Aortic Root Dilation In Patients Research Articles

Cardiac defects of hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders: a retrospective cohort study.

Defective connective tissue structure may cause individuals with hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorders (HSD) to develop cardiac defects. We conducted a retrospective chart review of adult patients treated in the EDS Clinic from November 1, 2019, to June 20, 2022 to identify those with cardiac defects. Echocardiogram data were collected using a data collection service. All EDS Clinic patients were evaluated by a single physician and diagnosed according to the 2017 EDS diagnostic criteria. Patient demographic, family and cardiac history were extracted from self-reported responses from a REDCap clinical intake questionnaire. Patients with at least 1 available echocardiogram (ECHO) were selected for the study (n = 568). The prevalence of aortic root dilation in patients with hEDS was 2.7% and for HSD was 0.6%, with larger measurements for males than females and with age. Based on self-reported cardiac history that was verified from the medical record, patients with hEDS with bradycardia (p = 0.034) or brain aneurysm (p = 0.015) had a significantly larger average adult aortic root z-score. In contrast, patients with HSD that self-reported dysautonomia (p = 0.019) had a significantly larger average aortic root z-score. The prevalence of diagnosed mitral valve prolapse in patients with hEDS was 3.5% and HSD was 1.8%. Variants of uncertain significance were identified in 16 of 84 patients that received genetic testing based on family history. These data reveal a low prevalence of cardiac defects in a large cohort of well-characterized hEDS and HSD patients. Differences in cardiovascular issues were not observed between patients with hEDS vs. HSD; and our findings suggest that cardiac defects in patients with hEDS or HSD are similar to the general population.

Beta-Blocker use During Pregnancy Correlates with Less Aortic Root Dilatation in Patients with Marfan Syndrome.

Background Pregnant patients with Marfan syndrome (MFS) are at an increased risk for adverse aortic outcomes. While beta-blockers are used to slow aortic root dilatation in non-pregnant MFS patients, the benefit of such therapy in pregnant MFS patients remains controversial. The purpose of this study was to investigate the effect of beta-blockers on aortic root dilatation during pregnancy in MFS patients. Methods This was a longitudinal single-center retrospective cohort study of females with MFS who completed a pregnancy between 2004 and 2020. Clinical, fetal, and echocardiographic data were compared in patients on vs off beta-blockers during pregnancy. Results A total of 20 pregnancies completed by 19 patients were evaluated. Beta-blocker therapy was initiated or continued in 13 (65%) of the 20 pregnancies. Pregnancies on beta-blocker therapy experienced less aortic growth compared to those off beta-blockers (0.10cm [IQR: 0.10-0.20cm] vs 0.30cm [IQR: 0.25-0.35cm]; P=0.03). Using univariate linear regression, maximum systolic blood pressures (SBP), increase in SBP, and absence of beta-blocker use in pregnancy were found to be significantly associated with greater increase in aortic diameter during pregnancy. There were no differences in rates of fetal growth restriction between pregnancies on vs off beta-blockers. Conclusions This is the first study to evaluate changes in aortic dimensions in MFS pregnancies stratified by beta-blocker use. Beta-blocker therapy was found to be associated with less aortic root growth during pregnancy in MFS patients.

Clinical Risk Factors for Aortic Root Dilation in Patients with 22q11.2 Deletion Syndrome: A Longitudinal Single-Center Study.

