Prognostic impact of shock in patients with type A acute aortic syndrome. Results of a nationwide multicenter study.

OBJECTIVE: To evaluate the clinical characteristics, imaging findings, treatment, and prognosis of patients with type A acute aortic syndrome (AAS-A) presenting with shock. To assess the impact of surgery on this patient population. METHODS: The study included 521 patients with A-AAS enrolled in the Spanish Registry of Acute Aortic Syndrome (RESA-III) from January 2018 to December 2019. The RESA-III is a prospective, multicenter registry that contains AAS data from 30 tertiary-care hospitals. Patients were classified into two groups according to their clinical presentation, with or without shock. Shock was defined as persistent systolic blood pressure <80 mmHg despite adequate volume resuscitation. RESULTS: 97 (18.6%) patients with A-AAS presented with shock. Clinical presentation with syncope was much more common in the Shock group (45.4% vs 10.1%, p = 0.001). Patients in the Shock group had more complications at diagnosis and before surgery: cardiac tamponade (36.2% vs 9%, p < 0.001), acute renal failure (28.9% vs 18.2%, p = 0.018), and need for orotracheal intubation (40% vs 9.1%, p < 0.001). There were no significant differences in aortic regurgitation (51.6% vs 46.7%, p = 0.396) between groups. In-hospital mortality was higher among patients with shock (48.5% vs 27.4%, p < 0.001). Surgery was associated with a significant mortality reduction both in patients with and without shock. Surgery had an independent protective effect on mortality (OR 0.03, 95% CI (0.00-0.32)). CONCLUSION: Patients with AAS-A admitted with shock have a heavily increased risk of mortality. Syncope and pericardial effusion at diagnosis are strongly associated with shock. Surgery was independently associated with a mortality reduction in patients with AAS-A and shock.

Specific Multiomic Profiling in Aortic Stenosis in Bicuspid Aortic Valve Disease.

INTRODUCTION AND PURPOSE: Bicuspid aortic valve (BAV) disease is associated with faster aortic valve degeneration and a high incidence of aortic stenosis (AS). In this study, we aimed to identify differences in the pathophysiology of AS between BAV and tricuspid aortic valve (TAV) patients in a multiomics study integrating metabolomics and transcriptomics as well as clinical data. METHODS: Eighteen patients underwent aortic valve replacement due to severe aortic stenosis: 8 of them had a TAV, while 10 of them had a BAV. RNA sequencing (RNA-seq) and proton nuclear magnetic resonance spectroscopy (1H-NMR) were performed on these tissue samples to obtain the RNA profile and lipid and low-molecular-weight metabolites. These results combined with clinical data were posteriorly compared, and a multiomic profile specific to AS in BAV disease was obtained. RESULTS: H-NMR results showed that BAV patients with AS had different metabolic profiles than TAV patients. RNA-seq also showed differential RNA expression between the groups. Functional analysis helped connect this RNA pattern to mitochondrial dysfunction. Integration of RNA-seq, 1H-NMR and clinical data helped create a multiomic profile that suggested that mitochondrial dysfunction and oxidative stress are key players in the pathophysiology of AS in BAV disease. CONCLUSIONS: The pathophysiology of AS in BAV disease differs from patients with a TAV and has a specific RNA and metabolic profile. This profile was associated with mitochondrial dysfunction and increased oxidative stress.

Changes in echocardiographic parameters over time in paradoxical low-flow low-gradient aortic stenosis.

AIMS: To assess the progression of the disease and evolution of the main echocardiographic variables for quantifying AS in patients with severe low-flow low-gradient (LFLG) AS compared to other severe AS subtypes. METHODS AND RESULTS: Longitudinal, observational, multicenter study including consecutive asymptomatic patients with severe AS (aortic valve area, AVA < 1.0 cm²) and normal left ventricle ejection fraction (LVEF ≥ 50%). Patients were classified according to baseline echocardiography into: HG (high gradient; mean gradient ≥ 40 mmHg), NFLG (normal-flow low-gradient; mean gradient < 40 mmHg, indexed systolic volume (SVi) > 35mL/m2), or LFLG (mean gradient < 40 mmHg, SVi ≤ 35 mL/m²). AS progression was analyzed by comparing patients' baseline measurements and their last follow-up measurements or those taken prior to aortic valve replacement (AVR). Of the 903 included patients, 401 (44.4%) were HG, 405 (44.9%) NFLG, and 97 (10.7%) LFLG. Progression of the mean gradient in a linear mixed regression model was greater in low-gradient groups: LFLG vs. HG (regression coefficient 0.124, P = 0.005) and NFLG vs. HG (regression coefficient 0.068, P = 0.018). No differences were observed between the LFLG and NFLG groups (regression coefficient 0.056, P = 0.195). However, AVA reduction was slower in the LFLG group compared to the NFLG (P < 0.001). During follow-up, in conservatively-managed patients, 19.1% (n = 9) of LFLG patients evolved to having NFLG AS and 44.7% (n = 21) to having HG AS. In patients undergoing AVR, 58.0% (n = 29) of LFLG baseline patients received AVR with a HG AS. CONCLUSION: LFLG AS shows an intermediate AVA and gradient progression compared to NFLG and HG AS. The majority of patients initially classified as having LFLG AS changed over time to having other severe forms of AS, and most of them received AVR with a HG AS.

