (20.0%) of the isolated MVP (p=0.007). After a mean clinical follow-up of 7.5±3.2 years, 10 patients required mitral surgery (6 prosthesis, 4 valvuloplasty), 22 (9.4%) presented atrial fibrillation, flutter or supraventricular tachycardia (SVT), and 2 (0.9%) SCD. After adjustment for the presence of MVP and time of follow-up, MAD was not associated with progression of MR (p=0.529) need for mitral surgery (p=0.096), atrial fibrillation-flutter or SVT (p=0.510) nor SCD. (p=0.997). Conclusions The prevalence of MAD in syndromic HTAD is high, especially in Marfan syndrome, and absent in vEDS. In this retrospective observational study, the presence of MAD in Marfan was not associated with mitral regurgitation evolution or arrhythmic events. Funding Acknowledgement Type of funding sources: None. Characteristics of MFS patientsPresence of significant MR by groups.

BACKGROUND Left ventricular noncompaction (LVNC) is a heterogeneous entity with uncertain prognosis. OBJECTIVES This study sought to develop and validate a prediction model of major adverse cardiovascular events (MACE) and to identify LVNC cases without events during long-term follow-up. METHODS This is a retrospective longitudinal multicenter cohort study of consecutive patients fulfilling LVNC criteria by echocardiography or cardiovascular magnetic resonance. MACE were defined as heart failure (HF), ventricular arrhythmias (VAs), systemic embolisms, or all-cause mortality. RESULTS A total of 585 patients were included (45 +/- 20 years of age, 57% male). LV ejection fraction (LVEF) was 48% +/- 17%, and 18% presented late gadolinium enhancement (LGE). After a median follow-up of 5.1 years, MACE occurred in 223 (38%) patients: HF in 110 (19%), VAs in 87 (15%), systemic embolisms in 18 (3%), and 34 (6%) died. LVEF was the main variable independently associated with MACE (P < 0.05). LGE was associated with HF and VAs in patients with LVEF > 35% (P < 0.05). A prediction model of MACE was developed using Cox regression, composed by age, sex, electrocardiography, cardiovascular risk factors, LVEF, and family aggregation. C-index was 0.72 (95% confidence interval: 0.67-0.75) in the derivation cohort and 0.72 (95% confidence interval: 0.71-0.73) in an external validation cohort. Patients with no electrocardiogram abnormalities, LVEF $50%, no LGE, and negative family screening presented no MACE at follow-up. CONCLUSIONS LVNC is associated with an increased risk of heart failure and ventricular arrhythmias. LVEF is the variable most strongly associated with MACE; however, LGE confers additional risk in patients without severe systolic dysfunction. A risk prediction model is developed and validated to guide management.

Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Spanish Ministry of Science, Innovation and Universities ; Instituto de Salud Carlos III Introduction The aorta is a 3D hollow, curvilinear elastic structure whose diseases have life-threatening consequences. Despite much effort has been paid to study aortic diameter, diameter is a poor predictor of events. Conversely, much less is known about aortic curvature, its distribution in the thoracic aorta and the potential impact of risk factors in aneurysms associated with different conditions. Currently, 4D flow magnetic resonance imaging (4D flow CMR) allows to obtain 3D geometry, 4D flow data and regional aortic stiffness. Purpose We aim to study regional aortic curvature in thoracic aorta aneurysms of different aetiologies and define its relationship with established risk factors. Methods One-hundred twenty patients (40 for each group, selected out of prospective cohorts of 156 bicuspid aortic valve – BAV-, 77 Marfan –MFS- and 67 patients with a degenerative aneurysm – TAVdeg-) were matched for age, sex and BSA via propensity score with 40 healthy volunteers (HV). The thoracic aorta was semi-automatically segmented from angiograms and the centreline was computed. Local curvature was assessed at 20 planes covering the thoracic aorta from the sinotubular junction to the proximal descending aorta (DAo) at the level of the pulmonary artery bifurcation. Local curvature was normalized by subject mean thoracic aorta curvature. Length was measured as centreline length. Aortic stiffness was measured in the DAo by pulse wave velocity (PWV). Aneurysm was defined by z-score ≥ 2 using diameters measured by double-oblique cine CMR. Results Matching was successful in all groups with the exception of a residual age difference between HV and TAVdeg. Curvature in HV showed a fairly smooth transition between the straighter ascending aorta (AAo) and DAo to a more curved aortic arch, with a peak in the mid aortic arch (Figure 1A). Conversely, all patients’ groups presented a peak in curvature in the proximal DAo and a decreased local curvature in the aortic arch and mid DAo close to the level of the pulmonary artery. BAV and TAVdeg patients showed also increased curvature in the mid AAo, were dilation is prevalent. Conversely, in the same area MFS showed a reduced curvature and limited prevalence of aneurysm. In the overall population, age, AAo and root diameters, mean blood pressure, DAo PWV and aortic length, all established risk factors for aortic events, were inversely related to curvature in the distal AAo and aortic arch (Figure 1B). Conclusions Aneurysms related to different aetiologies show similar abnormalities in aortic curvature, with limited curvature in the aortic arch and a peak soon after the third supra-aortic vessel. Age, aortic diameter, length, stiffness and blood pressure, all known risk factors, are all related to reduced curvature in the distal ascending aorta and aortic arch. Abstract Figure.

Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Spanish Ministry of Science, Innovation and Universities; Instituto de Salud Carlos III Introduction In Marfan syndrome (MFS) patients reduced longitudinal strain of the ascending aorta (AAo) as measured by applying feature-tracking on cine cardiac magnetic resonance (CMR) images predicts aortic root dilation and aortic events during the follow-up. Speckle-tracking is well established for cardiac deformation assessment but proximal aorta applications are challenging due to limited wall thickness and substantial cardiac motion. Moreover, echocardiography is widely used in the clinical assessment aortic diseases. Purpose We aimed to test a speckle-tracking tool for root longitudinal strain analysis in terms of comparison with CMR-derived AAo longitudinal strain and reproducibility and as predictor of dilation in MFS patients. Methods Thirty-five MFS patients diagnosed by original GHENT criteria, with maximum aortic root diameter of 45 mm and free from previous aortic dissection or cardiac/aortic surgery and non-severe aortic regurgitation were consecutive enrolled and followed-up. CMR and echocardiography were performed less than 2 months apart. Baseline and final aortic root diameter were measured on CMR images. To quantify aortic root cyclic elongation by echocardiography, two regions of interests were manually created covering both walls in a parasternal long-axis view and tracked along the cardiac cycle (Figure 1). Longitudinal strain was computed as the average of maximum increase in relative distance of several sub-regions covering both walls. CMR-derived AAo longitudinal strain was available in 29 patients. Intra-observer reproducibility was tested in 15 patients via intraclass correlation coefficient (ICC) for single-rater absolute agreement. Results Aortic root longitudinal strain by echocardiography was mildly related to CMR-derived AAo longitudinal strain (R = 0.27) and was larger compared to CMR-derived values (16.2 ± 6.0 vs 11.3 ± 4.3). Reproducibility was high, with ICC of 0.811, R = 0.802, p Conclusions The measurement of aortic root longitudinal strain by speckle-tracking echocardiography is feasible. Aortic root longitudinal strain is an independent predictor of progressive dilation in MFS patients. This may permit the improvement of risk-stratification in aortic diseases in large scale studies. Abstract Figure 1

