Abstract 18473: Rates and Risk Factors for Faster Aortic Root Growth in Pediatric Patients with Marfan Syndrome

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Nitya Viswanathan ◽  
Claudia Pedroza ◽  
Shaine A Morris
Keyword(s):  
Aortic Dissection ◽  
Root Growth ◽  
Marfan Syndrome ◽  
Aortic Root ◽  
Scoliosis Surgery ◽  
Z Score ◽  
Aortic Dilation ◽  
Significant Difference ◽  
Over Time ◽  
Infantile Type

Background: Marfan Syndrome (MS) is associated with progressive aortic dilation and aortic dissection. Aortic dissection is typically preceded by aortic dilation. The goal of this study is to identify factors associated with faster rates of aortic root dilation in children with MS. Methods: Patients undergoing serial transthoracic echocardiograms (TTE) with MS were retrospectively identified from an institutional database. Those with >2 TTEs over 1 year apart and <21 years of age at first TTE were included. TTEs performed after aortic surgery were excluded. Using multivariable longitudinal linear regression analysis, sex, medication, presence of ectopia lentis, need for scoliosis surgery and infantile type of MS were evaluated for associations with rate of change in aortic root dimension and aortic root z-score over time. Results: Of 240 patients with MS, 146 were included. Median age at first TTE was 8.1 yrs (range 0-20.9 years), median length of follow up 6.5 years (range 1.0-20.1 years), and median number of studies was 8 (range 2-25). Sixty-one percent were male. Of the 146 patients, 123 (84%) were documented to be on medical therapy: 14 angiotensin receptor blocker (ARB), 66 B-Blocker (BB), 10 prior history of both, 5 BB+ARB, 27 were in the Pediatric Heart Network medication trial, 1 ACE inhibitor. Sixteen patients underwent root replacement surgery at a median age of 14.6 years (range 1.8-24). No patients had aortic dissection. Three patients had infantile MS. All of these patients underwent root replacement at 1.8, 2 and 4 years of age. Two underwent subsequent aortic root replacement at 6 and 8 years old. Mean rate of aortic root growth in the cohort was 0.12cm/year, and mean change in z-score was 0.02/year (p=0.23 compared to expected rate of no change). The only variable associated with faster root growth was infantile MS (1.3cm/year, p<0.001; z-score change of 6.9/year, p<0.001). There was no significant difference in the rate of aortic root growth between patients who received therapy with BB vs. ARB vs. BB+ARB. Conclusions: Children with MS did not have a significant change in aortic root z-score over time, and the only factor associated with more rapid aortic root growth was infantile type MS.

Abstract 18498: Description of Aortic Root Growth and Outcomes in a Cohort of Pediatric Patients with Loeys-Dietz Syndrome

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Nitya Viswanathan ◽  
Shaine A Morris
Keyword(s):  
Aortic Dissection ◽  
Root Growth ◽  
Aortic Root ◽  
Aortic Surgery ◽  
Genetic Mutation ◽  
Root Diameter ◽  
Adverse Outcomes ◽  
Rate Of Change ◽  
Z Score ◽  
Repeat Surgery

Background: Loeys Dietz Syndrome (LDS) is associated with rapid aortic dilation and aortic dissection, but data on children with LDS are limited. The goal of this study is to describe aortic root growth and outcomes in children with LDS. Methods: Patients with LDS were identified from an institutional database. Data regarding genetic mutation, medications, aortic root dimensions by transthoracic echocardiography (TTE), aortic dissection and surgical intervention were collected. For those with >2 TTEs 1 year apart, rate of change in z-score was calculated using linear regression. TTEs performed after aortic surgery were excluded. We examined if variables were associated with rate of aortic root growth. Results: Of 16 patients, 8 were female. Five had a TGFBR1 and 9 had a TGFBR2 mutation; 2 patients did not have genetic data available. Median aortic root Z-score at diagnosis was 3.5 (range 0.5-25.4). Fifteen patients were on medication (2 beta-blocker (BB), 5 angiotensin receptor blocker (ARB), 5 BB+ARB, and 3 with past use of both). Four patients underwent prophylactic root replacement at ages 3.3, 6.7, 8.7, and 9.4 years at root dimensions 3.2, 6.5, 4.0, and 4.1 cm respectively. One patient had a Type A dissection at age 15y after prior root replacement and underwent repeat surgery. Another underwent heart transplant at 6.9 years old after prior root replacement. Ten patients had serial TTE data. Median change in aortic root diameter and Z-score was 0.11cm/year and 0.1/year respectively. Mean change in z-score per year for those on on BB was -0.1± (range -1.2 to 0.7), ARB 0.5 (range 0.1 to 1.1) and both 0.0 (range -0.2 to 0.2, p=NS). No variables studied were associated with faster aortic growth. Conclusions: Degree of aortic root dilation and rate of aortic root growth is highly variable in children with LDS, although factors associated more aggressive disease are unclear. The high proportion of patients with adverse outcomes including aortic dissection and surgery is concerning.