Multimodality imaging in thoracic aortic diseases: a clinical consensus statement from the European Association of Cardiovascular Imaging and the European Society of Cardiology working group on aorta and peripheral vascular diseases.

Imaging techniques play a pivotal role in the diagnosis, follow-up, and management of aortic diseases. Multimodality imaging provides complementary and essential information for this evaluation. Echocardiography, computed tomography, cardiovascular magnetic resonance, and nuclear imaging each have strengths and limitations in the assessment of the aorta. This consensus document aims to review the contribution, methodology, and indications of each technique for an adequate management of patients with thoracic aortic diseases. The abdominal aorta will be addressed elsewhere. While this document is exclusively focused on imaging, it is of most importance to highlight that regular imaging follow-up in patients with a diseased aorta is also an opportunity to check the patient's cardiovascular risk factors and particularly blood pressure control.

Prognosis of Paradoxical Low-Flow Low-Gradient Aortic Stenosis: A Severe, Non-critical Form, With Surgical Treatment Benefits.

Objectives: To determine the risk of mortality and need for aortic valve replacement (AVR) in patients with low-flow low-gradient (LFLG) aortic stenosis (AS). Methods: A longitudinal multicentre study including consecutive patients with severe AS (aortic valve area [AVA] < 1.0 cm2) and normal left ventricular ejection fraction (LVEF). Patients were classified as: high-gradient (HG, mean gradient ≥ 40 mmHg), normal-flow low-gradient (NFLG, mean gradient < 40 mmHg, indexed systolic volume (SVi) > 35 ml/m2) and LFLG (mean gradient < 40 mmHg, SVi ≤ 35 ml/m2). Results: Of 1,391 patients, 147 (10.5%) had LFLG, 752 (54.1%) HG, and 492 (35.4%) NFLG. Echocardiographic parameters of the LFLG group showed similar AVA to the HG group but with less severity in the dimensionless index, calcification, and hypertrophy. The HG group required AVR earlier than NFLG (p < 0.001) and LFLG (p < 0.001), with no differences between LFLG and NFLG groups (p = 0.358). Overall mortality was 27.7% (CI 95% 25.3-30.1) with no differences among groups (p = 0.319). The impact of AVR in terms of overall mortality reduction was observed the most in patients with HG (hazard ratio [HR]: 0.17; 95% CI: 0.12-0.23; p < 0.001), followed by patients with LFLG (HR: 0.25; 95% CI: 0.13-0.49; p < 0.001), and finally patients with NFLG (HR: 0.29; 95% CI: 0.20-0.44; p < 0.001), with a risk reduction of 84, 75, and 71%, respectively. Conclusions: Paradoxical LFLG AS affects 10.5% of severe AS, and has a lower need for AVR than the HG group and similar to the NFLG group, with no differences in mortality. AVR had a lower impact on LFLG AS compared with HG AS. Therefore, the findings of the present study showed LFLG AS to have an intermediate clinical risk profile between the HG and NFHG groups.

A systematic study and literature review of parental somatic mosaicism of FBN1 pathogenic variants in Marfan syndrome.