Background Aortic regurgitation (AR) can be evaluated by cardiac magnetic resonance (CMR).The most commonly used method to quantify AR is direct measurement using phase contrast (PC) imaging, at the aortic root (as close as possible to the aortic valve), for the calculation of regurgitant fraction (RF). Aortic distensibility (AD) may affect aortic valve dynamics and, as a result, aortic regurgitation grade. However, the impact of aortic distensibility in this evaluation remains unknown. Purpose The aim of the study was to evaluate the relationship between AD and AR in patients with different aortic valve anatomy. Methods 213 patients with different AR severity grades and aortic valve anatomy (tricuspid (TAV) and bicuspid valve (BAV) patients) were enrolled (32.2% female, 74% BAV, 55.5±15.4 years), excluding connective tissue disease. All patients underwent a CMR study with PC sequences for the evaluation of regurgitant fraction at the aortic valve level. AR was considered as mild (30%) depending on RF value. Furthermore we used cine-sequences to estimate aortic diameters and distensibilities, using Art Fun software. Distensibility was calculated as (change in aortic area between systole and diastole/diastolic area)/brachial pulse pressure. Results 159 (73.7%) AR were mild, 30 (14.1%) moderate and 24 (11.3%) severe. RF significantly correlated with aortic root diameter (r=0.337, p Conclusions In our study, aortic regurgitation is positively related to descending aorta distensibility in BAV patients, regardless of age and aortic root diameter. Thus, AD may play a role in the evaluation of AR in case of bicuspid valves. In contrast, in TAV patients, distensibility does not seem to influence the assessment of AR severity. Descending aorta distensibility Funding Acknowledgement Type of funding source: Other. Main funding source(s): Research grant provided by the Cardiopath PhD program

Background Thoracic endovascular aortic repair (TEVAR) is widely used for the treatment of blunt traumatic thoracic aortic injuries. Aortic flow dynamics and mechanical implications of this intervention are poorly investigated and may be of particular interest in the long-term follow-up of these mostly young patients. Purpose To assess whether the presence of TEVAR in a cohort of otherwise healthy subjects was related to dilation of the proximal aorta or increase in aortic stiffness and flow alterations. Methods Nineteen patients who underwent TEVAR implantation after a traumatic injury of the thoracic descending aorta (DAo) (10.0±6.1 years from intervention) and 44 healthy volunteers (HV) underwent 4D flow CMR to compute ascending aorta (AAo) pulse wave velocity (PWV), a marker of aortic stiffness, systolic flow reversal ratio (SFRR), quantifying backward flow during systole and in-plane rotational flow (IRF), measuring in-plane strength of helical flow. IRF and SFRR were assessed at 20 planes between the sinotubular junction and the mid thoracic DAo. Aortic diameters were measured using double-oblique cine CMR. Results Patients with TEVAR and HV did not differ in age, sex, body surface area, blood pressure and DAo diameter distal to TEVAR (Table). However, TEVAR patients presented larger diameters at the sinus of Valsalva and AAo, increased AAo PWV and strong flow alterations: IRF was reduced from the distal AAo to the proximal DAo, while SFRR was increased in the whole thoracic aorta (Figure). Conclusions In patients with blunt traumatic thoracic aortic injury treated with TEVAR the aorta proximal to TEVAR is dilated, stiffer and present potentially pathogenic flow conditions. Longitudinal studies are needed to assess whether these alterations have prognostic value and may improve clinical prevention and management of these patients. Figure 1. IRF and SFRR in healthy vs TEVAR Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): This study has been funded by Instituto de Salud Carlos III, Spanish Ministry of Science and Innovation (PI19/01480). Guala A. received funding from the Spanish Ministry of Science, Innovation and Universities (IJC2018-037349-I).

