Un mètode més precís per al diagnòstic de la dissecció aòrtica

La dissecció aòrtica és una malaltia vascular en què s'esquinça la paret de l'aorta i que té una elevada mortalitat. Determinar les variables que caracteritzen la dissecció (localització, extensió, dimensions...) condiciona el tractament dels pacients i la seva evolució per la qual cosa és important disposar d'unes bones eines de diagnòstic. Els mètodes tradicionals presenten tots la mateixa precisió però s'usen diferencialment en funció de la fase de la malaltia o del que es vulgui observar. Recentment, s'ha desenvolupat l'ecocardiografia transesofàgica tridimensional que permet obtenir una major precisió anatòmica en les 3 dimensions de l'espai. El paper d'aquesta tècnica en aquesta malaltia no havia estat validat. Investigadors de l'Hospital Universitari Vall d'Hebron n'han comparat els resultats amb els mètodes tradicionals.La disección aórtica es una enfermedad vascular en la que se rasga la pared de la aorta y que tiene una elevada mortalidad. Determinar las variables que caracterizan la disección (localización, extensión, dimensiones ...) condiciona el tratamiento de los pacientes y su evolución por lo que es importante disponer de unas buenas herramientas de diagnóstico. Los métodos tradicionales presentan todos la misma precisión pero se usan diferencialmente en función de la fase de la enfermedad o lo que se quiera observar. Recientemente, se ha desarrollado la ecocardiografía transesofágica tridimensional que permite obtener una mayor precisión anatómica en las 3 dimensiones del espacio, cuyo papel en esta enfermedad no había sido validado. Investigadores del Hospital Universitario Vall d'Hebron han comparado sus resultados con los de los métodos tradicionales

Actualización en válvula aórtica bicúspide y complicaciones asociadas

Bicuspid aortic valve (BAV) is the most common congenital heart disease affecting 1-2% of the population. It affects more frequently males than females with a ratio of 3:1. Despite the fact that it has been historically considered a relatively benign disease, 35% of individuals with BAV will develop complications throughout life such as valve dysfunction, aortic aneurysm or aortic dissection. In spite of the relevance of this disease many aspects are not still clarified. The aim of this article is to show an updated version of the basic aspects of this pathology with emphasis on the latest developments related to the diagnosis, evolution and associated complications from a cardiac imaging viewpoint.La válvula aórtica bicúspide (VAB) es la cardiopatía congénita más frecuente y afecta a un 1-2% de la población. Afecta de forma más habitual a varones que a mujeres, con una relación 3:1. A pesar de haberse considerado históricamente una patología de carácter relativamente benigno, un 35% de los individuos con VAB desarrollará a lo largo de la vida complicaciones derivadas como disfunción valvular, endocarditis, aneurisma aórtico o disección aórtica. A pesar de la relevancia de esta patología quedan aún muchos aspectos por dilucidar. El objetivo de este artículo es mostrar una revisión actualizada de los aspectos básicos de esta patología haciendo hincapié en las últimas novedades relacionadas con su diagnóstico, evolución y complicaciones asociadas desde el punto de vista de la imagen cardíaca

Trombo atrapado en foramen oval permeable causante de infarto agudo de miocardio

A 57 year-old male was derived to our institution because of an acute myocardial infarction due to paradoxical embolism caused by thrombus passing through a patent ovale foramen. We present the main clinical and imaging findings of the case, along diagnostic and management options for this condition.Se presenta el caso de un varón de 57 años derivado al centro de los autores por infarto agudo de miocardio secundario a una embolia paradójica causada por un trombo atrapado en un foramen oval permeable. Se describen los principales hallazgos de las técnicas de imagen, características clínicas, diagnósticas y opciones terapéuticas

Diagnostic value of quantitative parameters for myocardial perfusion assessment in patients with suspected coronary artery disease by single- and dual-energy computed tomography myocardial perfusion imaging

