CHILDHOOD AND ADOLESCENT AORTIC ROOT SIZE PREDICTS AORTIC SURGERY OR DISSECTION IN ADULTS WITH MARFAN SYNDROME

Abstract OBJECTIVES The goal of this study was to report the long-term outcomes of patients with Marfan syndrome who had aortic surgery on any aortic segment except for the replacement of the aortic root itself. METHODS An observational retrospective single-centre study was conducted with 115 Marfan syndrome patients who underwent 189 major aortic interventions from 1995 until 2018. Patients without aortic root replacement were identified and aortic root growth was analysed over time. RESULTS Eleven of 115 patients (9.5%) did not have aortic root replacement during a follow-up of 10.5 [standard deviation (SD) 5.7] years and a mean age at last follow-up of 53.9 (SD 13.4) years. Patients without root replacement did not suffer less frequently from any type of acute aortic dissection (type A 27% vs 25%, P = 0.999; type B 36% vs 25%, P = 0.474). Patients with native aortic roots did not undergo fewer aortic interventions than those with aortic root replacement [12/11, mean 1.09 (SD 0.54) operations/patient vs 177/104, mean 1.7 (SD 1.3); P = 0.128]. Progression of the aortic root dimension was 0.5 (SD 0.3) mm/year in the group of patients with native aortic roots. CONCLUSIONS Current data suggest that 10% of patients with Marfan syndrome with previous aortic surgery will be free from aortic root replacement until the sixth decade of life.

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Aortic root longitudinal strain by speckle-tracking echocardiography predicts progressive aortic root dilation in Marfan syndrome patients

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeaa356.177 ◽

2021 ◽

Vol 22 (Supplement_1) ◽

Author(s):

A Guala ◽

MI Pons ◽

A Ruiz-Munoz ◽

L Dux-Santoy ◽

L Madrenas ◽

...

Keyword(s):

Marfan Syndrome ◽

Aortic Root ◽

Speckle Tracking ◽

Longitudinal Strain ◽

Speckle Tracking Echocardiography ◽

Aortic Surgery ◽

Root Diameter ◽

Aortic Diseases ◽

Severe Aortic Regurgitation

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Spanish Ministry of Science, Innovation and Universities; Instituto de Salud Carlos III Introduction In Marfan syndrome (MFS) patients reduced longitudinal strain of the ascending aorta (AAo) as measured by applying feature-tracking on cine cardiac magnetic resonance (CMR) images predicts aortic root dilation and aortic events during the follow-up. Speckle-tracking is well established for cardiac deformation assessment but proximal aorta applications are challenging due to limited wall thickness and substantial cardiac motion. Moreover, echocardiography is widely used in the clinical assessment aortic diseases. Purpose We aimed to test a speckle-tracking tool for root longitudinal strain analysis in terms of comparison with CMR-derived AAo longitudinal strain and reproducibility and as predictor of dilation in MFS patients. Methods Thirty-five MFS patients diagnosed by original GHENT criteria, with maximum aortic root diameter of 45 mm and free from previous aortic dissection or cardiac/aortic surgery and non-severe aortic regurgitation were consecutive enrolled and followed-up. CMR and echocardiography were performed less than 2 months apart. Baseline and final aortic root diameter were measured on CMR images. To quantify aortic root cyclic elongation by echocardiography, two regions of interests were manually created covering both walls in a parasternal long-axis view and tracked along the cardiac cycle (Figure 1). Longitudinal strain was computed as the average of maximum increase in relative distance of several sub-regions covering both walls. CMR-derived AAo longitudinal strain was available in 29 patients. Intra-observer reproducibility was tested in 15 patients via intraclass correlation coefficient (ICC) for single-rater absolute agreement. Results Aortic root longitudinal strain by echocardiography was mildly related to CMR-derived AAo longitudinal strain (R = 0.27) and was larger compared to CMR-derived values (16.2 ± 6.0 vs 11.3 ± 4.3). Reproducibility was high, with ICC of 0.811, R = 0.802, p < 0.001. After a mean follow up of 76 ± 13 months, aortic root diameter grew in 20 patients with a rate of 0.29± 0.24 mm/year. Overall mean growth-rate was 0.87 ± 0.33 mm/year. In multivariable analysis corrected for age and baseline aortic root diameter, baseline longitudinal strain by echocardiography was independently and inversely related to progressive dilation (p = 0.033). Conclusions The measurement of aortic root longitudinal strain by speckle-tracking echocardiography is feasible. Aortic root longitudinal strain is an independent predictor of progressive dilation in MFS patients. This may permit the improvement of risk-stratification in aortic diseases in large scale studies. Abstract Figure 1

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Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome

Pediatric Cardiology ◽

10.1007/s00246-018-1916-6 ◽

2018 ◽

Vol 39 (7) ◽

pp. 1453-1461 ◽

Cited By ~ 4

Author(s):

Arvind Hoskoppal ◽

◽

Shaji Menon ◽

Felicia Trachtenberg ◽

Kristin M. Burns ◽

...

Keyword(s):

Marfan Syndrome ◽

Aortic Root ◽

Aortic Surgery

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Early and Long-term Outcomes after Aortic Surgery in Patients with Marfan Syndrome

The Thoracic and Cardiovascular Surgeon ◽

10.1055/s-0036-1571630 ◽

2016 ◽

Vol 64 (S 01) ◽

Author(s):

A. El-Sayed Ahmad ◽

N. Papadopoulos ◽

P. Risteski ◽

M. Radwan ◽

M. Ay ◽

...

Keyword(s):

Marfan Syndrome ◽

Aortic Surgery ◽

Long Term Outcomes

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Racial and ethnic differences in response to treatment for Marfan syndrome

Cardiology in the Young ◽

10.1017/s104795112100130x ◽

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.

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