Background Aortic dilation in bicuspid aortic valve (BAV) patients has been related to altered flow patterns, which contribute to aortic wall degeneration. However, preventive aortic replacement is currently based on a diameter threshold. Several studies on excised BAV reported wide variability of fusion extent. Purpose To unveil whether leaflet fusion extent can be quantified by CMR and is related to aortic dilation and flow abnormalities in non-dysfunctional BAV. Methods One hundred and twenty adults with non-dysfunctional BAV and no previous aortic or aortic valve surgery and 28 healthy volunteers underwent double-oblique cine and 4D flow CMR. BAV patients with two sinuses of Valsalva or left and non-coronary cusps fusion were excluded. Peak systolic circumferential wall shear stress (WSSc) and pulse wave velocity (PWV) in the ascending aorta (AAo) were assessed by 4D flow CMR. Fusion length between leaflets was measured using a stack of double-oblique cine CMR images of the aortic valve. Results The length of the fusion was effectively measured in 112/120 (93%) patients with good reproducibility (ICC = 0.826) and showed great variability (range 2.3–15.4 mm, 7.8±3.2 mm and tertiles cut-off points 6 and 9.3 mm). In multivariate analysis adjusted for clinical and demographic characteristics and PWV, fusion length was independently associated with the diameter at the sinus of Valsalva (p=0.002) and the AAo (p=0.02) (Table). WSSc progressively increased with larger fusion length (Figure), with statistical significance (p Conclusions Bicuspid aortic leaflet fusion length varies considerably, and it is independently associated with AAo and aortic root dilation, possibly through flow alterations. Figure 1. Maps of circumferential WSS Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): This study has been partially funded by Instituto Carlos III, Spanish Ministry of Science and Innovation (PI17/00381). Guala A. has received funding from the Spanish Ministry of Science, Innovation and Universities (IJC2018-037349-I).

Background EACVI recommends the use of an “integrative approach”, using several parameters, in aortic regurgitation (AR) quantification. This approach is easily achieved by echocardiography although cardiovascular magnetic resonance (CMR) remains the gold standard for the quantification of regurgitant fraction (RF). Purpose The aim of the study was to analyze the accuracy of descending aorta (DA) diastolic reverse flow in the assessment of chronic AR severity by CMR to identify an additional parameter. Methods 188 patients (34% female, 54.6±15.6 years) with different severity grades of chronic AR were enrolled. All patients underwent a CMR study. Aortic regurgitation was considered as absent (≤1%), mild (≤15%), moderate (≤15%) or severe (≥30%) depending on RF value at valve level. Furthermore, cine-sequences were used to estimate aortic diameters and distensibilities using Art Fun software. Velocity-time integral (VTI) of reverse flow in DA was calculated from maximum velocity curves by an in-house MatLab code. Results AR was absent in 21 (12%) patients, mild in 114 (62.9%), moderate in 23 (12.7%) and severe in 21 (11.6%).DA VTI reverse flow was significantly higher as was the RF at valve level (r=0.805, p Conclusions VTI reverse flow in DA correlates strongly with the degree of AR and may be useful in the assessment of its severity. Neverthless, owing to the influence of other factors (aortic distensibility and aortic root diameter), it cannot be used as a single parameter in the quantification of AR severity by CMR. Scatter Plot graphs Funding Acknowledgement Type of funding source: Other. Main funding source(s): research grant provided by the Cardiopath PhD program