Aortic root dilation (ARD) has been described in 22q11.2DS, even without congenital heart disease (CHD). However, the clinical implications and longitudinal course are unclear. In this study, we evaluated aortic root (AR) dimensions in 22q112.DS adolescents/adults without major intracardiac CHDs, analyzed the progression over time and investigated correlations with extracardiac comorbidities. AR dimensions were evaluated in 74 patients, measuring the sinus of Valsalva (VS) and proximal ascending aorta (AA), using Z-score to define mild, moderate and severe degrees. Changes in AR dimensions during longitudinal echocardiographic follow-up were investigated. Phenotypic characteristics have been collected. Twenty-four patients (32.4%) showed ARD in terms of VS Z-score (2.43; IQR 2.08-3.01), eight (33.3%) of a moderate/severe degree. Thirteen (54.2%) had concomitant AAD (Z-score 2.34; IQR 1.60-2.85). The risk of ARD was significantly directly related to skeletal/connective tissue disorders (OR 12.82, 95% CI 1.43-115.31; p = 0.023) and inversely related to BMI (OR 0.86, 95% CI 0.77-0.97; p = 0.011). A significant increase in AR diameter's absolute value (p = 0.001) over time has been detected. Isolated ARD is common in 22q11.2DS. Although some clinical risk factors have been identified, pathogenetic mechanisms and risk of complications are undefined. Regular cardiac evaluations should be part of the 22q11.2DS follow-up, and also in non-CHDs patients, to improve long-term outcome.

Growth of the aortic root in children and young adults with Marfan syndrome

ObjectivesThe primary aim was to gain insight into the growth of the aortic root in children and young adults with Marfan syndrome (MFS). Furthermore, we aimed to identify a clinical...

Aortic Root Dilation in Patients with 22q11.2 Deletion Syndrome Without Intracardiac Anomalies.

Aortic root dilation (ARD) has been reported in patients with 22q11.2 deletion syndrome (22q11.2DS) with and without congenital heart defects (CHDs). However, the long-term implications of isolated ARD in 22q11.2DS remain undefined. In this study, we measured aortic root size and estimated the probability of changing between normal aortic root size and ARD during follow up to understand the prevalence, longitudinal course, and clinical risk factors for ARD in patients with 22q11.2DS without intracardiac CHDs. Aortic root size was measured in 251 patients with 432 studies. Forty-one patients (16.3%) had ARD on at least one echocardiogram and the cohort sinus Z-score was increased on the last echocardiogram [mean (1.09, SD 1.24) and median (1.20, min - 1.90 and max 5.40)]. Transition probability analysis showed that 8.1% of patients developed ARD and 45.4% of patients with ARD reverted to normal at the next echocardiogram. The risk of ARD over time was significantly associated with male sex (OR 3.06, 95% CI 1.41-6.65; p = 0.004), but not with age or presence of an aortic arch anomaly. Compared to a sinus Z-score ≥ 2, initial Z-score < 2 was associated with 14.3 times lower risk of developing sinus Z-score ≥ 3 at follow up. Sinus Z-score overall decreased by age, and males had a higher Z-score than females (ß = 0.72, SE = 0.14, p < 0.001). Though only a few patients had a Z-score > 4, and patients with initial Z-scores < 2 seem unlikely to develop clinically significant disease, screening practices remain incompletely defined such that periodic evaluation appears warranted.

Postoperatif Fallot Tetralojili Çocuklarda Aort Kökü Dilatasyonu, Kısa ve Orta Dönem Sonuçlarımız

