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.

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.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Usefulness of Transesophageal Echocardiography in the Evaluation of Celiac Trunk and Superior Mesenteric Artery Involvement in Acute Aortic Dissection.

Mesenteric ischemia is a serious complication of acute aortic dissection (AAD), and its early diagnosis is vital for prognosis and appropriate treatment indication. Arteries affected by this complication are the celiac trunk and superior mesenteric artery, and their evaluation is usually based on computed tomographic angiography. Transesophageal echocardiography is also a useful technique for diagnosing AAD and is essential in monitoring surgical or endovascular treatment when computed tomographic angiography is not available. However, the usefulness of transesophageal echocardiography for evaluating celiac trunk and superior mesenteric artery involvement and mesenteric ischemia mechanisms in AAD is not well established. Real-time information on mesenteric malperfusion is needed at the bedside, in primary care facilities, and in the operating room to achieve prompt diagnosis and better therapeutic management. The aims of this review are to assess the role of TEE to diagnose celiac trunk and superior mesenteric artery involvement in AAD, determine the mechanisms that can cause flow obstruction in patients with mesenteric ischemia, and analyze possible implications in the treatment of this complication.

Predictors of Ascending Aorta Enlargement and Valvular Dysfunction Progression in Patients with Bicuspid Aortic Valve.

Bicuspid aortic valve (BAV) patients are at high risk of developing progressive aortic valve dysfunction and ascending aorta dilation. However, the progression of the disease is not well defined. We aimed to assess mid-long-term aorta dilation and valve dysfunction progression and their predictors. Patients were referred from cardiac outpatient clinics to the echocardiographic laboratories of 10 tertiary hospitals and followed clinically and by echocardiography for >5 years. Seven hundred and eighteen patients with BAV (median age 47.8 years [IQR 33-62], 69.2% male) were recruited. BAV without raphe was observed in 11.3%. After a median follow-up of 7.2 years [IQR5-8], mean aortic root growth rate was 0.23 ± 0.15 mm/year. On multivariate analysis, rapid aortic root dilation (>0.35 mm/year) was associated with male sex, hypertension, presence of raphe and aortic regurgitation. Annual ascending aorta growth rate was 0.43 ± 0.32 mm/year. Rapid ascending aorta dilation was related only to hypertension. Variables associated with aortic stenosis and regurgitation progression, adjusted by follow-up time, were presence of raphe, hypertension and dyslipidemia and basal valvular dysfunction, respectively. Intrinsic BAV characteristics and cardiovascular risk factors were associated with aorta dilation and valvular dysfunction progression, taking into account the inherent limitations of our study-design. Strict and early control of cardiovascular risk factors is mandatory in BAV patients.

The current role of echocardiography in acute aortic syndrome.

Acute aortic syndrome (AAS) comprises a range of interrelated conditions caused by disruption of the medial layer of the aortic wall, including aortic dissection, intramural haematoma and penetrating aortic ulcer. Since mortality from AAS is high, a prompt and accurate diagnosis using imaging techniques is paramount. Both transthoracic (TTE) and transoesophageal echocardiography (TEE) are useful in the diagnosis of AAS. TTE should be the first imaging technique to evaluate patients with thoracic pain in the emergency room. Should AAS be suspected, contrast administration is recommended when images are not definitive. TEE allows high-quality images in thoracic aorta. The main drawback of this technique is that it is semi-invasive and the presence of a blind area that limits visualisation of the distal ascending aorta near. TEE identifies the location and size of the entry tear, secondary communications, true lumen compression and the dynamic flow pattern of false lumen. Although computed tomography (CT) is the most used imaging technique in the diagnosis of AAS, echocardiography offers complementary information relevant for its management. The best imaging strategy for appropriately diagnosing and assessing AAS is to combine CT, mainly ECG-gated contrast-enhanced CT, and TTE. Currently, TEE tends to be carried out in the operating theatre immediately before surgical or endovascular therapy and in monitoring their results. The aims of this review are to establish the current role of echocardiography in the diagnosis and management of AAS based on its advantages and limitations.

Influence of Aortic Dilation on the Regional Aortic Stiffness of Bicuspid Aortic Valve Assessed by 4-Dimensional Flow Cardiac Magnetic Resonance: Comparison With Marfan Syndrome and Degenerative Aortic Aneurysm.