BACKGROUND: A proportion of de novo variants in patients affected by genetic disorders, particularly those with autosomal dominant (AD) inheritance, could be the consequence of somatic mosaicism in one of the progenitors. There is growing evidence that germline and somatic mosaicism are more common and play a greater role in genetic disorders than previously acknowledged. In Marfan syndrome (MFS), caused by pathogenic variants in the fibrillin-1 gene (FBN1) gene, approximately 25% of the disease-causing variants are reported as de novo. Only a few cases of parental mosaicism have been reported in MFS. METHODS: Employing an amplicon-based deep sequencing (ADS) method, we carried out a systematic analysis of 60 parents of 30 FBN1 positive, consecutive patients with MFS with an apparently de novo pathogenic variant. RESULTS: Out of the 60 parents studied (30 families), the majority (n=51, 85%) had a systemic score of 0, seven had a score of 1 and two a score of 2, all due to minor criteria common in the normal population. We detected two families with somatic mosaicism in one of the progenitors, with a rate of 6.6% (2/30) of apparently de novo cases. CONCLUSIONS: The search for parental somatic mosaicism should be routinely implemented in de novo cases of MFS, to offer appropriate genetic and reproductive counselling as well as to reveal masked, isolated clinical signs of MFS in progenitors that may require specific follow-up.

Predictive Value of Cardiac Magnetic Resonance Feature Tracking after Acute Myocardial Infarction: A Comparison with Dobutamine Stress Echocardiography.

In acute ST-segment elevation myocardial infarction (STEMI) late gadolinium enhancement (LGE) may underestimate segmental functional recovery. We evaluated the predictive value of cardiac magnetic resonance (CMR) feature-tracking (FT) for functional recovery and whether it incremented the value of LGE compared to low-dose dobutamine stress echocardiography (LDDSE) and speckle-tracking echocardiography (STE). Eighty patients underwent LDDSE and CMR within 5-7 days after STEMI and segmental functional recovery was defined as improvement in wall-motion at 6-months CMR. Optimal conventional and FT parameters were analyzed and then also applied to an external validation cohort of 222 STEMI patients. Circumferential strain (CS) was the strongest CMR-FT predictor and addition to LGE increased the overall accuracy to 74% and was especially relevant in segments with 50-74% LGE (AUC 0.60 vs. 0.75, p = 0.001). LDDSE increased the overall accuracy to 71%, and in the 50-74% LGE subgroup improved the AUC from 0.60 to 0.69 (p = 0.039). LGE + CS showed similar value as LGE + LDDSE. In the validation cohort, CS was also the strongest CMR-FT predictor of recovery and addition of CS to LGE improved overall accuracy to 73% although this difference was not significant (AUC 0.69, p = 0.44). Conclusion: CS is the strongest CMR-FT predictor of segmental functional recovery after STEMI. Its incremental value to LGE is comparable to that of LDDSE whilst avoiding an inotropic stress agent. CS is especially relevant in segments with 50-74% LGE where accuracy is lower and further testing is frequently required to clarify the potential for recovery.

Clinical Risk Prediction in Patients With Left Ventricular Myocardial Noncompaction.

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.

Aortic Branch Aneurysms and Vascular Risk in Patients With Marfan Syndrome.

BACKGROUND: Aortic branch aneurysms are not included in the diagnostic criteria for Marfan syndrome (MFS); however, their prevalence and eventual prognostic significance are unknown. OBJECTIVES: The goal of this study was to assess the prevalence of aortic branch aneurysms in MFS and their relationship with aortic prognosis. METHODS: MFS patients with a pathogenic FBN1 genetic variant and at least one magnetic resonance or computed tomography angiography study assessing aortic branches were included. Aortic events and those related to aneurysm complications were recorded during follow-up. RESULTS: A total of 104 aneurysms were detected in 50 (26.7%) of the 187 patients with MFS (mean age 37.9 ± 14.4 years; 54% male) included in this study, with the iliac artery being the most common location (45 aneurysms). Thirty-one patients (62%) had >1 peripheral aneurysm, and surgery was performed in 5 (4.8%). Patients with aneurysms were older (41.9 ± 12.7 years vs. 36.7 ± 14.8 years; p = 0.040) and had more dilated aortic root (42.2 ± 6.4 mm vs. 38.8 ± 8.0 mm; p = 0.044) and dyslipidemia (31.0% vs. 9.7%; p = 0.001). In a subgroup of 95 patients with no previous aortic surgery or dissection followed up for 3.3 ± 2.6 years, the presence of arterial aneurysms was associated with a greater need for aortic surgery (hazard ratio: 3.4; 95% confidence interval: 1.1 to 10.3; p = 0.028) in a multivariable Cox analysis adjusted for age and aortic diameter. CONCLUSIONS: Aortic branch aneurysms are present in one-quarter of patients with MFS and are related to age and aortic dilation, and they independently predict the need for aortic surgery. The systematic use of whole-body vascular assessment is recommended to identify other sites of vascular involvement at risk for complications and to define the subgroup of patients with more aggressive aortic disease.