Amaç: Tam düzeltme ameliyatı yapılmış Fallot tetralojili hastalarımızın klinik durumlarının değerlendirilmesi, ritim bozukluklarının araştırılması, ekokardiyografi ile ventrikül fonksiyonlarının ve aort kökü dilatasyonunun değerlendirilmesidir. Gereç ve Yöntemler: İstanbul Üniversitesi, İstanbul Tıp Fakültesi Çocuk Kardiyoloji bölümünde izlenen, düzeltme operasyonu uygulanmış Fallot tetralojili 50 çocuk çalışmaya dahil edildi. Düzeltilmiş Fallot tetralojili olgular aort kökü dilate olanlar ve dilate olmayanlar diye iki guruba ayrıldı. Her iki gurup arasında cinsiyet, tam düzeltme operasyonu yaşı, aort yetersizliği, triküspit yetersizliği, pulmoner yetersizlik, palyatif tedavi ve transanüler yama kullanımı açısından farklar değerlendirildi. Pulmoner kapak yetmezliği, tam düzeltme ameliyatı sırasında uygulanan transanüler yama yapılanlar ve yapılmayanlar olarak karşılaştırıldı. Düzeltilmiş Fallot tetralojili olguların ekokardiyografik olarak ölçülen aort kökü ve asendan aort çapları yaş, cinsiyet ve vücut yüzey alanlarına göre normal dağılımları ile değerlendirildi. Bulgular: Çalışmaya 24 Ocak-15 Mayıs 2014 tarihleri arasında 50 olgu alındı. Çalışmaya katılan olguların yaşları 2-21 yıl arasında değişmekte olup, ortalama 9.9 ±4.5 yıldır. Çalışmaya katılanların %52’si (n=26) erkek, %48’i (n=24) kızdır. Olguların total düzeltme yaşları 6 (0.5 yıl) ile 190 (15.8 yıl) ay arasında değişmekte olup, ortalama 28.3 ± 28.3 (2.3 ± 2.3 yıl) aydır. Postoperatif süreleri 1.3-17.8 yıl arasında değişmekte olup, ortalama 8.0 ± 4.1 yıldır. Olguların %28’ine (n=14) palyatif tedavi uygulanmıştır. Olguların aort kök ölçümleri 18.0 ile 42.5 mm arasında değişmekte olup, ortalama 27.9 ±5.3 mm’dir. Olguların %12’sinin (n=6) aort kökü dilatasyonu yok iken, %88’inin (n=44) aort kökü dilatasyonu vardır. Olguların tamamında (%100) sağ ventrikül dilatasyonu ve asendan aort dilatasyonu vardır. Transanüler yama uygulananlarda, orta ve ağır pulmoner yetmezlik daha az saptandı (p: 0.02). Sonuç: Olguların %88’inde (n: 44) aort kökü dilatasyonu saptandı. Yıllık aort kökü çapı ölçümlerinin verilmesi, Fallot tetralojili hastalarda ilerleyici aort kökü dilatasyonunun tedavisinde daha etkili bir stratejisinin planlanmasına yardımcı olacaktır.

Meta-analysis Examining the Usefulness of Angiotensin Receptor blockers for the Prevention of Aortic Root Dilation in Patients With the Marfan Syndrome

The Marfan syndrome (MFS) patients are highly predisposed to thoracic aortic aneurysm and/or dissection, with virtually every patient having evidence of aortic disease at some point during their lifetime. We conducted a meta-analysis to investigate the efficacy of angiotensin receptor blockers (ARBs) in slowing down the progression of aortic dilatation in MFS patients. PUBMED, EMBASE, and COCHRANE databases were searched for relevant articles published from inception to February 1, 2020. We included randomized clinical trials evaluating the effect of ARBs on aortic root size in patients with MFS with a follow-up period of at least 2.5 years. Seven studies were included with a total of 1,510 patients. Our analysis demonstrated a significantly smaller change in aortic root and ascending aorta dilation in the ARBs treated group when compared with placebo (mean difference 0.68; 95% confidence interval [CI] -1.31 to -0.04; p = 0.04, I2 = 94%, and mean difference -0.13, 95% CI -0.17 to -0.09; p < 0.00001, I2 = 0%, respectively). ARBs as an add-on therapy to beta-blockers resulted in a significantly smaller change in aortic root dilation when compared with the arm without ARBs (mean difference -2.06, 95% CI -2.54 to -1.58; p < 0.00001, I2 = 91%). However, there was no statistically significant difference in the number of clinical events (aortic complications/surgery) observed in the ARBs arm when compared with placebo (Risk ratio of 1.01, 95% CI 0.74 to 1.38; p = 0.94, I2 = 0%). In conclusion, ARBs therapy is associated with a slower progression of aortic root dilation when compared with placebo and as an addition to beta-blocker therapy.

Highlights From the Circulation Family of Journals.

Highlights From the Circulation Family of Journals.

Losartan for Preventing Aortic Root Dilatation in Patients with Marfan Syndrome: A Meta-Analysis of Randomized Trials.

