Proximal aorta longitudinal strain predicts aortic root dilation rate and aortic events in Marfan syndrome

Abstract 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.

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

European Heart Journal ◽

10.1093/eurheartj/ehz748.0573 ◽

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

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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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Outcomes of endovascular management of the aorta in connective tissue disorders

European Heart Journal ◽

10.1093/eurheartj/ehab724.2002 ◽

2021 ◽

Vol 42 (Supplement_1) ◽

Author(s):

X Yuan ◽

I Akin ◽

T Semple ◽

U Rosendahl ◽

C Nienaber

Keyword(s):

Connective Tissue ◽

Aortic Dissection ◽

Marfan Syndrome ◽

Aortic Root ◽

Endovascular Management ◽

Type B ◽

Connective Tissue Disorders ◽

Type B Aortic Dissection

Abstract Background Endovascular management of type B aortic dissection has been well established and accepted in the community. However, due to the lack of information on long-term outcome, endovascular management remains controversial in the setting of connective tissue disorders such as Marfan syndrome. Although surgical repair is recommended in type B aortic dissection with connective tissue disorders, recent studies demonstrated both feasibility and safety of endovascular interventions in those patients at least short term. Purpose Single centre experience and documentation of long-term outcomes after endovascular management of aortic conditions in patients with connective tissue disorders. Methods All patients with genetic confirmation of connective tissue disorders who underwent endovascular repair of the thoracic aorta were identified and evaluated retrospectively. Perioperative, procedure-specific and follow-up data were extracted from medical records of a structured surveillance program. Results Between 2002 and 2020, 18 patients were identified having undergone TEVAR procedures and protocol follow-up. The median age was 35.5 (IQL 30.0 – 42.7), and 12 (66.7%) were males; median ACEF II score was 0.6 (IQL 0.5 – 0.925). This cohort comprised 15 (83.3%) patients with genetically confirmed Marfan syndrome, two (11.1%) with Loeys-Dietz, and one (5.6%) with Ehlers-Danlos syndrome. Of these, 13 (72.2%) patients had undergone aortic root or aortic root/ascending aorta replacement independently in the past. The indication for a TEVAR procedure was seen in one case for degenerative aneurysmal disease (5.6%), in one case for elephant trunk stenosis (5.6%), in five cases for residual aortic dissection (27.8%), and in 11 cases for new type B dissection (61.1%). The most frequently used stent-graft was Valiant™ (Medtronic) in 10 cases (55.6%), followed by TAG® (Gore®) in four cases (22.2%), Zenith® (COOK®) in two cases (11.1%), and for Relay® (Bolton) and Sinus-XL® (Optimed) in one case each (5.6%). The average total covered length was 192.2±40.2 mm. Median follow-up duration was 77.5 months (IQR 35.8 - 131); three (16.7%) cases revealed failure of TEVAR and led to conversion to open surgery. Two patients (11.1%) had died including one patient in the perioperative phase. At the last follow-up, complete thrombosis of the false lumen was observed in 14 cases (82.3%), and 15 cases (88.2%) showed no aortic expansion or progression during the follow-up period resulting in an overall endovascular success rate of 72.2%. Conclusion Thoracic endovascular aortic repair can be performed for selected patients with connective tissue disorders at relatively low long-term mortality and morbidity. With improved devices and technical skills, mid- and long-term surveillance revealed promising outcomes and a low rate of aneurysmal degeneration. Structured surveillance remains crucial to detect emerging late complications and to accumulate more long-term data. FUNDunding Acknowledgement Type of funding sources: None.

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Abstract 15966: Abnormal Left Ventricular Strain Correlates With Left Ventricular Dysfunction but Not Aortic Pathology in Marfan Syndrome in Children

Circulation ◽

10.1161/circ.142.suppl_3.15966 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Patrick S CONNELL ◽

Shaine A Morris ◽

Tam Doan ◽

Justin Weigand

Keyword(s):

Marfan Syndrome ◽

Aortic Root ◽

Longitudinal Strain ◽

Circumferential Strain ◽

Secondary Analysis ◽

Imaging Study ◽

Left Ventricular ◽

Z Score ◽

Aortic Pathology ◽

Z Scores

Introduction: Cardiomyopathy has been described in some adults with Marfan syndrome (MFS), as demonstrated by low ejection fraction (EF) and abnormal left ventricular (LV) strain. Recent studies from our group show that LV dilation and decreased EF are common in children with MFS. However, LV strain remains understudied in children. Hypothesis: Abnormal strain is associated with lower EF, higher aortic root z-score and higher height-adjusted vertebral artery tortuosity index (VTI-h). In addition, abnormal strain with normal EF is associated with a subsequent decline in EF. Methods: Patients with MFS, defined as meeting Ghent 2010 criteria and a pathogenic FBN1 variant or ectopia lentis, <19 years of age at first cardiac magnetic resonance imaging study (CMR) from 2003-2018 were included. CMR LV circumferential and longitudinal strain were measured using feature tracking software. CMR aortic root Z-scores were calculated using both pediatric echocardiographic formulas and MRI reference data. VTI-h was measured using previously described methods. We evaluated initial CMR for associations between strain, EF, root z-scores, and VTI using Spearman correlation. A secondary analysis was performed limiting to patients with a normal EF on initial CMR, evaluating for associations between initial strain values and the outcomes of sustained normal EF versus development of low EF (<55%). Results: Of 31 included patients, median age at first CMR was 13.5 y (IQR 10.7-16.2 y). Worse circumferential strain was associated with lower EF (basal: rho = -0.586, p = 0.001; mid: rho = -0.573, p = 0.001; apical: rho = -0.406, p = 0.026), while longitudinal strain was not associated with EF (p = 0.063, 0.257, 0.260). Neither circumferential nor longitudinal strain were associated with aortic root z-score or VTI-h. Among patients with normal EF, initial circumferential strain was not different between patients who maintained normal EF or developed low EF. Conclusions: Worse LV circumferential strain was associated with lower EF in children with MFS, while longitudinal strain was not. Abnormal strain was not associated with aortic pathology or later development of reduced EF. This suggests that the cardiomyopathy seen in children with MFS is likely independent of aortic pathology.