A single nucleotide deletion resulting in a frameshift in exon 4 of TAB2 is associated with a polyvalular syndrome.

OBJECTIVE: Polyvalvularmyxomatous degeneration is a rare clinical condition. A 51-year-old male patient presented at our centre with all four heart valves with myxomatous degeneration and severe mitral and aortic regurgitation due to leaflet prolapse. The patient referred five further family members with valvular heart disease at different stages of presentation. The aim of this study was to investigate the genetic basis of this familial polyvalvularmyxomatous degeneration which was associated with mild dysmorphic facial anomalies and short stature. DESIGN: A detailed family history was recorded. Nine members of the family, affected or not by valvular heart disease, were studied clinically, echocardiographically and by detailed genetic analyses. RESULTS: Six of the nine family members had echocardiographic features of different degrees of degenerative heart valve disease. In addition, the affected subjects shared similar mild dysmorphic facial anomalies and short stature. Exome sequencing identified a rare heterozygous single nucleotide deletion in the TAB2 gene in all affected family members, which was absent in the unaffected members. CONCLUSIONS: A variant in the TAB2 gene is proposed as the cause of syndromic congenital heart disease, displaying congenital myxomatous degenerative heart valve disease, mild dysmorphic fascial anomalies and short stature in this family.

Type B Aortic Dissection in Young Individuals With Confirmed and Presumed Heritable Thoracic Aortic Disease.

BACKGROUND: To investigate clinical course of patients with type B aortic dissection (TBAD) occurring at a young age with confirmed or suspected heritable thoracic aortic disease. METHODS: Individuals with TBAD occurring at an age <50 years enrolled in the National Registry of the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Consortium were selected for analysis. Three cohorts were compared: Marfan syndrome (MFS TBAD), nonsyndromic familial TBAD (FTBAD), and sporadic TBAD. Demographics, comorbidities, aortic dissection details, and repair were compared. RESULTS: A total of 150 individuals met inclusion criteria (mean age at TBAD, 36.9 ± 9 years): 73 MFS TBAD, 42 FTBAD, and 35 sporadic TBAD. The cohort of sporadic TBAD had more male patients (71.4%) and fewer individuals of European descent (51.4%) compared with MFS TBAD (57.5% male, 84.9% European descent) and FTBAD (59.5% male, 90.5% European descent). There was a stepwise increase in hypertension prevalence across the cohorts (28.8% MFS, 59.5% FTBAD, 71.4% sporadic TBAD, P < .001). Repair of the descending thoracic aorta was performed in 92 cases (67.1% in MFS, 61.9% in FTBAD, and 48.6% sporadic TBAD, P = .18) at a mean of 3.4 ± 5.4 years from TBAD. The repair extent varied. The largest extent of repair was in MFS TBAD, in which thoracoabdominal aortic aneurysm repair was performed in 56.2% compared with 35.7% FTBAD and 17.1% sporadic TBAD (P < .001). CONCLUSIONS: Control of hypertension is an essential component of care to decrease the risk of TBAD. Over half of the young individuals with TBAD require aortic repair, and individuals with MFS undergo a larger anatomical extent of repair after TBAD.

Percutaneous cardiological intervention and cardiac surgery: patient-centered care. Position statement of the Spanish Society of Cardiology.

The percutaneous treatment of coronary artery disease and some structural cardiovascular diseases has undergone spectacular changes. More and more patients with different types of heart disease are being treated by percutaneous or transcatheter interventions, with no such increase in patients undergoing cardiac surgery. This situation has led to different types of approach, requiring an objective analysis that includes all the factors possibly influencing these changes. This document assesses the 2 scenarios where this problem is most evident: coronary revascularization and the treatment of aortic stenosis. The document analyzes the situation of coronary revascularization in Spain, and the causes that may explain the differences between the number of patients who currently undergo percutaneous revascularization and those who undergo coronary surgery. In contrast, treatment of aortic stenosis through transcatheter aortic valve implantation will lead to a foreseeable reduction in the number of candidates for surgical replacement. Several international scientific societies have published the requirements on training and experience and the necessary operator and center volumes to implement a transcatheter aortic valve implantation program, conditions that the Spanish Society of Cardiology, adopting a patient-centered approach, considers absolutely essential. Given that the 2 forms of intervention (percutaneous and surgical) are complementary, multidisciplinary patient assessment (Heart Team) remains crucial to offer the best treatment option. In this scenario of diverse approaches, a key figure is the clinical cardiologist. Finally, the changes currently occurring in the treatment of structural heart disease will, in future, lead to the performance of procedures requiring the participation of professionals from both specialties. This approach will require a redesign of current training programs.