IntroductionThe role of losartan in preventing aortic root dilatation in Marfan syndrome has been evaluated in many clinical trials; however, the results are conflicting.MethodsWe performed a computerized search of MEDLINE, EMBASE and COCHRANE databases through February 2019 for randomized clinical trials evaluating the effect of losartan in patients with Marfan syndrome. The main outcome was the change in the aortic root diameter in the losartan versus control groups.ResultsOur final analysis included seven randomized trials with a total of 1352 patients and average weighted follow-up of 37.8 months. Change in aortic root diameter was significantly smaller with losartan compared with control [weighted means: 0.44 vs. 0.58 mm, mean difference (MD) = −0.13; 95% CI −0.24 to −0.02; p = 0.02]. Subgroup analysis according to the control group showed no significant subgroup interaction when comparing losartan with beta-blockers versus with standard therapy (pinteraction= 0.27). The composite outcome of aortic surgery, dissection or mortality did not differ between the losartan and control groups (risk ratio = 1.03; 95% CI 0.72–1.49, p = 0.86).ConclusionIn this meta-analysis including seven randomized trials, the use of losartan was associated with a significantly smaller change in aortic root diameter in patients with Marfan syndrome.Electronic supplementary materialThe online version of this article (10.1007/s40119-019-00149-3) contains supplementary material, which is available to authorized users.

445Effects of losartan and beta-blockers on aortic root dilatation in patients with Marfan Syndrome - results of the extended COMPARE trial

Abstract Introduction Despite several randomized trials, beneficial effects of Losartan and β-blockers in adults with Marfan syndrome (MFS) are not entirely clear. The COMPARE trial previously showed a small but significant beneficial effect of Losartan on top of β-blocker use on aortic root dilatation rate. Yet, this effect was not reproduced by other trials, although in general a favourable effect of Losartan could be demonstrated. All trials in adults with MFS showed lower than expected aortic root dilatation rates, suggesting mildly affected study populations. Therefore we extended the follow-up period of the COMPARE trial up to 10 years and aimed to assess the clinical outcomes of the study cohort, as well as the effect of medication regimes on aortic root dilatation rates in the subgroup of patients with a native aortic root at initial randomization. Methods Patients previously enrolled in the COMPARE trial were retrospectively analyzed. Cardiovascular events (aortic dissections, ruptures and operations) and all-cause mortality were assessed. Individual aortic root dilatation rates were estimated in patients with a native aortic root at time of randomization on the basis of linear regression analysis of multiple transthoracic echocardiogram (TTE) results. Correlations between aortic root dilatation rates and cumulative Losartan or β-blocker treatment days were assessed with Spearman's rho (ρ). Results During a median follow-up of 8.0 years, two dissections and three deaths occurred in the 151 patients with a native aortic root at time of randomization. The 122 patients that were eligible for aortic root dilatation analysis, underwent a median of 6 TTEs. The median aortic root dilatation rate in these patients was 0.28 (interquartile range 0.09 - 0.59) mm/y. These patients were further classified as either patients with a stable aortic root (n=102) or with aortic root growth (n=20), based on the threshold of 0.9 mm per year. Patients with aortic root growth had significantly more aortic root replacements during follow-up (17/20 vs 18/102, P=0.001). Furthermore, aortic root dilatation rate was negatively correlated with the number of Losartan treatment days (ρ=−0.272, P=0.003), β-blocker treatment days (ρ=−0.217, P=0.017) and with the duration of follow-up (ρ=−0.437, P&lt;0.001). Conclusions Our results support previous findings that Losartan and β-blockers appear to be equally effective on aortic root dilatation rate in Marfan Syndrome patients. The low event rate in the long term follow-up of this subgroup of the COMPARE trial represent a relatively mildly affected study population and an aggressive prophylactic surgical regime.

Association of aortic root dilatation with left ventricular function in patients with postoperative ventricular septal defect.