To compare performance of visual and quantitative analyses for detecting myocardial ischaemia from single- and dual-energy computed tomography (CT) in patients with suspected coronary artery disease (CAD). Eighty-four patients with suspected CAD were scheduled for dual-energy cardiac CT at rest (CTA) and pharmacological stress (CTP). Myocardial CT perfusion was analysed visually and using three parameters: mean attenuation density (MA), transmural perfusion ratio (TPR) and myocardial perfusion reserve index (MPRI), on both single-energy CT and CT-based iodine images. Significant CAD was defined in AHA-segments by concomitant myocardial hypoperfusion identified visually or quantitatively (parameter < threshold) and coronary stenosis detected by CTA. Single-photon emission CT and invasive coronary angiography were used as reference. Perfusion-parameter cut-off values were calculated in a randomly-selected subgroup of 30 patients. The best-performing thresholds for TPR, MPRI and MA were 0.96, 23 and 0.5 for single-energy CT and 0.97, 47 and 0.3 for iodine imaging. For both CT-imaging modalities, TPR yielded the highest area under receiver operating characteristic curve (AUC) (0.99 and 0.97 for single-energy CT and iodine imaging, respectively, in vessel-based analysis) compared to visual analysis, MA and MPRI. Visual interpretation on iodine imaging resulted in higher AUC compared to that on single-energy CT in per-vessel (AUC: 0.93 vs 0.86, respectively) and per-patient (0.94 vs 0.93) analyses. Transmural perfusion ratio on both CT-imaging modalities is the best-performing parameter for detecting myocardial ischaemia compared to visual method and other perfusion parameters. Visual analysis on CT-based iodine imaging outperforms that on single-energy CT

Decreased rotational flow and circumferential wall shear stress as early markers of descending aorta dilation in Marfan syndrome : a 4D flow CMR study

Diseases of the descending aorta have emerged as a clinical issue in Marfan syndrome following improvements in proximal aorta surgical treatment and the consequent increase in life expectancy. Although a role for hemodynamic alterations in the etiology of descending aorta disease in Marfan patients has been suggested, whether flow characteristics may be useful as early markers remains to be determined. Seventy-five Marfan patients and 48 healthy subjects were prospectively enrolled. In- and through-plane vortexes were computed by 4D flow cardiovascular magnetic resonance (CMR) in the thoracic aorta through the quantification of in-plane rotational flow and systolic flow reversal ratio, respectively. Regional pulse wave velocity and axial and circumferential wall shear stress maps were also computed. In-plane rotational flow and circumferential wall shear stress were reduced in Marfan patients in the distal ascending aorta and in proximal descending aorta, even in the 20 patients free of aortic dilation. Multivariate analysis showed reduced in-plane rotational flow to be independently related to descending aorta pulse wave velocity. Conversely, systolic flow reversal ratio and axial wall shear stress were altered in unselected Marfan patients but not in the subgroup without dilation. In multivariate regression analysis proximal descending aorta axial (p = 0.014) and circumferential (p = 0.034) wall shear stress were independently related to local diameter. Reduced rotational flow is present in the aorta of Marfan patients even in the absence of dilation, is related to aortic stiffness and drives abnormal circumferential wall shear stress. Axial and circumferential wall shear stress are independently related to proximal descending aorta dilation beyond clinical factors. In-plane rotational flow and circumferential wall shear stress may be considered as an early marker of descending aorta dilation in Marfan patients. The online version of this article (10.1186/s12968-019-0572-1) contains supplementary material, which is available to authorized users

Aortic flow patterns and wall shear stress maps by 4D-flow cardiovascular magnetic resonance in the assessment of aortic dilatation in bicuspid aortic valve disease