Prognostic Value of Initial Left Ventricular Remodeling in Patients With Reperfused STEMI.

OBJECTIVES: This study sought to establish the best definition of left ventricular adverse remodeling (LVAR) to predict outcomes and determine whether its assessment adds prognostic information to that obtained by early cardiac magnetic resonance (CMR). BACKGROUND: LVAR, usually defined as an increase in left ventricular end-diastolic volume (LVEDV) is the main cause of heart failure after an ST-segment elevated myocardial infarction; however, the role of assessment of LVAR in predicting cardiovascular events remains controversial. METHODS: Patients with ST-segment elevated myocardial infarction who received percutaneous coronary intervention within 6 h of symptom onset were included (n = 498). CMR was performed during hospitalization (6.2 ± 2.6 days) and after 6 months (6.1 ± 1.8 months). The optimal threshold values of the LVEDV increase and the LV ejection fraction decrease associated with the primary endpoint were ascertained. Primary outcome was a composite of cardiovascular mortality, hospitalization for heart failure, or ventricular arrhythmia. RESULTS: The study was completed by 374 patients. Forty-nine patients presented the primary endpoint during follow-up (72.9 ± 42.8 months). Values that maximized the ability to identify patients with and without outcomes were a relative rise in LVEDV of 15% (hazard ratio [HR]: 2.1; p = 0.007) and a relative fall in LV ejection fraction of 3% (HR: 2.5; p = 0.001). However, the predictive model (using C-statistic analysis) failed to demonstrate that direct observation of LVAR at 6 months adds information to data from early CMR in predicting outcomes (C-statistic: 0.723 vs. 0.795). CONCLUSIONS: The definition of LVAR that best predicts adverse cardiovascular events should consider both the increase in LVEDV and the reduction in LV ejection fraction. However, assessment of LVAR does not improve information provided by the early CMR.

Proximal aorta longitudinal strain predicts aortic root dilation rate and aortic events in Marfan syndrome.

AIMS: Life expectancy in Marfan syndrome patients has improved thanks to the early detection of aortic dilation and prophylactic aortic root surgery. Current international clinical guidelines support the use of aortic root diameter as a predictor of complications. However, other imaging markers are needed to improve risk stratification. This study aim to ascertain whether proximal aorta longitudinal and circumferential strain and distensibility assessed by cardiac magnetic resonance (CMR) predict the aortic root dilation rate and aortic events in Marfan syndrome. METHODS AND RESULTS: One hundred and seventeen Marfan patients with no previous aortic dissection, cardiac/aortic surgery, or moderate/severe aortic regurgitation were prospectively included in a multicentre protocol of clinical and imaging follow-up. At baseline, CMR was performed and proximal aorta longitudinal strain and ascending aorta circumferential strain and distensibility were obtained. During follow-up (85.7 [75.0-93.2] months), the annual growth rate of aortic root diameter was 0.62 ± 0.65 mm/year. Fifteen patients underwent elective surgical aortic root replacement and four presented aortic dissection. Once corrected for baseline clinical and demographic characteristics and aortic root diameter, proximal aorta longitudinal strain, but not circumferential strain and distensibility, was an independent predictor of the aortic root diameter growth rate (P = 0.001, P = 0.823, and P = 0.997, respectively), z-score growth rate (P = 0.013, P = 0.672, and P = 0.680, respectively), and aortic events (P = 0.023, P = 0.096, and P = 0.237, respectively). CONCLUSION: Proximal aorta longitudinal strain is independently related to the aortic root dilation rate and aortic events in addition to aortic root diameter, clinical risk factors, and demographic characteristics in Marfan syndrome patients.

Comparison of distinctive clinical and cardiac magnetic resonance features between ST elevation myocardial infarction patients with incomplete myocardial rupture and those with moderate to severe pericardial effusion.