Comparability of different Z-score equations for aortic root dimensions in children with Marfan syndrome

2021 ◽  
pp. 1-7
Author(s):  
Dominiek W.E. Rutten ◽  
Ilse J.H. Aarts-Janssen ◽  
Marlies J.E. Kempers ◽  
Annette G. Reimer ◽  
Floris E.A. Udink ten Cate ◽  
...  
Keyword(s):  
Marfan Syndrome ◽  
Aortic Root ◽  
Major Complication ◽  
Root Diameter ◽  
Z Score ◽  
Sinus Of Valsalva ◽  
Aortic Dilation ◽  
Z Scores ◽  
The Mean ◽  
Mean Differences

Abstract Background: Aortic root dilation is a major complication of Marfan syndrome and is one of the most important criteria in establishing the diagnosis. Currently, different echocardiographic nomograms are used to calculate aortic root Z-scores. The aim of the present study was to assess the potential differences in aortic root measurements when aortic root Z-scores were obtained in a cohort of paediatric Marfan patients using several published nomograms. Methods: In a cohort of 100 children with Marfan syndrome, Z-scores for aortic root dimensions were calculated according to the nomograms of Pettersen et al, Gautier et al, Colan et al, and Lopez et al. Bland–Altman plots were used to estimate mean differences in Z-scores and to establish limits of agreement. Results: The mean Z-score of the sinus of Valsalva for Lopez et al was significantly higher compared to Gautier et al (p < 0.01) and Pettersen et al (p = 0.03). The nomogram of Lopez et al resulted in substantially higher Z-scores in patients with a large sinus of Valsalva diameter. Thirty-five percentage of the studied patients would have a Z-score ≥ 2 using Lopez et al compared to 20% for Pettersen et al, 21% for Gautier et al, and 33% for Colan et al. Conclusion: The currently available nomograms for calculating Z-scores of aortic dilation in children with Marfan syndrome lead to clinically relevant differences in Z-scores, especially in children with a relative large aortic root diameter. This could have impact on both the diagnosis and treatment of patients with Marfan syndrome.

Racial and ethnic differences in response to treatment for Marfan syndrome

2021 ◽  
pp. 1-8
Author(s):  
Rachel Ayers ◽  
Michael Kelleman ◽  
Glen Iannucci ◽  
Courtney McCracken ◽  
Matthew E. Oster
Keyword(s):  
Marfan Syndrome ◽  
Ethnic Differences ◽  
Aortic Root ◽  
Randomised Trial ◽  
Z Score ◽  
Treatment Groups ◽  
Black Patients ◽  
Aortic Root Enlargement ◽  
Racial Ethnic ◽  
Hispanic White

Abstract Objective: To determine whether racial/ethnic differences exist for the treatment of Marfan syndrome aortopathy. The 2014 Pediatric Heart Network randomised trial of losartan versus atenolol in Marfan syndrome paediatric and young adult patients showed no treatment differences in the rate of aortic root growth over 3 years; however, they did not examine racial/ethnic differences, and recent data suggest that angiotensin receptor blockers may have different pharmacologic effects in different racial/ethnic populations. Methods: We performed a secondary analysis of public-use data from the Pediatric Heart Network randomised trial comparing the differences by race/ethnicity (non-Hispanic White, non-Hispanic Black, and Hispanic patients) amongst the treatment groups for the primary outcome of rate of aortic root enlargement by z score and secondary outcome of rate of change of absolute diameter of aortic root, z score and absolute diameter of ascending aorta, and blood pressure changes. Results: For aortic root enlargement by z score amongst on-Hispanic White patients, patients on losartan exhibited an annual z score change of –0.090 ± 0.016, compared to –0.146 ± 0.015 for those on atenolol (p = 0.01), favouring atenolol. For Hispanic and non-Hispanic Black patients, there was no difference in primary or secondary outcomes between treatment groups. Conclusion: Non-Hispanic White patients had a small, but statistically significantly greater decrease in aortic root z score favouring atenolol over losartan. There were no significant differences amongst Hispanic or non-Hispanic Black patients, which may be due to relatively small size numbers. These findings may have important implications for medication selection by race/ethnicity in Marfan syndrome patients, which has not previously been evaluated in studies.