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Semi-automatic quantification of aortic root progressive dilation by automatic co-registration of computed tomography angiograms: a preliminary comparison with manual assessment in Marfan patients

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeaa356.224 ◽

2021 ◽

Vol 22 (Supplement_1) ◽

Author(s):

L Dux-Santoy ◽

G Teixido-Tura ◽

A Ruiz-Munoz ◽

L La Mura ◽

F Valente ◽

...

Keyword(s):

Computed Tomography ◽

Growth Rate ◽

Root Growth ◽

Marfan Syndrome ◽

Aortic Root ◽

Public Institution ◽

Maximum Diameter ◽

Automatic Quantification ◽

Root Growth Rate

Abstract Funding Acknowledgements Type of funding sources: Public Institution(s). Main funding source(s): Spanish Ministry of Science, Innovation and Universities Instituto de Salud Carlos III Background. Dilation of the aortic root is a key feature of Marfan syndrome and it is related to the occurrence of aortic events and death. On top of maximum diameter, rapid annual growth rate is suggested by guidelines for indication of aortic root replacement. Current gold-standard for aortic root diameter assessment is manual quantification on multiplanar reformatted 3D computed tomography (CT) or magnetic resonance angiogram. However, inter- and intra-observer reproducibility are limited and different measurement methods, i.e. cusp-to-cusp and cusp-to-commissure, may be used in different clinical centres, leading to difficulties in the clinical assessment of progressive dilation. Purpose. We aimed to test whether aortic root growth rate during follow-up can be reliably quantified by semi-automatic co-registration of two CT angiograms. Methods. Seven Marfan syndrome patients, free from previous aortic surgery, with a total of 11 pairs of CT were identified. Manual assessment of six aortic root diameters (right-non coronary -RN- , right-left -RL- and left-non coronary -LN- cusp-to-cusp and R, L and N cusp-to-commissure) was obtained from all CTs by an experienced researcher blind to semi-automatic results. The thoracic aorta and the outflow tract were semi-automatically segmented in the baseline CT and commissure and cusps were manually located. A 10 mm-thick region of interest containing the aortic wall was automatically generated from segmentation boundary. Co-registration was obtained with three, fully-automatic steps. Firstly, baseline and follow-up CT scans were aligned by means of a rigid registration. Then, scans were co-registered with multi-resolution affine followed by b-spline non-rigid registrations based on mutual information metric. The transformation pertaining to the location of baseline commissure and cusps points was used to locate the same points in the follow-up scan (Fig. 1 top). Results. Follow-up duration was 35 ± 22 (range 12-70.3) months. Automatic quantification of diameter growth during the follow-up was obtained in 62 out of 66 (94%) diameter comparisons. High Pearson correlation coefficients (R) and ICC were found between manual and semi-automatic assessment of growth rate, both for cusp-to-cusp and cusp-to-commissure diameters: R = 0.727 and ICC = 0.678 for RN; R = 0.822 and ICC = 0.602 for RL; R = 0.648 and ICC = 0.668 for LN; R = 0.726 and ICC = 0.711 for R; R = 0.911 and ICC = 0.895 for L and R = 0.553 and ICC = 0.482 for N. Scatter and Bland-Altman plots for all growth rates (Fig. 1) confirmed very good correlation (R = 0.810) but a slight tendency (R=-0.270) for underestimation at high growth rate. No correlation was found between follow-up duration and difference between techniques (R = 0.06). Conclusions. Semi-automatic quantification of aortic root growth rate by co-registration of pairs of CT angiograms is feasible for follow-up as short as one year. Larger studies are needed to confirm these preliminary data. Abstract Figure. CT measurements. Automatic vs manual.

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

Circulation ◽

10.1161/circ.130.suppl_2.18473 ◽

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.

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Aortic diseases

ESC CardioMed ◽

10.1093/med/9780198784906.003.0689_update_001 ◽

2018 ◽

pp. 2861-2863

Author(s):

Bernard Iung

Keyword(s):

Aortic Valve ◽

Aortic Dissection ◽

Marfan Syndrome ◽

Bicuspid Aortic Valve ◽

Young Women ◽

Beta Blockers ◽

Aortic Aneurysms ◽

Maximum Diameter ◽

Aortic Diseases

Aortic diseases encountered in young women are mainly associated with syndromic diseases, which are often heritable, or bicuspid aortic valve. The most frequent syndromic disease is Marfan syndrome. In Marfan syndrome, the risk of aortic dissection is low during pregnancy when the maximum diameter of the ascending aorta is less than 45 mm. Dissection may affect the thoracic ascending or descending aorta. The risk of aortic dissection is low in bicuspid aortic valve when the aortic diameter is less than 50 mm. Beta blockers are recommended throughout pregnancy in Marfan syndrome and are often used in other causes of aortic aneurysms. Close echocardiographic follow-up is needed during pregnancy and after delivery.

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