OBJECTIVES: This study sought to ascertain whether patients with a bicuspid aortic valve (BAV) have an intrinsic alteration in regional aortic stiffness compared with patients with tricuspid aortic valve (TAV) and Marfan syndrome (MFS) patients with similar aortic sizes, as well as to assess the influence of ascending aorta (AAo) dilation on regional stiffness parameters in BAV patients. BACKGROUND: Imaging biomarkers as predictors of BAV, MFS, and degenerative AAo aneurysms in TAV patients (DA-TAV) are lacking. Biomechanical characterization has been proposed as a possible tool for further aneurysm stratification. METHODS: A total 234 subjects (136 BAV, 44 MFS, and 18 DA-TAV patients and 36 healthy control subjects) were included. The cardiac magnetic resonance protocol comprised 4-dimensional flow to assess AAo and descending aorta (DAo) pulse wave velocities (PWVs) and double-oblique, 2-dimensional, steady-state free-precession cine cardiac magnetic resonance to compute aortic distensibility (AD). RESULTS: On adjusted analysis, nondilated BAV patients had similar PWV and AD as healthy control subjects in both AAo and DAo, whereas dilated BAV did not differ from DA-TAV. In contrast, AAo and DAo stiffness in MFS patients was markedly greater than in BAV patients, increasing slightly with dilation severity. AAo PWV showed a biphasic pattern in BAV patients: it first decreased and then increased throughout AAo dilation, with a clear turning point at 50 mm, whereas distensibility did not discern mildly dilated aorta. In multivariate analysis adjusted for clinical and demographic characteristics, only PWV was related to AAo dilation in BAV patients. CONCLUSIONS: The mechanical properties of AAo aneurysms are similar in BAV and TAV patients, whereas MFS patients have a stiffer aorta. Aortic stiffness strongly depends on dilation severity. AAo PWV resulted in a potentially clinically useful biphasic trend with respect to aneurysm diameter, whereas distensibility did not discern mildly dilated aorta. Beyond clinical risk factors, PWV but not AD was independently related to AAo dilation in BAV patients.

Increased rotational flow in the proximal aortic arch is associated with its dilation in bicuspid aortic valve disease.

AIMS: Aortic dilation in bicuspid aortic valve (BAV) might extend to the proximal arch. Arch flow dynamics and their relationship with this segment dilation are still unexplored. Using 4D-flow cardiovascular magnetic resonance, we analysed flow dynamics in the arch for each BAV morphotype and their association with this segment dilation. METHODS AND RESULTS: One hundred and eleven BAV patients (aortic diameters ≤55 mm, non-severe valvular disease), 21 age-matched tricuspid aortic valve (TAV) patients with dilated arch and 24 healthy volunteers (HV) underwent 4D-flow. BAV were classified per fusion morphotype: 75% right-left (RL-BAV), and per arch dilation: 57% dilated, mainly affecting the right-noncoronary (RN) BAV (86% dilated vs. 47% in RL-BAV). Peak velocity, jet angle, normalized displacement, in-plane rotational flow (IRF), wall shear stress, and systolic flow reversal ratio (SFRR) were calculated along the thoracic aorta. ANCOVA and multivariate linear regression analyses were used to identify correlates of arch dilation. BAV had higher rotational flow and eccentricity than TAV in the proximal arch. Dilated compared with non-dilated BAV had higher IRF being more pronounced in the RN-morphotype. RN-BAV, IRF, and SFRR were independently associated with arch dilation. Aortic stenosis and male sex were independently associated with arch dilation in RL-BAV. Flow parameters associated with dilation converged to the values found in HV in the distal arch. CONCLUSION: Increased rotational flow could explain dilation of the proximal arch in RN-BAV and in RL-BAV patients of male sex and with valvular stenosis. These patients may benefit from a closer follow-up with cardiac magnetic resonance or computed tomography.

Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease.

OBJECTIVE: Bicuspid aortic valve (BAV) is associated with early valvular dysfunction and proximal aorta dilation with high heterogeneity. This study aimed to assess the determinants of these complications. METHODS: Eight hundred and fifty-two consecutive adults diagnosed of BAV referred from cardiac outpatient clinics to eight echocardiographic laboratories of tertiary hospitals were prospectively recruited. Exclusion criteria were aortic coarctation, other congenital disorders or intervention. BAV morphotype, significant valve dysfunction and aorta dilation (≥2 Z-score) at sinuses and ascending aorta were established. RESULTS: Three BAV morphotypes were identified: right-left coronary cusp fusion (RL) in 72.9%, right-non-coronary (RN) in 24.1% and left-non-coronary (LN) in 3.0%. BAV without raphe was observed in 18.3%. Multivariate analysis showed aortic regurgitation (23%) to be related to male sex (OR: 2.80, p<0.0001) and valve prolapse (OR: 5.16, p<0.0001), and aortic stenosis (22%) to BAV-RN (OR: 2.09, p<0.001), the presence of raphe (OR: 2.75, p<0.001), age (OR: 1.03; p<0.001), dyslipidaemia (OR: 1.77, p<0.01) and smoking (OR: 1.63, p<0.05). Ascending aorta was dilated in 76% without differences among morphotypes and associated with significant valvular dysfunction. By contrast, aortic root was dilated in 34% and related to male sex and aortic regurgitation but was less frequent in aortic stenosis and BAV-RN. CONCLUSIONS: Normofunctional valves are more prevalent in BAV without raphe. Aortic stenosis is more frequent in BAV-RN and associated with some cardiovascular risk factors, whereas aortic regurgitation (AR) is associated with male sex and sigmoid prolapse. Although ascending aorta is the most commonly dilated segment, aortic root dilation is present in one-third of patients and associated with AR. Remarkably, BAV-RL increases the risk for dilation of the proximal aorta, whereas BAV-RN spares this area.