BACKGROUND: Whether patients with incomplete myocardial rupture (IMR) present distinctive clinical and cardiac magnetic resonance features from those with moderate-severe pericardial effusion (⩾10 mm (PE)) remains unknown. METHODS: We compared the clinical, angiographic and cardiac magnetic resonance characteristics of nine patients with IMR (diagnosed angiographically and/or by cardiac magnetic resonance) with 29 with PE, and also with 38 without IMR or PE with evidence of transmural necrosis (reference group) matched for age, gender and year of admission. RESULTS: Patients with IMR were younger than those with PE (p<0.001) but the two groups shared a higher rate of admission delay (78% and 41%) than those without IMR/PE (5%, p<0.001) and lower frequency of reperfusion therapy (44%, 55% and 100%, respectively, p<0.001). Thirteen patients with PE (45%) but only one IMR (11%) presented recurrent chest pain. IMR patients tended to present smaller infarct size at cardiac magnetic resonance (p=0.153 and 0.036) and number of segments with ⩾75% necrosis than PE patients and those without IMR/PE (p=0.098 and 0.029, respectively). Ten PE patients presented cardiac tamponade (35%). A control 2D-echocardiogram performed within two years in 71 patients (93%) documented a pseudoaneurysm in one PE and in one IMR patient. CONCLUSIONS: IMR is generally silent and occurs in younger patients with smaller infarct size than those with PE although both present late and are often untreated with reperfusion therapy. These findings may warrant imaging assessment in ST elevation myocardial infarction patients with delayed admission, particularly in absence of reperfusion, to rule out an IMR.

Aortic arch tortuosity, a novel biomarker for thoracic aortic disease, is increased in adults with bicuspid aortic valve.

INTRODUCTION: Arterial tortuosity has emerged as a predictor of adverse outcomes in congenital aortopathies using 3D reconstructed images. We validated a new method to estimate aortic arch tortuosity on 2D CT. We hypothesize that arch tortuosity may identify bicuspid aortic valve (BAV) patients at high risk to develop thoracic aortic aneurysms or aortic dissections (TAD). METHODS: BAV subjects with chest CT scans were retrospectively identified in our clinical records and matched to tricuspid aortic valve (TAV) controls by age, gender, and presentation with TAD. Subjects with prior ascending aortic intervention were excluded. Measurements included aortic arch tortuosity, length, angle, width and height. Total aortic tortuosity was estimated in subjects with available abdominal images. RESULTS: 120 BAV and 234 TAV subjects were included. Our 2D measurements were highly correlated with 3D midline arch measurements and had high inter- and intra-observer reliability. Compared to TAV, BAV subjects had increased arch tortuosity (median 1.76 [Q1-Q3: 1.62-1.95] vs. 1.63 [1.53-1.78], P < 0.01), length (149 [136-160] vs. 135 [122-152] mm, P < 0.01), height (46 [41-53] vs. 39 [34-47] mm, P < 0.01), and vertex acuity (70 [61-77] vs. 75 [68-81] degree, P < 0.01). In a multivariable analysis, arch tortuosity remained independently associated with BAV after adjusting for aortic diameter and other clinical characteristics. CONCLUSIONS: We found that aortic arch tortuosity is significantly increased in BAV and may identify BAV patients who are at increased risk for TAD. Further studies to evaluate the association between tortuosity and clinical outcomes are in progress.

Losartan Versus Atenolol for Prevention of Aortic Dilation in Patients With Marfan Syndrome.

BACKGROUND: Beta-blockers are the standard treatment in Marfan syndrome (MFS). Recent clinical trials with limited follow-up yielded conflicting results on losartan's effectiveness in MFS. OBJECTIVES: The present study aimed to evaluate the benefit of losartan compared with atenolol for the prevention of aortic dilation and complications in Marfan patients over a longer observation period (>5 years). METHODS: A total of 128 patients included in the previous LOAT (LOsartan vs ATenolol) clinical trial (64 in the atenolol and 64 in the losartan group) were followed up for an open-label extension of the study, with the initial treatment maintained. RESULTS: Mean clinical follow-up was 6.7 ± 1.5 years. A total of 9 events (14.1%) occurred in the losartan group and 12 (18.8%) in the atenolol group. Survival analysis showed no differences in the combined endpoint of need for aortic surgery, aortic dissection, or death (p = 0.462). Aortic root diameter increased with no differences between groups: 0.4 mm/year (95% confidence interval: 0.2 to 0.5) in the losartan and 0.4 mm/year (95% confidence interval: 0.3 to 0.6) in the atenolol group. In the subgroup analyses, no significant differences were observed considering age, baseline aortic root diameter, or type of dominant negative versus haploinsufficient FBN1 mutation. CONCLUSIONS: Long-term outcome of Marfan syndrome patients randomly assigned to losartan or atenolol showed no differences in aortic dilation rate or presence of clinical events between treatment groups. Therefore, losartan might be a useful, low-risk alternative to beta-blockers in the long-term management of these patients.