Altres ajuts: This study has been funded by , La Marató de TV3 (project number 20151330). Guala A. has received funding from the European Union Seventh Framework Programme FP7/People under grant agreement n° 267128.In patients with bicuspid valve (BAV), ascending aorta (AAo) dilatation may be caused by altered flow patterns and wall shear stress (WSS). These differences may explain different aortic dilatation morphotypes. Using 4D-flow cardiovascular magnetic resonance (CMR), we aimed to analyze differences in flow patterns and regional axial and circumferential WSS maps between BAV phenotypes and their correlation with ascending aorta dilatation morphotype. One hundred and one BAV patients (aortic diameter ≤ 45 mm, no severe valvular disease) and 20 healthy subjects were studied by 4D-flow CMR. Peak velocity, flow jet angle, flow displacement, in-plane rotational flow (IRF) and systolic flow reversal ratio (SFRR) were assessed at different levels of the AAo. Peak-systolic axial and circumferential regional WSS maps were also estimated. Unadjusted and multivariable adjusted linear regression analyses were used to identify independent correlates of aortic root or ascending dilatation. Age, sex, valve morphotype, body surface area, flow derived variables and WSS components were included in the multivariable models. The AAo was non-dilated in 24 BAV patients and dilated in 77 (root morphotype in 11 and ascending in 66). BAV phenotype was right-left (RL-) in 78 patients and right-non-coronary (RN-) in 23. Both BAV phenotypes presented different outflow jet direction and velocity profiles that matched the location of maximum systolic axial WSS. RL-BAV velocity profiles and maximum axial WSS were homogeneously distributed right-anteriorly, however, RN-BAV showed higher variable profiles with a main proximal-posterior distribution shifting anteriorly at mid-distal AAo. Compared to controls, BAV patients presented similar WSS magnitude at proximal, mid and distal AAo (p = 0.764, 0.516 and 0.053, respectively) but lower axial and higher circumferential WSS components (p < 0.001 for both, at all aortic levels). Among BAV patients, RN-BAV presented higher IRF at all levels (p = 0.024 proximal, 0.046 mid and 0.002 distal AAo) and higher circumferential WSS at mid and distal AAo (p = 0.038 and 0.046, respectively) than RL-BAV. However, axial WSS was higher in RL-BAV compared to RN-BAV at proximal and mid AAo (p = 0.046, 0.019, respectively). Displacement and axial WSS were independently associated with the root-morphotype, and circumferential WSS and SFRR with the ascending-morphotype. Different BAV-phenotypes present different flow patterns with an anterior distribution in RL-BAV, whereas, RN-BAV patients present a predominant posterior outflow jet at the sinotubular junction that shifts to anterior or right anterior in mid and distal AAo. Thus, RL-BAV patients present a higher axial WSS at the aortic root while RN-BAV present a higher circumferential WSS in mid and distal AAo. These results may explain different AAo dilatation morphotypes in the BAV population. The online version of this article (10.1186/s12968-018-0451-1) contains supplementary material, which is available to authorized users

Oxidative Stress in Structural Valve Deterioration : A Longitudinal Clinical Study

The cause of structural valve deterioration (SVD) is unclear. Therefore, we investigated oxidative stress markers in sera from patients with bioprosthetic heart valves (BHVs) and their association with SVD. Blood samples were taken from SVD (Phase A) and BHV patients during the first 24 (Phase B1) and >48 months (Phase B2) after BHV implantation to assess total antioxidant capacity (TAC), malondialdehyde (MDA), and nitrotyrosine (NT). The results show that MDA levels increased significantly 1 month after surgery in all groups but were higher at 6 months only in incipient SVD patients. NT levels increased gradually for the first 24 months after implantation in the BHV group. Patients with transcatheter aortic valve implantation (TAVI) showed even higher levels of stress markers. After >48 months, MDA and NT continued to increase in BHV patients with a further elevation after 60-72 months; however, these levels were significantly lower in the incipient and established SVD groups. In conclusion, oxidative stress may play a significant role in SVD, increasing early after BHV implantation, especially in TAVI cases, and also after 48 months' follow-up, but decreasing when SVD develops. Oxidative stress potentially represents a target of therapeutic intervention and a biomarker of BHV dysfunctio

Aortic disease in Marfan syndrome is caused by overactivation of sGC-PRKG signaling by NO

AbstractThoracic aortic aneurysm, as occurs in Marfan syndrome, is generally asymptomatic until dissection or rupture, requiring surgical intervention as the only available treatment. Here, we show that nitric oxide (NO) signaling dysregulates actin cytoskeleton dynamics in Marfan Syndrome smooth muscle cells and that NO-donors induce Marfan-like aortopathy in wild-type mice, indicating that a marked increase in NO suffices to induce aortopathy. Levels of nitrated proteins are higher in plasma from Marfan patients and mice and in aortic tissue from Marfan mice than in control samples, indicating elevated circulating and tissue NO. Soluble guanylate cyclase and cGMP-dependent protein kinase are both activated in Marfan patients and mice and in wild-type mice treated with NO-donors, as shown by increased plasma cGMP and pVASP-S239 staining in aortic tissue. Marfan aortopathy in mice is reverted by pharmacological inhibition of soluble guanylate cyclase and cGMP-dependent protein kinase and lentiviral-mediated Prkg1 silencing. These findings identify potential biomarkers for monitoring Marfan Syndrome in patients and urge evaluation of cGMP-dependent protein kinase and soluble guanylate cyclase as therapeutic targets.</jats:p