Proximal aortic enlargement is associated with an increased risk of heart failure and all-cause mortality. Recently, aortic root dilatation (ARD) was reported in postoperative patients with ventricular septal defects (VSDs). However, the impact of ARD on left ventricular (LV) function in postoperative VSD patients remains unclear. Thus, the aim of this study was to investigate the effect of ARD on LV function in patients with postoperative VSD. One hundred and thirty-five patients (> 15years of age) with surgically repaired isolated ventricular defects and who underwent transthoracic echocardiography in our institution between 2009 and 2013 were identified. ARD was defined as an observed aortic root diameter/body surface area > 2.1cm/m2. The propensity score estimating the probability of having ARD adjusted for anatomical and clinical characteristics was calculated. Forty-four patients (32.6%) had ARD. In unadjusted analyses, right ventricular systolic pressure, Tei index, and E/e' were significantly (p < 0.05) higher in patients with ARD than in those without ARD (31.3 ± 7.5 vs. 35.4 ± 13.7mmHg, 0.32 ± 0.10 vs. 0.44 ± 0.15, and 7.1 ± 1.7 vs. 9.5 ± 2.9, respectively). In the propensity score-adjusted analysis, significant differences in the Tei index and E/e' were confirmed between the two groups (Tei index difference: 0.11, 95% confidence interval 0.05-0.17; E/e' difference: 2.4, 95% confidence interval 1.3-3.5). However, there were no differences in the other echocardiographic measurements. The presence of ARD in patients with postoperative VSD was significantly associated with LV diastolic dysfunction. Thus, surgically repaired VSD patients require careful screening for aortic enlargement and LV function.

Aortic root dilatation in patients with mucopolysaccharidoses and the impact of enzyme replacement therapy.

Mucopolysaccharidoses (MPS) are disorders characterized by impaired glycosaminoglycan (GAG) catabolism as a consequence of a deficiency or the absence of lysosomal enzymes directly involved in their degradation. Multiple organ systems are involved in MPS, including the cardiovascular system. Recently, aortic root dilatation (ARD) has been described in these patients. Thus, we reviewed aortic root diameter measurements in 69 MPS patients from a single center from 2000 to 2016. Aortic root diameter z scores were calculated based on data published by Colan et al. according to the body surface area (BSA) determined using the Haycock formula. The overall incidence of ARD in MPS patients was 39.1%. Higher mean z scores were present in patients with MPS IVA and VI when compared to MPS I and II. Aortic root z scores were higher in older MPS IVA patients, which may suggest a progressive ARD change in this MPS type. No significant differences were found before and after enzyme replacement therapy (ERT) in 11 patients with available data (2 with MPS I; 4 with MPS II; 2 with MPS IVA, and 3 with MPS VI). This work provides further evidence that ARD is common in different types of MPS, being especially evident in MPS IVA, but with a significant occurrence also in MPS VI.

Factors associated with aortic root dilatation after surgically repaired ventricular septal defect.

Aortic root dilatation (ARD) in congenital heart disease is related to aortic aneurysm, rupture, and dissection. This study aimed to investigate the factors associated with ARD in patients with surgically repaired ventricular septal defect (VSD). This cross-sectional study included 152 patients with surgically repaired VSD. Two definitions of ARD were used: (1) observed Valsalva diameter to body surface area (BSA) ratio >2.1cm/m2 and (2) absolute value of Valsalva diameter ≥4.0cm. Odds ratios (ORs) and 95% confidence intervals (CIs) of ARD presence were calculated using multivariate logistic regressions. The prevalence of ARD ranged between 8.6% and 32.9%. Using the definition of observed aortic root diameter/BSA >2.1cm/m2 , patients with nonsubarterial VSD type were more likely to have ARD (OR 5.65, 95% CI; 1.83-17.44, P=.003) than those with subarterial type, and patients with preoperative right- or noncoronary cusp prolapse (R/NCCP) were more likely to have ARD (OR 3.68, 95% CI; 1.20-11.23, P=.022) than patients without preoperative R/NCCP after adjustment for sex, age at repair (ie, shunt duration), VSD size, and postoperative follow-up period. Using the definition of absolute Valsalva diameter ≥4cm, nonsubarterial VSD type and presence of R/NCCP were also significantly associated with ARD after adjustment for the same covariates. Anatomical and morphological features (nonsubarterial type and presence of preoperative R/NCCP) are independently correlated with ARD in patients with VSD regardless of the different definitions of ARD. Patients with surgically repaired VSD may need careful monitoring for potential ARD development.