Conservative management in very elderly patients with severe aortic stenosis: Time to change?

BACKGROUND: Despite current recommendations, a high percentage of patients with severe symptomatic aortic stenosis are managed conservatively. The aim of this study was to study symptomatic patients undergoing conservative management from the IDEAS registry, describing their baseline clinical characteristics, mortality, and the causes according to the reason for conservative management. METHODS: Consecutive patients with severe aortic stenosis diagnosed at 48 centers during January 2014 were included. Baseline clinical characteristics, echocardiographic data, Charlson index, and EuroSCORE-II were registered, including vital status and performance of valve intervention during one-year follow-up. For the purpose of this substudy we assessed symptomatic patients undergoing conservative management, including them in 5 groups according to the reason for performing conservative management [I: comorbidity/frailty (128, 43.8%); II: dementia 18 (6.2%); III: advanced age 34 (11.6%); IV: patients' refusal 62 (21.2%); and V: other reasons 50 (17.1%)]. RESULTS: We included 292 patients aged 81.5±9 years. Patients from group I had higher Charlson index (4±2.3), higher EuroSCORE-II (7.5±6), and a higher overall (42.2%) and non-cardiac mortality (16.4%) than the other groups. In contrast, patients from group III had fewer comorbidities, lower EuroSCORE-II (4±2.5), and low overall (20.6%) and non-cardiac mortality (5.9%). CONCLUSIONS: Patients with severe symptomatic aortic stenosis managed conservatively have different baseline characteristics and clinical course according to the reason for performing conservative management. A prospective assessment of comorbidity and other geriatric syndromes might contribute to improve therapeutic strategy in this clinical setting.

Incidence and predictors of obstetric and fetal complications in women with structural heart disease.

OBJECTIVE: Women with cardiac disease becoming pregnant have an increased risk of obstetric and fetal events. The aim of this study was to study the incidence of events, to validate the modified WHO (mWHO) risk classification and to search for event-specific predictors. METHODS: The Registry Of Pregnancy And Cardiac disease is a worldwide ongoing prospective registry that has enrolled 2742 pregnancies in women with known cardiac disease (mainly congenital and valvular disease) before pregnancy, from January 2008 up to April 2014. RESULTS: Mean age was 28.2±5.5 years, 45% were nulliparous and 33.3% came from emerging countries. Obstetric events occurred in 231 pregnancies (8.4%). Fetal events occurred in 651 pregnancies (23.7%). The mWHO classification performed poorly in predicting obstetric (c-statistic=0.601) and fetal events (c-statistic=0.561). In multivariable analysis, aortic valve disease was associated with pre-eclampsia (OR=2.6, 95%CI=1.3 to 5.5). Congenital heart disease (CHD) was associated with spontaneous preterm birth (OR=1.8, 95%CI=1.2 to 2.7). Complex CHD was associated with small-for-gestational-age neonates (OR=2.3, 95%CI=1.5 to 3.5). Multiple gestation was the strongest predictor of fetal events: fetal/neonatal death (OR=6.4, 95%CI=2.5 to 16), spontaneous preterm birth (OR=5.3, 95%CI=2.5 to 11) and small-for-gestational age (OR=5.0, 95%CI=2.5 to 9.8). CONCLUSION: The mWHO classification is not suitable for prediction of obstetric and fetal events in women with cardiac disease. Maternal complex CHD was independently associated with fetal growth restriction and aortic valve disease with pre-eclampsia, potentially offering an insight into the pathophysiology of these pregnancy complications. The increased rates of adverse obstetric and fetal outcomes in women with pre-existing heart disease should be highlighted during counselling.

Left atrial strain: a new predictor of thrombotic risk and successful electrical cardioversion.