Funding Acknowledgements Spanish Ministry of Economy and Competitiveness RTC-2016-5152-1, ISCIII PI17/00381, La Marató de TV3 (20151330), Eur FP7/People 267128 and CIBERCV Background Histological findings of fibrillin-1 deficiency in bicuspid aortic valve (BAV) ascending aorta (AAo), as observed in Marfan (MFS), supported the existence of intrinsic aortic wall abnormalities, but recent studies reported the absence of an intrinsic impairment in stiffness. A recent study in MFS showed that AAo longitudinal strain was reduced in MFS and predicted dilation and aortic events. This parameter has not been studied in BAV. Purpose We investigated whether ascending aorta longitudinal strain is intrinsically altered in BAV with respect to tricuspid aortic valve (TAV) individuals. Methods 80 BAV, 31 healthy volunteers (HV) and 29 TAV with AAo aneurysm, all without moderate valvular disease, were consecutively included. AAo dilation was defined as a z-score > 2. The 1.5T CMR protocol included a set of 2D cine CMR stacks covering the proximal aorta in saggital, coronal and axial views. AAo longitudinal strain was computed by an in-house Matlab code performing a feature tracking of the aortic valve in each of the cine images. Results Twenty (25%) of BAV had AAo dilation. AAo longitudinal strain was lower in non-dilated BAV compared to HV, but the difference was not significant in multivariate analysis adjusted for AAo diameter and systolic blood pressure. Similarly, the difference between dilated BAV and dilated TAV found in univariate analysis was not confirmed by multivariate analysis. On the other hand, both dilated BAV and TAV showed decreased AAo longitudinal strain compared to HV, which were confirmed in multivariate analyses. Conclusions AAo longitudinal strain, a marker of aortic stiffness with predictive value in MFS, is not altered in BAV patients compared to TAV matched for dilation prevalence. Reduced AAo longitudinal strain was independently associated with dilation in both BAV and TAV. Table 1 HV vs. NON-DILATED BAV DILATED BAV vs DILATED TAV HV vs. DILATED BAV HV vs. DILATED TAV HV NON-DILATED BAV Univariate /multivariate p-value DILATED TAV DILATED BAV Univariate /multivariate p-value Univariate/ Multivariate p-value Univariate p-value N 31 20 29 60 Age [years 35 ± 8 49 ± 16

Background Peripheral aneurysms are not included in the diagnostic criteria for Marfan syndrome (MFS); however, their real prevalence in MFS is unknown. Furthermore, they are commonly seen in other genetic entities such as Loeys-Dietz syndrome. We aimed to investigate the prevalence of peripheral aneurysm in Marfan syndrome. Methods Patients with clinical criteria of Marfan syndrome and identified FBN1 mutation were evaluated. Only patients with either MRI or CT angiography assessing peripheral vessels were included in this study. MRI and CT angiography studies were retrospectively evaluated to detect the presence of peripheral aneurysms. Aortic dissection-related arterial dilations were excluded. Aortic events and those related to aneurysm complications were collected during follow-up. Results Two hundred and nine patients with Marfan and FBN1 mutation were evaluated. Of these 136 (65.1%) had undergone either MRA or CTA with peripheral artery study during follow-up. Mean age at the last follow-up visit was 42.4±14.1yrs; 54.4% were men, and mean follow-up 7.3±3.1 years. Sixty-six aneurysms were identified in 42 (30.9%) patients. The most common locations were the iliac arteries in 23. The rest were: renal (7), vertebral (5), splenic (5), coeliac (3), brachiocephalic (1), subclavian (3), carotid (3), axillary (2), internal mammary (3), femoral (2), hypogastric (3), bronchial (2), coronary (1), hepatic (1), lumbar (1), gastroduodenal (1) and popliteal (1). Twenty-six patients (61.9%) had more than one peripheral aneurysm, and only 4 required surgery. Patients with peripheral aneurysms were older (47.2±14.3yrs vs 40.2±13.6yrs, p=0.06) and more frequently men (69.0% vs 47.9% p=0.026). Although patients with peripheral aneurysms did not more frequently have aortic dissection (16.7% vs 17.0%, p=0.586), they did more frequently have aortic surgery (73.8% vs 47.9% p=0.05). Conclusions Peripheral aneurysms are present in one third of Marfan syndrome patients and are related to age and more advanced aortic disease. Systematic use of whole-body vascular assessment in Marfan patients can provide a comprehensive evaluation of the entire arterial system, identifying other sites of vascular involvement at risk of potential complications, and the subgroup of patients with more aggressive vascular disease expression.