Natural history of aortic root dilation through young adulthood in a hypermobile Ehlers-Danlos syndrome cohort.

Hypermobile Ehlers-Danlos syndrome (hEDS) is a common inherited connective tissue disorder characterized by joint hypermobility. The natural history of aortic root dilation (AoD), a potential complication of EDS, has not been well characterized in this population. We describe the natural history of aortic root size in a large cohort of patients with hEDS. A cohort of 325 patients with HEDS was identified at Cincinnati Children's Hospital Medical Center (CCHMC), including 163 patients from a previous study. Medical records were reviewed and each participant's height, weight, and aortic dimensions from up to four echocardiograms were documented. Aortic root z-scores were calculated using two established formulas based on age (Boston or Devereux). Overall prevalence of AoD and prevalence by age were calculated and longitudinal regression was performed. The prevalence of AoD with a z-score ≥ 2.0 was 14.2% (46/325) and with a z-score of ≥3.0 was 5.5% (18/325). No significant increases in z-score were seen over time for patients with multiple echocardiograms. Participants under the age of 15 years had an average decline of 0.1 standard deviations (SDs)/year. No significant change was found after 15 of age. Between the ages of 15 and 21 years, Boston z-scores were 0.96 higher than Devereux z-scores. The nearly 1 z-score unit difference between formulas indicates caution prior to diagnosing AoD in patients with hEDS. In light of the low prevalence and lack of progression of AoD, routine echocardiograms may not be warranted for pediatric patients with hEDS.

Aortic root dilatation in patients with Alport’s syndrome

A total of 50 patients from 45 unrelated families diagnosed with Alport’s syndrome were examined. The diagnosis was based on molecular genetic testing or kidney biopsy. To assess changes in the aortic root, its volume was examined (calculation methods were given). Most patients with Alport’s syndrome were found to have cardiovascular disorders, such as hypertension; dilatation of the ring of the aortic valve; dilatation of the sinuses of Valsalva; some patients had dilatation of the sinotubular junction; dilatation of the ascending aorta; enlargement of the left ventricle; mitral and aortic insufficiency. In the patients with Alport’s syndrome, the median value of aortic root volume is 9.9 Z3, which substantially exceeds the permissible upper normal limit according to the general population data, which is 2.82 Z3. The rate of aortic root dilatation (volume increase) in the patients with Alport’s syndrome rises with an increase in the surface area of the body. In Alport’s syndrome, aortic root dilatation is unrelated to the presence of hypertension that occurs with a relative frequency of 0.52. Dilatation of the ascending aorta in Alport’s syndrome is diagnosed with a relative frequency of 0.37 and significantly more frequently diagnosed in male subjects (the statistical significance of a two-sided test is p=0.056; Fisher’s test is p=0.097; risk posed for men is OR = 7.2; 95% CI, 1.63-31.72. Changes in the cardiovascular system are also detected in female carriers of X-linked Alport’s syndrome; therefore they should be followed up by a cardiologist.

Aortic root dilatation in patients with mucopolysaccharidoses and the effect of enzyme replacement therapy

Aortic root dilatation in patients with mucopolysaccharidoses and the effect of enzyme replacement therapy

Abstract 15146: Updated Meta-Analysis of Role of Angiotensin Blockade in Reducing Aortic Dilatation in Patients With Marfan Syndrome