BACKGROUND: Left atrial deformation (LAD) parameters are new markers of atrial structural remodelling that seem to be affected in atrial fibrillation (AF) and atrial flutter (AFL). This study aimed to determine whether LAD can identify patients with a higher risk of thrombosis and unsuccessful electrical cardioversion (ECV). METHODS: Retrospective study including 56 patients with AF or AFL undergoing ECV, with previous transthoracic (TTE) and transoesophageal echocardiography (TEE) studies. Echocardiographic parameters analysed were as follows: left ventricle function, left atrium (LA) dimensions, LAD parameters (positive and negative strain peaks), left atrial appendage (LAA) filling and emptying velocities and the presence of thrombi. Strain values were analysed according to thrombotic risk and success of ECV. RESULTS: Lower mean values of peak-positive strain (PPS) in patients with prothrombotic velocities (<25 cm/s) and a higher incidence of thrombi in LAA were observed compared with those with normal velocities. Multivariate analysis revealed PPS normalised by LA maximum volume indexed by body surface area (BSA) to be associated with prothrombotic risk (odds ratio 0.000 (95% CI: 0.000-0.243), P 0.017), regardless of CHADs2VASC score. Peak-negative strain normalised by LA volumes indexed by BSA were associated with unsuccessful ECV. CONCLUSIONS: Atrial deformation parameters identify AF and AFL patients with a high risk of thrombosis and unsuccessful ECV. Therefore, these new parameters should be included in anticoagulation management and rhythm vs rate control strategies.

Predictors of obstetric complications in women with heart disease.

OBJECTIVES: The objective of this study is to evaluate obstetric outcomes in women with heart disease and determine whether current multidisciplinary management approaches adversely affect the mother, the neonate, or both. Also to compare the accuracy of several risk scores (RS) including the modified World Health Organization classification (mWHO) and CARPREG to predict obstetric and neonatal complications and to study the addition value of Uteroplacental-Doppler flow (UDF) parameters to predict obstetric complications. METHODS: A prospective cohort study examined outcomes in women with heart disease (HD), the majority of whom had corrective surgery and delivered between January 2007 and March 2012. RESULTS: One hundred and seventy-four patients with 179 pregnancies were included in the study. Obstetric complications, including premature labor, arose in 87 patients (48.6%). Neonatal complications were observed in 11 cases (7%). On multivariate analysis, maternal heart disease was predictive of adverse perinatal events (46 cases, 25.7%) and mode of delivery (Thierry's spatula) of third- or fourth-degree perineal tears (six cases, 3.2%). mWHO classification predicted obstetric complications (p = 0.0001) better than the CARPREG study. Impaired UDF (uterine artery pulsatility index-20 weeks and umbilical artery pulsatility index-32 weeks in HD versus healthy women: 20w 1.12 versus 1.34, p = 0.005; 32w 0.87 versus 1.09, p = 0.008) was associated with adverse obstetric and offspring outcome in the group of HD pregnant women. CONCLUSIONS: Nearly 50% of pregnancies were associated with an adverse obstetric outcome, particularly IUGR. mWHO was better at predicting obstetric and neonatal complications that CARPREG in all categories. Furthermore, compromised UDF combined with mWHO improved the prediction of obstetric and offspring complications in this population.

Evaluación del flujo retrógrado diastólico en la aorta descendente para cuantificar la insuficiencia aórtica / Descending Aorta Diastolic Retrograde Flow Assessment for Aortic Regurgitation Quantification

Introducción: El flujo diastólico retrógrado en la aorta descendente se ha relacionado con la gravedad de la insuficiencia aórtica; sin embargo, la mayoría de los parámetros vinculados con la velocidad del flujo no se encuentran validados con una técnica de imágenes de referencia. Objetivo: Evaluar la utilidad del flujo retrógrado diastólico en la aorta torácica descendente y la aorta abdominal en la cuan-tificación de la insuficiencia aórtica utilizando como referencia la resonancia magnética. Material y métodos: Se incluyeron 40 pacientes consecutivos en un estudio prospectivo de ecocardiografía y resonancia magnética. Por Doppler pulsado se analizaron los siguientes parámetros: a) la integral velocidad-tiempo del flujo retrógrado diastólico y b) la velocidad máxima telediastólica del flujo regurgitante, ambos en la aorta torácica, y c) el flujo holodiastólico inverso en la aorta abdominal. El protocolo de resonancia magnética incluyó secuencias de contraste de fase para calcular la fracción regurgitante. Valores > 30% se consideraron diagnósticos de insuficiencia aórtica grave. Resultados: Once pacientes (30%) tenían una fracción regurgitante > 30%. La integral velocidad-tiempo del flujo retrógrado diastólico demostró la mayor precisión en el diagnóstico de insuficiencia aórtica grave: ABC = 0,87; p < 0,001. Un punto de corte para la integral velocidad-tiempo del flujo retrógrado diastólico > 15 cm demostró una sensibilidad del 91% y una especificidad del 86% para detectar insuficiencia aórtica grave. El flujo pandiastólico inverso en la aorta abdominal mostró una excelente especificidad (100%) para el diagnóstico de insuficiencia aórtica grave, aunque con baja sensibilidad (50%). Conclusiones: La evaluación del flujo retrógrado diastólico en la aorta torácica descendente permite un diagnóstico adecuado de la insuficiencia aórtica grave. El flujo holodiastólico inverso, aunque es poco sensible, muestra una alta especificidad.