Background The impact of recent advances on the management of acute aortic syndrome (AAS) is usually reported by centres with great experience in aortic diseases. Current data on the management of this specific disease in Spain remains unknown. Purpose The Spanish Registry of Acute Aortic Syndrome (RESA-III) was established to assess current results in the management of AAS in a large cohort of hospitals from the same geographical area. Methods All patients admitted for AAS to 29 Spanish tertiary hospitals were enroled over 18 months (2017/2018). Results 574 patients, (68% men; mean age 64±14y; range 18–99) were prospectively and consecutively included. Aortic dissection was the underlying disease in 474 (82.6%) (375 type A, 99 type B), aortic haematoma in 76 (13.2%) (43 type A and 33 type B) and penetrating ulcer in 24 (4.2%) (7 type A and 17 type B). From the subgroup of type A AAS (74% n=425), 81% underwent surgical treatment and medical management was chosen in the remaining 19%. 78 patients did not undergo surgery principally because of severe comorbidities (n=34) or advanced age (n=24), patient refusal (n=7), or presence of an intramural haematoma (n=2). Regarding the cohort of patients with type B AAS (26% n=149), 52% were managed only medically, 37% with endovascular treatment, and 11% underwent open surgery. Endovascular treatment was indicated owing to recurrent pain (n=19), progressive vessel dilation (n=9), dissection expansion (n=5), peripheral (n=5) or visceral ischaemia (n=4), high blood pressure (n=18), peripheral bleeding (n=17) or haemodynamical instability (n=8). Overall type A mortality during hospitalisation was 36.4%; 26.4% in surgically treated and 79.4% in medically-treated patients (p=0.001). In type B AAS, overall mortality was 19.1%; 21.9% in the treated medically subgroup, 43.8% in those treated with open surgery and 7.8% in the endovascular treatment cohort (p=0.004). Conclusion Despite significant advances in acute aortic syndrome diagnosis and management, in-hospital mortality remains high. In type A AAS, medical management rate was too high (19%); however, in type B AAS, endovascular treatment yielded excellent results with less mortality than medical management (7.8% vs 19.1%, respectively). Our data support the need for continued improvement in the management of acute aortic syndrome.

Background The most common cardiovascular complications in Marfan syndrome (MFS) are aortic root dilation and type A aortic dissections. Elective aortic root surgery is indicated when maximum aortic diameter is larger than a defined threshold or in the case of fast-progressing dilation. However, maximum aortic diameter is limited for the prediction of aortic events. Indeed, a large international registry of acute aortic syndromes reported that as much as 40% of aortic dissections happen with maximum aortic diameter lower than 50 mm. Consequently, there is a need for new, non-invasive biomarkers to improve the prediction of aortic complications. Purpose The aim of the present study was to assess if proximal aorta circumferential and longitudinal strain and ascending aorta distensibility were associated with progressive aortic dilation and incidence of aortic events in Marfan syndrome patients. Methods Eighty seven Marfan syndrome patients free from previous cardiac/aortic surgery or dissection, were prospectively included in a multicenter follow-up. Patients were diagnosed by original Ghent criteria. Proximal aorta longitudinal and circumferential strain and distensibility were computed from baseline cine CMR images by means of feature-tracking. The predictive capacity of each stiffness biomarkers was separately tested with multivariable linear regression analysis (aortic growth) and with Cox logistic regression analysis (aortic events), both corrected for clinical and demographic variables, including baseline maximum aortic diameter. Results During a follow-up of 81.6±17 months, mean diameter growth-rate was 0.65±0.67 mm/year and z-score growth rate was 0.07±0.13 / year. Elective aortic root replacement was performed in 11 patients while two patients presented type A aortic dissection.Baseline proximal aorta longitudinal strain was independently related to diameter growth-rate (p=0.001), z-score growth-rate (p=0.018) and aortic events (p=0.018). Conversely, neither circumferential strain nor distensibility were independent predictors of diameter growth-rate (p=0.385 and p=0.381, respectively), z-score growth-rate (p=0.515 and p=0.484, respectively) and aortic events (p=0.064 and p=0.205, respectively). Conclusions Proximal aorta longitudinal strain predicts aortic root dilation and major aortic events in Marfan syndrome patients beyond aortic root diameter and clinical and demographic characteristics. Acknowledgement/Funding ISCIII PI14/0106, La Maratό de TV3 (20151330) and CIBERCV. Guala A. FP7/People n° 267128