Introduction: Patients with Marfan Syndrome have an increased risk of sudden death due to aortic dissection, mostly preceded by aortic dilatation.There has been considerable interest in evaluating the role of angiotensin blockade with Angiotensin Converting Enzyme (ACE) inhibitors or Angiotensin Receptor Blockers (ARB) on reducing the progression of aortic root dilatation in these patients. While earlier studies showed considerable benefit with ACE/ARB, recently large scale Randomized Control Trials showed conflicting results. The purpose of this meta-analysis was to assess the role of angiotensin blockade in reducing the rate of aortic root dilatation in patients with Marfan Syndrome. Methods: We searched PubMed, EMBASE, and Cochrane Central Register of Clinical Trials from the inception to May 30, 2015 using the terms “Marfan syndrome”, “angiotensin converting enzyme inhibitors” and “angiotensin II-receptor blockers”. Data was extracted using standardized protocol.The main outcome of the present analysis was the change in aortic root diameter with ACE/ABR therapy compared to control. The random effects model of DerSimonian and Laird was used. The statistical analysis was done in line with recommendations from the Cochrane Collaboration using Review Manager (RevMan) version 5.3. Results: Six studies enrolling a total of 1252 patients were included in the final analysis. Five were randomized controlled trials and one was an observation study. Analysis showed that there was no significant benefit with ACE/ARB in reducing aortic root dilatation rate compared to control [Mean Difference of -0.24 ; 95 % CI -0.36 to -0.13, P &lt; 0.00001]. Conclusion: Based on the results of this meta analysis which includes data from the most recent trials, there is no significant benefit with ACE/ARB treatment on reducing the rate of aortic root dilatation in patients with Marfan Syndrome.

Fusiform Aneurysms Are Associated with Aortic Root Dilatation in Patients with Subarachnoid Hemorrhage

Aortic root dimension (ARD), an echocardiographic marker of aortopathy, has recently been associated with intracranial aneurysm eccentricity, suggesting a common underlying pathophysiology between intracranial aneurysm and aortopathy. To identify specific intracranial aneurysm-related parameters that independently correlate with ARD in patients with subarachnoid hemorrhage. Clinical and aneurysm-related parameters obtained from 151 patients with ruptured intracranial aneurysms, and who also underwent echocardiography with ARD measured, were examined. Univariate and multivariate analysis were performed to determine correlation of these parameters with ARD. 151 patients who were evaluated from 2008 to 2013 were analyzed. Multivariate linear regression revealed that male gender and fusiform aneurysms were correlated with higher ARD (P < 0.01 and P = 0.041, respectively) after adjustment for other morphological and clinical variables. Fusiform aneurysms are correlated with larger ARD than saccular aneurysms, suggesting a shared pathophysiologic mechanism with aortopathy.

Abstract 12138: Angiotensin Blockade Reduces Aortic Dilatation in Patients With Marfan Syndrome: A Meta-Analysis

Introduction: Recently few studies evaluated the effects of ACE/ARB in patients with Marfan syndrome. However, as majority of the studies were small and mainly pilot studies, conclusive evidence of benefit with ACE/ARB in this scenario is still pending. The objective of the present meta-analysis was to evaluate the effect of ACE/ARB on the progression of aortic root dilatation in patients with Marfan syndrome. Hypothesis: Treatment with ACE/ARBs is effective for the prevention of aortic-root enlargement in patients with Marfan syndrome. Methods: We searched PubMed, EMBASE, and Cochrane Central Register of Clinical Trials from the inception to April 30, 2013. The main outcome of the present analysis was the change in aortic root diameter with ACE/ABR therapy compared to control. The random effects model of DerSimonian and Laird was used. Results: Four studies were included in the final analysis; three studies were randomized trial and one was observation study. ACE/ARB significantly reduced enlargement of aortic root diameters compared to control; mean difference of -0.97 (95% confidence interval, -1.42 to -0.52; P&lt;0.0001). Consistent benefit of ACE/ARB was observed in all sub-group and sensitivity analyses; against beta blocker, against placebo, in adult patients, with only ARB, analysis with the results from only RCTs. Conclusions: This is the first meta-analysis indicating a beneficial effect of ACE/ARB treatment on aortic root dilatation in patients with Marfan Syndrome. Current evidence supports the use of ARB, specially losartan for this indication.

Circulation: Clinical Summaries

<i>Circulation:</i> Clinical Summaries