Aortic root dilation in adult patients with Marfan syndrome: Does aortic root stiffness matter?Central MessagePerspective
Objective: Aortic root (AoR) size remains an imperfect predictor of rate of aortic dilation in Marfan syndrome (MFS). Indicators of vascular phenotype such as aortic stiffness have been proposed as additional predictors. In this study we assessed the rate of AoR dilation and stiffness in adult patie...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-06-01
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| Series: | JTCVS Open |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2666273622001632 |
| _version_ | 1818214270094016512 |
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| author | Kelly Cox, MD Yousi A. Oquendo, MSE David Liang, MD, PhD Elif Seda Selamet Tierney, MD |
| author_facet | Kelly Cox, MD Yousi A. Oquendo, MSE David Liang, MD, PhD Elif Seda Selamet Tierney, MD |
| author_sort | Kelly Cox, MD |
| collection | DOAJ |
| description | Objective: Aortic root (AoR) size remains an imperfect predictor of rate of aortic dilation in Marfan syndrome (MFS). Indicators of vascular phenotype such as aortic stiffness have been proposed as additional predictors. In this study we assessed the rate of AoR dilation and stiffness in adult patients with MFS. Methods: We performed a retrospective chart review. We included adult patients with MFS (aged 20-40 years) with at least 2 local echocardiograms 6 months apart (no aortic surgery in-between). A blinded observer analyzed the echocardiograms. AoR dilation rate and stiffness were calculated. Results: Thirty-two patients (53% women; median age, 21.1; interquartile range [IQR], 19-24 years at first echocardiogram) were included. AoR dilation rate in the entire cohort was 0 to 8 mm/year (median, 0.465; IQR, 0.23-1.45 mm/year). Multiple linear regression analysis showed that baseline AoR stiffness was associated with AoR dilation rate (β = 0.0004; P < .001 for elastic modulus), whereas baseline age and baseline AoR dimension were not. Eighteen of these 32 patients (56%) eventually had AoR surgery (Sx) and 14 did not have surgery (NSx). At baseline, Sx and NSx patients were similar in age. AoR dimension was larger (Sx, 4.27 cm; IQR, 4.05-4.49 cm vs NSx, 3.73 cm; IQR, 3.37-4.09 cm; P = .011) and AoR stiffness was higher in Sx patients (beta stiffness index: median, 23.2; IQR, 17.8-28.6 vs median, 15.6; IQR, 11.6-19.7; P = .024). AoR dilation rate was greater in Sx patients, independent of baseline AoR dimension (1.63 ± 0.41 mm/year vs 0.38 ± 0.08 mm/year; P = .01). Conclusions: Our results showed that AoR dilation rate varies among adult patients with MFS and is associated with baseline AoR stiffness, measured by echocardiography. Further studies are warranted to determine how aortic stiffness can be implemented clinically to refine management in patients with MFS. |
| first_indexed | 2024-12-12T06:17:31Z |
| format | Article |
| id | doaj.art-8aa75375ac7c4681900c0b3d015d6bae |
| institution | Directory Open Access Journal |
| issn | 2666-2736 |
| language | English |
| last_indexed | 2024-12-12T06:17:31Z |
| publishDate | 2022-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | JTCVS Open |
| spelling | doaj.art-8aa75375ac7c4681900c0b3d015d6bae2022-12-22T00:34:58ZengElsevierJTCVS Open2666-27362022-06-0110113120Aortic root dilation in adult patients with Marfan syndrome: Does aortic root stiffness matter?Central MessagePerspectiveKelly Cox, MD0Yousi A. Oquendo, MSE1David Liang, MD, PhD2Elif Seda Selamet Tierney, MD3Division of Cardiology, Department of Pediatrics, Lucile Packard Children's Hospital, Stanford University, Palo Alto, CalifSchool of Medicine, Stanford University, Palo Alto, CalifDivision of Cardiology, Department of Medicine, Stanford Hospital, Stanford University, Palo Alto, CalifDivision of Cardiology, Department of Pediatrics, Lucile Packard Children's Hospital, Stanford University, Palo Alto, Calif; Address for reprints: Elif Seda Selamet Tierney, MD, Division of Cardiology, Department of Pediatrics, Lucile Packard Children's Hospital, 750 Welch Rd, Palo Alto, CA 94306.Objective: Aortic root (AoR) size remains an imperfect predictor of rate of aortic dilation in Marfan syndrome (MFS). Indicators of vascular phenotype such as aortic stiffness have been proposed as additional predictors. In this study we assessed the rate of AoR dilation and stiffness in adult patients with MFS. Methods: We performed a retrospective chart review. We included adult patients with MFS (aged 20-40 years) with at least 2 local echocardiograms 6 months apart (no aortic surgery in-between). A blinded observer analyzed the echocardiograms. AoR dilation rate and stiffness were calculated. Results: Thirty-two patients (53% women; median age, 21.1; interquartile range [IQR], 19-24 years at first echocardiogram) were included. AoR dilation rate in the entire cohort was 0 to 8 mm/year (median, 0.465; IQR, 0.23-1.45 mm/year). Multiple linear regression analysis showed that baseline AoR stiffness was associated with AoR dilation rate (β = 0.0004; P < .001 for elastic modulus), whereas baseline age and baseline AoR dimension were not. Eighteen of these 32 patients (56%) eventually had AoR surgery (Sx) and 14 did not have surgery (NSx). At baseline, Sx and NSx patients were similar in age. AoR dimension was larger (Sx, 4.27 cm; IQR, 4.05-4.49 cm vs NSx, 3.73 cm; IQR, 3.37-4.09 cm; P = .011) and AoR stiffness was higher in Sx patients (beta stiffness index: median, 23.2; IQR, 17.8-28.6 vs median, 15.6; IQR, 11.6-19.7; P = .024). AoR dilation rate was greater in Sx patients, independent of baseline AoR dimension (1.63 ± 0.41 mm/year vs 0.38 ± 0.08 mm/year; P = .01). Conclusions: Our results showed that AoR dilation rate varies among adult patients with MFS and is associated with baseline AoR stiffness, measured by echocardiography. Further studies are warranted to determine how aortic stiffness can be implemented clinically to refine management in patients with MFS.http://www.sciencedirect.com/science/article/pii/S2666273622001632aortic rootstiffnessdilationMarfan syndrome |
| spellingShingle | Kelly Cox, MD Yousi A. Oquendo, MSE David Liang, MD, PhD Elif Seda Selamet Tierney, MD Aortic root dilation in adult patients with Marfan syndrome: Does aortic root stiffness matter?Central MessagePerspective JTCVS Open aortic root stiffness dilation Marfan syndrome |
| title | Aortic root dilation in adult patients with Marfan syndrome: Does aortic root stiffness matter?Central MessagePerspective |
| title_full | Aortic root dilation in adult patients with Marfan syndrome: Does aortic root stiffness matter?Central MessagePerspective |
| title_fullStr | Aortic root dilation in adult patients with Marfan syndrome: Does aortic root stiffness matter?Central MessagePerspective |
| title_full_unstemmed | Aortic root dilation in adult patients with Marfan syndrome: Does aortic root stiffness matter?Central MessagePerspective |
| title_short | Aortic root dilation in adult patients with Marfan syndrome: Does aortic root stiffness matter?Central MessagePerspective |
| title_sort | aortic root dilation in adult patients with marfan syndrome does aortic root stiffness matter central messageperspective |
| topic | aortic root stiffness dilation Marfan syndrome |
| url | http://www.sciencedirect.com/science/article/pii/S2666273622001632 |
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