Angiotensin receptor blockers and β blockers in Marfan syndrome: an individual patient data meta-analysis of randomised trials

Background: Angiotensin receptor blockers (ARBs) and β blockers are widely used in the treatment of Marfan syndrome to try to reduce the rate of progressive aortic root enlargement characteristic of this condition, but their separate and joint effects are uncertain. We aimed to determine these effects in a collaborative individual patient data meta-analysis of randomised trials of these treatments. Methods: In this meta-analysis, we identified relevant trials of patients with Marfan syndrome by systematically searching MEDLINE, Embase, and CENTRAL from database inception to Nov 2, 2021. Trials were eligible if they involved a randomised comparison of an ARB versus control or an ARB versus β blocker. We used individual patient data from patients with no prior aortic surgery to estimate the effects of: ARB versus control (placebo or open control); ARB versus β blocker; and indirectly, β blocker versus control. The primary endpoint was the annual rate of change of body surface area-adjusted aortic root dimension Z score, measured at the sinuses of Valsalva. Findings: We identified ten potentially eligible trials including 1836 patients from our search, from which seven trials and 1442 patients were eligible for inclusion in our main analyses. Four trials involving 676 eligible participants compared ARB with control. During a median follow-up of 3 years, allocation to ARB approximately halved the annual rate of change in the aortic root Z score (mean annual increase 0·07 [SE 0·02] ARB vs 0·13 [SE 0·02] control; absolute difference –0·07 [95% CI –0·12 to –0·01]; p=0·012). Prespecified secondary subgroup analyses showed that the effects of ARB were particularly large in those with pathogenic variants in fibrillin-1, compared with those without such variants (heterogeneity p=0·0050), and there was no evidence to suggest that the effect of ARB varied with β-blocker use (heterogeneity p=0·54). Three trials involving 766 eligible participants compared ARBs with β blockers. During a median follow-up of 3 years, the annual change in the aortic root Z score was similar in the two groups (annual increase –0·08 [SE 0·03] in ARB groups vs –0·11 [SE 0·02] in β-blocker groups; absolute difference 0·03 [95% CI –0·05 to 0·10]; p=0·48). Thus, indirectly, the difference in the annual change in the aortic root Z score between β blockers and control was –0·09 (95% CI –0·18 to 0·00; p=0·042). Interpretation In people with Marfan syndrome and no previous aortic surgery, ARBs reduced the rate of increase of the aortic root Z score by about one half, including among those taking a β blocker. The effects of β blockers were similar to those of ARBs. Assuming additivity, combination therapy with both ARBs and β blockers from the time of diagnosis would provide even greater reductions in the rate of aortic enlargement than either treatment alone, which, if maintained over a number of years, would be expected to lead to a delay in the need for aortic surgery. Funding: Marfan Foundation, the Oxford British Heart Foundation Centre for Research Excellence, and the UK Medical Research Council

Historia natural y tratamiento del síndrome aórtico agudo

El síndrome aórtico agudo es un proceso agudo de la pared aórtica que afecta a la capa media; incluye la disección aórtica, el hematoma intramural y la úlcera penetrante. En los últimos años, los avances en las técnicas de imagen han ayudado a conocer la historia natural de estas entidades y a comprender mejor el importante dinamismo de esta afección. A pesar de los importantes progresos en el tratamiento quirúrgico, la mortalidad en la fase aguda podría reducirse mediante la sospecha clínica precoz y la mejoría de la experiencia quirúrgica. La incorporación del tratamiento endovascular ha abierto nuevas perspectivas en el manejo de esta enfermedad y podría mejorar el pronóstico a largo plazo. En este artículo se revisan los conocimientos actuales de la historia natural y del manejo terapéutico de este síndrome