Short- and long-term outcomes of aortic root-sparing repair and replacement in acute type A aortic dissection repair

2021 ◽  
pp. 021849232110150
Author(s):  
Tillana Nirav Tarkas ◽  
Carla Stoicescu ◽  
Wahaj Munir ◽  
Mohamad Bashir ◽  
Benjamin Adams
Keyword(s):  
Decision Making ◽  
Aortic Dissection ◽  
Aortic Root ◽  
Type A ◽  
Acute Type ◽  
Type A Aortic Dissection ◽  
Definitive Answer ◽  
Significant Difference ◽  
Current Controversy ◽  
Short And Long Term

Acute type A aortic dissection is a surgical emergency with a high mortality rate if left untreated. Management of the aortic root in this setting constitutes an intricate decision-making framework, further complicated by the emergent nature of the dissection. There exists much controversy regarding pursuit of the aggressive aortic root replacement versus a conservative root-sparing repair, alongside considerations for valve-sparing root replacement. In this review, we critically appraise the current controversy in the literature considering the fate of the aortic root, discussing the aforementioned root interventions for which provides better outcomes for mortality and risk of re-intervention. Literature search was performed using electronic database through PubMed, Google scholar, and Embase focussing on studies reporting outcomes and re-intervention rates for these approaches. Limited by the heterogeneity in surgical strategy, with most studies being single-centred retrospective experiences, further fuel this ongoing debate. The literature reveals rather contrasting results whilst comparing root-sparing repair, versus the extensive root replacement; whilst some studies report no statistically significant difference, others show one superior over the other. There is greater consensus when considering risk of re-operation, with studies showing higher rates of re-operation in root-sparing group compared to replacement; however, many others show no statistically significant difference. In conclusion, the conflicting outcomes reported in the literature, with their inherent limitations, results in the current inability to reach a definitive answer. There remains support in the current literature for both approaches with much of the decision-making being surgeon-bound with many significant influencing factors on a case-by-case basis.

scholarly journals Aortic root growth in children with Marfan syndrome

1998 ◽  
Vol 31 ◽  
pp. 30
Author(s):  
M. Groenink ◽  
L. Rozendaal ◽  
M.S. Naoff ◽  
R.C. Hennekam ◽  
A.A. Hart ◽  
...  
Keyword(s):  
Root Growth ◽  
Marfan Syndrome ◽  
Aortic Root

P1821Proximal aorta longitudinal but not circumferential strain predicts aortic events and aortic root dilation rate in marfan syndrome patients

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A Guala ◽  
G Teixido Tura ◽  
J F Rodriguez-Palomares ◽  
A Ruiz Munoz ◽  
C Granato ◽  
...  
Keyword(s):  
Growth Rate ◽  
Marfan Syndrome ◽  
Aortic Root ◽  
Longitudinal Strain ◽  
Circumferential Strain ◽  
Aortic Diameter ◽  
Diameter Growth ◽  
Z Score ◽  
Aortic Dissections

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

scholarly journals A clinical appraisal of different Z-score equations for aortic root assessment in the diagnostic evaluation of Marfan syndrome

2013 ◽  
Vol 15 (7) ◽  
pp. 528-532 ◽  
Author(s):  
Roland R.J. van Kimmenade ◽  
Marlies Kempers ◽  
Menko-Jan de Boer ◽  
Bart L. Loeys ◽  
Janneke Timmermans
Keyword(s):  
Marfan Syndrome ◽  
Aortic Root ◽  
Diagnostic Evaluation ◽  
Z Score

Pregnancy and Aortic Root Growth in the Marfan Syndrome: A Prospective Study

2005 ◽  
Vol 14 (9) ◽  
pp. 35
Author(s):  
L.J. Meijboom ◽  
F.E. Vos ◽  
J. Timmermans ◽  
G.H. Boers ◽  
A.H. Zwinderman ◽  
...  
Keyword(s):  
Root Growth ◽  
Prospective Study ◽  
Marfan Syndrome ◽  
Aortic Root ◽  
A Prospective Study

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

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M M Van Andel ◽  
J Jalalzadeh ◽  
R Indrakusuma ◽  
R Balm ◽  
J Timmermans ◽  
...  
Keyword(s):  
Root Growth ◽  
Marfan Syndrome ◽  
Aortic Root ◽  
Favourable Effect ◽  
Study Cohort ◽  
Aortic Root Dilatation ◽  
Dilatation Rate ◽  
Β Blocker ◽  
Β Blockers

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<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.

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